Concluded Case

Identify with description : *Stains *Primary & Metastatic sites *Differential diagnosis *Cytogenetics *Rx


Concluded answer

ASPS Solid PAS-D & EM rhomboid crystals TFE3 + ve t(X;17) Fusion gene - TFE3 & ASPSCR1

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Alveolar soft part sracoma Tumor cells are large arranged in pseudoalveolar pattern separated by sinusoidal vascular channels. It's a disease of young .Common sites being extremities Female genital tract Breast Urinary bladder Head and neck region. Mets are usually to lung liver and brain (unlike other soft tissue tumors). Diff diagnosis Granular cell tumor Paragangliomas Renal cell carcinoma Stain PAS highlights intracytoplasmic PAS Positive crystals Desmin Positivity is seen. EM Rhomboid membrane bound crystals Treatment Surgical resection with adequate margins as local recurrence is common Chemotherapy and radiation combined with surgery C Met TK inhibitors and molecular targeted therapies under evaluation.

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Identify with description : *Stains : •H&E : Solid/Alveolar pattern, separated by fibrovascular septae, compactly arranged polygonal cells having abundant granular eosinophilic to clear cytoplasm with vesicular nucleus & prominent nucleolus.Minimal nuclear pleomorphism with rare mitosis. •PAS-D : positive showing intracytoplasmic rod like/ rhomboid crystals. *IHC : Positive for TFE3. *EM : Intracytoplasmic crystals+. *Primary sites : Thigh(Lower extremity);Tongue,orbit,eye(Head & neck);Viscera (Uterus,cervix, lung). *Metastatic sites : Lung,Liver,Bone,Brain. *Differential Diagnosis : •Rhabdomyosarcoma. *Paraganglioma. *Epithelioid Angiosarcoma. *Epithelioid Sarcoma. *Malignant Granular cell tumor. *Metastatic RCC/Melanoma/Adrenal gland carcinoma. *Cytogenetics : •t(X;17), Fusion gene - TFE3 & ASPSCR1 *Rx : Surgery + Angiogenic inhibitors, Tyrosine kinase inhibitors, Immunotherapy.

epitheloid hemangioendothelioma with abundant and eosinophilic and often vacuolated cytoplasm. stain for Weibel- Palade body WWTR1 on chromosome 3 and CAMTA1 on chromosome 1

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ASPS Solid PAS-D & EM rhomboid crystals TFE3 + ve t(X;17) Fusion gene - TFE3 & ASPSCR1