Ewing's sarcoma of the kidney.
Primitive neuroectodermal tumors (PNETs) are small, round-cell tumors of neural crest origin classically found in the central nervous system (CNS) but more recently characterized in the periphery. Peripherally located PNETs (pPNETs) are members of the Ewing's sarcoma family of tumors (EFTs). Renal localization of pPNETs is very rare, is found in young adults, and is characterized by an aggressive clinical course and poor prognosis. We present the case of a young man with renal pPNET characterized by psuedorosette formation, cluster of differentiation 99 (CD 99+), focally positive for neuron-specific enolase (NSE), with cytogenetic findings of the translocation t(11;22)(q24;q12) and the unique abnormality of trisomy 7. To our knowledge, we report the first case of trisomy 7 and PNET.
Angel
Joel R
JR
Department of Medicine, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, USA. jangel@mcw.edu
Alfred
Anushayanthan
A
Sakhuja
Ankit
A
Sells
Ryan E
RE
Zechlinski
Joseph John
JJ
D000071443:12E7 Antigen; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015815:Cell Adhesion Molecules / Q000032:analysis; D017024:Chemotherapy, Adjuvant; D002880:Chromosomes, Human, Pair 11; D002892:Chromosomes, Human, Pair 22; D002897:Chromosomes, Human, Pair 7; D006801:Humans; D007680:Kidney Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology / Q000601:surgery; D008297:Male; D009392:Nephrectomy; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D012512:Sarcoma, Ewing / Q000737:chemistry / Q000235:genetics / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic; D016896:Treatment Outcome; D014314:Trisomy
D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D010751:Phosphopyruvate Hydratase