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Table of Contents
Year : 2022  |  Volume : 15  |  Issue : 2  |  Page : 104-107

Primary colonic liposarcoma presenting as intussusception: A case report from the Kashmir valley

Department of General and Minimal Access Surgery, SKIMS Medical College, Srinagar, Jammu and Kashmir, India

Date of Submission21-Nov-2021
Date of Acceptance01-Dec-2021
Date of Web Publication04-Jul-2022

Correspondence Address:
Yaqoob Hassan
Department of General and Minimal Access Surgery, SKIMS Medical College, Srinagar, Jammu and Kashmir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/hmj.hmj_76_21

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Rationale: Soft tissue sarcoma (STS) arises from mesenchymal tissue and can occur at any anatomic site and age. These tumors can originate from skeletal muscle, adipose tissue, vascular tissues, or connective tissues. Liposarcoma (LPS) is the most frequent type of sarcoma and is commonly found in the retroperitoneal region. Patient Concerns: A case of colonic liposarcoma in a young female patient presented with sub-acute intestinal obstruction due to intussusception. The patient was only 17-years-old, much younger than the average age of occurrence, and thus represents the first case report of primary colonic liposarcoma in this age group. Diagnosis: The patient was resuscitated and subjected to standard investigations. An ultrasonography abdomen/ pelvis revealed colo-colic intussusception and CECT abdomen showed evidence of an enhancing mass within the lumen of the transverse colon. Interventions: The patient was subjected to exploratory laparotomy with segmental colonic resection and primary colo-colic anastomosis. Outcomes: The histopathology and immunohistochemistry revealed the morphology of high grade colonic liposarcoma. The patient is doing well eight months after surgery and is being monitored on a regular basis. Lessons: The primary gastrointestinal liposarcoma tumors are uncommon and colonic variants are extremely rare, especially in the second decade of life, and limited to case reports which can be counted on fingertips. Surgical resection with negative margins is the only curative treatment.

Keywords: Age, case report, colon, liposarcoma, rare, sarcoma

How to cite this article:
Hassan Y, Rather AA, Akhtar Z. Primary colonic liposarcoma presenting as intussusception: A case report from the Kashmir valley. Hamdan Med J 2022;15:104-7

How to cite this URL:
Hassan Y, Rather AA, Akhtar Z. Primary colonic liposarcoma presenting as intussusception: A case report from the Kashmir valley. Hamdan Med J [serial online] 2022 [cited 2022 Sep 26];15:104-7. Available from: http://www.hamdanjournal.org/text.asp?2022/15/2/104/344195

  Introduction Top

Soft-tissue sarcomas (STS) are rare tumours that develop from mesenchymal tissue and can afflict people of all ages and anatomic sites. Half of these tumours are found in the extremities, and 15% in the retroperitoneum, representing 1% of the adult malignancies and 15% of paediatric malignancies.[1] There are many histological subtypes of sarcoma, which can broadly be of two types: low-grade sarcoma (desmoid, liposarcoma [LPS] with low-grade features, etc.) and high-grade sarcoma (malignant fibrosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma). LPS is the soft-tissue tumour that develops from adipose tissue. LPS is the most common histological subtype in adults, accounting for 24% of extremity STS and 45% of retroperitoneal STS.[1] The World Health Organization updated the five distinct subtypes of LPS in 2013, including well-differentiated/dedifferentiated, pleomorphic, myxoid and mixed LPS.[2] Depending on the location of tumour, the patients may present with different symptoms and signs such as painful swelling, palpable lump developing under, skin numbness, fatigue, abdominal/chest pain, nausea, vomiting, urinary or swallowing difficulty and decreased range of motion of joints and non-specific symptoms of fevers, chills, fatigue, night sweats and weight loss. Radiation or certain chemical exposure and a few familial syndromes (Gardner syndrome) are some of the risk factors of LPS. Although common in the extremities and retroperitoneum, LPS rarely involves the gastrointestinal tract.[3],[4],[5] Herein, we present a rare case of a 17-year-old female with transverse colon LPS who presented to our emergency room with subacute intestinal obstruction (intussusception) and the treatment she received. The work has been reported in line with the SCARE 2020 guidelines.[6]

  Case Report Top

Miss X, a 17-year-old, female student in 11th grade, normotensive, non-diabetic and euthyroid from a rural area of Kashmir valley, was referred to our accident and emergency unit from a community health-care centre with the presentation of a 5-day history of pain in the abdomen and few episodes of vomiting. A detailed history including, previous surgical, medical, obstetric, drug, personal and family history, all of which were not significant. Any history of radiation exposure, as well as familial diseases, was ruled out. The patient was thoroughly examined, resuscitated and subjected to standard baseline investigations [Table 1]. On physical and systemic examination, her pulse rate was 88 bpm, the tongue was dry, blood pressure was 120/70 mmHg, respiratory rate was 18 breathes/min, temperature 98.8°F and her saturation was 96% at room air. The abdomen was mild-to-moderately distended, and the supraumbilical area was mildly tender. The digital rectal examination was unremarkable and the finger was stained with normal color stools. As a first radiological investigation, ultrasonography abdomen/pelvis was performed, revealing 'telescoping of one gut loop into another for a length of 4.2 cm seen in the periumbilical region with 87 mm × 34 mm well-defined heterogeneous lesion'. Because of the ultrasonographic findings of colo-colic intussusception, the patient was admitted to our tertiary care hospital, Department of General and Minimal Access Surgery, Medical Record Department Number 128642 (confidential), for evaluation and management.
Table 1: Biochemical-haematological parameters and tumour markers

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The patient was advised to withhold oral feeds and kept nothing per oral, resuscitated with intravenous Ringer's lactate fluid, antibiotics (I. V. ceftriaxone-sulbactam, tinidazole) and strict monitoring of vitals, urine output, serial per-abdomen examinations, abdominal girth and serum electrolytes. Patients were subjected to contrast-enhanced computed tomography (CECT) scanning of the abdomen and pelvis and results were reported within 6 h of admission. A CECT scan showed evidence of an enhancing mass with non-enhancing components measuring 8.8 cm × 4.3 cm in size within the lumen of the transverse colon, with the proximal transverse colon entering the distal colon (Intussusception) [Figure 1]. The mild mesenteric stranding was observed in the affected region. There were a few enlarged locoregional nodes, the largest of which was 12 mm × 9.8 mm in size and small gut loops abut the affected area of the transverse colon. The rest of the viscera in the abdomen were normal. Carcinoembryonic antigen, cancer antigen (CA19-9) and CA-125 tumour markers were all within the normal limits [Table 1]. The patient was managed conservatively and colonoscopy was deferred due to symptoms of obstruction. A colonoscopy revealed a large polypoidal mass with intussusception and edematous gut lumen. Cope could not pass beyond the mass and any attempt to do so was thwarted. Colonoscopic biopsy was avoided with the idea of avoiding the perforation, dissemination and upgradation of the tumour. The rest of the distal colon appeared to be normal [Figure 1].
Figure 1: (a) Contrast-enhanced computed tomography abdomen and pelvis and (b) xolonoscopic photographs showing transverse colon mass with intussusception

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The patient was taken up for an exploratory laparotomy and proceeded. On laparotomy, a visible mass measuring 8 cm × 5 cm was found in the transverse colon, with intussusception and mild dilatation of the proximal colon [Figure 2]. There was no invasion of the serosal surface, and there were no palpable or visible mesenteric nodes. Pelvis and liver were free of any metastasis. There was no ascitic fluid, no visible lymph nodes or metastasis. The appendix, omentum, mesentery, stomach, ascending and descending sigmoid colon and rectum, liver and spleen were all normal. The transverse and ascending colons were mobilised, and a segmented resection with oncological margins of more than 5 cm on both sides of growth was performed, followed by a double-layer primary colo-colic anastomosis. The specimen was dissected in the operating room and revealed a mass of 7 cm × 6 cm in size, soft to firm in consistency, arising from the ante-mesenteric bowel wall with mucosal petechial patches and edematous surrounding gut wall, and was sent for histopathological examination [Figure 2]. The mesenteric defect was closed, complete haemostasis was achieved, peritoneal cavity washes with normal saline were performed, a 28F pelvic tube drain was left in situ and the incision was closed back in layers. The patient was uneventfully extubated and shifted to the high dependency unit of the general surgery ward for post-operative monitoring. After 12 h of surgery, the patient was encouraged to ambulate, and the Foleys' catheter was removed. After 72 h, liquid orals were introduced, followed by semisolids and solids. The tube drain was removed on the 4th post-operative day, and the patient was discharged on the 5th day and attached to our outpatient department for weekly follow-up in the 1st month and monthly thereafter. The patient is doing well 8 months after surgery and is being monitored regularly.
Figure 2: Intraoperative photograph and on-table axially sectioned gross specimen

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The section studied shows a polypoid mass with tumour cells dispersed in an edematous background on microscopic and immunohistochemical examination [Figure 3]. The cells had hyperchromatic large nuclei, inconspicuous nucleoli and scant cytoplasm. The superficial colonic mucosa was ulcerated, and an inflammatory infiltrate was present in the background. Both resection margins were tumour-free. Mitotic activity was also seen (5/50 HPF). There was no evidence of metastasis in any of the 11 lymph nodes that had been dissected. Tumour cells were positive for SMA, CD34(membrane glycoprotein), MDM2, and CDK4 but negative for S100, HMB45, DOG1, CD117, MELAN A, EMA, ALK, HHV8, SMAD4, ERG, CD31, DESMIN, HMB45 and DOG1.
Figure 3: Microscopic and immunohistochemistry slides. (a) Haematoxylin and eosin-stained sample reveals high-grade liposarcoma (b) positive for lipoblast and lipogenic cell, CD34

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  Discussion Top

LPS is the mesenchymal tumour arising from adipocytes. Among the 50 recognised entities of STS, LPS is the most common type in adults. It represents 20%–25% of adult STSs with the peak age of onset in the fifth–seventh decades of life.[7],[8],[9] The tumour can arise from various locations in the body but is common in extremities and retroperitoneum.[10] The primary gastrointestinal LPS tumours are uncommon and colonic types are extremely rare, limited to case reports which can be counted on fingertips. It is a slow-growing tumour with a pleomorphic subtype that is the most aggressive and metastasis-prone type, with a 40% tumour-related mortality rate.[11] The symptoms and presentation of patients with colonic LPS vary depending on the anatomical site and location. It can be completely asymptomatic and go unnoticed until the disease is advanced, or it can present with non-specific symptoms. The presentation varies from patient to patient, ranging from simple abdominal pain to partial or complete obstruction, diarrhoea to constipation, anaemia to frank haematochezia and weight loss.

LPS behaviour ranges from silent non-metastatic disease to aggressive disease with the potential to recur and metastasise. As a result, a multimodal approach is used for accurate definitive diagnosis, staging and management of these lesions. CECT scanning and magnetic resonance imaging of the abdomen and pelvis are the radiological investigations of choice for defining the site, size, origin and relationship to surrounding viscera and neurovascular structures, invasion or compression and ruling out metastasis.[12],[13],[14] Biopsy for histopathological examination improves radiological assessment and is a critical step in reaching a diagnosis, and thus provides optimal patient care. The preferred treatment for primary colonic LPS is complete surgical resection with negative resection margins. Chemotherapy sensitivity is determined by histological type and grade, which is especially important in unresectable and advanced LPSs.[15] Some of the factors accepted as indicators of poor prognosis are age >45 years, the presence of round cells, necrotic areas within the tumour and disease dissemination.[16],[17]

We report a case of primary colonic LPS, a rare mesenchymal tumour with literature available in case reports. Only a few cases of primary colonic LPS have been reported in the literature, with Wood and Morgenstern reporting the first case in 1989.[18] The management has been similar to retroperitoneal LPS including a combination of surgical resection and targeted chemotherapy. To the best of our knowledge, only 16 cases of primary colonic LPS and two primary colonic liposarcomatosis have been reported in the English medical literature. What makes our case unique is that the patient was only 17 years old, much younger than the mean age of occurrence, and thus represents the first case report of primary colonic LPS in this age group.

  Conclusion Top

The primary gastrointestinal LPS tumours are uncommon, and colonic types are extremely rare, especially in the second decade of life, and limited to case reports which can be counted on fingertips. For a definitive diagnosis, a multimodal approach of radiology and histopathological examination is required. The only curative treatment is surgical resection with negative margins. Due to the scarcity of available literature, a large number of cases must be collected to definitively determine treatment guidelines, recurrence patterns and outcomes.

Declaration of patient consent

The authors certify that they have obtained the parents' consent for the patient images and clinical information to be reported in the journal. The parents understand that their child's name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Ashley SW, Cance WG, Chen H. Soft tissue sarcoma. In: ACS Surgery: Principles and Practice. Ch. 8. Ontario: Decker Intellectual Properties; 2014.  Back to cited text no. 1
Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. World Health Organization Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon: IARC Press; 2013.  Back to cited text no. 2
Choi JH, Hwang IG, Cha SJ, Lee TJ, Jang JS. Occurrence of colonic liposarcoma after retroperitoneal liposarcoma. Korean J Intern Med 2015;30:125-8.  Back to cited text no. 3
Gutsu E, Ghidirim G, Gagauz I, Mishin I, Iakovleva I. Liposarcoma of the colon: A case report and review of literature. J Gastrointest Surg 2006;10:652-6.  Back to cited text no. 4
Türkoğlu MA, Elpek GÖ, Doğru V, Calış H, Uçar A, Arıcı C. An unusual case of primary colonic dedifferentiated liposarcoma. Int J Surg Case Rep 2014;5:8-11.  Back to cited text no. 5
Agha RA, Franchi T, Sohrabi C, Mathew G, Kerwan A, SCARE Group. The SCARE 2020 guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines. Int J Surg 2020;84:226-30.  Back to cited text no. 6
Coindre JM, Pédeutour F, Aurias A. Well-differentiated and dedifferentiated liposarcomas. Virchows Arch 2010;456:167-79.  Back to cited text no. 7
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van Vliet M, Kliffen M, Krestin GP, van Dijke CF. Soft tissue sarcomas at a glance: Clinical, histological, and MR imaging features of malignant extremity soft tissue tumors. Eur Radiol 2009;19:1499-511.  Back to cited text no. 9
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Downes KA, Goldblum JR, Montgomery EA, Fisher C. Pleomorphic liposarcoma: A clinicopathologic analysis of 19 cases. Mod Pathol 2001;14:179-84.  Back to cited text no. 11
Thomas JM. Retroperitoneal sarcoma. Br J Surg 2007;94:1057-8.  Back to cited text no. 12
Hughes TM, Spillane AJ. Imaging of soft tissue tumours. Br J Surg 2000;87:259-60.  Back to cited text no. 13
Hoffman A, Lazar AJ, Pollock RE, Lev D. New frontiers in the treatment of liposarcoma, a therapeutically resistant malignant cohort. Drug Resist Updat 2011;14:52-66.  Back to cited text no. 14
Jones RL, Fisher C, Al-Muderis O, Judson IR. Differential sensitivity of liposarcoma subtypes to chemotherapy. Eur J Cancer 2005;41:2853-60.  Back to cited text no. 15
Enzinger FM, Weiss SW. Soft Tissue Tumours. 3rd ed. St. Luis, MO: Mosby, Inc.; 1995.  Back to cited text no. 16
D'Annibale M, Cosimelli M, Covello R, Stasi E. Liposarcoma of the colon presenting as an endoluminal mass. World J Surg Oncol 2009;7:78.  Back to cited text no. 17
Wood DL, Morgenstern L. Liposarcoma of the ileocecal valve: A case report. Mt Sinai J Med 1989;56:62-4.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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