Odontogenic And Nonodontogenic Tumors Short Notes

Question 1. Describe odontome.
Answer:

• Odontoma is a tumor of odontogenic origin, in which an atypical formation of enamel and dentin occurs.
• It is considered as a hamar- tomatous malformation rather than a true neoplasm because it has both epithelial and mesenchymal components.

Odontoma Etiology: Definite etiology is unknown. Local trauma or infection is the most recognized cause.

Read And Learn More: Oral Medicine and Radiology Question And Answers

Odontoma Types:

1. Compound composite odontoma: The enamel and dentine are laid down orderly so that they superficially resemble a tooth structure.
2. Complex composite odontoma: The calcified tissues are arranged as an irregular pattern without any resemblance to the tooth morphology. This form is rare.

Odontoma Clinical Features:

• There is a male predilection for the occurrence.
• Maxilla is more common (67%) than mandible (33%).
• Compound odontoma occurs in the anterior maxilla, whereas complex odontomas have a preference for posterior jaws.
• Odontoma usually remains smaller than a tooth, but rarely it becomes larger and produces bony expansion and facial asymmetry.
• Mostly these are asymptomatic.

Odontoma Complication: Occasional development of dentigerous cyst.

Odontoma Radiographic Features

Present as a dense radiopaque mass surrounded by a thin radiolucent boundary.

• Stage 1 (or) initial stage: Radiolucent due to the absence of calcified structures.
• Stage 2 (or) intermediate stage: Partial calcification gives rise to a mixed pattern of radiopacity and radiolucency.
• Stage 3 (or) mature stage: Radiopaque stage with surrounding radiolucent halo.

Odontoma Treatment: Surgical excision.

Question 2. Ossifying fibroma.
Answer: Ossifying fibroma (OF) is a benign lesion of jaw bones in which the affected site is composed of fibrous tissue and calcified structures like cementum and bone instead of normal bone architecture.

Ossifying fibroma Synonyms:

• Cementifying fibroma
• Cemento-ossifying fibroma
• Juvenile ossifying fibroma.

Ossifying fibroma Histological Types:

• Juvenile trabecular ossifying fibroma (JTOF)
• Juvenile psammomatoid ossifying fibroma (JPOF).

Ossifying fibroma Location:

• Ossifying fibroma has prevalence for the mandibular molar-premolar region.
• Ossifying fibroma presents as small, painless lesions that are occasionally diagnosed on routine radiographs. They are slow-growing and expansive.

Ossifying fibroma Radiographic Appearance:

• They are unilocular lesions with a well-defined, smooth, radiopaque border.
• In the initial stage of development, the lesion resembles a cyst with a radiolucent appearance.
• In the later course of the lesion, it appears radiopaque with a well-defined radiolucent boundary.

Ossifying fibroma Management: Surgical resection.

Question 3. Describe adenomatoid odontogenic tumor.
Answer:

• The adenomatoid odontogenic tumor is a tumor originating from the odontogenic epithelium.
• The tumor presents with both intraoral and extraoral swellings in the maxilla (involves both bone and soft tissue).
• It is referred to as a “two­third tumor” because in 2/3rd of reported cases, it occurred in the maxilla in 2/3rd cases, the gender reported is females of young age group and 2/3rd of the diagnosed patients had an unerupted tooth in the affected site and canine is involved in nearly 2/3 cases.
• It is a well-capsulated tumor and hence benign.
• The tumor has a high predilection for females.
• Root resorption, displacement of roots, and bone perforation are rarely associated findings.
• Histological findings reveal calcification in the form of irregular masses or as a pattern of Liesegang rings.

Adenomatoid odontogenic tumor Radiographic Appearance:

• The adenomatoid odontogenic tumor occurs as both intraosseous and extraosseous types.
• The intraosseous type takes either follicular (pericoronal) or extrafollicular form.

Adenomatoid odontogenic tumor Intraosseous Type:

1. Follicular form: Appear as a distinct radiolucent lesion around the crown of an unerupted tooth and hence frequently misdiagnosed as a dentigerous cyst.
2. Extrafollicular form: Appear as a definite radiolucent lesion overlaying the root of an unerupted tooth. Sometimes tiny radiopaque structures are present within the radiolucency.

Extraosseous (Peripheral or Gingival) Type: The peripheral type is seldom observed on radiographs. Many times mild erosion of the alveolar bone is the only positive radiographic feature.

Adenomatoid odontogenic tumor Treatment: Curettage is the preferred treatment. Recur¬rence is uncommon.

Question 4. Calcifying epithelial odontogenic tumor
(or)
Pindborg tumor.
Answer:

• This tumor is rare and is known by the name of Dr. J J Pindborg, who described it first. It is locally invasive and presents as a unilocular or multilocular swelling in the molar—ramus region and thus resembles ameloblastoma.
• This tumor forms an amorphous amyloid or amyloid-like material. It calcifies in a concentrically lamellar form resem¬bling tree-ring pattern and is known as Liesegang calcifications.
• A calcifying epithelial odontogenic tumor (CEOT) is nonencapsulated but does not infiltrate into the deep tissues.
• Radiographically, this tumor appears radiolucent but if calcifications are present, it appears radiopaque. The central location of the tumor in the jaw with an expansion of cortex and areas of spotty calcification are radiographic characteristics for the Pindborg tumor.
• Enucleation or local block excision is the treatment of choice.

Question 5. Write about verruca vulgaris
(or)
Oral warts.
Answer:

• It is focal papillary hyperplasia induced by human papillomaviral infection (HPV-2, HPV-4, and HPV-40).
• The lesion is contagious and spreading from autoinoculation to other parts of the skin, and mucous membrane in the affected individual is common.

Verruca vulgaris Clinical Features:

• Pointed, exophytic surface projection with a narrow stalk, and presents as multiple or clustered lesions.
• It enlarges rapidly and reaches a higher diameter of 5 mm.
• Present on lips and intraoral presentation is rare and seen in the palate, and gingiva.
• It appears white due to surface keratinization

Verruca vulgaris Diagnosis:

• Clinical presentation.
• Histopathological features of thin, long, and finger-like projections, hyperkeratosis, and koilocytosis (HPV-altered epithelial cells).
• Demonstration of HPV-antigen by immuno- histochemistry.

Verruca vulgaris Treatment:

• Excision of the lesion with the base in which the stalk is attached.
• Liquid nitrogen cryotherapy.
• Topical keratolytic agents.

Question 6. Discuss condyloma acuminatum.
Answer:

• It is a type of oral wart caused by human papillomavirus-6 (HPV-6).
• It is a sexually transmitted infection that occurs either single or as multiple.

Condyloma acuminatum Clinical Features:

• Commonly present on lips, commissure of mouth, and gingiva.
• Coral pink in color with a warty surface.
• The lesions may proliferate and coalesce to form clusters. Size is variable between 2 cm and 3 cm.

Condyloma acuminatum Diagnosis: Histopathological features of intranuclear inclusion bodies in epithelial cells.

Condyloma acuminatum Treatment:

• Surgical excision.
• Topical application of keratolytic agents.

Question 7. Pyogenic granuloma.
Answer:

• It is a localized reactive lesion to mild irritation and manifests as a tissue overgrowth.
• Pyogenic granuloma is a misused terminology because it is neither associated with pus formation nor with a histological resemblance to granuloma.

Pyogenic granuloma Etiology: Low-grade, chronic irritation caused by trauma, calculus, and overhanging resto¬rations.

Pyogenic granuloma Clinical Features:

• Commonly occur on the gingiva, especially in the interdental region of the anterior maxillary teeth. May also occur in the lip, tongue, and buccal mucosa.
• It appears as an elevated, sessile or pedunculated mass with erythematous over¬lying mucosa. The surface is smooth or lobulated.
• Spontaneous bleeding is common, and even a slight provocation causes profuse bleeding.
• It is painless and soft in consistency.
• It develops rapidly and reaches full size, a few millimeters to several centimeters, then remains static.
• Long-standing pyogenic granulomas may show fibrous changes and undergo dystrophic calcification.

Pyogenic granuloma Diagnosis:

• Clinical features
• Intraoral periapical radiograph to rule out bone resorption.
• Histopathological features.

Pyogenic granuloma Treatment: Surgical excision.

Question 8. Discuss fibroma.
Answer:

• Fibroma is the most prevalent form of benign, soft tissue tumor of the oral cavity.
• It is a connective tissue, a reactive tumor that arises in response to trauma or local irritation.

Fibroma Clinical Features:

• May occur at any site of the oral cavity, but buccal mucosa along the plane of occlusion is the common site.
• It appears as an elevated, sessile, nodular growth with a smooth surface.
• The overlying mucosa is normal in color. It is firm in consistency and non-tender.
• Common in the 3rd, 4th, and 5th decades of life and females are affected more in a ratio of 1:2, male: female.

Fibroma Differential Diagnosis: Peripheral giant cell granuloma, neurofibroma and mucocele.

Fibroma Treatment: Surgical excision.

Question 9. Lipoma.
Answer:

• Lipoma is a slow-growing, benign adipose tissue tumor comprising of mature fat cells. It is relatively rare in the oral cavity.
• Intramuscular lipoma arises in the skeletal muscles and occurs in mid and late adulthood.

Lipoma Clinical Features:

• Well-circumscribed, sessile or pedunculated growth with smooth or lobulated surface and slippery margins.
• Soft in consistency, freely movable, and non-tender.
• Deeper lesions are more diffuse and only slightly elevated.
• Buccal mucosa, tongue, and floor of the mouth are the common sites.
• Multiple fibromas are associated with:
  • Gardner’s syndrome
  • Proteus syndrome
  • Multiple familial lipomatosis syndrome
  • Encephalocraniocutaneous lipomatosis
• Histological types:
  • Fibrolipoma
  • Angiolipoma
  • Myxolipoma
  • Adenolipoma

Lipoma Treatment: Surgical excision.

Question 10. Hemangioma.
Answer: Hemangiomas are vascular malformation that develops within the first month of life, proliferate rapidly, and then involute within the childhood period.

Hemangioma Types:

1. Capillary hemangioma
2. Cavernous hemangioma
3. Angioblastic hemangioma
4. Racemose hemangioma
5. Port-wine stain
6. Hereditary hemorrhagic telangiectasia.

Hemangioma Oral Manifestations:

• Flat or raised, well-circumscribed lesions of the mucosa. Deep red or bluish red.
• Soft and compressible.
• Lip, tongue, buccal mucosa, and palate are commonly affected.
• Central hemangioma of the maxilla and mandible may occur but the involvement of the mandible is more common.

Syndromes-associated with Hemangiomas

• Osler-Weber-Rendu syndrome
• Sturge-Weber syndrome (Port-wine stain)
• Maffucci syndrome—(Hemangioma of mucous membrane and dyschondroplasia).
• Von Hippel-Lindau syndrome.

Diascopy — chair side investigation In hemangioma, there will be complete blanching of the lesion when pressure is applied on the surface.

Hemangioma Radiographic Features:

• Central hemangioma shows a honeycomb appearance on the radiographs with radia¬ting spicules extending at the periphery forming a sunray appearance.
• Ultrasonography is useful in determining the angiomatous nature.
• Contrast-enhanced MRI is used to differentiate hemangioma from lymphangioma.

Hemangioma Treatment: Usually undergoes spontaneous remission. In case of failure to undergo remission, treatment option depends on the type of lesion and amount of residual deformity.

• Surgery
• Radiotherapy
• Intralesional injection of sclerosing agents
• Cryotherapy.

Question 11. Paget’s disease
(or)
osteitis deformans.
Answer:

• Paget’s disease is a chronic, noninflam-matory, bone remodeling disease that affects the adult population (> 40 years).
• Males are affected more than females. Its prevalence is worldwide and is the most common bone disorder next to osteoporosis.
• It is characterized by excessive bone resorption together with rapid, disorganized bone remodeling. The etiology is unknown but genetic predisposition is noted.
• The possibility of slow-virus infection is also considered as a causative factor (paramyxo¬virus nucleocapsid particles and canine distemper virus have been observed in osteoclasts).
• It preferably involves the axial skeleton (spine, pelvis, femur, sacrum, and skull in descending order of frequency), but may affect any bone.
• Leontiasis ossia or lion¬like face is the term used to characterize the overgrowth of the facial and cranial bones in Paget’s disease.
• The involvement of the maxilla is two times more than that of the mandible. Progressive enlargement of the maxilla, alveolar bone expansion, and flattened appearance of the palate are common manifestations.
• Migration of teeth causes spacing and mobility. Edentulous patients complain difficulty in wearing dentures because of increasing tightness after the alveolar expansion.
• The common symptoms include tinnitus, vertigo, deafness, malocclusion, enlarged maxilla, and cranial nerve disorders.

Three phases are stated in Paget’s disease progression:

1. Paget’s disease starts with the lytic phase. Osteoclasts resorb normal bone. Bone turnover rates increase up to 20 times normal.
2. The second stage is the mixed phase. Numerous osteoblasts are involved in rapid bone formation. The new bone contains haphazardly arranged collagen fibers and appears morphologically abnormal.
   • As the osteoclastic and osteoblastic activities recur, a high- degree of bone turnover occurs.
   • In the sclerotic phase, bone formation is more but in a disorganized pattern (woven bone). The woven bone is weaker than normal adult bone and allows the infiltration of fibrous tissue and blood vessels to the bone marrow leading to a hypervascular state.
3. After an undefined period, osteoclastic activity decreases, but abnormal bone formation continues. Over a period, osteoblastic activity also reduces, and the bone becomes quiescent known as a burned-out phase.

• Sarcomatous changes of pagetic bone are rare but tedious complications.

Paget’s disease Oral Complications: Increased incidents of oral infections and pathological fracture of jaw bone are the associated complications.

Paget’s disease Investigation: Serum alkaline phosphatase and bone-specific alkaline phosphatase levels are valuable markers in the diagnosis of Paget’s disease.

Paget’s Disease Management;

• Treatment does not cure Paget’s disease, but it can control it. Drug therapy for Paget’s disease should include:
  • Bisphosphonate treatment with serial monitoring of bone markers.
  • Around 1,000-1,500 mg of calcium and vitamin D 400 IU, daily in conjunction with bisphosphonate treatments.
  • Chemotherapy and radiotherapy are used to treat neoplasms arising from the pagetic bone.
  • Morbidity from Paget’s disease can be extensive. A more central lesion carries an unfavorable prognosis.

Question 12. Lymphangioma.
Answer:

• Lymphangiomatous lesions are congenital malformations of the lymphatic system. It has a predilection for the cervicofacial area. The proliferating vessels contain lymph — a clear protein-rich fluid with few cells.
• They occur either as isolated lesions or in asso¬ciation with hemangiomas. It commonly occurs on the tongue like a soft mass without clear boundaries.
• Lymphangiomas are classified as microcystic (capillary lymphangiomas), macrocystic (cavernous lymphangiomas), and cystic hygromas based on the size of the lymphatic cavities. When a lymphangioma is confined to solid organs like the tongue, it manifests as cavernous lymphangioma, but when it develops in a loose fascia of the neck, a cystic lesion occurs.

Lymphangioma Management:

• Surgical resection is the preferred treatment.
• Sclerotherapy using dextrose, tetracycline, doxycycline, bleomycin, and OK-432 can reduce the complications of surgery.

Question 13. Schwannoma
(or)
Neurilemmoma.
Answer: Schwannoma is a benign tumor of Schwann cells or neurilemma cells.

Schwannoma Clinical Features:

• A slow-growing, circumscribed, tumor.
• Occur on the tongue, palate, floor of the mouth, buccal mucosa, gingival, and lip.
• Central lesions may occur within the mandible and arise from the mandibular nerve.
• Central lesions produce significant bone destruction and expansion of the cortical palate.
• Usually painless. Pain occurs when they exert pressure on adjacent nerves.

Schwannoma Treatment: Surgical excision.

Question 14. Neurofibroma.
(or)
Von Recklinghausen’s disease
(or)
Neurofibromatosis.
Answer: Neurofibroma is a derivative of the nerve sheath cells. It is a benign tumor that occurs either as an independent lesion or as a part of neurofibromatosis syndrome.

Neurofibroma Clinical Features:

• The tumor occurs as discrete nodules with normal mucosal color.
• Involvement trigeminal nerve causes pain.
• In the case of multiple endocrine neoplasia syndrome (MEN type 2), mucosal neuromas are the early manifestation.
• Central lesions may occur within the mandible and are associated with the mandibular nerve.
• Sarcomatous transformation of neurofibromatosis is also described as fibrosarcoma, neurogenic sarcoma, and spindle cell carcinoma.

Neurofibroma Radiographic Findings: Central lesion with mandibular nerve involvement reveals blunderbuss expansion of infe¬rior alveolar foramen with uniformly expanded alveolar canal.

Neurofibroma Treatment:

• Surgical excision for single neurofibroma.
• Removal of multiple lesions is not possible and should be considered only when malignant transformation is suspected.

Question 15. Hereditary hemorrhagic telangiectasia.
(or)
Osler-Weber-Rendu syndrome.
Answer:

• It is a triad of telangiectasia, recurrent epis- taxis, and a positive family history of this disorder.
• The major complication associated with this condition is multiorgan arteriovenous malformation. The main organs are the GI tract, pulmonary vasculature, brain, and nasal mucosa.
• Skin lesions are common on the face, neck, and chest. Intraoral lesions involve the lip, gingiva, buccal mucosa, tongue, and floor of the mouth.
• A defect in the perivascular supporting tissue results in the weakening of small vessels of skin and mucosa. Both bleeding and clotting time are normal in affected individuals.
• Pressure packs control spontaneous hemorrhage. Telangiectatic areas can be treated with radiotherapy, cauterization, or surgery.

Question 16. Sturge-Weber syndrome
(or)
Encephelo- trigeminal angiomatosis.
Answer:

• It is a congenital hamartomatous malformation that affects the eye, skin, and central nervous system at different times.
• This syndrome is characterized by venous angiomas of leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, skull, jaws, and oral soft tissues.
• The facial cutaneous capillary venous angiomas (Port-wine nevi) is the first component of the syndrome to be observed at birth. It is confined to the skin supplied by the trigeminal nerve.
• Intracranial calcifications are the second common finding that are noted on radio-graphs.
• Neurological manifestations are common and include convulsions, spastic hemiplegia with/without mental retardation

Neurofibroma Management: Neurological care for seizures.

Question 17. Odontogenic myxoma.
Answer:

• Odontogenic myxomas are benign tumors derived from embryonic mesenchymal elements of dental papilla, follicle or periodontal ligament. It is an unencapsulated, locally infiltrating tumor.
• They are slow-growing, painless, and site-aggressive tumors. Since asymptomatic, the lesions enlarge significantly before the patient reports. Extensive lesions may cause migration of teeth and bone expansion.
• On radiologic examination, the lesions appear either as unilocular or multilocular patterns with well-defined or diffused margins.
• Fine, bony septa within the radio- lucency are known as wispy trabeculations and are characteristic of this tumor. They give a honeycombed, soap bubble, or tennis racket appearance to the lesion. The tumor is scalloped between the roots, but root resorption is infrequent.
• The preferred treatment is surgical excision by enucleation, curettage, or block resec¬tion.

Question 18. Ameloblastic fibroma.
Answer:

This is a tumor of childhood, seldom is it seen beyond age 20.

• Site: The preferred location for this tumor is the posterior mandible followed by the posterior maxilla.
• Symptoms: Patients present with a pain¬less swelling in the jaw. The lesion may disturb the eruption of teeth in the area. An impacted tooth may often be associated with the tumor in approximately in % of the reported cases.
• Some lesions are asymptomatic, and detected upon routine dental radiographs.
• Radiographically, ameloblastic fibromas are unilocular lesions, or multilocular if when large. They have smooth well-demarcated borders. Cortical expansion may or may not be seen on radiographs. As lesions are frequently associated with unerupted teeth, often they are misdiagnosed as dentigerous cysts.
• Both odontogenic epithelium and odontogenic ectomesenchyme constitute this tumor.
• Surgical excision with removal of the affected teeth is the treatment of choice. Recurrence is rare.

Question 19. Cementoblastoma
(or)
True cementoma.
Answer:

• Cementoblastoma is a benign tumor of odontogenic origin derived from ecto- mesenchyme. The most common site
  for occurrence is a mandibular molar region with 50% of the cases involving the mandibular first molar. It may be completely asymptomatic or rarely causes pain and swelling.
• This tumor typically occurs around the roots of the lower posterior teeth. When they cause bony expansion, pain is experienced.
• Radiographically, it appears as a well-defined, ball-like radiopacity with a thin halo of radiolucency attached to the root apex.
• Cementoblastoma continues to grow until it is removed, and hence it is a true tumor—a true cementoma, in contrast to cementoma which is present around the root apices of the lower incisor with self-limiting growth.

Cementoblastoma Treatment: Surgical: Removal of the tumor along with the affected tooth, and peripheral ostectomy.

Question 20. Giant cell granuloma.
Answer:

• Giant cell granuloma is a benign tumor. The cause of occurrence is unknown and the lesion is uncommon. They may be either centrally located within the jaw or peripherally located on the gingiva.
• The central lesion of the jaw may be aggressive or non-aggressive. Aggressive lesions are equal to or greater than 5 cm in size and tend to overgrow and cause tooth displacement, root resorption, cortical bone perforation or thinning out. Recurrence is common following the curettage.
• Non-aggressive lesions are slow growing, asymptomatic, and do not recur following the surgical treatment.
• Giant cell granuloma Treatment:
  • Curettage
  • Intralesional corticosteroids, calcitonin injections, and interferon-alpha therapy. They are alternatives as well as adjuncts to the surgical procedure.
• Peripheral giant cell granuloma is the frequently occurring extraosseous giant cell lesion. It looks as a bluish-purplish- red nodule and is soft in consistency and resembling the pyogenic granuloma in appearance.
• Cupping or superficial resorption of the underlying alveolar crest is present. Treatment comprises surgical resection.

Question 21. Granular cell tumor.
Answer:

• A granular cell tumor is a nodular hamartomatous lesion occurring on the tongue and gingiva (granular cell epulis). The nodular growth has overlying pseudoepithelio- matous hyperplasia and hence leads to a misdiagnosis of squamous cell carcinoma.
• The granular cells are the derivatives of neural crest cells (primitive Schwann cells).
• The lesion may occur as single or multiple. Surface ulceration and pain are common.

Granular cell tumor Investigation: On immunochemical study, neuron-specific enolase, and S-100 protein markers are seen on the tumor cells.

Granular cell tumor Treatment: Surgical excision. Recurrence is unusual.

Question 22. Nevoid basal cell carcinoma syndrome.
(or)
Basal cell nevus syndrome.
(or)
Gorlin syndrome.
Answer: It is a hereditary condition characterized by the following major and minor features.

Gorlin syndrome Major Features:

• Orofacial structures:
  • Odontogenic keratocysts has a predilection for mandibular angle and maxillary 3rd molar region. The affected bone is extensively involved in the cystic cavity before the patient realizes the symptoms like pain and migration of teeth.
  • Ameloblastoma and fibrosarcoma of jaw bones, but the incidence is minimal.
  • Bifid ribs and underdeveloped ribs.
  • Skin: Epidermoid cysts, multiple basal cell carcinoma, palmar and plantar pits.
  • Calcification of falx cerebri.

Gorlin syndrome Minor Features:

• Macrocephaly
• Cleft lip and palate, frontal bossing, sunken eyes, broad nasal bridge and mandibular prognathism.
• Syndactyly and pectus deformity.
• Medulloblastoma and cerebellar tumor of childhood.
• The presence of any two major or one major and two minor features will establish the diagnosis.

Question 23. Non-Hodgkin’s lymphoma.
Answer:

• It is a lymphoproliferative malignancy involving lymph nodes and lymphoid organs as well as extranodal organs and tissues.
• Different types of Non-Hodgkin’s lymphoma are identified, among which large B-cell lymphoma and follicular lymphoma are common.
  • Human T-cell leukemia virus is identified as the cause, and older adults are affected commonly. The other causative factors include immunosuppression, Helicobacter pylori, and advancing age.
  • Lymphadenopathy is a persistent finding.
  • Other associated symptoms include fever, night sweats, fatigue, weight loss, chest pain, and cough.
  • Non-Hodgkin’s tumor generally occurs in lymphatic vessels, tonsils, adenoids, bone marrow, spleen, and thymus.
  • Oral findings include diffuse swelling in the buccal vestibule, hard palate or gingiva that are erythematous, purplish, and ulcerated.
  • Treatment is radiation and chemotherapy.

Question 24. Langerhans cell histiocytosis.
Answer:

• Langerhans cell histiocytosis (LCH) is an idiopathic histiocytosis disorder characterized by the presence of granulomas.
• They are composed of proliferating bone marrow-derived Langerhans cells including T-cells, macrophages, eosinophils, and inflammatory cells.

It comprises three types of lesions:

1. Eosinophilic granuloma: Single or multiple bone lesions are present in affected children and young adults. The extraskeletal site involvement is not seen.
2. Hand-Schuller: Christian syndrome: Extensive lesions involving both skeletal and extraskeletal regions. It affects children and progresses gradually.
3. Letterer-Siwe disease: Disseminated acute or subacute condition. It is a fatal disease due to their extensive involvement. Children younger than 3 years of age are affected.

• • Jaw lesions are common in Langerhans cell granulomatosis. The mandibular body- angle region is the most common site of involvement. In the oral cavity, localized swelling, pain, ulcers, gingival necrosis, alveolar bone destruction, tooth mobility, and exfoliation are the notable findings.
  • Systemic complications are rare. Hence the majority of patients are diagnosed during the routine radiological investigation.

Langerhans cell histiocytosis Radiographic Findings:

• Localized, punched-out radiolucent lesion without peripheral sclerosis.
• Severe alveolar bone resorption produces the appearance of teeth floating in space.

Langerhans cell histiocytosis Management: Surgical curettage, radiotherapy, or chemotherapy or a combination of these modalities.

Question 25. Nodular fasciitis.
Answer:

• It is a fibrous proliferative tumor in sub-cutaneous tissue having a fast-growing tendency.
• It is otherwise known as subcutaneous fibromatosis or pseudosarcomatous fasci¬itis.
• Head and neck regions, trunk, and hands are frequently involved. Facial skin, parotid, tongue, oral mucosa, and buccinator muscle are the affected sites in the orofacial region.
• It is a self-limiting lesion, and the size reaches up to 2 cm, sometimes may exceed but always less than 5 cm. The etiology is unknown, but trauma is considered as a precipitating factor. Histologically it is composed of fibroblasts and myofibroblasts.
• The lesion is well-circumscribed, freely movable, and tender.
• Treatment is surgical excision. The tumor does not recur.