IN THE NAME OF GOD Dr. Kheirandish Oral and maxillofacial pathology

IN THE NAME OF GOD Dr. Kheirandish Oral and maxillofacial pathology

ORAL FOCAL MUCINOSIS Uncommon Tumorlike Cutaneous myxoid cyst Overproduction of hyaluronic acid by firoblasts Young adults Female Gingiva > hard palate Sessile or pedunculated Painless Smooth

Histopathologic Features o Well-localized but nonencapsulated o Loose, myxomatous connective tissue o Rete ridges o Firoblasts (within the mucinous area) ovoid, stellate o Few capillaries o Mucinous product is hyaluronic acid

GRANULAR CELL TUMOR Uncommon neoplasm Oral cavity Schwann cells Solitary Multiple (black patients)

Fourth to sixth decades Female Asymptomatic Sessile nodule Pink \ yellow Tongue > buccal mucosa

Histopathologic Features o Large, polygonal cells o (pale eosinophilic, granular cytoplasm \ dark or vesicular nuclei) o Sheets \ cords \ nests o Not encapsulated o Overlying epithelium o S-100 protein

CONGENITAL EPULIS (CONGENITAL EPULIS OF THE NEWBORN; CONGENITAL GRANULAR CELL LESION) Uncommon Alveolar ridges of newborns Slight microscopic resemblance to the granular cell tumor Pink-to-red Smooth-surfaced Polypoid mass Maxillary ridge Females 90%

Histopathologic Features o Large, rounded cells o (Abundant granular, eosinophilic cytoplasm and round o To oval, lightly basophilic nuclei) o Overlying epithelium o S-100 protein

MALIGNANT MESENCHYMAL TUMOR

Sarcoma Carcinoma Oral cavity : Rare o Fibrosarcoma o Malignant Fibrous Histiocytoma o Liposarcoma o Malignant schwannoma o Olfactory Neuroblastoma o Angiosarcoma o Kaposi's Sarcoma o Leiomyosarcoma o Rhabdomyosarcoma o Metastases to the Oral Soft Tissues

Fibrosarcoma Malignant tumor of fibroblasts Extremities Head and neck :10% Slow-growing Anywhere in the head and neck young adults and children

HISTOPATHOLOGIC FEATURES Well-differentiated : Fascicles Spindle-shaped cells Herringbone pattern Mitotic figures Poorly-differentiated : Less organized Rounder or ovoid More frequent mitotic activity Poorly differentiated tumors tend to produce less collagen than do well-differentiated tumors.

TREATMENT AND PROGNOSIS Surgical excision Wide margin of adjacent normal tissue Recurrence 50% 5-year survival : 40% to 70%.

MALIGNANT FIBROUS HISTIOCYTOMA Both fibroblastic and histiocytic features Adult Expanding mass May be painful or ulcerated HISTOPATHOLOGIC FEATURES Most common type : storiform-pleomorphic(short fascicles of plump spindle cells arranged in a storiform pattern, admixed with areas of pleomorphic giant cells) Myxoid/ giant cell/ inflammatory/ angiomatoid

TREATMENT AND PROGNOSIS Aggressive Radical surgical resection Recurrences : 40% Metastases : 40% Oral tumors : worse than other body sites

LIPOSARCOMA Malignant neoplasm of fatty origin Most common soft tissue sarcoma in adults Head and neck : rare Most common site of the head and neck : Neck Tongue and cheek Soft Slow-growing Ill -defined Normal in color or yellow Pain or tenderness : uncommon

HISTOPATHOLOGIC FEATURES 1. Well-differentiated liposarcoma/atypical lipomatous tumor Most common in the oral cavity Resemble benign lipomas Scattered lipoblasts and atypicalcells 2. Myxoid/round cell liposarcoma Myxoid stroma Rich capillary network The round cell liposarcoma is a more aggressive form of myxoid liposarcoma 3. Pleomorphic liposarcoma Extreme cellular pleomorphism Bizarre giant cells 4. Dedifferentiated liposarcomas Well -differentiated liposarcoma + poorly differentiated, nonlipogenic sarcomatous

TREATMENT AND PROGNOSIS Radical excision Recurrences : 15% to 20% Histopathologic subtype is important in prognosis : Pleomorphic liposarcomas is much worse than other types Oral liposarcoma : favorable (well-differentiated and small) metastasis: rare

MALIGNANT SCHWANNOMA Young adults Neurofiromatosis type I (29 to 36 years) Head&neck (14% to 19%) Mandible, lips, and buccal mucosa Pain

Histopathologic Features o Fascicles of atypical spindle-shaped cells o Firosarcoma o Wavy or comma-shaped nuclei o S-100 protein

Treatment and Prognosis Radical surgical excision Adjuvant radiation therapy and chemotherapy Prognosis poor Survival (39% to 60% at 5 years, and 26% to 45% at10 years)

OLFACTORY NEUROBLASTOMA (ESTHESIONEUROBLASTOMA) Arise from the olfactory epithelium Adult Nasal cavity Nasal obstruction Anosmia Epistaxis Pain

HISTOPATHOLOGIC FEATURES Small, round to ovoid basophilic cells Sheets and lobules Rosette and pseudorosette

TREATMENT AND PROGNOSIS Surgical excision Radiation therapy Chemotherapy Prognosis : Stage A (nasal cavity) / 72%-90% Stage B (paranasal Sinuses) / 59% -70% Stage C (beyond the nasal cavity and sinuses) / 50% Death : local recurrence Metastasis

Angiosarcoma Rare Vascular endothelium <50%...Head and neck Scalp and forehead Tongue and mandible Older adult Early lesions...simple bruise Enlarge \ elevated \ nodular \ ulcerated

Histopathologic Features o Infitrative proliferation of endothelium-lined blood vessels o endothelial cells hyperchromatic and atypical o Mitotic activity o CD31 \ factor VIII

Treatment and Prognosis Radical surgical excision Radiation therapy Prognosis poor 10-year survival (14% to 21%)

KAPOSI'S SARCOMA Unusual vascular neoplasm HIV HHV-8 Endothelial cells Four clinical presentations : 1. Classic 2. Endemic (African) 3. Iatrogenic immunosuppression associated 4. AIDS related

CLINICAL FEATURES CLASSIC TYPE : Chronic Late adult Men Italian. Jewish, or Slavic Multiple blue-purple macules and plaques Skin of the lower extremities Grow slowly Many years Painless(tumor nodules) Oral : rare ( palate)

ENDEMIC TYPE : Africa 1. Benign nodular : Similar to classic 2. Aggressive or infiltrative : Locally invasive Soft tissues and bone 3. Florid : Rapidly progressive Widely disseminated Aggressive Visceral involvement 4. Iymphadenopathic : Young black children Generalized Rapidly growing Lymph nodes Visceral Skin

IATROGENIC TYPE : Iatrogenic Immunosuppression Organ transplants Loss of cellular immunity Immunosuppressive drugs

HISTOPATHOLOGIC FEATURES Three stages: 1. Patch (macular) Endothelial cells Scattered lymphocytes and plasma cells 2. Plaque Proliferation vascular channels Spindle cell 3. Nodular Spindle cells Extravasated erythrocytes Slitlike vascular

TREATMENT AND PROGNOSIS Clinical subtype and stage Classic: radiation therapy Surgical excision Chemotherapy Classic / Lymphadenopathic

LEIOMYOSARCOMA Malignant neoplasm of smooth muscle differentiation Most common sites : uterine wall \ gastrointestinal tract Oral cavity : rare Middle-aged and older adults Enlarging mass Secondary ulceration

HISTOPATHOLOGIC FEATURES Fascicles Spindle-shaped cells Blunt-ended, cigar-shaped nuclei Five or more mitoses (per 10 HPF): malignant Masson trichrome stain Myogenic markers

TREATMENT AND PROGNOSIS Radical surgical excision The prognosis for oral tumors is guarded

RHABDOMYOSARCOMA Malignant neoplasm of skeletal muscle differentiation Young children (first decade) Most common sarcomas in childhood Most frequent site: head and neck (orbit / palate) Second most common location: genitourinary tract Males

Embryonal ( Botryoid / Spindle / NOS ) Most common in the first 10 years of life (60% of all cases) Alveolar ( Classic and Solid ) Between 10 and 25 years Pleomorphic Less than 5% of all cases Older than 40 years Extremities Most head and neck lesions: embryonal or alveolar types Painless Infiltrative

HISTOPATHOLOGIC FEATURES EMBRYONAL TYPE Small round or oval cells with hyperchromatic nuclei and indistinct cytoplasm Botryoid (grape like) : arise within a cavity demonstrate an exophytic. polypoid growth pattern that resembles a cluster of grapes. ALVEOLAR TYPE Classic pattern : poorly differentiated round to oval cells separated by fibrous septa. Solid pattern: small round basophilic cells without fibrovascular septa.

TREATMENT AND PROGNOSIS Local surgical excision Chemotherapy Radiation therapy Good prognosis : EMBRYONAL type

METASTASES TO THE ORAL SOFT TISSUES Uncommon Lymphatic Blood-borne Most common site : gingiva Next most common site : tongue Pyogenic granuloma Adjacent teeth : loosened (underlying destruction of the alveolar bone)

Male Middle-aged and older adults Men : lung cancer, renal carcinoma and melanoma Women : breast cancer, malignancies of the genital organs, lung, bone, and kidney Resemble the tumor of origin Most cases : carcinomas Metastatic sarcomas to the oral region are rare

TREATMENT AND PROGNOSIS Prognosis poor