BENIGN MESENCHYMAL TUMOR

Transcription

1 IN THE NAME OF GOD

2 BENIGN MESENCHYMAL TUMOR

3 o Giant Cell Fibroma o Fibrous Histiocytoma o Fibromatosis o Myofibroma o Lipoma o Neurilemoma o Neurofibroma o Neurofibromatosis Type I o Multiple Endocrine Neoplasia Type 2B

4 o Hemangioma and Vascular Malformations o Sturge-Weber Angiomatosis o Nasopharyngeal Angiofibroma o Lymphangioma o Leiomyoma o Rhabdomyoma o Osseous and Cartilaginous Choristomas

5 GIANT CELL FIBROMA Nodule Sessile or pedunculated Asymptomatic Papillary Papilloma Female Gingiva(50%) Mandibular gingiva(2) Tongue and palate

6 RETROCUSPID PAPILLA Gingiva lingual to the mandibular cuspid Bilateral Small, pink papule Common 25% to 99% Children and young adults Normal anatomic variation Disappears with age

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8 HP Vascular firous connective tissue Hallmark numerous large, stellate firoblasts within the superfiial connective tissue. Epithelium thin and atrophic Rete ridges narrow and elongated

9 FIBROMATOSIS Intermediate tumor Fibrous proliferations Head and neck: juvenile aggressive fibromatosis Bone : desmoplastic fibromas Firm Painless Rapid growth Children or young adults Most common oral site : paramandibular soft tissue Destruction of adjacent bone

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11 HISTOPATHOLOGIC FEATURES Cellular proliferation Spindle-shaped Streaming fascicles Poorly circumscribed Infiltrates the adjacent tissues

12 TREATMENT AND PROGNOSIS Locally aggressive Wide excision (margin of adjacent normal Tissues) Recurrence

13 FIBROUS HISTIOCYTOMA Firoblastic and histiocytic diffrentiation Dermatofiroma / sclerosing hemangioma / firoxanthoma / nodular subepidermal fibrosis True neoplasm Most common (skin of the extremities) Oral and perioral region (rare) Painless Nodular mass Vary in size Deeper tumors larger

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15 HP o Spindle-shaped firoblastic cells o Short, intersecting fascicles o Storiform pattern o Histiocyte-like cells o Multinucleated giant cells can be seen Larger lesions of the deeper soft tissues have a greater potential to recur

16 LIPOMA Benign tumor of fat Most common mesenchymal neoplasm Trunk and proximal of the extremities Oral and maxillofacial : less frequent More common in obese people Metabolism independent of the normal body fat

17 Nodular masses Sessile or pedunculated Soft Smooth-surfaced Asymptomatic Less than 3 cm Most common intraoral sites : buccal mucosa and buccal vestibule Tongue. floor of the mouth, and lips 40 y/o

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20 HISTOPATHOLOGIC FEATURES Mature fat cells Well circumscribed Lobular arrangement Microscopic variants : o Fibrolipoma o Angiolipoma o Myxoid lipoma o Pleomorphic lipomas o Intramuscular (infiltrating) lipomas

21 TREATMENT AND PROGNOSIS Conservative local excision Recurrence : rare Intramuscular lipomas : higher recurrence

22 NEURILEMOMA (SCHWANNOMA) Benign neural neoplasm of Schwann cell Uncommon Head and neck : 25% to 48% Slow-growing Encapsulated

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24 Pushes the nerve Asymptomatic Young and middle-aged adults Most common location : tongue Intraosseous : posterior mandible / unilocular or multilocular radiolucencies

25 HISTOPATHOLOGIC FEATURE Encapsulated Two microscopic patterns : 1)Antoni A 2)Antoni B

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27 Antoni A o Streaming fascicles of spindle-shaped Schwalm cells o Palisaded arrangement around central acellular, eosinophilic areas known as Verocay bodies Verocay bodies consist of reduplicated basement membrane and cytoplasmic processes Antoni B o Spindle cells o Less cellular and less organized o Randomly arranged

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29 Ancient neurilemomas : Hemorrhage Hemosiderin deposits Inflammation Fibrosis Nuclear atypia Still benign

30 TREATMENT AND PROGNOSIS Surgical excision Malignant transformation

31 NEUROFIBROMATOSIS TYPE I (VON RECKLINGHAUSEN'S DISEASE OF THE SKIN) Hereditary Most common : neurofibromatosis type I (NFl) Autosomal dominant trait Mutations : NF1 gene

32 Multiple neurofibromas (skin) : Small papules to larger soft nodules to massive baggy (elephantiasis neuromatosa) Plexiform neurofibroma : Bag of worms" Pathognomonic for NF 1 During puberty Wide variability in the expression of the disease : Only a few neurofibromas Hundreds or thousands of tumor

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35 Cafe au lait (coffee with milk) pigmentation on the skin: Another highly characteristic feature Macules Vary in diameter At birth (during the first year) Axillary freckling (Crowe's sign) : Highly suggestive sign Lisch nodules: Translucent brown-pigmented spots on the iris All

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38 The most common finding : enlargement of the fungiform papillae

39 Radiographic findings: 1. Enlargement of the mandibular foramen 2. Enlargement or branching of the mandibular canal 3. Increased bone density 4. Concavity of the medial surface of the ramus 5. Increase in dimension of the coronoid notch Unilateral enlargement that mimics hemifacial hyperplasia

40 TREATMENT AND PROGNOSIS No specific therapy Prevention or management of complications Cancer development : Malignant peripheral nerve sheath tumor Pheochromocytoma Leukemia Rhabdomyosarcoma Wilms tumor Average lifespan (15 years less than the general population)

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42 MULTIPLE ENDOCRINE NEOPLASIA TYPE 2B (MULTIPLE ENDOCRINE NEOPLASIA TYPE 3; MULTIPLE MUCOSAL NEUROMA SYNDROME) MEN 3 / MEN 2B o MEN syndromes o Group of rare conditions o Tumors or hyperplasias of the neuroendocrine tissues MEN type 1 (benign tumors of the pancreatic islets. adrenal cortex. Parathyroid glands. pituitary gland) MEN type 2A (adrenal pheochromocytomas and medullary thyroid carcinoma) MEN type 2B (mucosal neuromas of oral mucousa)

43 Marfanoid body : thin / elongated limbs / muscle wasting / narrow face Lips : thick and protuberant Oral mucosal neuromas : first sign Neuromas : o Soft o Painless o Papules or nodules o Lips and anterior tongue o Bilateral neuromas of the commissural mucosa (highly characteristic) Pheochromocytomas Neuroendocrine tumors Medullary carcinoma (thyroid gland) : 90% / 18 and 25

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45 LABORATORY VALUES Medullary carcinoma of the thyroid gland Calcitonin (serum or urinary) Pheochromocytomas Urinary vanillylmandelic acid (VMA) Epinephrine-to-norepinephrine ratios

46 HISTOPATHOLOGIC FEATURES Mucosal neuromas : hyperplasia of nerve bundles

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48 TREATMENT AND PROGNOSIS Removal of the thyroid gland at an early age

49 HEMANGIOMA AND VASCULAR MALFORMATIONS Hemangiomas Benign tumors of infancy Rapid growth Endothelial cell proliferation Cannot be recognized at birth (during the first 8 weeks) Vascular malformations o Structural anomalies of blood vessels o Without endothelial proliferation o Present at birth and persist throughout life o Categorized : type of vessel / hemodynamic features

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51 HEMANGIOMA OF INFANCY Most common tumors of infancy Females Whites Most common location: head and neck Single lesions Rapid development

52 Superficial tumors : Bosselated Bright-red color Strawberry hemangioma Firm and rubbery Blood cannot be evacuated by applying pressure Deeper tumors : Bluish hue

53 5 y/o 9 y/o Permanent changes : atrophy, scarring, wrinkling, or telangiectasias Complications : Most common problem : ulceration (secondary infection) Tumors in the neck and laryngeal region : airway obstruction

54 PHACE(S) syndrome Posterior fossa brain anomalies Hemangioma Arterial anomalies Cardiac defects Eye anomalies.sternal cleft Kasabach-Merritt phenomenon Serious coagulopathy (associated with hemangioma)

55 VASCULAR MALFORMATIONS 1. Port wine stains : o Common o Capillary malformation o Newborns o Trigeminal nerve o Sturge-Weber angiomatosis o Pink or purple macule

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57 2. Venous malformations : o At birth o Grow with the patient o Secondary thrombosis 3. Arteriovenous malformations : o Persistent direct arterial and venous communication o Bruit o Overlying skin (warmer.pain. bleeding. Ulceration)

58 INTRABONY VASCULAR MALFORMATIONS Arteriovenous malformations First 3 decades Females Mandible Radiographic appearance : variable Multilocular radiolucent (honeycomb or soap bubble) Ill-defined radiolucent Well.defined(cystlike radiolucency ) o Sunburst" radiographic

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60 HISTOPATHOLOGIC FEATURES Early hemangiomas of infancy: Numerous plump endothelial Indistinct vascular lumina Juvenile or cellular hemangiomas Mature lesions: Endothelial cells (flattened) Capillary-sized vascular spaces GLUTI

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62 TREATMENT AND PROGNOSIS Hemangiomas Port wine stains Venous malformations Surgical excision Sclerotherapy

63 LYMPHANGIOMA Benign Hamartomatous tumors of lymphatic Three types of lymphangioma: I. Lymphangioma simplex (capillary lymphangioma) 2. Cavemous lymphangioma : mouth 3. Cystic lymphangioma (cystic hygroma) : neck and axilla

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65 Head and neck : 50% to 75% At birth : 50% 2 years of age : 90% Oral lymphangiomas : Two thirds of the tongue (macroglossia) Pebbly surface (frog eggs or tapioca pudding) Small lymphangiomas on the alveolar ridge in black neonates

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67 HISTOPATHOLOGIC FEATURES lymphatic vessels lining endothelium : thin Intraoral tumors : lymphatic vessels Beneath the epithelial surface Translucent, vesicle

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69 TREATMENT AND PROGNOSIS Surgical excision Recurrence is common (cavernous lymphangiomas)

70 LEIOMYOMA Oral 1. Solid leiomyomas 2. Vascular leiomyomas (angiomyomas) 3. Epithelioid leiomyomas

71 Mucosal nodule Firm Slow-growing Asymptomatic Most common sites : lips, tongue,palate, and cheek Rare intraosseous (unilocular radiolucencies)

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75 RHABDOMYOMA 1) Adult rhabdomyomas o Middle-aged and older (Men) o Pharynx, oral cavity. and larynx o Intraoral lesions(floor of the mouth, soft palate and base of tongue) o Rapid growth 2) Fetal rhabdomyomas Young children Face and periauricular region

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77 OSSEOUS AND CARTILAGINOUS CHORISTOMAS Tumorlike growth Normal tissue in an abnormal location Most common choristomas : bone, cartilage, or both Soft tissue osteomas Soft tissue chondromas Not true neoplasms

78 Sessile or pedunculated nodule Firm Smooth-surfaced Females Tongue : 85% Most common location : posterior tongue near the foramen cecum

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