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CASE REPORT
Year : 2021  |  Volume : 54  |  Issue : 5  |  Page : 196-199

Primary pleomorphic leiomyosarcoma of descending mesocolon


Department of Surgical Oncology, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India

Date of Submission21-Oct-2020
Date of Decision22-Apr-2021
Date of Acceptance02-Aug-2021
Date of Web Publication12-Oct-2021

Correspondence Address:
Suhaildeen Kajamohideen
Department of Surgical Oncology, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_196_20

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  Abstract 


Mesenteric tumors range from benign cysts to aggressive malignancies and pose both diagnostic and therapeutic challenge. Leiomyosarcomas are rare tumors of the digestive tract comprising <0.1% of colorectal tumors. Similarities between mesenchymal tumors of the digestive tract make histological diagnosis difficult. We report a case of 53-year-old female diagnosed with leiomyosarcoma of descending mesocolon, who has undergone surgery following neoadjuvant chemotherapy and has been on regular follow-up for 2 years. This case signifies the diagnostic difficulties encountered in these rare tumors with a review of the literature.

Keywords: Gastrointestinal, mesenchymal tumor, mesocolon, leiomyosarcoma


How to cite this article:
Kajamohideen S, Venkitaraman B, Shivkumaran SM, Premkumar P. Primary pleomorphic leiomyosarcoma of descending mesocolon. Formos J Surg 2021;54:196-9

How to cite this URL:
Kajamohideen S, Venkitaraman B, Shivkumaran SM, Premkumar P. Primary pleomorphic leiomyosarcoma of descending mesocolon. Formos J Surg [serial online] 2021 [cited 2021 Nov 28];54:196-9. Available from: https://www.e-fjs.org/text.asp?2021/54/5/196/327883




  Introduction Top


Leiomyosarcomas are aggressive tumors of smooth muscle cells commonly seen in uterine, gastrointestinal and soft tissue origin. Differentiation between leiomyosarcoma from gastrointestinal stromal tumors (GISTs) is only possible after the advent of immunohistochemistry (IHC) and many GIST in the past may have been mistakenly labeled as leiomyosarcoma. Primary leiomyosarcoma of mesentery and mesocolon is rare tumors with aggressive behavior. Origin from smooth muscles of the blood vessels has been suggested. Common sites are ileal mesentery followed by transverse and sigmoid mesocolon. The occurrence of primary mesocolic leiomyosarcoma is extremely rare and very few cases have been reported.


  Case Report Top


This patient, a 53-year-old female with no comorbid illness, was evaluated for lower abdominal pain and abdominal mass. A diagnosis of a retroperitoneal tumor possibly a pedunculated fibroid was made at another hospital. She was taken in for surgery. Intraoperatively, the tumor was found in retroperitoneal plane at the left lumbar region infiltrating the descending and sigmoid colon. Hence, the tumor was deemed in-operable and the patient was referred to our institute.

Preoperative images were reviewed and showed a lobulated heterogeneously enhanced mass in the left abdominopelvic region, displacing the small bowel loops superiorly, suspicious involvement of descending colon [Figure 1]. Trucut biopsy of the tumor showed atypical pleomorphic cells with many giant cells and mitosis of 12/10 hpf [Figure 2]. IHC staining was positive for vimentin, smooth muscle actin, focally positive for desmin, negative for S-100, CD117 and DOG-1, and Ki-67 of 20%, suggestive of a leiomyosarcoma [Figure 2]. The patient received three cycles of neoadjuvant chemotherapy with gemcitabine and paclitaxel. Reassessment with positron emission tomography computed tomography scan showed stable disease without signs of distant metastasis [Figure 1].
Figure 1: (a and b) Prechemotherapy computed tomography scan of the abdomen showing retroperitoneal mass displacing small bowel loops superiorly, and with suspicious involvement of the descending colon. (c and d) Positron emission tomography computed tomography showing residual soft tissue density in the left abdominopelvic region

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Figure 2: (a) H and E, ×400, Multinucleated pleomorphic cells (arrows), with atypical mitosis (arrowhead). (b) Immunohistochemistry for smooth muscle actin ×400, cells showing cytoplasmic positivity for smooth muscle actin

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On laparotomy, the mass was found within the descending mesocolon, encasing the vessels with restricted mobility and feeder vessels from the left gonadal vessels. An extended left hemicolectomy was done and colorectal continuity was restored. The patient had an uneventful postoperative recovery.

Postoperative histopathological examination showed an 11.2 cm × 9.4 cm globular encapsulated mass. Cut surface showed a solid gray white mass with gray-black areas, pleomorphic grade III leiomyosarcoma with 40% necrosis. Margins were free.

The patient completed six cycles of chemotherapy postoperatively followed by adjuvant radiation and is disease-free on year-2 of regular follow-up.


  Discussion Top


Primary tumors arising from mesentery and mesocolon are rare. The most common tumors are lymphoma followed by mesenchymal tumors. Majority of the mesenchymal tumors are benign. Mesenchymal malignant tumors arise from stromal tumors such as leiomyosarcoma, liposarcoma, fibrosarcoma, and undifferentiated sarcomas. In our case, the biopsy was done preoperatively as it had been deemed inoperable elsewhere.

Mesenteric leiomyosarcoma in an adult was firstly described by Yannopoulos and Stout et al.[1] These tumors are seen in the middle age group with a female predisposition. The proliferation of smooth muscle in response to estrogen has been suggested for this predisposition. They frequently present with abdominal mass or distension followed by pain. Infrequent presentations are abscess, intraperitoneal hemorrhage, or ileus. Most of the tumors attain a large size before becoming symptomatic since the mobility and elasticity of the mesentery allow tumors to occupy a large intra-peritoneal space without causing any obstructive symptoms. The sizes of the reported cases of mesocolic leiomyosarcoma range between 14 and 20 cm [Table 1].
Table 1: Reported cases of mesocolic leiomyosarcoma (n=8, included this case)

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Since 1998, when Hirota et al. demonstrated the expression of c-KIT proto-oncogene in GIST. IHC study is essential for the diagnosis of leiomyosarcoma and to differentiate it from GIST.[9] Before 1998, several GIST haves been misclassified as leiomyosarcoma, and thus overestimating the number of cases of leiomyosarcoma.

Ranchod and Kempson showed the frequency of mitoses to be the most useful indicator of malignant potential.[10] Other prognostic factors include the size and grade of the lesion. The overall prognosis of these tumors is dismal, with 5-year survival between 20% and 30%.[11] Complete primary surgical resection with negative margins is critical for achieving the best outcome.[11]

This patient received neoadjuvant chemotherapy, as she was deemed inoperable and received gemcitabine and docetaxol. The use of neoadjuvant chemotherapy is not well established in leiomyosarcoma. These drugs are used as an extrapolation of the efficacy of these drugs in uterine leiomyosarcoma and soft tissue sarcoma by the French sarcoma study group.[12],[13],[14] Neoadjuvant chemotherapy is aimed to improve negative resection margin and determine the response of tumor to chemotherapy. There is high incidence of metastatic disease, as seen in five out of eight patients in our review of mesocolon leiomyosarcoma and adjuvant chemotherapy may be considered for added benefit. The role of adjuvant radiation after complete resection is of limited value and is considered in selected cases targeting defined anatomical boundaries presumed to be at higher risk, thereby delaying any local recurrence.

Guidelines for adjuvant management of mesocolic or mesenteric leiomyosarcoma are unclear, probably due to the small number of cases reported.


  Conclusion Top


Mesocolic tumors pose a diagnostic and therapeutic challenge. Although surgery is the main modality of treatment, recurrences are frequent. Early detection and surgical resection will improve the prognosis of this type of lesion.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, patient has given consent for her images and other clinical information to be reported in the journal. The patient has given her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Yannopoulos K, Stout AP. Primary solid tumors of the mesentery. Cancer 1963;16:914-27.  Back to cited text no. 1
    
2.
Agarwal K, Nangia A, Bajaj P, Niveditha SR. Leiomyosarcoma of the mesocolon–A case report. Indian J Pathol Microbiol 2000;43:467-9.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Fukunaga M. Neuron-specific enolase-producing leiomyosarcoma of the mesentery. APMIS 2004;112:105-8.  Back to cited text no. 3
    
4.
Koczkowska M, Lipska BS, Grzeszewska J, Limon J, Biernat W, Jassem J. Primary leiomyosarcoma of the mesentery in two sisters: Clinical and molecular characteristics. Pol J Pathol 2013;64:59-63.  Back to cited text no. 4
    
5.
Mizobe T, Akagi Y, Ishikawa H, Shiratsuchi I, Oka Y, Kinugasa T, et al. Gemcitabine with paclitaxel therapy against mesocolic leiomyosarcoma: A case report. Anticancer Res 2013;33:2929-33.  Back to cited text no. 5
    
6.
Jai SR, Farah RH, Hamdaoui B, Boufettal R, Chehab F. Pleomorphous leiomyosarcoma of the mesocolon: A case report. Pan Afr Med J 2015;22:322.  Back to cited text no. 6
    
7.
Kato T, Noda H, Abe I, Alonso S, Yokoyama N, Rikiyama T. Curative resection for leiomyosarcoma of the descending mesocolon with metachronous liver metastasis: A case report and literature review. Mol Clin Oncol 2016;5:53-6.  Back to cited text no. 7
    
8.
Ilias AS, Yaacob H, Wan Zain WZ, Zakaria AD. Rare case of primary leiomyosarcoma of sigmoid mesocolon. BMJ Case Rep 2017;2017:r-219826.  Back to cited text no. 8
    
9.
Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998;279:577-80.  Back to cited text no. 9
    
10.
Ranchod M, Kempson RL. Smooth muscle tumors of the gastrointestinal tract and retroperitoneum: A pathologic analysis of 100 cases. Cancer 1977;39:255-62.  Back to cited text no. 10
    
11.
Sidhic AK, Ranjith M, Ali KP, Tej PR. Leiomyosarcoma of the mesentry, a rare mesentric tumour. Int J Surg Case Rep 2015;7C: 58-60.  Back to cited text no. 11
    
12.
Lee HY, Shin SJ, Kim HS, Hong SJ, Han JW, Lim ST, et al. Weekly gemcitabine and docetaxel in refractory soft tissue sarcoma: A retrospective analysis. Cancer Res Treat 2012;44:43-9.  Back to cited text no. 12
    
13.
Pautier P, Floquet A, Penel N, Piperno-Neumann S, Isambert N, Rey A, et al. Randomized multicenter and stratified phase II study of gemcitabine alone versus gemcitabine and docetaxel in patients with metastatic or relapsed leiomyosarcomas: A Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) French Sarcoma Group Study (TAXOGEM study). Oncologist 2012;17:1213-20.  Back to cited text no. 13
    
14.
Gallup DG, Blessing JA, Andersen W, Morgan MA; Gynecologic Oncology Group Study. Evaluation of paclitaxel in previously treated leiomyosarcoma of the uterus: A gynecologic oncology group study. Gynecol Oncol 2003;89:48-51.  Back to cited text no. 14
    


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