Benign And Malignant Tumors Of The Oral Cavity Oral Pathology Question And Answers

Benign And Malignant Tumors Of The Oral Cavity Question And Answers

Question 251. Define hemangioma.
Answer. Hemangiomas are relatively common benign proliferative lesions of vascular tissue origin, which may be present either at birth or may arise during early childhood.

Question 252. In which intraoral sites the hemangiomas commonly develop?
Answer. Intraorally hemangiomas frequently occur over the facial skin, tongue, lips, buccal mucosa, palate, within facial muscles and salivary glands, etc. It can also develop from within the jaw bones as a central lesion.

Read And Learn More: Oral Pathology Short Notes Question And Answers

Question 252a. Among the above sites; which one is the commonest intra oral location for hemangioma?
Answer. Upper and lower lips.

Question 253. How the initial lesion of hemangioma looks like?
Answer. Initially, it appears as pale macule with a thread-like telangiectasia over the skin and mucous membrane.

Question 254. What is the life cycle of hemangioma?
Answer. Since inception the lesion is fully recognizable at about 8 weeks of life. After the initial development some of these lesions enlarge continuously while others become static and make no further progress. However some lesions of hemangiomas may even resolve slowly and fially disappear completely.

Question 255. How the mucosal lesions of hemangioma clinically appear?
Answer. Hemangiomas of the oral mucosa are usually raised, localized, multinodular or flt lesions. The colour of the lesion ranges from distinctly red to blue or to purple colours.
The deeper lesions appear as slightly raised mass with only a bluish hue.

Question 256. What is a strawberry hemangioma?
Answer. Sometimes the bright red bosselated lesion of hemangioma of the skin is called strawberry hemangioma.

Question 257. Why does a lesion of hemangioma blanch upon pressure?
Answer. Hemangioma lesions look red as they have hundreds of blood vessels inside them, each one filed with blood; when a hemangioma lesion is compressed, it blanches (reddish color disappears) because the erythrocytes are pushed out of the vascular channels due to pressure. However, once the pressure is released, its reddish appearance returns back due to refiling of the tumor vessels with blood.

Question 258. Why auscultation is important during clinical examination of hemangioma?
Answer. Hemangioma lesions are filed with hundreds of blood vessels and in case the tumor is connected with a major vessel, ‘bruits’ can be heard during auscultation with a stethoscope (Bruit means a blowing sound or murmur heard in auscultation).

Question 259. Why hemangiomas are dangerous lesions to handle?
Answer. Because of the large number of vessels inside them, any traumatic or surgical injury can initiate excessive bleeding from hemangioma lesions; which will be diffiult to control.

Question 260. Describe the clinical appearance of intramuscular hemangioma.
Answer. Muscular hemangioma commonly produces a diffuse, pulsatile swelling with occasional pain. The lesion often has a spongy feeling with normal surface color and during examination the lesion can be moved across the long axis of the muscle, but it cannot be moved along it.

Question 261. What is port-wine stain?
Answer. Port-wine stain is a special type of hemangioma (mostly capillary type) which is often encountered over the face. It is characterized by a diffuse, purplish, unilateral, macule with irregular borders and is sharply demarcated from the normal skin.

Question 262. What is Sturge-Weber syndrome?
Answer. Sturge-Weber syndrome is a unique phenomenon, which includes unilateral port-wine stain of the face, intracranial hemangiomas and epilepsy.

Question 263. Which disease shows the unique radiographic appearance of tram-line calcifiations (Parallel, curved radiopaque lines of varying length)?
Answer. Intracranial hemangioma; as it may produce calcifications within the walls of the meningeal vessels, which lead to an unique radiographic appearance of parallel radiopaque lines and this have been termed as “tram-line” calcifiations.

Question 264. What is a central hemangioma?
Answer. Central hemangioma represents benign vascular proliferation within the bone; the lesion can be either capillary or cavernous type.

Question 265. In which bones hemangioma occurs more frequently.
Answer. Intraosseous hemangiomas affect vertebrae and skull bones more often than any other bone.

Question 266. Among the jawbones, which one is more frequently affected by hemangioma?
Answer. Mandible is affected more often than maxilla.

Question 267. Describe the clinical features of central hemangioma.
Answer.

• Slow enlarging, non-tender, expansile jaw swelling
• Severe erosion can make the affected bone pulsatile, thin and compressible
• Loosening of teeth and spontaneous gingival bleeding in the affected area is common
• Occasionally, there can be throbbing pain and anesthesia or paresthesia in the affected part of the jaw.

Question 268. How central hemangiomas of the jaw appear radiographically?
Answer. Radiographically central hemangiomas present multilocular radiolucent area, with a typical soap-bubble appearance. Larger lesions often cause severe thinning and expansion of the cortical plates of bone with occasional sun-burst appearance.

Question 269. Describe the histological appearance of capillary hemangioma.
Answer.

• Capillary hemangiomas histologically present numerous, minute, small, endotheliallined capillaries in the lesion, which are densely packed with erythrocytes
• The cells of the endothelial lining are usually well-formed, spindle-shaped and single-layered; these cells are usually supported by a loose connective tissue stroma
  Although, well-formed capillaries are mainly present in capillary hemangioma; sometimes there may be focal proliferating endothelial cells in the form of small aggregates or rosettes and these cells often lack in their attempt at vessel formation.

Question 270. Name one lesion, which looks very similar to capillary hemangioma histologically.
Answer. Pyogenic granuloma.

Question 271. How pyogenic granuloma differs from capillary hemangioma histologically?
Answer. Features like intercellular edema and chronic inflmmatory cell infitration, etc. are very common in pyogenic granuloma but are not seen in case of capillary hemangioma.

Question 272. Describe the histological appearance of cavernous hemangioma (Cavernous means an area or a tumor filed with cavities or hollow spaces).
Answer.

• Cavernous hemangiomas histologically reveal large, irregularly shaped, dilated,‘endothelial-lined sinuses’, which contain large aggregates of erythrocytes
• These blood-pooled sinuses are often intercommunicating with one another and each one is lined by a single layer of flttened endothelial cells
• A mature firous connective tissue stroma often separates one sinus from the other.
• Large areas of hemorrhage and hemosiderin pigmentations are often seen.

Question 273. Name the common imaging techniques used in the diagnosis of hemangioma?
Answer. Doppler angiography and contrast time-lapse angiography.

Question 274. Which lesions are to be considered during the differential diagnosis of hemangioma?
Answer.

• Pyogenic granuloma
• Mucoceles
• Kaposi’s sarcoma
• Salivary gland neoplasm
• Inflammatory hyperplasia of the tissue.

Question 275. What is the treatment of hemangioma?
Answer. Local excision is the treatment of choice in small lesions; however, larger lesions are treated by excision following pretreatment of the lesion with sclerosing agents to reduce their size.

Question 276. Name the sclerosing agents commonly use during the treatment of hemangioma.
Answer. Hot water and 95 percent ethanol (Sclerosing means thickening or hardening of tissue,as of an artery, especially from excessive formation of firous interstitial tissue).

Question 277. What is function of sclerosing agents in the treatment of hemangioma?
Answer. Since hemangiomas are vascular lesions, it is difficult to excise them (particularly the larger lesions) because of the risk of excessive hemorrhage. To overcome this, sclerosing agents are repeatedly injected intra-lesionally prior to surgery, which causes destruction of the intralesional vessels with subsequent firosis. Once there is firosis in hemangioma, it is much smaller in size and does not cause hemorrhage during surgery.

Question 278. What is the treatment of hemangioma in small children?
Answer. Hemangiomas in children may be left untreated until puberty, anticipating their spontaneous regression.

Question 279. What is lymphangioma (Lymph is the clear, watery flid, derived from body tissues that contains white blood cells and circulates throughout the lymphatic vessels)?
Answer. Lymphangioma is an uncommon benign hematogenous neoplasm; characterized by excessive proliferation of the lymphatic vessels.

Question 280. What are the different types of lymphangiomas?
Answer. Lymphangiomas are of three types, which are as follows:

• Capillary lymphangioma: containing numerous small lymphatic capillaries in the tumor.
• Cavernous lymphangiom: containing large dilated lymphatic vessels in the tumor.
• Cystic hygroma: massive diffuse lesion of the neck, containing macroscopic cyst-like spaces of lymphatic vessels.

Question 281. What is the common age of occurrence of lymphangioma?
Answer. Most of the lesions are present at birth or they can arise during childhood (90 percent lesions occur within 2 years of age).

Question 282. What is commonest intraoral site for development of lymphangioma?
Answer. Tongue.

Question 283. Describe the clinical appearance of oral lymphangioma.
Answer. Intraoral superficial lymphangioma usually presents a painless, felt or nodular or vesicle-like translucent swelling over the oral mucosa. The surface of the lesion is pebbly and often resembles ‘frog-eggs’ or ‘tapioca pudding’ (Pebbles means small stones, which are worn smooth by erosion).

Question 284. Clinically, what is the color of lymphangioma?
Answer. The color of the lesion is usually pale and it is often lighter than the color of the surrounding normal mucosa, however on few occasions; lymphangiomas may sometimes produce a red-blue discoloration of the surface (Pale means dim or fade in color).

Question 285. How lymphangioma feels during palpation?
Answer. Palpation often reveals a flctuant swelling in lymphangioma with atypical crepitate sound, the later occurs due to sudden movement of the intra-lesional lymphatic flid from one part of the tumor to the other because of the pressure from palpation (Fluctuant swelling means whose size increases and decreases or varies irregularly).

Question 286. What is the commonest change in the tongue with lymphangioma?
Answer. Lymphangioma often produces diffuse and extensive swelling in the tongue resulting in macroglossia.

Question 287. What is the commonest cause of macroglossia?
Answer. Lymphangioma.

Question 288. Mention the possible complications in case of lymphangioma.
Answer. Cervical lymphangiomas sometimes attend a very large size and they may even extend to the mediastinum, some lesions may cause respiratory distress.

Patients with Down’s or Turner’s syndrome often have a tendency to develop cystic hygromas.

Question 289. Describe the brief histological appearance of lymphangioma.
Answer. Lymphangioma present numerous proliferating, thin walled, markedly dilated lymphatic vessels lined by plump endothelial cells. The lumens of the lymphatic vessels contain an eosinophilic or pinkish proteinaceous coagulum (results from fiation of lymph) with occasional presence of erythrocytes and lymphocytes in it.

Question 290. Name the lesions commonly included in the differential diagnosis of lymphangioma.
Answer.

• Hemangioma
• Mucocele
• Branchial cleft cyst
• Lipoma
• Tuberculosis.

Question 291. Name the special investigations often advised for lymphangioma?
Answer. CT scan, MRI (magnetic resonance imaging), lymphatic scintigraphy and carotid angiogram, etc.

Question 292. Define osteoma.
Answer. Osteoma is a benign neoplasm of bone (osseous tissue); which consists of either mature compact bone or cancellous bone.

Question 293. What are the types of osteomas?
Answer. Osteomas are of two types, which are as follows:

• Periosteal osteoma—arises peripherally from the outer surface of bone.
• Endosteal osteoma—lesions arising centrally within the medullary region of bone.

Question 294. Describe the clinical appearance of osteomas.
Answer. Osteoma clinically produces an asymptomatic, slow growing, nodular, solitary or multiple, exophytic, bony hard growth in the jaw. Some lesions are large and may cause facial deformity.

Question 294a. In case of soft tissue osteoma, what is the most common intraoral site?
Answer. Tongue.

Question 295. Name the syndrome in which osteoma is an integral part.
Answer. Gardner syndrome.

Question 296. Enumerate the features of Gardner syndrome.
Answer. The syndrome consists of multiple osteomas of bone, intestinal polyposis, many unerupted normal or supernumerary teeth, epidermoid cyst and desmoid firomas of skin, etc.

Question 297. Mention the radiographic appearance of osteoma.
Answer. Osteoma radiographically presents well-circumscribed, solitary or multiple, round or oval, dense radiopacities in bone.

Question 298. Describe the brief histological appearance of osteoma.
Answer. Microscopically, osteoma presents a neoplastic osseous tissue being composed of dense cortical bone with a distinct lamellar pattern; the cortical bone is sclerotic and relatively avascular while the medullary bone is denser than normal bone with reduced marrow spaces.

Question 299. Name the lesions, which are often clinically and radiographically confused with osteoma?
Answer. Odontomas, antrolith, bony exostoses, osteoblastoma, sclerotic cemental masses and focal sclerosing osteomyelitis.

Question 300. What is osteoid osteoma?
Answer. Osteoid osteoma is a benign intraosseous neoplasm, which has striking clinical, radiographic, and histological resemblance to osteoblastoma.

Question 301. What is its most signifiant clinical fiding of osteoid osteoma?
Answer. The lesion is almost always painful, especially when digital pressure is applied on it; the cause of pain could be either due to the presence of numerous peripheral nerves in the tumor or due to the synthesis of prostaglandins by the tumor cells.

Question 302. Describe the radiographic appearance of osteoid osteoma.
Answer. Radiographically osteoid osteoma presents a small, round or oval, well-defined, radiolucent area that is surrounded by an area of increased radiodensity (reactive sclerosis). The central area of radiolucency is often called the nidus, which may exhibit some evidence of spotty calcification (Nidus means a point or place at which something originates).

Question 303. What is an osteoblastoma?
Answer. Osteoblastoma is a benign intraosseous neoplasm, which has striking clinical, radiographic and histological resemblance to osteoid osteoma; however, the size of the lesion is usually much bigger than osteoid osteoma.

Question 304. Describe the clinical appearance of osteoblastoma.
Answer. Osteoblastoma clinically presents a progressively expansile swelling of the jaw with a greater tendency to cause local bony expansion. The size of osteoblastoma is usually more than 1 cm in diameter (average is 2–4 cm) and it is also painful. However, the intensity of pain is much less that of compared to osteoid osteoma.

Question 305. Describe the radiographic appearance of osteoblastoma.
Answer. Osteoblastoma radiologically presents a well-defied, large, radiolucent area; containing patchy areas of mineralization and it has a faint bony margin.

Question 306. How cementoblastoma differs radiographically from osteoblastoma?
Answer. Cementoblastoma is radiologically similar to osteoblastoma but it always occurs in continuity with the root portion of a molar tooth.

Question 307. Define chondroma.
Answer. Chondroma is a common benign neoplasm of cartilaginous tissue origin and is consists of mature chondrocytes.

Question 308. Name the common orofacial sites for chondromas.
Answer. Chondromas mostly arise from the vestigial cartilaginous rests present in different parts of the jaws i.e. anterior part of maxilla, the symphysis, premolar-molar area, condyle and coronoid process of mandible, etc. nasal septum is also sometimes affected.

Question 309. Mention the brief clinical appearance of chondroma.
Answer. Chondroma produces a slow enlarging, painless, bony hard swelling of the jawbone with normal overlying epithelium. Few lesions are aggressive in nature and produces expansion and distortion of the cortical plates with mobility of the regional teeth.

Question 310. Name the syndromes with which multiple chondromas can be associated.
Answer. Ollier’s syndrome and Maffucci’s syndrome.

Question 311. Describe the brief histopathological appearance of chondroma.
Answer.

• The lesion consists of well-defined lobules of hyaline cartilage, containing multiple mature chondrocytes; each lobule is separated from one another by an intervening firous tissue septa
• The neoplastic chondrocytes are round or oval in shape with pale cytoplasm and a single nucleus; few tumor cells however have double nuclei
• There are many areas of calcification within the lesion and moreover, there can be some areas of hemorrhage and tissue necrosis.

Question 311a. Which type of cartilage tissue is seen in chondroma?
Answer. Hyaline cartilage.

Question 312. What is a benign chondroblastoma?
Answer. Benign chondroblastoma is a rare benign neoplasm arising from the epiphyseal ends of the long bones.

Question 313. Give the clinical features of benign chondroblastoma.
Answer. The neoplasm clinically presents a relatively large, bony hard lesion, often causing bulging of the jawbones. Displacement of the regional teeth and pain in the area may be present especially during palpation.

Question 314. Describe the histological features of benign chondroblastoma.
Answer. Histologically, benign chondroblastoma presents a highly cellular structure consisting of numerous round or polyhedral ‘chondroblast-like’ cells within a thin firous tissue stroma.
Few areas of calcifiation are seen within the tumor with occasional presence of multiple multinucleated giant cells.

Question 315. Define leiomyoma.
Answer. Leiomyoma is the benign neoplasm of smooth muscle cells. In the oral cavity they are usually derived from the smooth muscle cells of the blood vessels.

Question 316. What is the most common intraoral site for leiomyoma?
Answer. Tongue.

Question 317. Give the brief clinical presentation of leiomyoma.
Answer. Clinically leiomyoma appears as a slow growing, painless, yellow-colored, submucosal nodule with smooth surface; which is often covered by normal appearing epithelium.

Question 318. Describe the histological appearance of leiomyoma.
Answer.

• Leiomyomas exhibit proliferation of spindle-shaped smooth muscle cells in solid sheets; the cells often resemble firoblasts
• The cells have indistinct cell margin and they contain elongated, blunt-ended, pale nuclei, which often produce a ‘cigar-shaped’ appearance
• These spindle-shaped tumor cells also produce perivascular concentric laminations of parallel fascicles.

Question 319. What are the different histological types of leiomyoma?
Answer. Histologically leiomyomas are divided into three types—solid type, vascular type and the epitheloid type.

Question 320. Why special staining is mandatory for confimatory diagnosis of leiomyoma?
Answer. Histologically, the spindle-shaped smooth muscle cells of leiomyoma often resemble firoblasts, moreover both appear “pink” with routine hematoxylin and eosin stain; therefore it is often difficult to differentiate between leiomyoma from neurofiroma or firoma. For this reason special investigations are required.

Question 321. Name the special stains used in the confimatory diagnosis of leiomyoma.
Answer.

• Masson’s Trichome stain: With this special stain, the smooth muscle cells of leiomyoma appear ‘pink’ while the collagenous structures of firoblasts appear blue or green.
• Mallory’s phosphotungstic acid: Hematoxylin stain helps to demonstrate myofirils in leiomyoma.
• Van-Gieson stain: It stains the collagen fiers red and the smooth muscle fiers yellow.
• Immunohistochemical analysis using the monoclonal antibodies to muscle-specifi actin may be useful in confiming the diagnosis of leiomyoma.

Question 322. Define Rhabdomyoma.
Answer. Rhabdomyomas are rare benign neoplasms of striated (skeletal) muscles.

Question 323. Name the common sites for development of rhabdomyoma.
Answer. Rhabdomyomas of the oral cavity often develops from base of tongue, flor of the mouth and soft palate. Some lesions may also develop from the lip, larynx, pharynx and uvula,etc.

Question 324. Describe the clinical appearance of rhabdomyoma.
Answer. The neoplasm clinically presents a slow growing, well-circumscribed, uninodular or multinodular, painless mass in the oral cavity.
Some other lesions can be multicentric in nature and occur at different locations at a time.
Larger and untreated lesions of rhabdomyoma in the pharyngeal or laryngeal region may sometimes cause airway obstruction.

Question 325. Describe the brief histopathological appearance of rhabdomyoma.
Answer.

• Microscopically rhabdomyomas appear as sharply outlined, unencapsulated mass;consisting of large, round or oval striated muscle cells
• The neoplastic cells have a granular eosinophilic cytoplasm and multiple cytoplasmic acuoles, which gives a spidery appearance to the cell
• Irregular cross-striations are often seen and there can be presence of several multinucleated cells.

Question 326. What is the confimatory investigative procedure for diagnosis of rhabdomyoma?
Answer. The diagnosis of rhabdomyoma can be confimed by the electron microscopic demonstration of myofirils in the tumor cells.

Question 327. Name the lesions included in the differential diagnosis of rhabdomyoma.
Answer.

• Leiomyoma
• Granular cell myoblastoma
• Giant cell firoma
• Neurofiroma.

Question 328. What is granular cell myoblastoma?
Answer. Granular cell myoblastoma is a benign neoplastic condition (recent terminology granular cell tumor), which is commonly seen in the oral cavity and skin.

Question 329. Where from the neoplasm granular cell myoblastoma originates?
Answer. The true origin of this lesion is controversial.
According to some investigators granular cells myoblastomas arise from the striated muscle cells as a degenerative disease process.
However, other investigators believe that this neoplasm is of neural tissue (Schwann cell) origin as S-100 protein can be demonstrated from these tumor cells.

Question 330. Describe the common sites for development of granular cell myoblastoma.
Answer. Intraorally tongue is the most frequent site, besides that lip, flor of the mouth, gingiva,palate and uvula, etc. are often affected.
The extra-oral sites for the tumor include—breast, skeletal muscles, skin and subcutaneous tissue, etc.

Question 331. Describe the clinical appearance of granular cell myoblastoma.
Answer.

• The lesion clinically presents a slow enlarging, painless, well-circumscribed lump on the dorsum of the tongue, just beneath the covering epithelium
• The size of the lesion is around 2 centimeter in diameter; it is fim, nodular and nonmovable
• The overlying covering epithelium usually appears normal but sometimes it exhibits a yellow or orange tinge; there may be even a ‘leukoplakia-like’ appearance to the overlying mucosa
• The tumor is often mistaken for carcinoma of tongue or median rhomboid glossitis.

Question 332. Describe the microscopic appearance of granular cell myoblastoma.
Answer.

• The unencapsulated neoplasm consists of diffuse sheets of large, oval or polygonal cells, with distinct cytoplasmic membrane
• The cells lie in close proximity to the overlying epithelium and characteristically contain large number of discrete, punctate, eosinophilic granules
• The overlying epithelium sometimes exhibits an unusual proliferative response,which is referred to as the pseudoepitheliomatous hyperplasia.

Question 332a. Which lesion does granular cell myoblastoma resembles histologically?
Answer. Congenital epulis of new-born.

Question 333. What is pseudoepitheliomatous hyperplasia?
Answer. Pseudoepitheliomatous hyperplasia is characterized by elongated and branched rete pegs of the epithelium resembling the pattern of a neoplastic growth, especially squamous cell carcinoma.

Question 333a. If a tumor of the tongue histologically shows pseudoepitheliomatous hyperplasia,the most likely diagnosis will be?
Answer. Granular cell myoblastoma.

Question 334. Name the lesions which can exhibit pseudoepitheliomatous hyperplasia.
Answer.

• Granular cell myoblastoma
• Cryptococcosis
• Necrotizing sialometaplasia
• Blastomycosis
• Verruciform xanthoma.

Question 335. Name the lesions commonly included in the differential diagnosis of granular cell myoblastoma.
Answer.

• Congenital epulis of new born
• Epidermoid carcinoma
• Neurofiroma
• Neurilemmoma
• Fibroma
• Salivary gland neoplasms.

Question 336. Name the common benign neoplasms of neural tissue origin.
Answer.

• Neurilemmoma or Schwannoma
• Neurofiroma
• Melanotic neuroectodermal tumor of infancy.

Question 337. Define Neurilemmoma.
Answer. Neurilemmomas are benign neoplasms derived from the Schwann cells. These cells are neuro-ectodermal in origin and they envelope the axons of the peripheral nerves in the form of a membrane.

Question 338. Which gender is more often affected by neurilemmoma?
Answer. Females are more affected than males.

Question 339. Name the common intraoral sites for neurilemmoma.
Answer. Intraorally dorsum of the tongue is the most favored location; other sites include palate,flor of the mouth, buccal mucosa, gingiva and lips, etc. Central jaw lesions often occur in relation to the inferior alveolar nerve; at the posterior part of mandible.

Question 340. Describe the clinical appearance of soft tissue lesion of neurilemmoma of oral cavity.
Answer. Neurilemmoma clinically presents a slow enlarging, exophytic, painless, nodule in the oral cavity.
The lesion has a smooth surface, is fim in consistency and often appears as a movable swelling beneath the mucosa.
The size of the lesion ranges between few millimeters to several centimeters in diameter.
The small, lobulated gingival lesions of neurilemmoma often simulate the firous epulis.

Question 341. Describe the clinical appearance of central neurilemmoma of the jaw.
Answer.

• Central neurilemmoma of the jaw presents a well-demarcated, bony hard lesion that causes expansion of the cortical plates and sometimes displacement of the regional teeth
• These lesions may grow at a faster pace as compared to their soft tissue counterparts and may cause pain and paresthesia in the jaw.

Question 342. Describe the radiographic appearance of neurilemmoma.
Answer.

• Radiographically central neurilemmomas present well-defied, unilocular or multilocular radiolucent areas in the jawbone, with expansion and distortion of the cortical plates
• Large lesions may cause extensive bone destruction and may even cause perforation of the cortical plates
• Lesions developing from the inferior alveolar nerve of mandible often cause enlargement of the mandibular canal.

Question 343. Describe the histological appearance of neurilemmoma.
Answer. Microscopically the encapsulated tumor presents proliferating spindle-shaped, neoplastic
Schwann cells, having elongated nuclei; which are arranged in two distinct patterns:

• Antony-A type
• Antony-B type.

Question 344. Describe the Antoni-A tissue in neurilemmoma.
Answer.

• In neurilemmoma a unique pattern of arrangement of neoplastic Schwann calls is seen, which is referred to as the Antoni-A-tissue and is characterized by parallel rows of palisading nuclei of Schwann cells (regimentation of nuclei)
• The cell cytoplasm cannot be readily delineated since they are blended with the surrounding tissue and as a result often there is presence of two palisaded confiurations that are separated from one another by a cell-free zone.

Question 345. What are verocay bodies in relation to neurilemmoma?
Answer. The verocay bodies are eosinophilic acellular areas; consisting of reduplicated basement membrane and cytoplasmic processes. The nuclei in Antoni-A tissue are often arranged in organoid swirls and are referred to as verocay bodies.

Question 346. Describe the Antony-B tissue of neurilemmoma.
Answer. The cells in Antoni-B tissue in neurilemmoma exhibit randomly arranged cells with oval nuclei within a loose myxomatous stroma; this tissue is typically less cellular and less organized. Normally the Antoni-A tissue forms multiple nodules, which are interspersed by Antoni-B tissues.

Question 347. Name the type of lesions which can be included in the differential diagnosis of neurilemmoma.
Answer.

• Neurofiroma
• Fibroma
• Fibroepithelial polyp
• Leiomyoma
• Peripheral giant cell granuloma.

Question 348. Define Neurofiroma.
Answer. Neurofiromas are most common benign neural tissue neoplasms arising from the perineural firoblasts.

Question 349. How many types of neurofiromas develop?
Answer. They may occur either as solitary lesion in the oral cavity or as multiple lesions in association with neurofiromatosis.

Question 349a. Between neurilemmoma and neurofiroma, which one has more tendency for malignant transformation?
Answer. Neurofiroma.

Question 350. What is neurofiromatosis?
Answer. Neurofibromatosis is an autosomal dominant hereditary condition characterized by development of multiple neurofiromas on the skin and oral mucosa, along with ‘café-au-lait’ pigmentation of the skin.

Question 351. Describe the clinical appearance of soft tissue neurofiroma.
Answer. Clinically neurofiroma presents a small, painless, soft or fim, freely movable,submucosal growth with multilobulated surface.

Question 351a. Café-au-lait pigmentation of skin is a feature of which neural tumor?
Answer. Neurofiroma.

Question 352. What are the features of central jaw lesion of neurofiroma?
Answer. Central jaw lesions of neurofiroma in mandible or maxilla often produce slow growing,expansile, swelling of the jawbone with expansion of cortical plates and displacement of teeth. Pain and paresthesia are rarely present in these lesions.

Question 353. Describe the clinical features of neurofiromatosis?
Answer. Neurofiroma lesions in relation to multiple neurofiromatosis can be either nodular or diffuse lesions.

• Nodular lesions: The nodular lesions are few millimeters to several centimeters in diameter, they are spherical in shape and often produce multiple, dome-shaped elevations of the skin.
  The number of nodules in a patient varies from only few to several hundreds
• Diffuse lesions: These lesions produce massive flbby soft masses, which emanate from the neck or involve the subcutaneous tissue of the face and scalp
• Café-au-lait spots: This is a classic feature of neurofiromatosis and is characterized by one or more, large, diffuse, macular brown pigmentations of the skin.

Question 354. Biologically how the solitary neurofiromas differ from those occurring as part of multiple neurofiromatosis?
Answer. Solitary neurofiromas are mostly innocuous lesions; however, those neurofiromas occurring as part of the disease, multiple neurofiromatosis exhibit a higher tendency for malignant transformation.

Question 355. Name the other pathological conditions, which can be associated with multiple neurofiromatosis.
Answer. Pathological conditions occurring in association with neurofiromatosis include hemangiomas and melanomas of the skin, meningeal, cerebral and endocrine neoplasms; and disturbance in the growth and development of bone, etc.

Question 356. Describe the oral manifestations of neurofiromatosis.
Answer. In the oral cavity, neurofiromatosis causes massive swelling with deformity of lips,macrognathia, macroglossia and deformity of mandible and maxilla, etc.

Question 357. Describe the histopathological features of neurofiroma.
Answer.
Histologically neurofiromas exhibit well circumscribed areas of proliferating spindle-shaped neurofiroblasts having typically wavy nuclei
These cells are often arranged haphazardly in interlacing bundles and sometimes the ground substance in the connective tissue exhibits a myxoid appearance.

Question 358. What is neuroectodermal tumor of infancy?
Answer. Neuroectodermal tumor of infancy is a rare benign, pigmented neoplasm of the jawbone,which is derived from the primitive neural crest cells.

Question 359. At what age neuroectodermal tumor of infancy is found in the mouth?
Answer. The lesion occurs mainly in infants before the age of 6 months; some lesions are present at birth.

Question 360. Neuroectodermal tumor of infancy mostly affects which part of the jaw.
Answer. Majority of the neoplasms originate from the anterior part of maxilla; however, mandible is also sometimes affected (about 25% cases).

Question 361. Describe the clinical features of neuroectodermal tumor of infancy.
Answer.

• The tumor clinically presents fast enlarging painless, swelling of the jawbone (especially anterior maxilla) in small children with expansion and distortion of the cortical plates
• The swelling often causes elevation of the lip with facial asymmetry and displacement of developing teeth in the jaw. The surface of the lesion exhibits brown or black pigmentations.

Question 362. Explain the radiographic appearance of neuroectodermal tumor of infancy.
Answer.

• Predominantly the tumor exhibits a cyst-like well-defied radioluceny in the jaw
• Bone destruction in the tumor is sometimes associated with bone formation (osteogenesis) and such lesions may radiographically exhibit a typical ‘sun-ray’ appearance
• Few lesions even exhibit ill-defied ragged borders due to irregular pattern of bone destruction and thereby resemble invasive tumor.

Question 363. Describe the microscopic appearance of neuroectodermal tumor of infancy.
Answer. The lesion is composed of two types of cells:

• Pigmented cells: They are large, flttened or cubical cells with an open nucleus and a lightly staining cytoplasm, which occasionally contains coarse melanin granules.
• Non-pigmented cells: They are small with dark, dense nuclei and a scanty cytoplasm and they often resemble lymphocytes.

The unpigmented cells are arranged in clusters within the connective tissue stroma and sometime these cells are surrounded by the pigmented cells.

Question 364. Describe a special feature of neuroectodermal tumor of infancy.
Answer. Patients with neuroectodermal tumor of infancy exhibit high urinary levels of vanillylmandelic acid (It is an end-stage metabolite of epinephrine and nor-epinephrine).

Question 365. What is a sarcoma?
Answer. It is the malignant neoplasm of the mesenchymal tissue origine.

Question 365a. Defie firosarcoma.
Answer. Fibrosarcomas are malignant neoplasms of the firoblast cells, which often exhibit an aggressive and destructive behavior.

Question 366. Name the common orofacial sites for the development of firosarcomas.
Answer. Cheek, tongue, gingiva, palate, flor of the mouth, jawbones, maxillary sinus and other paranasal sinuses and the pharynx, etc.

Question 367. Name a pre-existing lesion from which firosarcoma may develop.
Answer. Occasionally firosarcomas may develop following radiotherapy in a pre-existing bony lesion, e.g. firous dysplasia of bone.

Question 368. Describe the clinical appearance of firosarcoma.
Answer.

• Fibrosarcoma mostly produces a fast enlarging, painful, bulky, lobulated flshy mass in the oral cavity. The surface of the lesion is smooth and fim; there may be induration and secondary infections
• Intrabony lesions of firosarcoma often produce severe swelling and destruction of the affected bone with loosening and exfoliation of the regional teeth. Pain,anesthesia or paresthesia is often present.

Question 369. Mention in brief the radiographic appearance of firosarcoma.
Answer.

• Fibrosarcoma of the jawbone radiographically produces a sharply defied radiolucent area with severe expansion and destruction of bone
• Marked thinning of cortical bone, displacement of teeth and resorption of roots, etc. are common.

Question 370. Describe the microscopic appearance of firosarcoma.
Answer.

• Proliferation of numerous spindle shaped, ‘tad-pole’ like, malignant firoblast cells.
  (Tad-pole means—tailed larva of a baby frog)
• The malignant firoblast cells proliferate in a typical streaming fashion (cells prolifetate as a continuous stream) within the connective tissue.
• Individual malignant tumor cell contains a large, uniformly stained, elongated hyperchromatic nucleus and a thin scanty cytoplasm
• In many cases, the tumor cells exhibit abnormal mitotic activity in the form of bi-radiate or tri-radiate mitosis
• The malignant firoblast cells synthesize very minimum collagen and often these collagen bundles are arranged in a typical herringbone pattern (an arrangement or pattern resembling the bones in a fih).

Question 371. Name the lesions which look very similar to firosarcoma.
Answer.

• Malignant firous histiocytoma
• Fibroblastic variant of osteosarcoma
• Rhabdomyosarcoma
• Liposarcoma
• Neurogenic sarcoma
• Nodular fascisitis.

Question 372. Define malignant firous histiocytoma.
Answer. Malignant firous histiocytomas are a group of aggressive malignant neoplasms, arising from the undifferentiated mesenchymal cells that differentiate along both firoblastic and histiocytic pathways.

Question 373. Why malignant firous histiocytoma is such an important lesion?
Answer. Because this is probably the most common soft tissue sarcoma of the adults.

Question 374. What are the common orofacial sites of malignant firous histiocytoma.
Answer. Maxillary antrum, tongue, buccal mucosa and maxillary or the mandibular bones, etc.

Question 375. Describe the clinical features of malignant firous histiocytoma.
Answer.

• The neoplasm clinically presents a fast expanding, exophytic, lobulated, flshy mass with pain and surface ulceration
• Secondary infection, hemorrhage, anesthesia or paresthesia, etc. are common Intra-osseous lesions often produce large, painful, expansile growth in the jawbone with mobility or spontaneous exfoliation of the regional teeth
• Facial asymmetry or gross facial disfigurement is often associated with this lesion.

Question 376. Describe the radiological appearance of malignant firous histiocytoma.
Answer.

• Malignant firous histiocytoma radiographically presents a large, multilocular radiolucent area in the jawbone, with severe expansion and distortion of the cortical plates
• Teeth in the affected area often appear to be ‘flating’ within the radiolucent zone.

Question 376a. Describe the histopathological appearance of malignant firous histiocytoma.
Answer. The neoplasm reveals actively proliferating, numerous polyhedral or oval shaped malignant histiocytes and many spindle-shaped malignant firoblast cells.

Short fascicles of malignant cells are often arranged in a typical cart-wheel or storiform pattern and there may be presence of multiple multinucleated giant cells in the tumor.

Question 377. Defie liposarcoma.
Answer. Liposarcomas are malignant neoplasms derived from cells that differentiate along adipose tissue lines and show some evidence of fat synthesis.

Question 378. What is the common oral site for liposarcoma.
Answer. In the oral cavity, the lesions frequently develop from the cheek or buccal mucosa.

Question 379. Describe the clinical features of liposarcoma.
Answer.

• Clinically liposarcoma produces a slow-growing, lobulated, soft, occasionally painful, submucosal swelling
• The overlying epithelium may be either normal or yellow in color and sometimes the lesion is so soft and flctuant that it can be clinically mistaken for a large cyst.

Question 379a. What is the histological appearance of liposarcoma?
Answer. Histologically liposarcomas are more cellular lesions than lipomas and they consist of multiple numbers of foamy and fat-containing malignant lipoblast cells.

Many cells with signet-ring appearance (vacuolated cytoplasms) are found and the nuclei in the tumor cells are prominently displaced to the side of a large vacuole.

There can be presence of irregularly shaped giant cells in few lesions and while other lesions produce large amount of fat within the tumor thereby giving rise to a myxoid appearance.

Question 380. Name the histological types of liposarcoma.
Answer. There are fie histologic types of liposarcomas; which are as follows:

• Myxoid type
• Round cell type
• Well-differentiated type
• De-differentiated type
• Pleomorphic type.

Question 381. What is an angiosarcoma?
Answer. Angiosarcoma is a malignant neoplasm of vascular tissue or angiomatous tissue origin.

Question 382. What are the different types of angiosarcoma?
Answer. There are in three distinct types of angiosarcomas—hemangioendothelioma, hemangiopericytoma, and the Kaposi’s sarcoma.

Question 382a. What is the commonest sarcoma of the angiomatous tissue?
Answer. Kaposi’s sarcoma is considered to be the commonest among all sarcomas of the angiomatous tissue.

Question 383. What are endothelial cells?
Answer. The endothelial cells are specialized cells, which make a lining on the inner aspect of the
basement membrane of vessels and capillaries.

Question 384. What are pericytes?
Answer. Pericytes are specialized cells, which embrace the capillaries from outside. These cells usually help in the process of contraction and dilatation of the vessels.

Question 385. What is a hemangioendothelioma?
Answer. Hemangioendothelioma is a malignant angiomatous neoplasm of mesenchymal tissue origin, which is derived from the endothelial cells of the blood vessels or lymphatic vessels.

Question 386. Describe the clinical appearance of hemnagioendothelioma.
Answer. Hemangioendothelioma clinically presents a fast enlarging, localized, painful, nodular swelling; surface ulceration, paresthesia or anesthesia of the affected area is also common. Some lesions clinically exhibit hemangioma-like appearance.

Question 387. Can hemangioendothelioma develop inside the jawbone?
Answer. Yes, the central jaw lesions often produce expansile, destructive growths with swelling, pain, and cortical expansion, etc. Mobility of the regional teeth and bleeding from the localized gingiva are common.

Question 388. Give the brief histological appearance of hemangioendothelioma.
Answer.

• Hemangioendothelioma microscopically presents neoplastic proliferation of malignant endothelial cells, which are pleomorphic, large, polyhedral in nature with hyperchromatic nuclei
• These neoplastic cells proliferate within the lumen of the vessels as a single layer; however in some cases there may be proliferation of cells in large masses, which fils up the entire lumen of the involved vessel (It should be noted that hemangiopericytoma, the other important angiomatous sarcoma exhibits proliferation of malignant cells on the outer surface of the vessels as the tumor develops from pericytes present around the outer margin of vessels).

Question 389. Name the special stain used in the detection of hemangioendothelioma?
Answer. In silver-reticulin stain is used as the special stain; with this stain the tumor cells of hemangioendothelioma are seen to be lying within the delicate reticulin sheath encircling the blood vessel.

Question 390. Defie hemangiopericytoma.
Answer. Hemangiopericytomas are malignant neoplasms arising from pericytes, these are contractile cells present around the outer aspect of the capillary walls.

Question 391. Describe the clinical appearance of hemangiopericytoma.
Answer.

• Hemangiopericytoma clinically presents a slow enlarging, painless, well-circumscribed, nodular growth, which are often fim in consistency
• The surface may be normal or reddish, which could be indicative of its vascular origin.
  However, the superficial lesions may have vascular prominence and pigmentations
• Some neoplasms can be fast enlarging and they produce large, painful, nodular swellings with surface ulceration.

Question 392. Give the brief histology of hemangiopericytoma.

• There will be actively proliferating spindle-shaped malignant pericytes, exhibiting cellular pleomorphism, nuclear hyperchromatism, and increased abnormal mitotic activity
• These cells group around the outer aspect of several capillary-like tubules often lined on the inner aspect by a single layer of flattened endothelial cells
• The blood vessels in the tumor often exhibit irregular branching and therefore produce a typical staghorn or antler-like appearance.

Question 393. What is a Kaposi’s sarcoma?
Answer. Kaposi’s sarcoma is a malignant neoplasm arising from the endothelial cells of the blood capillaries.

Question 394. Why the tumor is called ‘Kaposi’s sarcoma’?
Answer. Kaposi’s sarcoma is named after Moritz Kaposi who fist reported this tumor in 1872.

Question 395. What are the different forms of Kaposi’s sarcoma?
Answer. Kaposi’s sarcoma predominantly occurs in two form—Endemic form and epidemic form.
(Endemic means a disease regularly found among particular people or in a certain area) (Epidemic means sudden breakout of a disease in a community at a particular time; e.g. fl epidemic).

Question 395a. What is morbidity?
Answer. The relative incidence of a particular disease.

Question 395b. What is mortality?
Answer. The state of being subject to death.

Question 396. Endemic form of Kaposi’s sarcoma occurs among which category of people?
Answer. Endemic form of the disease occurs among elderly persons of Central European or Mediterranean origin and also among children and young black AfricAnswer.

Question 397. Epidemic form of Kaposi’s sarcoma occurs among which category of people?
Answer. Among AIDS patients with history of homosexuality.

Question 398. What are common intraoral sites of Kaposi’s sarcoma in AIDS patients?
Answer. Oral lesions of Kaposi’s sarcoma most often affect the palate; this is followed by maxillary gingiva and tongue.

Question 399. What are different clinical stages of Kaposi’s sarcoma?
Answer. Kaposi’s sarcoma is usually present in three different clinical stages:

• Patch stage
• Plaque stage
• Nodular stage.

Question 400. Describe the clinical appearance of ‘patch stage’ of oral Kaposi’s sarcoma?
Answer. Patch stage is the initial stage of the disease and during this stage a pink, red or purple macule appears over the oral mucosa.

• Nodular lesion of Kaposi’s sarcoma consists of sheets of spindle-shaped cells in a background of scattered blood vessels and slit-like spaces containing RBC
• Marked hemorrhage, hemosiderin pigmentation, lymphocyte and macrophage infitrations are also commonly present.

• Pyogenic granuloma
• Hemangioma
• Melanoma
• Angiosarcoma.

• The tumor causes rapid swelling in the affected part of bone with severe pain
• Expansion of the jawbone with paresthesia or anesthesia of the area
• Unexplained loosening of the tooth
• Occasional perforation the cortical plate of bone with protrusion of tumor outside bone as a soft tissue mass
• In the later stages, the neoplasm develops surface ulceration
• Patients with Ewing‘s sarcoma may develop moderate fever, leukocytosis, anemia, and raised ESR.

• Radiographically Ewing’s sarcoma presents a radiolucent area in the jaw showing widespread destruction of alveolar bone with ill-defied margins.
• The periosteum of the bone characteristically exhibits lamellar layering (an osteophytic reaction), which is known as onion-skin appearance.

• Microscopically Ewing’s sarcoma presents numerous, proliferating, closely packed,small round cells, which resemble the lymphocytes
• The malignant cells are arranged either in diffuse sheets or in loosely arranged lobules, which are separated from one another by a thin firous band, containing small blood vessels, chronic inflmmatory cells, areas of necrosis and hemorrhage, etc.

• First are the small round cells with darkly staining nuclei and well-delineated cytoplasm
• The other cells are larger with fiely granular nuclei and ill-defied, faint cytoplasm.

• Embryonal rhabdomyosarcoma
• Garre’s osteomyelitis
• Lymphoma
• Metastatic carcinoma
• Leukemia
• Myeloma
• Small cell osteosarcoma.

• Primary chondrosarcoma: Lesion arising directly from the bone as a malignant neoplasm
• Secondary chondrosarcoma: Lesion arising from the pre-existing benign cartilaginous neoplasms such as chondromas or osteochondromas, etc.

• Posterior part of mandible (at the site of Meckel’s cartilage)
• Mandibular symphysis, coronoid or the condylar processes
• Anterior part of maxilla at the site of nasal cartilage
• Nasal septum and the para-nasal sinuses.

• Chondrosarcoma often produces a fast enlarging swelling with pain, tenderness, anesthesia or paresthesia in the region along with extensive local tissue destruction
• Jaw swelling often leads to severe expansion of the bone and loosening of teeth
• Chodrosarcomas occurring in the anterior maxilla may extend into the deeper tissue and cause nasal obstruction, epistaxis, photophobia, visual loss and breathing difficulties, etc.

• Radiographically chondrosarcoma produces large radiolucent area with a typical moth-eaten appearance, having ill-defied borders. Within the area of radiolucency,multiple flcks or blotchy areas of radiopacities are found; which occur due to calcifiation or ossifiation of the cartilage matrix
• Widening of the periodontal ligament space of the adjoining teeth and ‘sun-ray’ appearance etc, may be seen occasionally but not as often as seen in osteosarcomas.

• The lesion shows proliferation of malignant chondrocytes, which are pleomorphic and hyperchromatic cells; many of them are bi-nucleated
• The well-differentiated lesions resemble benign cartilaginous neoplasms and produce large amount of cartilage within the tumor, however other lesions could be anaplastic in nature and are composed of spindle-shaped malignant cells, with little or no evidence of cartilage formation.

• Grade- I
• Grade- II
• Grade- III.

• Paget’s disease of bone
• Fibrous dysplasia
• Giant cell tumor of bone
• Osteochondroma
• Bone infarct
• Chronic osteomyelitis
• Osteogensis imperfecta.

• Medullary osteosarcoma
• Periosteal osteosarcoma
• Parosteal osteosarcoma (arising from the external surface of bone)
• Soft tissue or extra-skeletal osteosarcoma.

• Osteolytic type of osteosarcoma
• Osteoblastic type of osteosarcoma
• Mixed type.

• Osteoblastic type of osteosarcoma
• Chondroblastic type of osteosarcoma
• Fibroblastic type of osteosarcoma
• Telangiectatic type of osteosarcoma.

• A very fast enlarging, fim, painful swelling of the jaw
• Expansion and distortion of the cortical plates with severe facial deformity
• Toothache-like pain in the jaws
• Displacement and loosening of teeth
• Diffiulty in taking food due to restricted jaw movements
• Reddening and inflmmation of the overlying skin and mucosa
• Paresthesia of the lips, epistaxis, nasal obstruction and pressure in the eye-ball, etc.
• Vascular prominence in the skin of the affected area
• Ulceration, hemorrhage and possible pathological fracture of bone.

• A large, irregular, radiolucent area in the bone with ill-defied or indistinct margin
• The lesion often has a typical moth-eaten appearance
• Expansion, destruction and perforation of the cortical plates are common
• Few lesions cause resorption of roots of the teeth with tapered narrowing of the teeth (spiking resorption)
• Multiple, irregular, foci of radiopacities are seen within the lesion; which occurs due to deposition of bone within the tumor
• Deposition of new bone on the surface of the lesion in a radiating fashion often produces a typical “sun-ray” or a “sunburst” appearance
• At the margin of the tumor there is lifting of the periosteum and new bone formation;
• this phenomenon is often known as ‘Codman’s triangle’
• Early osteosarcomas are characterized by localized, symmetric widening of the periodontal ligament space around the regional teeth
• Loss of supporting bone often causes displacement of the regional teeth
• Many lesions radiologically present the evidence of pathological fracture of the affected bone.

• To demonstrate intramedullary extention of tumor
• Soft tissue involvement
• Tumor calcification
• Cortical invilvement.

• Osteosarcoma histologically presents proliferating, spindle-shaped, oval or angular,malignant osteoblast cells characterized by cellular pleomorphism and nuclear hyperchromatism with features of abnormal mitosis
• Multiple areas of newly formed bone or osteoid tissues are often present within the tumor and these osteoids are always bordered at the periphery by malignant tumor cells.

• Chondrosarcoma
• Fibrosarcoma
• Garre’s osteomyelitis
• Fibrous dysplasia of bone
• Osteoblastoma
• Eosinophilic granuloma.

• Hodgkin’s lymphoma
• Non-Hodgkin’s lymphoma.

• Age: It is more common above 60 years
• Family history: Several members of the same family can be affected
• Immunosuppressive drug treatment.
• Infections such as HIV/AIDS, Epstein-Barr virus infection, Hepatitis C, etc.
• Radiation therapy
• Cytotoxic drug therapy
• Rheumatoid arthritis
• Sjogren’s syndrome
• Benign lymphoepithelial lesions.

• Chronic lymphocytic leukemia/small lymphocytic lymphoma
• B-cell prolymphocytic leukemia
• Lymphoplasmacytic lymphoma (such as Waldenström macroglobulinemia)
• Splenic marginal zone lymphoma
• Plasma cell neoplasms:
  • Plasma cell myeloma
  • Plasmacytoma
  • Monoclonal immunoglobulin deposition diseases
  • Heavy chain diseases
• Extranodal marginal zone B cell lymphoma, also called MALT lymphoma
• Nodal marginal zone B cell lymphoma (NMZL)
• Follicular lymphoma
• Mantle cell lymphoma
• Diffuse large B cell lymphoma
• Mediastinal (thymic) large B cell lymphoma
• Intravascular large B cell lymphoma
• Primary effusion lymphoma
• Burkitt lymphoma/leukemia

• T cell prolymphocytic leukemia
• T cell large granular lymphocytic leukemia
• Aggressive NK cell leukemia
• Adult T cell leukemia/lymphoma
• Extranodal NK/T cell lymphoma, nasal type
• Enteropathy-type T cell lymphoma
• Hepatosplenic T cell lymphoma
• Blastic NK cell lymphoma
• Mycosis fungoides/sezary syndrome
• Primary cutaneous CD30-positive T cell lymphoproliferative disorders
  • Primary cutaneous anaplastic large cell lymphoma
  • Lymphomatoid papulosis
• Angioimmunoblastic T-cell lymphoma
• Peripheral T cell lymphoma, unspecified
• Anaplastic large cell lymphoma

• Classical Hodgkin’s lymphomas are
  • Nodular sclerosis
  • Mixed cellularity
  • Lymphocyte-rich
  • Lymphocyte depleted or not depleted
• Nodular lymphocyte-predominant Hodgkin’s lymphoma.

• Nodular pattern
• Diffuse pattern.

• DNA hybridization study reveals: Epstein-Barr virus DNA in the malignant
• B-lymphocytes
• Bone marrow biopsy
• Liver biopsy
• Laparotomy (making an incision in the abdomen)
• Bone scan
• Liver scan
• Blood picture
• CT scan
• Bone marrow biopsy
• Immunohistochemistry: monoclonal nature of the malignant lymphocytes can be recognized by the production of kappa and lamda light chains only.

• Endemic form (African type)
• Non-endemic or sporadic (non-African) form
• Immunodeficiency-associated form.

• Classic Burkitt’s lymphoma
• Burkitt’s lymphoma with plasmacytoid proliferations
• Atypical Burkitt’s/Burkitt-like lymphoma.

• Advanced lesion of Burkitt’s lymphoma produces massive painless swelling of the face with gross deformity
• Expansion of the jaw results in displacement of teeth, derangement of the dental arch and malocclusion, etc.
• Often there is massive enlargement of the gingiva or the alveolar process in the jaw; as a result, many deciduous or even permanent teeth are pushed out of their socket and many of which may exfoliate prematurely
• Sometimes the tumor is so large that it may fil up the entire oral cavity; in such cases a large tumor mass often protrudes outside the mouth, which contains many rootless teeth
• Paresthesia or anesthesia of the mental and infraorbital nerves is common.

• Lymphocyte predominant type
• Mixed cellularity type
• Lymphocyte depletion type
• Nodular sclerosis type.

• Biopsy of the lymph nodes to see the characteristic histopathologic fidings
• Complete blood count
• Chest X-ray and tomography
• Radiographic skeletal survey
• Technetium bone scans
• Liver function test and scan
• Bone marrow biopsy
• Lymphangiogram to see the size of the node
• Laparotomy to see the extent of the disease.

• Routine hemogram: anemia, neutropenia, thrombocytopenia and greatly raised ESR
• Blood serum: hyperglobulinemia and reversal of the serum albumin-globulin ratio
• Abnormal increase in the production of light chain proteins (known as Bence-Jones protein) by the tumor cells; as a result the serum protein level may become very high (measuring up to 8–16 g%).

• Polycythemia
• Leukemia
• Lymphosarcoma
• Hodgkin’s disease.

• Embryonal rhabdomyosarcoma (60–70 percent)
• Alveolar rhabdomyosarcoma (20–30 percent)
• Pleomorphic rhabdomyosarcoma (5 percent or less).