Basicmedical Key

Lymphoepithelioma-Like Carcinoma of the SkinHigh magnification of lymphoepithelioma-like carcinoma of the skin (LELCS) shows a prominent lymphohistiocytic infiltrate obscuring a background of large, undifferentiated epithelioid-appearing cells . Low Magnification of…

 Gorlin-Goltz syndrome: Multiple cutaneous basal cell carcinomas, odontogenic keratocysts of jaw, palmar and plantar pits, rare medulloblastomas, and skeletal abnormalities • Rare cases not associated with NBCCS also reported Clinical Issues…

 Range: 0.3-6.5 cm Microscopic • Well circumscribed and lobulated • Mostly composed of mature hyaline cartilage • Variable amounts of calcification • Ossification common • Granulomatous inflammation in 15% of cases • Rare tumors with extensive…

 Considered to fall on morphologic spectrum with myopericytoma, myofibroma, and angioleiomyoma Clinical Issues • Most common in young adults (20-40 years) • Female predilection in subungual tumors • Most common in distal extremities…

 Cells are markedly enlarged and atypical to bizarre-appearing Top Differential Diagnoses • Melanoma in situ, superficial spreading type • Melanoma in situ, lentigo maligna type • Atypical (dysplastic) junctional melanocytic nevus • Irritated/traumatized nevi…

 Includes angiomyolipoma (AML), clear cell sugar tumor of lung (CCST), lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumor of falciform ligament/ligamentum teres Etiology/Pathogenesis • Only AML, CCST, and LAM are associated with tuberous…

 Spindle cells much more common  Epithelioid cells usually dispersed individually throughout lesion • Melanocytes “mature” by becoming smaller from superficial to deep • Kamino bodies: Eosinophilic globules at dermal-epidermal junction • Artifactual clefting…

 Enlarges after upper respiratory tract infection • Along anterior border of sternocleidomastoid muscle • Initial work-up of suspected branchial cleft anomaly (in order)  Intravenous or oral antibiotics (if infected), FNA, endoscopy &/or…