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Year : 2023  |  Volume : 66  |  Issue : 1  |  Page : 213-215
Metastatic dedifferentiated liposarcoma of ileum presenting as an ileal mass with intussusception—A rare case with literature review

Department of Pathology, Seth GS Medical College, KEM Hospital, Parel, Mumbai, Maharashtra, India

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Date of Submission06-Oct-2021
Date of Acceptance12-Jan-2022
Date of Web Publication18-Jan-2023

How to cite this article:
Chaturvedi RA, Patil RD, Sonavane TS, Tripathi N. Metastatic dedifferentiated liposarcoma of ileum presenting as an ileal mass with intussusception—A rare case with literature review. Indian J Pathol Microbiol 2023;66:213-5

How to cite this URL:
Chaturvedi RA, Patil RD, Sonavane TS, Tripathi N. Metastatic dedifferentiated liposarcoma of ileum presenting as an ileal mass with intussusception—A rare case with literature review. Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Mar 20];66:213-5. Available from:

Dear Editor,

Small intestinal tumors are rare, comprising only 2% of gastrointestinal tract malignancies.[1] Secondary tumors are more common and arise from uterus, cervix, colon, lung, and breast; and most are melanomas and adenocarcinomas on histology.[2] Metastatic liposarcomas are rare in small intestine, still rare is its dedifferentiated type which is the most aggressive type.[3] Here, we report an unusual case of a metastatic dedifferentiated liposarcoma (DDLS), presenting as an ileal mass with intussusception.

A 63-year-old man referred to our tertiary care hospital with complaints of acute abdominal pain and distension. He also reported intermittent diarrhea and constipation for 15–20 days. There was no history of vomiting, fever, melena, or jaundice. On physical examination, his BP was 90/40 mm Hg and pulse rate was 120/min. Abdominal examination showed a lump in left iliac fossa with no evidence of melena or per rectal bleeding. His CECT report showed ileo-ileal intussusception with proximal bowel dilatation [Figure 1]a and an absent left kidney. The patient underwent emergency exploratory laparotomy, and intraoperatively a gangrenous bowel of about 100 cm length was seen, approximately 120 cm from ileocolic junction, along with a 20-cm segment of intussuscepted ileum. Resection of the gangrenous segment with double-barrel ileal stoma was performed. Postoperatively, the patient was shifted to ICU and was started on antibiotics. We received a specimen of the small intestine showing a large polypoidal intraluminal mass measuring 8.5 x 5 x 4 cm with overlying ulcerated mucosa, and a grayish white, firm cut surface [Figure 1]b and [Figure 1]c. Microscopy showed a tumor predominantly in submucosa, focally extending up to mucosa causing ulceration [Figure 2]a, and also into muscularis propria [Figure 2]b and mesentery [Figure 2]c. The tumor showed high cellularity with spindle and lipomatous areas, showing many bizarre atypical cells having hyperchromatic nuclei and few cells resembling lipoblasts [Figure 2]d, [Figure 2]e, [Figure 2]f with many mitotic figures. On immunohistochemistry (IHC), the spindle cells expressed smooth muscle actin (SMA) [Figure 3]a, while CD117, CD34, and desmin were negative. Lipomatous areas expressed strong positivity for S100 [Figure 3]b. Sections from the rest of the bowel showed mild ischemic changes. A diagnosis of “Dedifferentiated liposarcoma of small intestine with intussusception” was rendered. Meanwhile, in view of the absence of one kidney on CT, he was thoroughly interviewed and he revealed a history of previous surgical excision, performed 2 years back for a retroperitoneal mass with left nephrectomy and adrenalectomy, and his pathological report showed a diagnosis of dedifferentiated liposarcoma. In view of the above, our histopathological report was revised as “Metastatic dedifferentiated liposarcoma of small intestine with intussusception.” The patient, however, did not recover from his sepsis and eventually succumbed on postoperative day 5.
Figure 1: Ileo-ileal intussusception (red arrow) (a), dilated bowel with tumor (b), gray-white cut surface (c)

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Figure 2: Mucosal ulceration (a, 40X HE), invasion of muscularis propria (b, 40X HE) and mesentery (c, 100X HE), atypical spindle and lipomatous cells (d, e, f, 400X HE)

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Figure 3: SMA (a) and S100 (b) positivity

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According to the study on tumor spectrum of adult intussusceptions,[4] the incidence of colonic intussusceptions was significantly higher (60%) than that of enteric intussusception (20%) and primary adenocarcinoma and lymphoma were the most common underlying malignant colonic lesions. In the small bowel, the intussusception was more common due to benign neoplasms, with lipoma and Peutz–Jeghers adenoma being the most common, and among the malignant neoplasms; lymphoma, gastrointestinal stromal tumor (GIST), and metastatic melanoma were the most common followed by metastatic leiomyosarcoma and carcinomas. None were liposarcomas.[4]

DDLS accounts for approximately 18% of all liposarcomas. It most frequently arises from the retroperitoneal region[5] and affects adults in the age-group of 60 to 70 years[5] as found in our case too. DDLS grows faster and spreads to adjacent organs with a higher recurrence rate and has poorer prognosis than well-differentiated liposarcoma.[1],[3] It commonly metastasizes to lung, liver, soft tissue, brain, and bone. Its metastasis to the gastrointestinal tract (GIT), especially to the small intestine as seen in our case, is very rare.[3] Primary liposarcoma of GIT is extremely rare, though occasional cases are described in stomach, small intestine, and colon.[1] Thus, a possibility of metastasis must always be excluded before making a diagnosis of primary in GIT. In our case, we initially made a diagnosis as primary LPS; however, we could arrive at the correct diagnosis of metastatic LPS after thoroughly eliciting the previous history. On thorough Web search over the period of 23 years, we found only seven cases of liposarcoma metastatic to the small intestine [Table 1], of which four presented with documented intussusception and at least three arose in the retroperitoneum. However, the dedifferentiated morphology was seen only in two, of which none metastasized from retroperitoneum to the small intestine as seen in our case; and both recurred in much shorter span (3 and 7 months) as compared to ours (2 years).
Table 1 (Original): Review of the literature

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Histological diagnosis of liposarcoma is otherwise straightforward in many cases; however, DDLS type can be difficult to diagnose. Multiple sections are always essential to look for any dedifferentiated areas for exact histological typing.[8] IHC is usually positive for S100 protein in well-differentiated component, but can be negative in dedifferentiated areas,[8] and specific markers like MDM2 and CDK4 would be useful for definite diagnosis,[9] which were not available with us. However, in our case, many lipomatous areas were present along with classical lipoblasts showing S100 positivity on IHC; hence, all other possibilities were ruled out. Though spindle (dedifferentiated) areas could give rise to a differential diagnosis of gastrointestinal stromal tumor, leiomyosarcoma, and tumors of neural origin, IHC for CD117, CD34, desmin, and S100 was negative in these areas.

Shibata et al.[3] have mentioned an extremely aggressive post-metastatic course of DDLS with a median post-metastatic survival of only 5 months. Our patient also succumbed on the fifth postoperative day. All liposarcomas are treated by wide local excision, and adjuvant radiotherapy improves the local control rate; however, chemotherapy has not established its effectiveness.[3] Our patient underwent wide local excision and received both chemotherapy and radiotherapy.


The authors thank Dr. Amita Joshi (Professor and Head, Department of Pathology, Seth GSMC and KEM Hospital, Mumbai) and Dr. Varsha Kulkarni (Professor, Department of General surgery, Seth GSMC and KEM Hospital, Mumbai).

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Odze RD, Goldblum JR. Odze and Goldblum Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas, 3rd edition, Philadelphia: Elsevier Saunders, 2015.  Back to cited text no. 1
Dwivedi RC, Kazi R, Agrawal N, Chisholm E, St. Rose S, Elmiyeh B, et al. Comprehensive review of small bowel metastasis from head and neck squamous cell carcinoma. Oral Oncol 2010;46:330–5.  Back to cited text no. 2
Shibata Y, Sato K, Kodama M, Nanjyo H. Metastatic liposarcoma in the jejunum causing intussusception: Report of a case. Surg Today 2008;38:1129–32.  Back to cited text no. 3
Chiang J-M, Lin Y-S. Tumor spectrum of adult intussusception. J Surg Oncol 2008;98:444–7.  Back to cited text no. 4
Luo P, Cai W, Yang L, Wu Z, Chen Y, Zhang R, et al. Retroperitoneal dedifferentiated liposarcoma: Analysis of 61 cases from a large institution. J Cancer 2018;9:3831-8.  Back to cited text no. 5
Rowe SP, Coquia SF, Johnson PT, Fishman EK. Liposarcoma metastases to the small bowel presenting as fat-density intraluminal lesions. Radiol Case Rep 2016;11:296–8.  Back to cited text no. 6
Gomez D, Rahman S, Guillou P. Spontaneous mesenteric haematoma: A diagnostic challenge. Ann R Coll Surg Engl 2006;88:W1–3.  Back to cited text no. 7
Rekhi B, Navale P, Jambhekar N. Critical histopathological analysis of 25 dedifferentiated liposarcomas, including uncommon variants, reviewed at a tertiary cancer referral center. Indian J Pathol Microbiol 2012;55:294-302.  Back to cited text no. 8
  [Full text]  
Binh MB, Sastre-Garau X, Guillou L, de Pinieux G, Terrier P, Lagacé R, et al. MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes: A comparative analysis of 559 soft tissue neoplasms with genetic data. Am J Surg Pathol 2005;29:1340-7.  Back to cited text no. 9

Correspondence Address:
Rachana A Chaturvedi
Seth G. S. Medical College (GSMC), KEM Hospital, Parel, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_994_21

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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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