International Journal of Biological & Medical Research

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1 Int J Biol Med Res. 2012; 3(1): Int J Biol Med Res Volume 2, Issue 4, Jan BioMedSiDiret Publiations Contents lists available at BioMedSiDiret Publiations International Journal of Biologial & Medial Researh Journal homepage: International Journal of BIOLOGICAL AND MEDICAL RESEARCH Case report Giant presaral-pelvi ysti shwannoma presenting as a right ilia fossa mass - megaolon in a poliomyelitis patient : a rare ase report & review of literature a b d e Ambikavathy.M *, Mohan Kumar. K, Kumar.S, Uday Kumar, Iram Shaikh a *Asst Professor, Dept of General Surgery,Sri Devaraj Urs Medial College& Researh Centre Kolar. b Professor, Dept of General Surgery,Sri Devaraj Urs Medial College & Researh Centre Kolar. Asst Professor,Dept of Mediine,Sri Devaraj Urs Medial College & Researh Centre Kolar. d Professor,Dept of Pathology,Sri Devaraj Urs Medial College & Researh Centre Kolar. e Junior Resident,Dept of General Surgery,Sri Devaraj Urs Medial College & Researh Centre Kolar. A R T I C L E I N F O A B S T R A C T Keywords: Giant ysti shwannoma Megaolon Poliomyelitis Reurrene Saral & presaral tumours are unommon & our in approximately 1 in 40,000 hospital admissions.shwannoma is one of the tumours that our in these areas and oasionally presents with enormous dimensions known as Giant shwannoma.it usually grows slowly and is often found inidentally beause it presents with vague and non-speifi symptoms. Tumour removal is a surgial hallenge due to the diffiult approah and vasularity.we report a rare ase of Giant presaral shwannoma presenting as a right ilia fossa mass with subaute intestinal obstrution- megaolon in a hildhood poliomyelitis patient,who underwent total exision of the tumour and HPE report was benign shwannoma with ysti degeneration and onfirmed by Immunohistohemistry. The patient was relieved of his bowel symptoms (obstrution). Reurrene was not noted even after 2 yrs of follow up. Copyright 2010 BioMedSiDiret Publiations IJBMR - ISSN: 0976:6685. All rights reserved. 1. Introdution Giant presaral shwannomas are unommon and our 1 in hospital admissions [1,2]. Benign shwannomas are generally slow growing and painless tumours originating from Shwann ells of peripheral nerve sheath of the neural rest [1,2]. Symptoms our late unless they are large enough to produe pressure effet. These are slow growing and often found inidentally [3-5]. Due to its non speifi linial and imaging findings preoperative diagnosis is very diffiult [3]. FNAC is diagnosti in differentiating benign from malignant shwannoma [6-7]. We report a rare ase of giant presaral shwannoma in a patient with poliomyelitis who presented with subaute intestinal obstrution. 2.Case Illustration A 26 yrs old male patient with hildhood poliomyelitis {FIG-1} presented with low bak ahe sine 2 months, vomiting,distension of abdomen and onstipation from 2 weeks.on linial examination two firm masses were papable per abdomen,one in the right ilia region 10 x 7ms non tender,non mobile and another in the suprapubi region measuring 6 x 3ms The upper pole was reahable and lower pole was diffuse. Impaired note was heard over both the masses. On manupulation of the masses,the patient omplained of nausea and sensation to defeate. A linial diagnosis of lobulated retroperitoneal soft tissue tumour with subaute intestinal obstrution was made. FIG 1; Photograph of 26 year old male patient with poliomyelitis: with Giant presaral shwannoma with subaute intestinal obstrution. * Corresponding Author : Dr. Ambikavathy. M Asst Professor,Dept of General Surgery Sri Devaraj Urs Medial College&RC Kolar Karnataka, India. Ph: ambikaashri67@live.om Copyright 2010 BioMedSiDiret Publiations. All rights reserved.

2 1443 All linial and lab investigations were within normal limits. USG abdomen and pelvis revealed a ysti swelling in the pelvis[fig-2]. A large dilated fluid filled olon imaged in the right ilia region on supine abdomen x-ray. Origin of mass ould not be ommented. CT of the pelvis and MRI sans revealed, a large dilated air fluid filled sigmoid megaolon of 13 ms diameter [Fig-3,CT,MRI]. FIG-2;A supine abdomen x-ray shows soliosis to left side,saral bone destrution,b/l femoral bone deformity,dilated olon on right side. adherent to the anterior surfae of sarum. The ysti tumour was opened up and ontents apirated. The inner yst wall had multiple septations,alified, neroti and haemorrhagi areas. The ysti presaral tumour was totally exised and haemostasis was well maintained as it was very vasular and lose to saral veins. On histopathologial examination [HPR NO:B/1165/08 dated 89-08] multiple setions studied from the yst shows fibrohyalinised ollagenous tissue with overlying shwanian ells in hyper ellular areas. Areas of haemorrhage, hyalinization, alifiation and yst hange seen. The HPE report was suggestive of benign shwannoma with ysti degeneration.[hpe-figs]. Postoperative reovery was good. Patient was relieved of bowel obstrution ompletely and was disharged after a week. Patient is disease free and no reurrene or sensory neurologial defiits noted even after 2 yrs of follow up. FIG-A&B Coronal and Saggital images showing large mass in the pelvis with a hypointense peripheral pseudoapsule[arrow] and internal septations. A pelvi tumour in the presaral region measuring 13 x14 x14 ms,arising to the left of midline ysti, enapsulated mass with septations. The tumour was attahed to the anterior surfae of the sarum with pressure erosions. Tumour had fine peripheral alifiation and small foi of the same within the tumour. A diagnosis of benign retroperitoneal ysti tumour with erosion of saral ortex with sigmoid megaolon was made. The patient was taken up for surgery under general anaesthesia and by transabdominal/transperitoneal approah. The presaral ysti tumour was totally exised. Peroperative findings revealed hugely distended megaolon in the right lumbar and ilia region.[perop Fig].A large well enapsulated 15 x13 ms tumour was noted adherent to the anterior surfae of sarum ompressing over the sigmoid olon,whih was the ause for bowel obstrution [perop Fig]. Due to its large size to make spae for dissetion,the tumour was aspirated and the ontent was 600 ml of haemorragi fluid[perop-fig]. By blunt dissetion the ysti tumour was freed all around though the apsule was densely

3 1444 Axial CT setions of pelvis showing large mass with erosions of saral ortex on left side[arrow] and mass effet over sigmoid olon. Op Fig 2: Showing the presaral ysti tumour. Op Fig 3: Showing aspiration of haemorrhagi fluid from the presaral ysti tumour to deompress Op Fig 4: presaral ysti tumour being opened up and ontents aspirated. Op Fig 1: Showing hugely distended sigmoid olon ( mega olon).

4 1445 Op Fig 5 : Showing megaolon & the ut open presaral ysti tumour. Immuno histohemistry Mirophotograph (200X) showing S-100 immunoreativity in the tumour ells. Op Fig 6: Showing totally exised speimen of presaral ysti tumour. 3. Disussion Giant presaral Shwannomas are benign neoplasm arising from the myelinated nerve sheaths. Malignant shwannomas our denovo or from transformation of plexiform neurofibromatosis [7-12]. However our patient did not have the disease. HP Report 1: Mirophotograph (200X, H & E) showing palisading of the nulei with hyaline thikening of the blood vessel. Pelvi shwannoma is rare and aounts for 1% of all Benign shwannoma.11 As these are slow growing and painless tumours, symptoms our late,unless they are large enough for pressure effet.they are low bakahe,pain in the abdomen and pelvis, urinary and digestive symptoms from bladder and retal ompression.6 However linial manifestation of onstipation and intestinal obstrution with megaolon as noted in our ase is very rare. Giant presaral shwannomas arise from peripheral nerve sheaths within the sarum or adjaent to saral foramen and grow out side the bony frame work, to a large size. Erosion of the bone and ompressive symptoms our rather late.4,5,12 Anient variant of shwannoma is rare and two ases have been reported in literature so far. These undergo degenerative hanges suh as yst, haemorrhagi, alifiation, and fibrosis [11,13]. The mehanism of degeneration is attributed to vasular insuffiieny [4]. HP Report 2: Mirophotograph (400X, H & E) showing tumour omposed of fibrohyalinized ollagenous tissue with overlying Shwann ells in hyper ellular areas benign ysti shwannoma. Preoperative diagnosis of presaral shwannoma is diffiult. CT and MRI are widely used as imaging modalities in evaluation of retroperitoneal soft tissue tumours [4,13]. The MRI harateristis of presaral shwannoma are homogenous or heterogenous / ysti appearane with well defined margin and ISO/ISO intensity,iso/high intensity on T1/T2W1 [4]. CT an aurately detet bony destrution and saro ilia joint involvement4. Definitive diagnosis is based on histo pathology of the biopsied speimen, that an differentiate benign from malignant shwannomas. Immunohistohemistry is onfirmatory of benign shwannomas as they show diffuse immune reativity to S-100 protein [7,9].

5 1446 Total tumour exision is onsidered the treatment of hoie for presaral shwannomas [5]. Sine tumour reurrene and malignant transformation almost never ours in benign shwannomas loal tumour exision an be onsidered [5,9]. Utmost are is required in surgial removal of retroperitoneal and intrapelvi shwannomas [5]. Generally anterior transabdominal or retroperitoneal approah should be performed for presaral shwannomas to gain ontrol of vasular plexus and protetion of intrapelvi organs [14]. If the tumour or tumour apsule is densely adherent to the presaral venous plexus, bleeding an be hazardous and haemostasis an pose a problem. Hene pieemeal or subtotal exision of the tumour an be onsidered [5]. Though, the benign nature of the the tumour has to be onfirmed by histopathology. If the HPE onfirms malignany,loal reurrene after marginal exision has to be expeted in upto 72%, while wide marginal exision has been reported in 11.7% 9. In a ase report by Foote et al, the attempt to exise a large retroperitoneal shwannoma was abandoned beause of danger of unontrollable bleeding [5,8]. In our ase the tumour apsule was densely adherent to sarum and by blunt dissetion total exision of the ysti tumour was done. We had blood loss of 500ml. However there was no extra/intradural extention of the tumour noted. The nerve of origin ould not be made out due to the huge size of the tumour. [5] Theodosopoulus T, stafyla VK, Tsiantoula P, Ylallourou Marinis A, kondi_pafitis A, et al. Speial problems enountering Surgial management of large retro-peritoneal Shwannomas. World. J. Surg on. 2008; 6: 107. [6] Sero Andonian, pierre I, Karakiewiz, and Harry W. Her presaral ysti Shwannoma in man. Urology 2003, 62(3)555VII-55IX. [7] Corton, Kumar, Robbins. pathologi Basis Disease, 5th Edition, pg 1353,MPNST. [8] Foote MN, Luongo V, Marino ER. Benign giant retroperitoneal neurilemoma. Ann surg 1963; 157: [9] Oliver S Shindler and John H Dixon. Retroperitoneal giant Shwannomas. J ortho Surg 2002 ; 10(1): [10] Salvant JB, young HF. Gaint intrasaral Shwannoma : anunusual ause of lumbosaral radiulopathy. Surg Neurol ; 41 : [11] L.Harzallah, M.A Jellai, B. Sriha, T.Yaoubi, H. Amara, D. Baker. Anient pelvi retroperitoneal shwannoma mimiking an adnexal mass. Euro J of Radiology 2004:67-70 [12] Takeyamma M, Koshino T, Nakazawa A, Nihi H, Nakamura J, Sait T Giant intrasaral ellular shwannoma treated with saral amputation. Spine 26:E216-E219. [13] Krandorz M J, Murphy MD (1997) Neurogeni tumours 1 imaging of soft tissue tumours. Saunders,Philadelphia, PP [14] Abernathy CD, Onofrio SM,Sheithauer B, Pairo lero PC, Shievs TC. Surgial management of Giant Saral shwannomas. J Neurosurg. 1986; 65: Regular follow up of the patient for the last 2 years has shown marked improvement of bowel symptoms and he has no reurrene\ sensory neurologial defiits and the patient is disease free till date. 4. Conlusion The duration of symptoms in a patient with giant presaral/pelvi shwannoma is very long and patient presents late when pressure symptoms predominate. Pain abdomen, urinary symptoms, onstipation, may be the presenting features. CT /MRI is the preoperative imaging of hoie in demonstrating tissue heterogeneity and anatomi loation of tumour. Surgery is the treatment of hoie for patients with presaral/pelvi shwannomas as it is assoiated with funtional improvement. Total exision is the standard surgial modality. However pieemeal or subtotal exision an be onsidered in benign tumours. Regular follow up is mandatory. 5.Referenes: [1] Whit taker LD, Remberton JD. Tumor Ventral to the sarum Ann surg. 1938; 107 : [2] Klimo P, Rao G, Shmidt MH. Nerve Sheath tumors involving the sarum : Case Report and lassifiation Sheme. Neurosurg Fous ; 15 : E12 [3] Turner ML, Mulheen CB, Dalinka MK. Lesions of the Sarum. Differential diagnosis and radiolologial Evaluation JAMA,2008. [4] Hughes MJ, Thomas JM, Fisher C. MOSKOVIC EC. Imaging features of retroperitoneal and pelvi shwannomas. Clin Radiol ; 60 ; Copyright 2010 BioMedSiDiret Publiations IJBMR - ISSN: 0976:6685. All rights reserved.