A rare entity of Primary Ewing sarcoma in kidney
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CASE REPORT
A rare entity of Primary Ewing sarcoma in kidney Li Cheng1, Yujie Xu1, Hong Song2, Houbao Huang1 and Dong Zhuo1*
Abstract Background: Ewing sarcoma (ES) or primitive neuroectodermal tumors (PNET) represents a spectrum of poorly differentiated and aggressive malignancies. It rarely arises from the kidney and accounts for less than 1% of renal mass. Given the uncharacteristic clinical symptoms and imaging features, renal Ewing sarcoma (RES) is often diagnosed by postoperative pathology. Case presentation: Herein, we depicted a case of RES, which was administrated in our institution by chief complaints of intermittent left plank pain and palpable abdominal mass. We demonstrated the aggressive behavior of this renal malignancy and summarized its therapeutic modalities and outcomes. Conclusion: The diagnosis of RES relies on integrated analysis including histomorphology, immunohistochemical staining and confirmation of molecular-genetic testing. Despite the surgery and adjuvant therapy, optimized and potent therapeutic regimes are still urgently needed to improve the poor prognosis of RES. Keywords: Renal ewing sarcoma (RES), Primitive neuroectodermal tumor (PNET), Nephrectomy, Chemotherapy Background Ewing sarcoma (ES), also known as primitive neuroectodermal tumors (PNET), is a group of undifferentiated tumors that originates from neuroectoderm. It typically encountered in the bone and soft tissue of children and young adults [1]. The occurrence of ES/PNET in kidney is firstly depicted in 1975 [2]. Up to date, there emerges limited reported cases of ES/PNET in kidney by the rarity of this disease. Renal Ewing sarcoma (RES) presents highly malignant, grows rapidly, and metastases early to the lung, bone and lymph node [3, 4]. Thus, it is indispensable to distinguish RES from other renal entities. Herein, we present a case of an adult patient with RES who was diagnosed and administered in our hospital. We report this case in accordance with the CARE-Guideline [5].
*Correspondence: [email protected] 1 Department of Urology, Anhui Province, The First Affiliated Hospital of Wannan Medical College, Wuhu, People’s Republic of China Full list of author information is available at the end of the article
Case presentation A 31-year-old female patient was admitted with chief complaints of intermittent pain in the left plank and palpable abdominal mass. No urinary symptoms such as haematuria and dysuria were found. Her past medical history and family history of malignancy were unremarkable. Results of routine blood and urine examination as well as tumor markers including AFP, AFP-L3, CEA, CA199, CA125 and SCC were within the normal range. Enhanced computed tomography (CT) demonstrated a hypoechoic mass (18 cm × 14.5 cm × 14 cm) from the left renal with central necrosis, no invasion to renal veins or inferior vena cava (IVC) was reported (Fig. 1a, b). Further clinical investigations including chest HRCT showed no evidence of metastasis. 3-dimensional imaging of the neoplasm was conducted (Fig.
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