Abstract
We report a 21-year-old man with a primitive neuroectodermal tumor (PNET) of the kidney that was difficult to distinguish from other small round cell tumors, e.g., small cell carcinoma, malignant lymphoma, and Wilms’ tumor. Pathologically, a primitive rosette-like pattern was shown by H&E staining; expression of MIC-2 was indicated by an immunohistochemical test; rather primitive organelles were observed by an ultrastructural method; and translocation of chromosome 22 was confirmed by FISH. We therefore diagnosed the current case as PNET. The patient had undergone a right radical nephrectomy more than 1.5 years earlier. After neither metastases nor recurrences for 0.5 year, imaging examinations revealed masses in his liver. He received chemotherapy and underwent surgery again, but the masses were not composed of viable tumor cells. PNET of the kidney is extremely rare; fewer than 30 cases have been reported in the English literature, and there are few data on the expression of p53, ki67, and bcl2. We investigated the relationships between these markers in the current case using immunohistochemical tests and observed strong expression of p53, Ki-67, and bcl-2. Such results generally indicate poor prognosis, and the patient eventually had some masses in his liver, but no viable tumor cells were found. The prognostic significance of these various markers in PNET of the kidney still remains unclear, but p53, ki-67, and bcl-2 might not be so important as indicators of prognosis in the kidneys as they are in other organs. Further studies are needed to investigate this, and we hope that the patient recovers completely.
Similar content being viewed by others
References
Montogomery E (2006) Soft tissue tumors. In: Silverberg SG, DeLellis RA, Frable WJ, LiVolsi VA, Wick MR (eds) Silverberg’s principles and practice of surgical pathology and cytopathology, 4th edn. Churchill Livingstone/Elsevier, Philadelphia, pp 385–386
Ewing J (1921) Diffuse endothelioma of bone. Proc N Y Pathol Soc 21:17–24
Stout A (1918) A tumor of the ulnar nerve. Proc N Y Pathol Soc 18:2–22
Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH (1979) Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer (Phila) 43(6):2438–2451
Dogra PN, Goel A, Kumar R, Das PK, Gupta SD (2002) Extraosseous Ewing’s sarcoma of the kidney. Urol Int 69:150–152
McCluggage WG, Sumathi VP, Nucci MR, Hirsch M, Cin PD, Wells M, Flanagan AM, Fisher C (2007) Ewing family of tumours involving the vulva and vagina: report of a series of four cases. J Clin Pathol 60:674–680
Kang SH, Perie MA, Nonaka D, Zhu H, Chan W, Assay GCH (2007) Primitive Ewing sarcoma / PNET of the kidney: fine-needle aspiration, histology, and dual color break-apart FISH assay. Diagn Cytopathol 35(6):353–357
Rosai J (1996) Extraskeletal Ewing’s sarcoma/PNET. In: Rosai J (ed) Ackerman’s surgical pathology, 8th edn. Mosby, St. Louis, pp 2105–2106
Nielsen GP, Layfield LJ, Rosenberg AE (2006) Neoplastic and tumor-like lesions of bone. In: Silverberg SG, DeLellis RA, Frable WJ, LiVolsi VA, Wick MR (eds) Silverberg’s principles and practice of surgical pathology and cytopathology, 4th edn. Churchill Livingstone/Elsevier, Philadelphia, pp 746–750
Bridge RS, Rajaram V, Dehner LP, Pfeifer JD, Perry A (2006) Molecular diagnosis of Ewing sarcoma /primitive neuroectodermal tumor in routinely processed tissue: a comparison of two FISH strategies and RT-PCR in malignant round cell tumors. Mod Pathol 19:1–8
Seemayer TA, Thelom WL, Bolande RP, Wiglesworth FW (1975). Peripheral neuroectodermal tumors. Perspect Pediatr Pathol 2:151–172
Romero FR, Rais-Bahrami S, Muntener M, Permpongkosol S, Fine SW, Paidas CN, Jarrett TW (2007) Metastatic primitive neuroectodermal tumor to the kidney. Urol Int 78:286–288
Kuroda N, Takahashi T, Moriki T, Okanoue Y, Mizobuchi H, Miyazaki E, Hayashi Y, Lee G-H (2006) Askin tumor with metastasis to the scalp: a histochemical, immunohistochemical and ultrastructural study. Med Mol Morphol 39:221–225
Kato S, Takeuchi T, Asano T, Ban B, Yamada T, Hasegawa T, Yamamoto N (2007) Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. Scand J Urol Nephrol 41:75–76
Rodriguez-Galindo C, Marina NM, Fletcher BD, Parham DM, Bodner SM, Meyer WH (1997) Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer (Phila) 79(11): 2243–2250
Angervall L, Enzinger FM (1975) Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer (Phila) 36:240–251
Kushner BH, Hajdu SI, Gulati SC, Erlandson RA, Exelby PR, Lieberman PH (1991) Extracranial primitive neuroectodermal tumors: the memorial Sloan-Kettering cancer center experience. Cancer (Phila) 67:1825–1829
Ishii H, Joshita T, Matsuyama N, Uchida T, Ishikawa A, Ebihara Y (2006) Immunohistochemical and ultrastructural investigation on cutaneous neuroendocrine carcinoma: report of a case and review of the literature. Med Mol Morphol 39: 164–168
Burns A, Jaros E, Cole M, Perry R, Pearson AJ, Lunec J (2002) The molecular pathology of p53 in primitive neuroectodermal tumours of the central nervous system. Br J Cancer 86:1117–1123
Abudu A, Mangham DC, Reynoids GM, Pynsent PB, Tillman RM, Carter SR, Grimer RJ (1999) Overexpression of p53 protein in primary Ewing’s sarcoma of bone: relationship to tumour stage, response and prognosis. Cancer (Phila) 79(7/8):1185–1189
Alava ED, Antonescu CR, Panizo A, Leung D, Meyers PA, Houvos AG, Pardo-Mindán FJ, Healey JH, Ladanyi M (2000) Prognostic impact of p53 status in Ewing sarcoma. Cancer (Phila) 89(4):783–792
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ishii, H., Ogaki, K. Primitive neuroectodermal tumor of the kidney. Med Mol Morphol 42, 175–179 (2009). https://doi.org/10.1007/s00795-009-0453-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00795-009-0453-z