title: Primary primitive neuroectodermal tumor of kidney: a rare case report with diagnostic challenge.
abstract: Primary primitive neuroectodermal tumors (PNETs) of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm's tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES)/PNET have a specific t(11;22) which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient's condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.
authors.forename: Sunita
authors.initials: S
authors.lastname: Kakkar
authors.affiliation: Department of Pathology and Laboratory Science, Armed Forces Medical College, Command Hospital, Pune, Maharashtra, India.
authors.forename: Devika
authors.initials: D
authors.lastname: Gupta
authors.forename: Gurpreet
authors.initials: G
authors.lastname: Kaur
authors.forename: Vandana
authors.initials: V
authors.lastname: Rana
mesh_terms: D000071443:12E7 Antigen; D015703:Antigens, CD / Q000032:analysis; D015815:Cell Adhesion Molecules / Q000032:analysis; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006651:Histocytochemistry; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D008297:Male; D008840:Microfilament Proteins / Q000032:analysis; D008853:Microscopy; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000473:pathology*; D011860:Radiography, Abdominal; D013902:Radiography, Thoracic; D018160:Receptors, Cytoplasmic and Nuclear / Q000032:analysis; D014057:Tomography, X-Ray Computed; D015534:Trans-Activators; D055815:Young Adult
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; C098179:FLII protein, human; D008840:Microfilament Proteins; D018160:Receptors, Cytoplasmic and Nuclear; D015534:Trans-Activators