Abstract
Peripheral primitive neuroectodermal tumor/Ewing’s sarcoma (pPNET/EWS) is an aggressive type of sarcoma that is rarely observed in the kidney. pPNET of the kidney principally occurs in young patients (<50 years old) and is very rare in older patients (≥50 years old). Additionally, only six cases of pPNET of the kidney have been reported in the literature in older patients (≥50 years old), and pPNET as a secondary primary tumor has rarely been reported. The current study presents a case of renal pPNET in a 51-year-old female who had been surgically treated for breast carcinoma and administered with adjuvant chemotherapy five years prior to hospitalization for pPNET. A computed tomography scan identified a tumor in the lower pole of the right kidney, which was treated by nephrectomy. Immunohistochemistry demonstrated diffuse, strong membranous positivity for cluster of differentiation (CD)99, positive nuclear staining for friend leukemia integration 1, and negative staining for Wilms’ tumor 1 and other markers. Fluorescence in situ hybridization (FISH) analysis of the EWS breakpoint region 1 (EWSR1) demonstrated the characteristic EWSR1 translocation. The patient declined chemotherapy or radiotherapy but accepted traditional Chinese medicine. No evidence of recurrence was observed eight months after diagnosis. Only two cases of renal pPNET with a history of an earlier or synchronous primary cancer were reported in the literature from the USA and Germany, respectively. To the best of our knowledge, the present case is the first FISH-confirmed renal pPNET in an older patient following breast adenocarcinoma.
Highlights
Peripheral primitive neuroectodermal tumor/Ewing's sarcoma is an aggressive type of sarcoma characterized by the t(11; 22)(q24;q12) translocation and overexpression of the cluster of differentiation (CD)99/MIC2 gene [1]. peripheral primitive neuroectodermal tumor (pPNET) is predominantly considered to be a malignant bone or soft tissue tumor of children and young adults that is uncommon in individuals aged >30 years [2]. pPNET of the kidney is very rare, and is even rarer in older patients or in patients who have received treatment for a different type of cancer
Discussion pPNET is a small round‐cell tumor that predominantly occurs in bone and soft tissue; pPNET is occasionally reported in visceral organs, such as urogenital, intra‐abdominal and intrathoracic organs. pPNET of the kidney is uncommon [3,4,5] and was first reported in 1975 [6]
Since the first report, ~150 cases have been published in the medical literature, with the largest series of 24 cases reported by Zöllner et al [7]. pPNET of the kidney is an aggressive disease with a high metastatic potential that predominantly occurs in children and young adults and exhibits a slight male predominance [8]. pPNET of the kidney following or synchronous with a different primary cancer is extremely rare, with only two reported cases [9,10]
Summary
Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (pPNET/EWS) is an aggressive type of sarcoma characterized by the t(11; 22)(q24;q12) translocation and overexpression of the cluster of differentiation (CD)99/MIC2 gene [1]. pPNET is predominantly considered to be a malignant bone or soft tissue tumor of children and young adults that is uncommon in individuals aged >30 years [2]. pPNET of the kidney is very rare, and is even rarer in older patients or in patients who have received treatment for a different type of cancer. The current study presents the first known case of renal pPNET following breast adenocarcinoma in a 51‐year‐old female. The patient had been treated by radical mastectomy and adjuvant chemotherapy for a left breast adenocarcinoma five years prior to the current hospitalization. No evidence of breast cancer recurrence was observed. The patient received radical nephrectomy with resection of tumor thrombi in the IVC and right renal vein. Vague rosette‐like patterns or perivascular concentrations of tumor cells were observed (Fig.). ZHONG et al: pPNET OF THE KIDNEY IN A 51‐YEAR‐OLD FEMALE FOLLOWING BREAST CANCER. No loss of integrase interactor‐1 (INI‐1) protein expression levels were observed, as determined by the nuclear positivity for INI‐1 (Fig. 3C), and the Ki‐67 nuclear labeling index was ~10%. Recurrence or metastasis taxol interferon and doxorubicin ifosfamide and Vincristine, mesna, Chemotherapeutic agents
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