OGH Reports 2017; 6(1): 35-37 Case Report Peer Reviewed Journal in Oncology, Gastroenterology and Hepatology www.oghreports.org | www.journalonweb.com/ogh

A Giant primary mesenteric Liposarcoma in an adolescent male: A rare case with presentation in unusual age and location

Vandana Rana1, Ranjan Praveer1, Singh Giriraj2, Rathi Khushi Ram1

ABSTRACT Primary mesenteric Liposarcoma is a rare neoplasm and only few documented cases are there in literature that too in adults. We report a case of giant primary mesenteric Liposar- coma in an adolescent male. Our patient is a 17 yrs old male who presented with gradual abdominal distension and was found to have huge multilobated and heterogeneous abdominal tumor arising from transverse mesocolon on imaging. The excised tumor weighed 19 kg and was reported as well differentiated Liposarcoma (WDLPS) on histomorphology. Atypical Lipomatous Tumor and WDLPS are synonyms describing lesions that are identical morpho- logically, karyotypically and in terms of biological potential. In deep visceral sites where wide excision margin are difficult to attain, the term WDLPS is preferred. Myxoid Liposarcoma is the most common variant of LPS seen in children and young adults with lower extremities being commonest site of occurrence. Our case is unique in rarity of lesion, its dimensions, location and uncommon histomorphology for age. Key words: Well differentiated Liposarcoma, Atypical lipomatous Tumor, , Giant, Adolescent. Key Messages: Liposarcoma is usually a malignancy of adults and constitutes <3% of sarcomas in children. Lower extremities and retroperitonium are its usual sites of occurrence. Retroperitoneal Liposarcoma are known to grow to large sizes. Primary mesenteric Liposar- coma is a rare entity and can also reach great dimensions and should be kept as one of the differential of gradual abdominal distension.

INTRODUCTION CASE HISTORY Vandana Rana1, Ranjan Liposarcoma (LPS) is the most common type of soft The patient is a 17 years old male presented in our Praveer1, Singh Giriraj2, tissue sarcoma in adults.[1] According to WHO classi­ institute with complaints of abdominal distension 1 and pain since last three years. Distension was insid­ Rathi Khushi Ram fication it is divided in to four subtypes—Atypical ious in onset, gradually progressive and associated lipomatous tumor (ALT)/ Well differentiated LPS 1Department of Pathology, Command with pain in which was dull aching and Hospital (SC), Armed Forces Medical (WDLPS), Myxoid/ Round cell, Pleomorphic and associated with two to three episodes of vomiting College, Pune-411040, INDIA. [2] initially. He was thin built and was not gaining 2 Dedifferentiated LPS. Among these WDLPS are the Department of Radiodiagnosis, weight. There was no history of altered bowel hab­ Command Hospital (SC), Armed Forces commonest in adults and are seen more in the deep its, malena or urinary complaints. General exami­ Medical College, Pune-411040, INDIA. soft tissue of the limbs (75%) followed by retroperito­ nation showed thin built and presence of pallor. On nium.[3] The common age group involved is between Correspondence per abdominal examination, he was having grossly 50 to 70 years.[1] These sarcomas are very rare in distended abdomen with dilated and flat, Dr Vandana Rana, Assoc Prof, Department of Pathology, Command Hospital (SC), childhood and constitute about 2% of all childhood stretched umbilicus (Figure 1). On palpation there AFMC, Pune-40,INDIA. soft tissue sarcoma with peak incidence in second was a huge, firm and painless lump filling almost the whole of abdomen. The lump was smooth, with Phone no: 919823988983 decade of life. Myxoid is most common in Email: [email protected] irregular margins and moved with respiration. No children with lower extremity as common primary clinical evidence of free fluid in the abdomen noted. [4] History site. Primary Intraperitoneal or mesenteric LPS Normal bowel sounds were present. Rest of the • Submission Date: 1-10-2015; are very rare and only few cases that too in adults systemic examination was normal. Serum Tumor • Review completed: 20-03-2016; [5,6] markers were not raised. On USG it appeared ill • Accepted Date: 12-04-2016. have been reported in literature. Retroperitoneal defined heterogeneously hyper echoic intraperitoneal and abdominal LPS are known to grow to large sizes DOI : 10.5530/ogh.2017.6.1.10 mass lesion extending from epigastric region to the however, after exhaustive search of written English . No significant vascularity noted on Doppler. Article Available online literature; we report most probably the first case of Differential of intraperitoneal benign teratoma was http://www.oghreports.org/v6/i1 a huge intra- abdominal LPS in an adolescent male. offered. Plain and contrast CT abdomen showed Copyright © 2016 Phcog.Net. This is an open- Cite this article: Rana V, Praveer R, Giriraj S, Ram RK. A Giant primary mesenteric Liposarcoma access article distributed under the terms in an adolescent male: A rare case with presentation in unusual age and location. OGH Reports. of the Creative Commons Attribution 4.0 2017;6(1):35-7. International license.

OGH Reports, Vol 6, Issue 1, Jan-Jun, 2017 35 A case of giant well differentiated abdominal Liposarcoma in an adolescent male intraperitoneal bizarre soft tissue mass measuring 20.5×28×38 cm (AP X TR X CC) extending from the epigastric, filling both the hypochondrias and reaching to pelvic region (Figure 2). It was having density of soft tissue, fat, fluid and multiple areas of calcification. Multiple septations were also noted. The mass was seen displacing the abdominal organs superiorly and posterior-laterally. It is seen extending through the trans­ verse mesocolon. Severe compression and displacement of mesenteric vessels also present. No organomegaly, signs of obstructive uropathy, significant lymphadenopathy or free fluid was present. Exploratory laparotomy and excision of the tumour was done. Per operative find­ ings confirmed the origin from transverse mesocolon. No infiltration into the surrounding structures was seen. The excised specimen was re­ ceived in our lab as multiple nodular masses with largest mass measuring 45×37×14 cm and the whole specimen weighed 19 kg (Figure 3) The specimen was multilobated, ivory to yellow coloured, soft to firm with semitransparent membranous capsule. Most of the lobes on cut sections showed yellowish gelatinous appearance with many of them showing Figure 1: 17 yrs old male with abdominal distension. the secondary changes in the form of haemorrhage, calcifications, focal cystic degeneration and few myxoid looking areas. Microscopy revealed encapsulated lesion comprising of mature adipocytic areas with trans­ gressing capillaries and fibrous septas along with many sclerosing areas having scattered bizarre stromal cells and some multinucleated floret cells (Figure 4). Occasional multivacuolated lipoblast seen. Areas of hemorrhage, cystic degeneration, focal calcification and myxoid change present. Few foci show perivascular presence of scanty lymphocytic cell infiltrate. No areas of necrosis or mitotic activity seen. The lesion was reported as Atypical lipomatous tumor/ Well differentiated LPS (WDLPS) and patient is kept on follow up. DISCUSSION Liposarcoma is one of the most common soft tissue sarcomas of adult life with relative incidence of 9.8% to 16% among other sarcomas but they rarely occur in children.[1] The common age group involved is between 50 to 70 years.[1] Our patient was just 17 yrs of age at presentation with a history of progressive distension of abdomen for last three years. There was a history of growth compromise and inability to gain weight. Figure 2: Contrast enhanced CT scan axial images with sagittal and coro- Although, approximately less than hundred cases of LPS in children and nal reformation show the massive heterogeneous abdomino-pelvic mass adolescents has been reported in written literature till date still they epicentred in the small bowel mesentery, indenting the superiorly account for <3% of all paediatric sarcomas.[4] One of the largest case series and inferiorly with displacement of bowel loops to the of LPS in young patients found lower limb to be the commonest involved left. location with only three cases to be of abdominal LPS out of 82 cases. All the three cases were histologically proven myxoid LPS.[7] Intra abdominal or primary mesenteric LPS are rare occurrence and approximately less than 20 cases mostly in adults have been reported in English literature till date.[5,6] Our patient was detected to have primary mesenteric WDLPS which is highly unusual for this age and anatomic location. The most recent WHO classification of soft tumors recognizes four ­cat egories of LPS: ALT/WDLPS (Intermediate grade), Dedifferentiated, Myxoid/round cell and pleomorphic LPS.[2] WDLPS is the most com­ mon form of LPS accounting for 40–45% of all LPS and are encountered in late adult life and 75% of the cases are known to develop in the deep muscles of the extremities, 20% in the retroperitonium and the remaining in the groin, spermatic cord and miscellaneous sites.[1,3] ALT and WDLPS are synonyms describing lesions that are identical morphologically, karyotypically and in terms of biological potential. In deep visceral sites like retroperitonium and mediastinum where wide excision margin are difficult to attain, the term WDLPS is recommended and justifiable.[2] In our case the anatomic site being intraabdominal, the lesion was reported as WDLPS. ALT/WDLPS show three main morphological patterns: adi­ Figure 3: Gross specimen post operatively and after fixation. pocytic, sclerosing and inflammatory and they all can be seen in same lesion as seen in our case also.

36 OGH Reports, Vol 6, Issue 1, Jan-Jun, 2017 A case of giant well differentiated abdominal Liposarcoma in an adolescent male

Surgical resection with clear margins is the treatment of choice for primary mesenteric LPS. Role of Radiotherapy and systemic chemotherapy remain to be established. Our patient is presently asymptomatic and is on follow up after surgical removal of tumor. CONCLUSON Primary mesenteric Liposarcoma though a rare entity and more so in children, should be kept as one of the differential of gradual abdominal distension. Awareness about lesion and correlation with clinical presen­ tation, Imaging and histopathology helps to clinch the diagnosis. ACKNOWLEDGEMENT We want to thank Surg Cmde C S Naidu, VSM Prof & HOD Surgery, AFMC and his whole surgical team for the remarkable surgery and all the support and encouragement.

Figure 4: H& E Stained sections show mature adipocytic areas with areas CONFLICT OF INTEREST of sclerosis bearing few bizarre large cells. Nil. REFERENCES 1. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft tissue tumors. 5th ed. Histologic grade is one of the most important predictors of outcome, Mosby Elsevier. 2008; 477-92. with low grade myxoid tumors having significant improved survival 2. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens. WHO Classification of rates then round cell, pleomorphic and dedifferentiated types. ALT/ tumours of soft tissue and . 4th ed. Lyon: IARC Press; 2013;33–36. WDLPS are histologic grade 1 tumors with usually low Ki 67 prolif­ 3. Weiss SW, Rao VK. Well-differentiated Liposarcoma (atypical lipoma) of deep soft tissue of extremities, retroperitonium and miscellaneous sites: a follow-up erative index. They are characterized by supernumerary ring and giant study of 92 cases with analysis of the incidence of “dedifferentiated”. Am J Surg marker chromosomes which can be detected by cytogenetic and FISH Pathol. 1992;16:1051. http://dx.doi.org/10.1097/00000478-199211000-00003 4. Huh WW, Yuen C, Munsell M, Jordan AH, Lazar A, Patel S et al. Liposarcoma studies. Murine double minute 2(MDM2) gene is consistently ampli­ in children and young adults: A multi-institutional experience. Pediatr Blood fied and over expressed in cases of ALT/WDLPS. MDM2 and or CDK4 Cancer. 2011;57:1142–6. http://dx.doi.org/10.1002/pbc.23095 ; PMid:21394894 PMCid:PMC3134599 nuclear immunopositivity is present in most cases and also found to be 5. Jain SK, Mitra A, Kaza RCM, Malagi S. Primary mesenteric Liposarcoma: An [2,8] correlated with higher Ki 67 proliferation index. In our case the Ki 67 unusual presentation of a rare condition. J Gastrointest Oncol. 2012;3:147–50. index was low and no areas of necrosis or dedifferentiation was present. PMid:22811883 PMCid:PMC3397650 6. Cerullo G, Marrelli D, Rampone B, Perrotta E, Caruso S, Roviello F. Giant Dedifferentiation occurs in up to 10% of WDLPS with risk being higher Liposarcoma of the mesentry. Report of a case. Ann Ital Chir. 2007;78:443–5. in deep sited LPS.[2] There is substantial amplification of MDM2 in PMid:18338555 Dedifferentiated LPS. 7. Alaggio R, Coffin CM, Weiss SW, Bridge JA, Issakov J, Oliveira AM et al. Liposar- coma in young patients: A study of 82 cases occurring in patients younger than LPS are known to grow in large dimensions especially in retro perito­ 22 years of age. Am J Surg Pathol. 2009;33:645–58. http://dx.doi.org/10.1097/ neum due to presence of potential space and delayed symptoms. Reports PAS.0b013e3181963c9c ; PMid:19194281 8. Putri RI, Siregar NC, Siregar B. Overexpression and amplifation of Murine dou- of many giant retroperitoneal LPS are there in literature with largest ble minute 2 as a diagnostic tool in large lipomatous tumor and its correlation reported to be weighing 42 kg.[9] However, largest abdominal or mesenteric with Ki 67 proliferative index: An institutional experience. IJPM. 2014;57:558–63. LPS reported in literature is not more than 15 Kg.[5,6,10] Sato et al found 9. Yol S, Talvi S, Talvi L, Belviranli M, Yosunkaya A. Retroperitoneal and scrotal giant liposarcoma: report of a case. Surg Today. 1998;28:339–42. http://dx.doi. that the tumor size greater than 20 cm predicted a significantly poorer org/10.1007/s005950050136 ; PMid:9548324 prognosis.[5] The tumor in present case was huge and weighed together as 10. Korukluoglu B, Ergul E, Sisman I C, Yalcin S, Kusdemir A. Giant primary syn- chronously bilateral mesenteric dedifferentiated Liposarcoma with hyperpara- 19 kgs. After thorough search of literature, ours is most probably the first thyroidism, hyperthyroidism, Type 2 diabetes mellitus and hypertension. J Pak case of giant intra abdominal WDLPS in an adolescent male. Med Assoc. 2009;59:563–5. PMid:19757707

Cite this article: Rana V, Praveer R, Giriraj S, Ram RK. A Giant primary mesenteric Liposarcoma in an adolescent male: A rare case with presentation in unusual age and location. OGH Reports. 2017;6(1):35-7.

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