Is primitive neuroectodermal tumor of the kidney a distinct entity?

• Published in: Cancer 1997-06, Vol.79 (11), p.2243-2250
• Main Authors: Rodriguez‐Galindo, Carlos, Marina, Neyssa M., Fletcher, Barry D., Parham, David M., Bodner, Sara M., Meyer, William H.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Child, Preschool; Combined Modality Therapy; Female; Humans; intrarenal neuroblastoma; Kidney Neoplasms - mortality; Kidney Neoplasms - pathology; Kidney Neoplasms - therapy; Kidneys; Male; malignant rhabdoid tumor; Medical sciences; Middle Aged; Neoplasm Metastasis; Nephrology. Urinary tract diseases; Neuroectodermal Tumors, Primitive - mortality; Neuroectodermal Tumors, Primitive - pathology; Neuroectodermal Tumors, Primitive - therapy; peripheral neuroepithelioma; primitive neuroectodermal tumor (PNET); renal neoplasms; Tumors of the urinary system; Wilms' tumor
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/(SICI)1097-0142(19970601)79:11<2243::AID-CNCR24>3.0.CO;2-V

BACKGROUND Primitive neuroectodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectodermal origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton in adolescents and young adults. As a soft tissue neoplasm, PNET arising in the kidney has not been well described, with only three cases previously reported. METHODS Four patients with PNET of the kidney were diagnosed and treated at St. Jude Children's Research Hospital. The authors reviewed the clinical, radiologic, and pathologic features and outcomes of these cases and of those previously described. RESULTS The authors' patients were age 4‐20 years. They presented with unilateral renal masses and metastatic disease in the lymph nodes (three patients), lungs (three patients), bone (two patients), liver (one patient), and bone marrow (one patient). Treatment included surgery, radiotherapy, and multiagent chemotherapy. Three of the patients died of progressive disease within 14 months of diagnosis. Features and outcomes were similar to those of the three previously reported cases. CONCLUSIONS PNET of the kidney appears to be a distinct entity. Although rare, it must be included in the differential diagnosis of renal tumors in children and young adults. Patients usually present with advanced disease and show poor response to combined‐modality therapy. Cancer 1997; 79:2243‐50. © 1997 American Cancer Society. This article presents four cases of primitive neuroectodermal tumor of the kidney and a review of the literature. The tumor appears to be a distinct entity with very aggressive behavior and poor response to therapy, and it must be differentiated from other childhood renal neoplasms.