Testicular desmoplastic small round cell tumor: a case report and review of literature

World Journal of Surgical Oncology, Jul 2014

Background Desmoplastic small round cell tumor (DSRCT) is an uncommon and highly aggressive malignancy with undetermined histogenesis and poor prognosis. To date, no case of testicular DSRCT has been reported in the literature. Case A 42-year-old Chinese man presented with painless swelling of his left testis and a painless palpable nodule in his left inguinal region. Computed tomography showed a solid mass in the left testis and multiple metastases in the body. Laboratory tests gave no abnormal results. Left radical orchiectomy was performed, and histopathological and molecular pathological examination showed typical features of DSRCT. Six cycles of chemotherapy were administrated after the operation, leading to partial remission. Postoperative 9-month follow-up indicated no progression.

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Testicular desmoplastic small round cell tumor: a case report and review of literature

World Journal of Surgical Oncology Testicular desmoplastic small round cell tumor: a case report and review of literature Gui-Ming Zhang 0 2 Yao Zhu 0 Hua-Lei Gan 1 Ding-Wei Ye 0 0 Department of Urology, Fudan University Shanghai Cancer Center , No.270, Dongan Rd, Shanghai 200032 , China 1 Department of Pathology, Fudan University Shanghai Cancer Center , Shanghai , China 2 Department of Oncology, Shanghai Medical College, Fudan University , Shanghai , China Background: Desmoplastic small round cell tumor (DSRCT) is an uncommon and highly aggressive malignancy with undetermined histogenesis and poor prognosis. To date, no case of testicular DSRCT has been reported in the literature. Case: A 42-year-old Chinese man presented with painless swelling of his left testis and a painless palpable nodule in his left inguinal region. Computed tomography showed a solid mass in the left testis and multiple metastases in the body. Laboratory tests gave no abnormal results. Left radical orchiectomy was performed, and histopathological and molecular pathological examination showed typical features of DSRCT. Six cycles of chemotherapy were administrated after the operation, leading to partial remission. Postoperative 9-month follow-up indicated no progression. Desmoplastic small round cell tumor; Testis; Immunohistochemistry; Chemotherapy - Background Desmoplastic small round cell tumor (DSRCT) is an extremely rare malignancy with undetermined histogenic origin. To date, about 300 cases have been reported in the literature, since it was first described as a mesenchymal entity with distinct clinicopathological features by Gerald and Rosai in 1989 [1,2]. DSRCT is prone to develop in adolescents and young adults, and the incidence in males is more than three times that in females [3]. The majority of DSRCTs originate from the abdominal cavity and pelvis, thus the retroperitoneum, omentum, and mesentery are often involved, in addition to multiple peritoneal tumors [4]. Although rarer, invasion of extraperitoneal sites, such as the central nervous system, nasal sinus, lung, bone and soft tissues, kidney, ovary and paratesticular region, has been documented [5-11]. However, no DSRCT derived from testis has been reported to date. Herein, we report a case of solid neoplasm in testis that was confirmed pathologically to be testicular DSRCT. Case presentation A 42-year-old Chinese man was admitted to the Department of Urology at our hospital, with an 8-month history of painless swelling of his left testis and a 6-month history of a painless palpable nodule in his left inguinal region. He had no relevant personal or family history of malignancy. On physical examination, an egg-size solid lump was found in the left testis, and a palpable, slightly mobile, solid nodule, 2 3 cm in size, in the left inguinal region. Laboratory tests, including -fetoprotein, -human chorionic gonadotropin, and lactate dehydrogenase, gave no abnormal results. Computed tomography (CT) showed a confined solid mass, 2 3 cm in size with homogenous attenuation in the left testis, and multiple lesions in the right diaphragmatic crus, retroperitoneal region, and peritoneal and pelvic cavity, in the left inguinal region, and in the near-bilateral iliac vessels, suggestive of multiple metastases (Figure 1). In addition, the patients left renal pelvis and ureter were dilated. The preoperative diagnosis was left testicular tumor with multiple metastases. Left radical orchiectomy was carried out, and the cut surface of the tumor showed fish-like changes. Histopathological examination of the resected specimen revealed a malignant tumor composed of well-defined nests of small round blue cells, which were separated by abundant desmoplastic stroma (Figure 2). The spermatic cord and epididymis were also involved. Immunohistochemical evaluation exhibited the following: Cytokeratin Pan (+), Epithelial Membrane Antigen (+), Cytokeratin 7(), vimentin (+), desmin (+) (Figure 3), Inhibin (/+), Chromogranin A (weakly +), synaptophysin Figure 1 Computed tomography showed multiple lesions in left inguinal region, and near-bilateral iliac vessels, suggestive of multiple metastases. (+/), CD56 (), Placental ALK. Phosphatase (/+),Wilms Tumor-1 (WT-1) protein (), Myogenin (/+), MyoD1 () and Neuron-specific enolase (+). Molecular evidence of t(11;22) (p13;q12) was detected by fluorescent in situ hybridization (FISH). Based on the abovementioned evidence, a diagnosis of testicular DSRCT was confirmed (pT3 N2 M1 S0). The patient was discharged on the fifth postoperative day in a good condition. In the third postoperative week, he was readmitted to the Department of Oncology at our hospital and received the VAC multi-agent systemic chemotherapy regimen consisting of vincristine (2 mg, day 1), adriamycin (75 mg/m2, day 1) and cyclophosphamide (1.2 g/m2, day 1). After six cycles of chemotherapy, the patient was appraised as being in partial remission. Postoperative 9-month follow-u (...truncated)


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Gui-Ming Zhang, Yao Zhu, Hua-Lei Gan, Ding-Wei Ye. Testicular desmoplastic small round cell tumor: a case report and review of literature, World Journal of Surgical Oncology, 2014, pp. 227, 12, DOI: 10.1186/1477-7819-12-227