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Benign Connective Tissue Tumors.pptx
- 1. DR. AMRUTHA
- 2. BENIGN TUMOURS MALIGNANT TUMOURS TUMOUR-LIKE LESIONS CONTENTS
- 3. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 4. FIBROSARCOMA OSTEOSARCOMA HODGKIN’S LYMPHOMA BURKITT’S LYMPHOMA KAPOSI’S SARCOMA PLASMACYTOMA MULTIPLE MYELOMA MALIGNANT TUMOURS
- 5. PERIPHERAL GIANT CELL GRANULOMA CENTRAL GIANT CELL GRANULOMA ANEURYSMAL BONE CYST TUMOUR-LIKE LESIONS
- 6. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 7. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 8. Irritational fibroma, Traumatic fibroma, Focal fibrous hyperplasia, Fibrous nodule Most common benign soft tissue neoplasm of oral cavity Mostly reactive focal fibrous hyperplasia secondary to trauma FIBROMA
- 9. 4th to 6th decades M:F = 1:2 Can occur anywhere in the mouth but common sites are: Buccal mucosa, along the bite line Gingiva Labial mucosa Tongue Size: from few mm to several cms. Mostly > 1.5cms FIBROMA Clinical features
- 10. Well defined, asymptomatic; slow growing lesion Smooth-surfaced nodule; similar in colour to surrounding mucosa Sometimes white in colour: hyperkeratosis from continued irritation Sometimes inflammed Superficial ulceration + pain may be present Sessile or pedunculated FIBROMA Clinical presentation
- 13. Nodular mass of fibrous connective tissue covered with stratified squamous epithelium Non-encapsulated lesion; fibrous tissue blends into the surrounding connective tissue CONNECTIVE TISSUE Dense and collagenized; scattered inflammation Collagen fibres are arranged in radiating, circular or haphazard pattern EPITHELIUM Atrophy;flat rete ridges or thin and elongated rete ridges Sometimes hyperkeratosis (clinically white) FIBROMA Histopathology
- 15. Conservative surgical excision Recurrence is extremely rare FIBROMA Treatment
- 16. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 17. Calcifying or ossifying fibroid epulis; peripheral fibroma with calcifications Relatively common gingival growth Reactive rather than neoplastic Mineralised product: origin from cells of periosteum or periodontal ligament PERIPHERAL OSSIFYING FIBROMA
- 18. Common in children and young adults; 10-19 yrs M:F = 1:2 to 2:3 Occurs exclusively in the gingiva Slight predilection for the maxillary arch More than 50% occur in incisor cuspid region Adjacent teeth: usually unaffected; sometimes can cause migration and loosening PERIPHERAL OSSIFYING FIBROMA Clinical features
- 19. Nodular mass Pedunculated or sessile Usually arises from the interdental papilla Red to pink in colour; usually ulcerated Mostly less than 2cms DIFFERENTIAL DIAGNOSIS Red lesions: pyogenic granuloma Pink non-ulcerated lesions: irritational fibroma RADIOGRAPHICALLY: superficial erosion PERIPHERAL OSSIFYING FIBROMA Clinical presentation
- 21. Fibrous proliferation associated with the formation of mineralised product EPITHELIUM Intact or ulcerated layer of stratified squamous epithelium When ulcerated: surface is covered by fibrinopurulent membrane + subjacent zone of granulation tissue CONNECTIVE TISSUE Cellular mass of connective tissue showing large numbers of proliferating fibroblasts Has delicate fibrillar stroma PERIPHERAL OSSIFYING FIBROMA Histopathology
- 22. Several forms of CALCIFICATION occur Bone: in the form of single or multiple interconnecting trabeculae of bone or osteoid; older lesions demonstrate mature lamellar bone Cementum-like material: ovoid droplets of basophilic cementum-like material which closely resembles acellular cementum Dystrophic calcifications: present as multiple granules, tiny globules or irregular masses of basophilic mineralised material; these calcifications are more in early ulcerated lesions Occasionally GIANT CELLS may be present PERIPHERAL OSSIFYING FIBROMA Histopathology
- 24. Local surgical excision down to the periosteum Should be submitted for histopathologic examination Adjacent teeth should be thoroughly scaled to prevent further irritation Recurrence rate of 8-16% is reported PERIPHERAL OSSIFYING FIBROMA Treatment
- 25. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 26. Benign tumour of fat tissue First described by ROUX (in 1848) as yellow epulis Relatively rare intraoral tumour; more frequent in subcutaneous tissues of the neck The cells of lipoma differ metabolically from the normal fat cells even though they are histologically similar LIPOMA
- 27. Usually found in adults above 40yrs No gender predilection Buccal mucosa and buccal vestibule are the most common sites Can also occur on tongue, floor of the mouth and gingiva LIPOMA Clinical features
- 28. Slow growing; soft, smooth- surfaced nodular mass Sessile or pedunculated Mostly less than 3cms in size INTRAORAL LIPOMAS can be classified into Superficial form Well encapsulated Yellow in colour Soft; freely movable beneath the mucosa Diffuse form Present in deeper surfaces; produces surface elevation More diffuse and gives the feel of a fluid on palpation LIPOMA Clinical presentation
- 30. Neurofibromatosis Gardner syndrome Encephalo-cranio-cutaneous lipomatosis Multiple familial lipomatosis Proteus syndrome LIPOMA Multiple lipomas……
- 31. Lipoma is composed predominantly of mature adipocytes or fat cells Well demarcated from the surrounding connective tissue by a fibrous capsule Lobular pattern is seen: collagenous streaks can be seen seperating the fat cells into lobules Sometimes lesional fat cells infiltrate surrounding tissues in the form of long thin extensions Extensive involvement of a wide area: LIPOMATOSIS LIPOMA Histopathology
- 33. Conservative local excision Recurrence is rare LIPOMA Treatment
- 34. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 35. A hemangioma is a benign and usually self- involuting tumor (swelling or growth) of the endothelial cells. HEMANGIOMA
- 36. Occur in infants and children; for central hemangiomas of jaws: peak age is second decade M:F = 1:3 TO 1:5 Whites are more commonly affected than the dark- skinned individuals MOST COMMON LOCATION IS THE HEAD AND NECK: ACCOUNTING FOR 60% OF ALL CASES HEMANGIOMA Clinical features
- 37. Superficial tumours: appear raised and bosselated with a bright red colour; firm and rubbery on palpation Deep tumours: slightly raised with a bluish hue HEMANGIOMA Clinical presentation
- 38. Fully developed hemangiomas are rare at birth It presents as a pale macule with thread like telangiectasias on the skin Proliferative phase This phase lasts for a few weeks where rapid pace of growth is observed 6-10 months after the proliferative phase the tumour begins to involute Colour changes to deep- purple hue; by the age of 5 yrs most of the red colour is gone Lesion feels less firm in palpation HEMANGIOMA Course of hemangioma…
- 39. 50% of the tumours show complete resolution by 5 yrs; 90% resolve by 9 yrs AFTER TUMOUR REGRESSION Normal skin might be restored Or permanent changes might be skin which include Atrophy Scarring Wrinkling Telangiectasias Complications might also occur which include ulceration and hemorrhage HEMANGIOMA Course of hemangioma…
- 41. Rendu- Osler- Weber syndrome Sturge- Weber syndrome Maffuci syndrome von Hippel Lindau syndrome HEMANGIOMA Syndromes associated…
- 42. Flat/ raised lesion of the mucosa Deep red/ bluish red in colour Readily compressible and fills slowly when released Common sites: lips, tongue, buccal mucosa and palate Usually traumatised, undergoes ulceration and secondary infection INTRAMUSCULAR AND CENTRAL HEMANGIOMAS are also reported in the oral cavity HEMANGIOMA Oral manifestations…
- 44. Central hemangioma: Occur in maxilla and mandible; 2/3rds in mandible First 2 decades of life bone destructive lesion: honey comb appearance in radiograph Root resorption is seen in some cases; vitality is not affected HEMANGIOMA Oral manifestations…
- 45. Angiography Ultrasonography Contrast enhanced MRI: can differentiate between hemangioma and lymphangioma MRI HEMANGIOMA Radiographic imaging
- 46. Three common types Cellular hemangioma Capillary hemangioma Cavernous hemangioma Cellular hemangioma Extensive endothelial proliferation Numerous plump endothelial cells Indistinct vascular lumina It may develop into a simple hemangioma or involute HEMANGIOMA Histopathology
- 48. Capillary hemangioma Many small capillaries lined by a single layer of endothelial cells Connective tissue stroma is present Compared to cellular hemangioma the endothelial cells are flat vascular spaces become evident During involution, vascular spaces become less prominent and are replaced by fibrous connective tissue HEMANGIOMA Histopathology
- 52. Cavernous hemangioma Large dilated blood sinuses with thin walls showing endothelial cells Sinusoidal spaces are usually filled with blood Sometimes lymph vessels may be present HEMANGIOMA Histopathology
- 56. Many congenital hemangiomas undergo spontaneous regression Case that do not show regression or those that arise in older persons have to be treated Surgery Radiation therapy Sclerosing agents injected into the lesion Carbon dioxide snow Cryotherapy Compression Recurrence and malignant transformation are rare HEMANGIOMA Treatment
- 57. Vascular malformations are structural anomalies of blood vessels without endothelial proliferation Present at birth and persist throughout life Are in continuity with the normal vasculature HEMANGIOMA vs VASCULAR MALFORMATION
- 58. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEURILEMMOMA NEUROFIBROMA BENIGN TUMOURS
- 59. Benign hamartomatous hyperplasia of lymphatic vessels Developmental malformations that arise from sequestrations of lymphatic tissue that do not communicate normally with rest of the lymphatic system CLASSIFICATION Lymphangioma simplex Cavernous lymphangioma Cystic lymphangioma LYMPHANGIOMA
- 61. 50% of the lesions are noted at birth and around 90% develop by 9yrs of age M = F 50-70% of lesions occur in head and neck; most common location being the lateral neck LYMPHANGIOMA Clinical features
- 62. Most common location is the anterior two-thirds of the tongue Other locations: palate, buccal mucosa, gingiva and lips Usually superficial in location Demonstrates pebbly surface which resembles a cluster of translucent vesicles The clinical appearance is simulated to tapioca pudding or frog eggs Secondary haemorrhage may cause the vesicles to appear purple Deep tumours present as soft ill-defined masses LYMPHANGIOMA Oral manifestations
- 64. Small lymphangiomas less than 1cm occur on the alveolar ridge: common in black neonates These lesions occur bilaterally on the mandibular ridge M:F = 2:1 They resolve spontaneously Central lymphangiomas are also reported : not common in the oral cavity LYMPHANGIOMA Oral manifestations
- 66. Lymphangiomas consist of multiple intertwining lymph vessels in a loose fibrovascular stroma UNENCAPSULATED The lining endothelium is typically thin; contains single layer of endothelial cells with flattened nuclei They usually contain lymph Some channels may contain RBCs: likely represent secondary hemorrhage SOME MAY BE ACTUAL EXAMPLES OF HEMANGIO-LYMPHANGIOMA LYMPHANGIOMA Histopathology
- 69. In INTRAORAL TUMOURS Lymphatic vessels are characteristically located beneath the epithelial surface They replace the connective tissue papillae: little or no connective tissue is present between the lymph vessels and the epithelium This superficial location results in the appearance of translucent vesicle- like appearance Extension into deeper tissues might also be seen LYMPHANGIOMA Histopathology
- 71. Lymphangioma simplex: small, thin walled lymphatics are seen Cavernous lymphangioma: dilated lymphatic vessels with surrounding adventitia Cystic lymphangioma: huge, macroscopic lymphatic spaces with surrounding fibrovascular tissues LYMPHANGIOMA Histopathology
- 72. THE SIZE OF THE VESSELS MAY DEPEND ON THE NATURE OF THE SURROUNDING CONNECTIVE TISSUE CAVERNOUS LYMPHANGIOMA: more frequent in the mouth, here denser surrounding connective tissue and skeletal muscle limit vessel expansion CYSTIC LYMPHANGIOMAS: neck and axilla, here loose adjacent connective tissue allows for expansion of the vessels LYMPHANGIOMA Histopathology
- 73. Spontaneous regression is rare Radioresistant and insensitive to sclerosing agents Surgical excision is the treatment of choice Recurrence is common, especially for cavernous lymphangiomas of the oral cavity: because of their infiltrative nature Surgical debulking of the tumour is the typical treatment provided and additional debulking procedures might be required as the child grows LYMPHANGIOMA Treatment
- 74. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEUROFIBROMA NEURILEMMOMA BENIGN TUMOURS
- 75. Neurofibroma is a benign tumour of nerve tissue origin Most common type of peripheral nerve neoplasm ORIGIN cells that constitute the nerve sheath, including SCHWANN CELLS and PERINEURAL FIBROBLASTS It can occur either as a solitary lesion or as a part of the syndrome: NEUROFIBROMATOSIS I NEUROFIBROMA
- 76. AGE: young adults No gender predilection LOCATION: skin is the most common location; intraoral locations are not uncommon INTRAORALLY Tongue and buccal mucosa are the most common sites CENTRAL LESIONS also occur; but are rare Occur in the mandible associated with the mandibular nerve NEUROFIBROMA Clinical features
- 77. Slow growing Soft, painless lesion May vary in size from small nodules to large masses If Trigeminal nerve involvement: causes facial pain or enlargement Central lesions present radiographically as enlargement of the mandibular canal, when mandibular nerve is involved As well demarcated or poorly defined unilocular or multilocular radiolucency NEUROFIBROMA Clinical presentation
- 79. Well- circumscribed: especially when the proliferation occurs within the perineurium of the involved nerve Tumours that proliferate outside the perineurium blend with the adjacent connective tissue Tumour is composed of Interlacing bundles of spindle shaped cells Cells often exhibit wavy nuclei Delicate collagen bundles Myxoid matrix Sparsely distributed small axons are present within the lesional tissue : demonstrated by silver stains NEUROFIBROMA Histopathology
- 82. Local surgical excision Recurrence is rare Malignant transformation is less but not rare NEUROFIBROMA Treatment
- 83. Also known as von Recklinghausen’s disease Incidence: 1 in 3000 individuals Etiology Mutation in neurofibromin gene Inherited as an autosomal dominant trait NEUROFIBROMATOSIS I
- 84. Patients have multiple neurofibromas that can occur anywhere on the body Most common on the skin Tumours may be present at birth; often appear during puberty They continue to develop slowly through adulthood Size varies from small papules to large nodules NEUROFIBROMATOSIS I Clinical features
- 86. Some patients have few lesions ; some have hundreds to thousands 2/3 of the patients affected with the syndrome suffer mild disease Accelerated growth is seen during pregnancy ELEPHANTIASIS NEUROMATOSA Massive baggy pendulous masses present on the skin PLEXIFORM NEUROFIBROMA On palpation gives the feeling of a bag of worms NEUROFIBROMATOSIS I Clinical presentation
- 87. Presence of café au lait spots Pigmentation on the skin; smooth-edged, yellow-tan to dark brown macules Vary in diameter from 1-2mm to several cms Present during birth or develop during the first year of life Axillary freckling Also known as Crowe’s sign Lisch nodules Translucent brown pigmentation spots on the iris Seen in all the affected individuals NEUROFIBROMATOSIS I Clinical presentation
- 89. 7-20% show oral manifestations Discrete non-ulcerated nodules which are of same colour of the mucosa Location: buccal mucosa, palate, alveolar ridge, vestibule and tongue Sometimes may present as diffuse masses involving tongue (presents as macroglossia) and alveolar ridge Enlargement of fungiform papillae is seen in 50% of patients Central lesions can also occur NEUROFIBROMATOSIS I Oral manifestations
- 90. Two or more of the following features 6 or more café au lait spots More than 5mm in prepubertal and more than 15mm in postpubertal individuals 2 or more neurofibromas or one plexiform fibroma Axillary freckling Optic gliomas Two or more Lisch nodules Sphenoid dysplasias or thinning f long bone cortex First degree relative with diagnosis of NF-1 NEUROFIBROMATOSIS I Diagnosis
- 91. No specific therapy Treatment directed towards prevention and management of complications Facial neurofibromas can be removed for cosmetic purposes Genetic counselling and evaluation of the other family member is required NEUROFIBROMATOSIS I Treatment
- 92. MPNST: seen in 5% of individuals Other sarcomas like fibrosarcoma and neurosarcoma may also occur NEUROFIBROMATOSIS I Complications
- 93. FIBROMA PERIPHERAL OSSIFYING FIBROMA LIPOMA HEMANGIOMA LYMPHANGIOMA NEUROFIBROMA NEURILEMMOMA BENIGN TUMOURS
- 94. Neurolemmoma, Schwannoma, Perineural Fibroblastoma, Neurinoma, Lemmoma Benign neural neoplasm of Schwann cell origin NEURILEMMOMA
- 95. Most common in young and middle aged adults; but can arise in any age, even during the first year of life M = F Few mm to several cm NEURILEMMOMA Clinical features
- 96. Encapsulated Slow- growing tumour Typically arises in association with a nerve trunk As it grows it pushes the nerve aside Tenderness and pain is not a common condition and usually occurs due to pressure on adjacent nerves NEURILEMMOMA Clinical presentation
- 97. Tongue is the most common location May also arise centrally and cause bony destruction with expansion of cortical plates Pain and paraesthesia accompany central lesions NEURILEMMOMA Oral manifestations
- 98. Encapsulated tumour Demonstrates two microscopic patterns in varying amounts ANTONI A ANTONI B ANTONI A Streaming fascicles of spindle shaped schwann cells These cells form a palisaded arrangement around central, acellular , eosinophilic areas: VEROCAY BODIES Verocay bodies: consist of reduplicated basement membrane and cytoplasmic processes NEURILEMMOMA Histopathology
- 101. ANTONI B pattern Shows tissue which is less cellular and less organized Spindle cells are randomly arranged within a loose myxomatous stroma No neurites are present within the tumour mass Degenerative changes can be seen in older tumours Hemorrhage Hemosiderin deposits Inflammation Fibrosis NUCLEAR ATYPIA NEURILEMMOMA Histopathology
- 104. Surgical excision Recurrence and malignant transformation are rare NEURILEMMOMA Treatment
- 105. Autosomal dominant condition Caused by mutation of gene producing the protein merlin CHARACTERISTIC FEATURES Bilateral neurilemmomas of auditory vestibular nerve Neurilemmomas of peripheral nerves Meningiomas of CNS SYMPTOMS Sensorineural deafness Dizziness Tinnitus NEURILEMMOMA Neurofibromatosis II
Editor's Notes
- Buccal mucosa along the biteline: presumably this is the consequence of trauma from cheek biting Gingiva: most gingival fibromas represent fibrous maturation of a pre-existing pyogenic granuloma