Submit Your Article CMED MEACR meeting
Home Print this page Email this page Users Online: 307
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 3  |  Issue : 6  |  Page : 548-550

Giant retroperitoneal liposarcoma: A very rare case report


Department of Surgery, Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi, India

Date of Web Publication11-Oct-2014

Correspondence Address:
Umesh Jethwani
Vardhman Mahavir Medical College and Safdarjang Hospital, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.142672

Rights and Permissions
  Abstract 

Soft-tissue sarcomas are very rare entity, seen in <1% of all malignancies. Sarcoma accounts for about one-third of all retroperitoneal malignancies. Management of retroperitoneal sarcoma is challenge for surgeons because of its rare presentation and no standard guidelines for it. A 65-year-old male presented to surgery out-patient department with the complaints of abdominal distention and pain in abdomen since 3 years. On imaging, he was diagnosed as case of retroperitoneal tumor and managed by radical surgery including resection of the tumor with left kidney, spleen, and descending colon. Postoperative period was uneventful.

Keywords: Giant retroperitoneal sarcoma, liposarcoma, retroperitoneal tumor


How to cite this article:
Mohil R S, Jethwani U, Singh G J, Arora J, Saroha R, Verma R, Siraj F. Giant retroperitoneal liposarcoma: A very rare case report. Clin Cancer Investig J 2014;3:548-50

How to cite this URL:
Mohil R S, Jethwani U, Singh G J, Arora J, Saroha R, Verma R, Siraj F. Giant retroperitoneal liposarcoma: A very rare case report. Clin Cancer Investig J [serial online] 2014 [cited 2019 Nov 17];3:548-50. Available from: http://www.ccij-online.org/text.asp?2014/3/6/548/142672


  Introduction Top


Retroperitoneal sarcomas (RPSs) are very rare tumors, constituting 1-2% of all solid tumors. [1] These malignant tumors arise from mesenchymal cells and are usually located in muscle, fat, and connective tissues. [2] However, it has been observed rarely in the gastrointestinal system. Liposarcoma accounts for more than 20% of all sarcomas in adults and up to 41% of all RPSs. [3],[4]

Imaging studies plays a huge role in management of RPSs as they are present in anatomically challenging sites. Imaging provides the surgical pathway for resection as it tells about the local extent, lymph node status and distant metastasis. They are usually diagnosed in their advance stage. Surgery is the main stay of management as neoadjuvant chemo and radiotherapy are inferior to the radical surgery. In tumors that have been adequately excised, the final outcome depends on the tumor grade. [5] We are reporting a rare case of giant RPS in a 65-year-old male, which was managed by radical resection.


  Case report Top


A 65-year-old male presented with the complaints of abdominal pain and distention since 3 years. History of weight loss was present. On examination, a large, firm mass was palpable occupying whole of the abdomen. All laboratory investigations were normal.

Contrast-enhanced computed tomography (CECT) abdomen revealed large hypo dense fatty attenuation septated mass lesion. The mass is found to be displacing left kidney and colon to the right crossing the midline. Multiple enhancing nodular masses with septa are noted within the mass lesion [Figure 1]. On laparotomy huge retroperitoneal tumor encasing left kidney, spleen, mesentry of splenic flexure and descending colon with no lymphadenopathy was found. En bloc resection of the tumor with left kidney, spleen, splenic flexure and descending colon followed by colocolic (transverse-sigmoid) stapler anastomosis was done.
Figure 1: Contrast-enhanced computed tomography abdomen image

Click here to view


Gross examination of specimen revealed large, lobulated soft tissue mass measuring 40 cm × 30 cm and weighing 10.6 kg. Histopathology examination revealed well-differentiated liposarcoma [Figure 2] and [Figure 3]. Postoperative period was uneventful and patient was discharged on day 11.
Figure 2: Histopathology-lobules of fat with presence of atypical round to spindle cells in the septa

Click here to view
Figure 3: Histopathology (×40) resence of lipoblast within tumor

Click here to view



  Discussion Top


The overall incidence of RPS is 0.3-0.4% per 100,000 of the population, and usually patient present in their fifth to sixth decade of life. [6] Primary tumors of the retroperitoneum can be of many types: Neoplasms arising in the kidney, adrenal gland, retroperitoneal lymph nodes (malignant lymphomas). The retroperitoneal soft-tissue sarcomas are also presented in this location, as liposarcoma, lipoma, malignant fibrous histiocytoma, leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, vascular tumors, peripheral nerve tumors, synovial sarcoma, etc., World Health Organization has divided liposarcoma into five subtypes according to the differentiation:

  1. Well-differentiated
  2. Dedifferentiated
  3. Myxoid
  4. Pleomorphic
  5. Mixed type. [7]


Retroperitoneal sarcoma most commonly presents as an asymptomatic abdominal mass in 80% of cases because tumors smaller than 5 cm usually remain silent. 20% of cases present with nonspecific signs and symptom, e.g. vague abdominal discomfort or pain, weight loss, and early satiety whereas some cases may present with intestinal obstruction or bleeding. They can also present as lower extremity swelling or pain. [8]

Contrast-enhanced computed tomography abdomen is very helpful in preoperative planning and demonstrates relationship of tumor to the nearby structures and vascular invasion. Sensitivity of CT scan in low grade lesion is 100% and there is virtually no role of preoperative treatment of these lesions. It also helpful in detection of synchronous and metastatic lesion in liver. [9]

Optimal management of resectable RPS is complete surgical removal with tumor free margin, which sometimes requires en bloc resection of adjoining structures. Most commonly resected structures are kidney, large bowel, ureter etc.

Similar case reports of giant RPSs have been reported in literature. Morandeira et al. [10] have reported a case of giant RPS in a 63-year-old woman. She presented with the complaints of abdominal mass, edema lower extremity and cahexia. CECT abdomen revealed heterogeneous mass with necrosis pushing intestine. En bloc resection of tumor with right nephrectomy, appendectomy, hysterectomy was done.

Prognosis depends on grade of the tumor, completeness of resection and multifocality of disease. Hassan et al. reported series of 97 patients of RPS with complete resection in 78% of cases with 5 years survival in 51% cases and 5 years recurrence in 44% cases. [11]

Local recurrence is the main cause of failure, ranging from 40% to 80%, respectively. [4] About 75% of sarcoma-related deaths involve uncontrolled local recurrence. [4],[12]

Follow-up is by CECT scans at every 6 months intervals to rule out any recurrence since most recurrence are seen within 2 years of initial surgery. [13]

Radical surgery provides the cure and is standard of care in RPSs. The concept of "compartmentalization" that applies to limb sarcomas is difficult to extrapolate directly to RPS. [14] It's difficult to get microscopic margins in huge RPS. No effective chemotherapy exists to influence survival in patients with RPS.


  Conclusion Top


No standardized guidelines have been established for its treatment because too small a number of cases have been reported, but surgical resection was considered the treatment of choice in our case. These tumors require an aggressive surgical approach. A larger number of cases will be necessary to determine whether additional treatment such as chemotherapy and radiotherapy is necessary or effective in liposarcoma of colon.

 
  References Top

1.
Goss G, Demetri G. Medical management of unresectable, recurrent low-grade retroperitoneal liposarcoma: Integration of cytotoxic and non-cytotoxic therapies into multimodality care. Surg Oncol 2000;9:53-9.  Back to cited text no. 1
    
2.
Erzen D, Sencar M, Novak J. Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the institute of oncology, Ljubljana. J Surg Oncol 2005;91:1-9.  Back to cited text no. 2
    
3.
Shibata D, Lewis JJ, Leung DH, Brennan MF. Is there a role for incomplete resection in the management of retroperitoneal liposarcomas? J Am Coll Surg 2001;193:373-9.  Back to cited text no. 3
    
4.
Lewis JJ, Leung D, Woodruff JM, Brennan MF. Retroperitoneal soft-tissue sarcoma: Analysis of 500 patients treated and followed at a single institution. Ann Surg 1998;228:355-65.  Back to cited text no. 4
    
5.
Kumar V, Misra S, Chaturvedi A. Retroperitoneal sarcomas-a challenging problem. Indian J Surg Oncol 2012;3:215-21.  Back to cited text no. 5
    
6.
Daugaard S. Current soft-tissue sarcoma classifications. Eur J Cancer 2004;40:543-8.  Back to cited text no. 6
    
7.
Fletcher CD, Unni KK, Mertens F. Pathology and Genetics of Tumors of Soft Tissue and Bone. World Health Organization Classification of Tumors. Lyon, France: IARC Press; 2002. p. 227-32.  Back to cited text no. 7
    
8.
Stawicki SP. Retroperitoneal sarcomas. OPUS 12 Sci 2007;1:17-8.  Back to cited text no. 8
    
9.
Windham TC, Pisters PW. Retroperitoneal sarcomas. Cancer Control 2005;12:36-43.  Back to cited text no. 9
    
10.
Morandeira A, Prieto J, Poves I, Sánchez Cano JJ, Díaz C, Baeta E. Giant retroperitoneal sarcoma. Can J Surg 2008;51:E79-80.  Back to cited text no. 10
    
11.
Hassan I, Park SZ, Donohue JH, Nagorney DM, Kay PA, Nasciemento AG, et al. Operative management of primary retroperitoneal sarcomas: A reappraisal of an institutional experience. Ann Surg 2004;239:244-50.  Back to cited text no. 11
    
12.
Ki EY, Park ST, Park JS, Hur SY. A huge retroperitoneal liposarcoma: Case report. Eur J Gynaecol Oncol 2012;33:318-20.  Back to cited text no. 12
    
13.
Gupta AK, Cohan RH, Francis IR, Sondak VK, Korobkin M. CT of recurrent retroperitoneal sarcomas. AJR Am J Roentgenol 2000;174:1025-30.  Back to cited text no. 13
    
14.
Strauss DC, Hayes AJ, Thway K, Moskovic EC, Fisher C, Thomas JM. Surgical management of primary retroperitoneal sarcoma. Br J Surg 2010;97:698-706.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed1290    
    Printed29    
    Emailed0    
    PDF Downloaded53    
    Comments [Add]    

Recommend this journal