Stesura Seveso 291Archivio Italiano di Urologia e Andrologia 2014; 86, 4 CASE REPORT Peripheral primitive neuroectodermal tumor of seminal vesicles: Is there a role for relatively aggressive treatment modalities? Alessandro Crestani 1, Andrea Guttilla 1, Mario Gardi 2, Marina Gardiman 3, Fabrizio Dal Moro 1, Claudio Valotto 1, Filiberto Zattoni 1 1 Department of Gastroenterological, Oncological and Surgical Sciences, Urology Clinic, University of Padua, Italy; 2 Department of Urology, S. Antonio Hospital, Padua, Italy; 3 Department of Oncological and Surgical Sciences, Division of Pathology, University Hospital of Padua, Italy. A 50 year old white man received an incidental ultrasound diagnosis of hypoechoic mass interesting the right seminal vesicle. A CT scan showed the presence of a 7.8 cm roundish cyst, originating from the right seminal vesicle. He had been followed by the removal of the right seminal vesicle and both the cystic lesion. The histological findings of the specimen documented the presence of small round cells compatible with Ewing’s sarcoma/PPNET. The patient received also adjuvant chemotherapy and radiation treat- ment. After 10 years, the follow-up is still negative. KEY WORDS: Seminal vesicles, Sarcoma, Peripheral primitive neuroectodermal tumor. Submitted 27 August 2013; Accepted 30 September 2014 Summary No conflict of interest declared. urine analysis were all normal. The general physical exam- ination did not reveal anything particular. At DRE, a pal- pable mass was recognized at the level of the right portion of the anterior rectal wall dislocating the prostate gland. The abdominal ultrasound examination of the pelvis showed a roundish cyst within the right seminal vesicle of 8 cm in the widest diameter, with septa and corpuscular material, inside (Figure 1). The further imaging diagnostic work up consisted in a pelvic computed tomography (CT) scan and intravenous pyelography (IVP). The CT scan con- firmed the presence of a 7.8 cm roundish cyst, originating from the right seminal vesicle; the urinary bladder result- ed compressed and anteriorly dislocated (Figure 2). No wall contrast enhancement was detected and a plane of dissection was appreciable from the bladder, rectum and the lateral pelvic wall (internal obturator muscle). CT scan revealed another oval-shaped cyst of 3 cm in the widest diameter originating from the right seminal vesicle with the same densitometric features. The IVP confirmed also that the right supero-external margin of the bladder wall was compressed (Figure 3). The patient underwent a TC guided fine needle agobiopsy (FNAB) of the larger lesion that gave no significant findings. Therefore the patient was surgically explored through a midline incision of the lower abdomen. A cystic lesion was found against the right sem- inal vesicle that raised-up the bladder. Frozen section biopsies of the cystic lesion documented fibrous tissue with chronic inflammation. The removal of the right sem- inal vesicle and both the cystic lesions was performed. The post operative period was uneventful and the patient was discharged in few days. The histological findings of the specimen documented the presence of small round cells compatible with Ewing’s sarcoma/PPNET (Figures 4-5). After surgery the patient received adjuvant chemotherapy with a total of 13 cycles (Adriamycin, Vincristine, Cyclophosphamide, Actinomycin D, Ifosfamide, Etopo - side), as well as radiation treatment (48 Gy) on the pelvis. After 10 years, the follow-up is still negative: PET total body was always negative and the patient is in a good gen- eral condition with normal urinary continence and good erectile function. DOI: 10.4081/aiua.2014.4.291 INTRODUCTION The seminal vesicles are an unusual site of primary malig- nancy in the urinary tract and primary malignant Ewing’s sarcoma or its variant peripheral primitive neuroectodermal tumor (PPNET) are even more rare (1-2). The prognosis of extraskeletal PPNET is generally poor and an aggressive, multimodal approach is usually required (3). At our knowl- edge only one case of PPNET involving the seminal vesicles has been reported, with scarce information about the fol- low-up. Herein is described a case of PPNET arising from the right seminal vesicle with a ten year follow-up. CASE REPORT A 50 year old white man received an incidental ultra- sound diagnosis of hypoechoic mass interesting the right seminal vesicle. At the time, he was 174 cm in height, 82 kg in weight and the body surface area was 1,97 m2; the performance status was grade 0. The family and his own personal medical history was not significant. He did not refer any professional exposure to carcinogens and used to smoke 7-8 cigarettes per day. Complete blood cell count, routine chemistry profile and Crestani_Stesura Seveso 14/01/15 13:00 Pagina 291 Archivio Italiano di Urologia e Andrologia 2014; 86, 4 A. Crestani, A. Guttilla, M. Gardi, M. Gardiman, F. Dal Moro, C. Valotto, F. Zattoni 292 way was related to CT scan images that showed only roundish cystic lesions without any contrast enhance- ment in the cystic wall and the presence of a well defined plane of dissection from the bladder, rectum and lateral pelvic wall. Usually in case of sarcoma of the seminal vesicles, they are treated by means of an aggressive surgical approach as radical prostatectomy or radical cystectomy with an inevitably negative impact on the quality of life of the patients. In the case here described, the very conserva- tive surgical approach was followed by an adjuvant ther- apy: such a treatment provided good clinical results, last- ed in the long term period. Figures are posted in Supplementary Materials” on www.aiua.it REFERENCES 1. Agrawal V, Kumar S, Sharma D, et al. Primary leiomyosarcoma of the seminal vesicle. Int J Urol. 2004; 11:253-5. 2. Baschinsky DY, Niemann TH, Maximo CB, Bahnson RR. Seminal vesicle cystadenoma: a case report and literature review. Urology. 1998; 51:840-5. 3. Lawrentschuk N, Appu S, Chao I, et al. Peripheral primitive neu- roectodermal tumor arising from the seminal vesicle. Urol Int. 2008; 80:212-5; discussion 216. 4. Llombart-Bosch A, Machado I, Navarro S, et al. Histological het- erogeneity of Ewing's sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support. Virchows Arch. 2009; 455:397-411. 5. Berg T, Kalsaas AH, Buechner J, Busund LT. Ewing sarcoma- peripheral neuroectodermal tumor of the kidney with a FUS-ERG fusion transcript. Cancer Genet Cytogenet. 2009; 194:53-7. 6. Zanetti G, Gazzano G, Trinchieri A, et al. A rare case of benign fibroepithelial tumor of the seminal vesicle. Arch Ital Urol Androl. 2003; 75:164-5. DISCUSSION PPNET, histologically characterized by the presence of small round cells extracranially in soft tissues and bones. Ewing’s sarcoma and primitive peripheral neuroectodermal tumor (PPNET) have been originally described as two distinct pathologic entities (4). Because of their similar histologic and cytogenetic charac- teristics, these tumors are now considered to derive from a common origin cell and to be a part of a spectrum of neo- plastic diseases known as the Ewing sarcoma family of tumors (ESFT), which also includes extraosseous Ewing sar- coma (EES), adult neuroblastoma, malignant small-cell tumor of the thoracopulmonary region (Askin tumor), par- avertebral small-cell tumor, and atypical Ewing’s sarcoma. They all derive from embryonal neural crest cells. The essential feature to diagnose PPNET or Ewing’s sarcoma is the histoimmunochemistry with CD99. Ewing family of tumors (EFTs) represents a neoplastic entity character- ized by specific chromosomal rearrangements. The most commonly detected translocation involves the fusion of EWSR1 to one of the genes encoding ETS fam- ily of transcription factors, usually FLI1 or ERG. The detection of specific translocations by fluorescence in situ hybridization (FISH), reverse transcription-polymerase chain reaction (RT-PCR), or both has become the diag- nostic hallmark for the EFTs (5). PPNETs are uncommon and the primary PPNET of the seminal vesicles is quite exceptional. Differential diagnosis must include rhab- domyosarcomas, leyomiosarcomas, phylloides tumors, malignant fibrous histiocytomas, chondrosarcomas. Differential diagnosis must also include benign condi- tions like benign fibroepithelial tumor (6), prostatic utri- cle cyst, the prostatic abscess, the hydrops, the cyst and the empyema of seminal vesicles or the ectopic uretero- cele. Usually surgical treatment of sarcomas of the pelvis is extremely aggressive. The choice to treat the present patient in a conservative Correspondence Alessandro Crestani, MD (Corresponding Author) alessandro.crest@gmail.com Andrea Guttilla, MD Fabrizio Dal Moro, MD Claudio Valotto, MD Filiberto Zattoni, MD Department of Gastroenterological, Oncological and Surgical Sciences Urology Clinic, University of Padua Via Giustiniani, 2 - Padova, Italy Mario Gardi, MD Department of Urology, S. Antonio Hospital, Padova, Italy Marina Gardiman, MD Department of Oncological and Surgical Sciences, Division of Pathology, University Hospital of Padua Via Giustiniani, 2 - Padova, Italy Crestani_Stesura Seveso 14/01/15 13:00 Pagina 292