Case Report
Urologia
Received: February 27, 2006 Accepted after revision: August 21, 2006
Urol Int 2008;80:212–216 DOI: 10.1159/000112616
Internationalis
Peripheral Primitive Neuroectodermal Tumor Arising from the Seminal Vesicle Nathan Lawrentschuk a Sree Appu a Ian Chao a Yee Chan a John Rogerson a Ian D. Davis b a Department of Surgery and b Departments of Medicine and Oncology, University of Melbourne, Austin Hospital, Heidelberg, Vic., Australia
Key Words Seminal vesicles Neuroectodermal tumors, primitive Ewing’s sarcoma Magnetic resonance imaging Positron emission tomography
Abstract In the genitourinary tract, masses in the seminal vesicles, particularly primary tumors, are an unusual event. One must consider all diagnoses including Ewing’s sarcoma or its variant, peripheral primitive neuroectodermal tumor (PPNET). In this case report we describe a case of PPNET arising from the seminal vesicle. The prognosis of such tumors is generally poor and requires aggressive multimodal management if it is to be successful. It is essential that unusual tumors are considered when confronted by a seminal vesicle mass and that appropriate investigation and management are initiCopyright © 2008 S. Karger AG, Basel ated.
Introduction
The seminal vesicles are an unusual site of malignancy in the genitourinary tract. Masses in the seminal vesicle raise the possibility of unusual diagnoses, including Ew© 2008 S. Karger AG, Basel 0042–1138/08/0802–0212$24.50/0 Fax +41 61 306 12 34 E-Mail
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ing’s sarcoma or its variant, peripheral primitive neuroectodermal tumor (PPNET). We describe the first case of PPNET arising from the seminal vesicle and outline the appropriate investigation and management.
Case Report A previously well 26-year-old male presented with lower urinary tract symptoms (irritative) but urine culture yielded no pyuria or growth. His examination was unremarkable. Transabdominal ultrasound demonstrated a mass adjacent to the prostate which was confirmed on computed tomography (fig. 1). To further delineate the mass, an MRI scan was performed (fig. 2). At cystoscopy, the bladder mucosa was normal with an external mass protruding into the bladder lumen. Transrectal ultrasound and biopsy then demonstrated a PPNET on histology (fig. 3). Aggressive combined chemotherapy for six cycles was commenced (ifosfamide/etoposide/cyclophosphamide/adriamycin/vincristine). An initial positron emission tomography study displayed high uptake in the tumor but radioisotope activity was severely reduced after chemotherapy (fig. 4) so salvage excision of the tumor was undertaken. A lower midline abdominal incision was used as the surgical approach which remained extraperitoneal throughout. A standard retropubic radical prostatectomy was undertaken after careful lymph node sampling bilaterally up to the bifurcation of the common iliac vessels extending from and including the obturator fossa. Nerve sparing was undertaken as part of the radi-
Nathan Lawrentschuk, MB, BS Department of Surgery, University of Melbourne, Austin Hospital, Heidelberg, Vic. 10 Mary Street Beaumaris 3193 (Australia) Tel. +61 3 9415 8219, Fax +61 3 9496 3765, E-Mail
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Fig. 1. Initial ultrasound (a) demonstrating a mass (marked by cross) adjacent to the right side of the prostate that was also seen on computed tomography scanning (b).
Fig. 2. Magnetic resonance images of the tumor in coronal (a) and sagittal (b) sections. The tumor is up to 10.7 cm in diameter and is clearly compressing the prostate and protruding into but not infiltrating the bladder.
cal prostatectomy only on the right and care was taken to avoid rectal injury by developing the correct plane from the apex of the prostate and moving proximally (the bowel was prepared preoperatively in the event of bowel resection, which was not required). This was difficult as the normal separation of Denonvilliers’ fascia, when removing the prostate and seminal vesicles away from the rectum, was inhibited by inflammation and extension of the large tumor mass arising from the left seminal vesicle. However, rectal preservation was possible as the seminal vesicles, particularly the left with its mass, were carefully dissected away from the rectum using a sharp technique and no
diathermy. The remainder of the operation involved the reconstruction and anastomosis of the bladder to the urethra as per a standard retropubic radical prostatectomy. His postoperative recovery was uneventful. On histology minor areas of viable tumor remained and the tumor was clearly arising from the left seminal vesicle (fig. 5) but with clear surgical margins and lymph nodes free of disease. He has remained well following surgery and has not required adjuvant radiotherapy. His overall survival has now been almost 24 months since diagnosis with survival after surgery at 12 months.
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Fig. 3. a Hematoxylin and eosin staining at high power demonstrating collagenous fibrous tissue containing islands and nests of cellular tumor with cells being small and having small amounts of amphophilic cytoplasm but a high nuclear:cytoplasmic ratio. Nuclei are rounded, generally with finely dispersed nucleoplasm. !400. b Immunohistochemical stain for CD99 demonstrates focal, sharp membranous staining of tumor cells with the antibody to CD99 in keeping with the diagnosis of peripheral primitive neuroectodermal tumor. !400.
Discussion
PPNETs are highly cellular round-cell neoplasms that occur extracranially in soft tissue and bone, derived from embryonal neural crest cells and are in the same spectrum as Ewing’s sarcoma [1]. Sharing light microscopic features, cytogenetic anomalies and affecting young people, distinguishing the two is important as Ewing’s sarcoma has a better prognosis [1, 2]. Immunohistochemistry with CD99 (a monoclonal antibody recognizing a characteristic glycoprotein) is an essential feature to diagnose PPNET or Ewing’s sarcoma [3]. To distinguish the two conditions histologically, PPNET has well-defined rosettes, immunohistochemical reactivity with neural markers or evidence of neural processes and dense granules (fig. 2) compared to Ewing’s sarcoma [2]. Seminal vesicle malignancies are rare [4, 5]. Urinary tract PPNET is exceptionally rare with only occasional reports of Ewing’s sarcomas in the kidneys and genitalia [6, 7]. In the pelvis, the primary imaging modality should be ultrasound but MRI is also essential to delineate the accurate anatomy of masses adjacent to or involving the seminal vesicles [8]. This is extremely relevant when planning surgery. PET is a useful guide when monitoring the response to treatment of many malignancies and was helpful in this case in deciding to proceed to salvage surgery. 214
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Fig. 4. PET studies before (a) and after (b) chemotherapy with 18
F-FDG uptake diminishing within the tumor. A small amount of excreted 18F-FDG is visible in the right superior portion of the bladder lumen in both studies with the amount increasing after the chemotherapy study (b) because the protruding tumor had shrunk.
With Ewing’s sarcoma and PPNET, aggressive combination chemotherapy is essential [9]. Encouraging results have also been found with localized disease when combining surgery with radiotherapy [10]. Despite combined modalities, most patients are dead within 2 years of diagnosis [9]. However, each case needs an individual management plan. The prognosis of PPNET is somewhat better than Ewing’s and pathologists need to be aware of the Lawrentschuk /Appu /Chao /Chan / Rogerson /Davis
Fig. 5. a Hematoxylin and eosin staining at high power demonstrating viable PPNT despite chemotherapy. !400. b The tumor arose from the left seminal vesicle which is apparent in the lowermost part of the figure with the tumor extending above. !100.
differences. In the case reported, chemotherapy followed by surgical resection has thus far extended survival, which will hopefully be sustained in the future. Finally, urologists need to consider rare tumors of the genitourinary tract, particularly in young men, and obtain appropriate imaging and biopsies if necessary.
Acknowledgements We would like to thank Dr. R. Kalnins and Dr. R. Cameron of the Austin Hospital Pathology Department for providing the histopathological slides.
References 1 Ahmad R, Mayol BR, Davis M, Rougraff BT: Extraskeletal Ewing’s sarcoma. Cancer 1999; 85:725–731. 2 Devoe K, Weidner N: Immunohistochemistry of small round-cell tumors. Semin Diagn Pathol 2000;17:216–224. 3 Lee KM, Wah HK: Primary Ewing’s sarcoma family of tumors arising from the broad ligament. Int J Gynecol Pathol 2005; 24: 377– 381. 4 Agrawal V, Kumar S, Sharma D, Singh UR, Gupta A: Primary leiomyosarcoma of the seminal vesicle. Int J Urol 2004;11:253–255.
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5 Thiel R, Effert P: Primary adenocarcinoma of the seminal vesicles. J Urol 2002;168:1891– 1896. 6 Balakrishnan R, Khairullah QT, Giraldo A, Provenzano R: Extraskeletal Ewing’s sarcoma in a kidney transplant patient. Am J Kidney Dis 1999;33:1164–1167. 7 Jimenez-Verdejo J, Fernandez PL, Haddad A, Garcia-Carriazo A, Zuluaga A, Nogales F: Extraskeletal Ewing’s sarcoma metastatic to penis. Br J Urol 1992;70:206–207.
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8 Murphy JO, Power RE, Akhtar M, Torreggiani WC, McDermott TE, Thornhill JA: Magnetic resonance imaging in the diagnosis of seminal vesicle cysts and associated anomalies. J Urol 2003;170:2386. 9 Hoppin EC, Rives RK: Small-cell carcinoma of the seminal vesicle responding to chemotherapy. Ann Oncol 1997;8:503. 10 Krasin MJ, Rodriguez-Galindo C, Davidoff AM, Billups CA, Fuller CE, Neel MD, et al: Efficacy of combined surgery and irradiation for localized Ewings sarcoma family of tumors. Pediatr Blood Cancer 2004;43:229–236.
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Clinical Comment M. Froehner Universitätsklinikum ‘Carl Gustav Carus’ der Technischen Universität Dresden, Dresden, Germany
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It is tantalizing to attribute rare soft tissue malignancies to a certain organ of origin to raise interest in an apparently unique case [1, 2]. With the images presented in this case [3], seminal vesicle origin is not obvious. The tumor did not arise within a seminal vesicle and was certainly too large to assign it to any single pelvic organ. Therefore, the term ‘pelvic peripheral primitive neuroectodermal tumor with seminal vesicle involvement’ should be preferred. Nevertheless, this case report delivers some useful clinical information on the management of such rare tumors, although a longer follow-up is needed to the definite success of the treatment.
References 1 2 3
Sanghvi DA, Purandare NC, Jambhekar NA, Thakur MH, Joshi MS: Primary rhabdomyosarcoma of the seminal vesicle. Br J Radiol 2004;77:159–160. Ahmadzadeh M, Bosse A: Leiomyoma of the seminal vesicle. Urol Int 1996;57:252–254. Lawrentschuk N, Appu S, Chao I, Chan Y, Rogerson J, Davis ID: Peripheral primitive neuroectodermal tumor arising from the seminal vesicle. Urol Int 2008;80:212–216.
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