Pelvic Schwannoma in the Right Parametrium: The First Case (Congress code 121108) N. Machairiotis1,*, E. Karatrasoglou2, G. Sotiropoulou2, A. Floreskou2, E. Chatzi2, P. Zarogoulidis3, A. Stylianaki1, A. Karamani2, N. Katsikogiannis1, N. Courcoutsakis4, C.Machairiotis 2 1 Surgery Department (NHS), University General Hospital of Alexandroupolis, Greece 2 Obstetric Gynecology Department, ``Thriasio`` General Hospital, Athens, Greece 3 Pulmonary Department, ``G. Papanikolaou`` General Hospital, Aristotle University of Thessaloniki, Greece 4 Radiology Department, University General Hospital of Alexandroupolis, Democritus University of Thrace, Greece *Corresponding author E-mail address: nikolaosmachairiotis@yahoo.com SUMMARY We report the case of a 58- year- old female with pelvic schwannoma, measuring 6.5 x 5.5 cm in size, in the right parametrium. Based on the rarity of this tumor, we performed laparotomy with total abdominal hysterectomy and en-block tumor excision. Frozen section was taken during the surgery before complete resection of the mass, which was ambiguous. Histological examination showed a benign neoplasm, originating from the cells of peripheral nerve sheaths, with a diagnosis of a schwannoma. Pelvic schwannomas are very rare neoplasms that can undergo the diagnosis or they can be misdiagnosed. Laparoscopy is a safe and efficient option for approaching benign pelvic tumors and might offer the advantage of better visualization of structures. Keywords: INTRODUCTION Neurillemomas are benign usually encapsulated nerve sheath tumors deriving from the Schwann cells. They constitute one of the most common types of benign peripheral nerve sheath tumors. These tumors may be very common in cranial and peripheral nerves, but they are rarely located in the pelvis [1, 2]. Schwannomas can occur sporadically or as manifestations of genetic conditions such as neurofibromatosis 1 and 2. Pelvic schwannomas have no specific radiologic features and are often considered to be urologic diseases or gynecologic masses [3]. MATERIAL AND METHODS A 58-year-old Caucasian woman was admitted to gynecology out-patient department complaining for increasing abdominal distension and sustained pain in right bottom abdominal quadrant, independent of activities without any signs of neurovascular deficit, the last three months. The last 48 hours the pain was located in the right iliac fossa. Her medical and family history was unremarkable, including an appendectomy as a child and five natural childbirths. Bimanual pelvic exam revealed an agile cervix and a large, solid, ovoid, palpable, particularly sensitive in touch mass in the right parametrium. Computer tomography showed a mass located in the right ovary, corresponding to ovarian schwannoma. (Figure 1) Due to the preoperative findings, we had to deal with a retroperitoneal tumor of unknown pathology, in a 58year-old menopausal woman. In order to ensure the optimum treatment and survival for our patient we performed laparotomy. During the surgery frozen section was taken which was ambiguous. Thereafter, taking under consideration the possibility of malignancy, we performed total abdominal hysterectomy and en-block tumor excision , with pelvic blunt dissection. A huge retroperitoneal mass was noted in the right parametrium, measuring 6, 5 x 5.5 cm in size and showed a close proximity to the internal iliac vein, which was ligated.(Figure 2) RESULTS Histological examination showed a benign neoplasm, originating from the cells of peripheral nerve sheath, with a diagnosis of schwannoma. Immunohistochemical studies were performed using a panel of antibodies including S-100, SMA, and CD68. The cells were strongly positive for S100 protein and were negative for smooth muscle actin (SMA). Immunostaining for CD68 showed numerous histiocytes in the intermediary layer. Postoperatively, the patient recovered well and she was discharged home on postoperative day six (6). CONCLUSIONS Neurillemomas are non aggressive, slowly growing solitary neoplasms with extremely low possibility of malignant transformation or recurrence after excision [4]. Immunohistochemistry is positive for S-100, vimentin, and neuron-specific enolase, but negative for smooth muscle actin and CD117 [4]. As a result of their slow rate of growing and their anatomic location, pelvic schwannomas remain asymptomatic and either they are incidentally discovered during a medical investigation for unrelated symptoms or they are discovered as soon as they obtain large dimensions and cause a mass effect [4,5]. More specifically, the mass effect can lead to pain in pelvic area and lower back as well as a sense of heaviness accompanied with urinary and digestive symptoms caused by bladder and bowel compression [5]. Their therapy is considered to be the complete excision of the tumor either laparoscopically or with an open abdomen surgery [6]. Taking under consideration that the vast majority of schwannomas are benign tumors simple tumor enucleation could also be effective [4]. Laparoscopy might greatly facilitate dissection due to magnification of the anatomic elements in the narrow pelvis [6]. It is also very interesting to refer that there are many case reports in which the pelvic schwannomas were misdiagnosed and were discovered during an operation that was considered to be the optimal therapy for the initial diagnosis. Laparoscopy is a safe and efficient option for approaching benign pelvic tumors and might offer the advantage of better visualization of structures due to the magnification of laparoscopic view, especially in narrow anatomic spaces [6]. ACKNOWLEDGMENTS (if there are) REFERENCES 1. Rao W, Wang G, Xiu D. Laparoscopic resection of a retroperitoneal schwannoma adherent to vital vessels. Surg Laparosc Endosc Percutan Tech. Feb 2009;19(1):e21-23. 2. Funamizu N, Sasaki A, Matsumoto T, Inomata M, Shiraishi N, Kitano S. Laparoscopic resection of a retroperitoneal schwannoma behind the lesser omental sac. Surg Laparosc Endosc Percutan Tech. Jun 2004;14(3):175-177. 3. Daneshmand S, Youssefzadeh D, Chamie K, et al. Benign retroperitoneal schwannoma: a case series and review of the literature. Urology. Dec 2003;62(6):993-997. 4. Strauss DC, Qureshi YA, Hayes AJ, Thomas JM. Management of benign retroperitoneal schwannomas: a single-center experience. Am J Surg. Aug 2011;202(2):194-198. 5. Sinha R, Sundaram M, Hegde A, Mahajan C. Pelvic schwannoma masquerading as broad ligament myoma. J Minim Invasive Gynecol. Mar-Apr 2008;15(2):217-219. 6. Konstantinidis K, Theodoropoulos GE, Sambalis G, et al. Laparoscopic resection of presacral schwannomas. Surg Laparosc Endosc Percutan Tech. Sep 2005;15(5):302-304. Legend to the figures FIGURE 1. Axial, enhanced CT image of the pelvis demonstrates a mass in the anatomic region of the right ovary, corresponding to ovary’s schwannoma. The enlarged ovary is well delineated, with low density internal, enhancing peripherally (uterus [UT], urinary bladder [BL:]). FIGURE 2. Surgically resected lesion. Work published in Proceedings 17th World Congress on Controversies in Obstetrics, Gynecology & Infertility (COGI) –November 8-11, 2012, Lisbon, Portugal, edited by Z. BenRafael, Monduzzi Editoriale, Milano, Italy, 2012, pp. 177-180. PLEASE SEND TABLES AND FIGURES AS SEPARATE FILES