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CASE REPORT
Year : 2021  |  Volume : 58  |  Issue : 2  |  Page : 267-272
 

Mesenteric leiomyosarcoma in a pregnant woman: A case report and review of literature


1 Surgical Oncology, Ruby Hall Clinic, Pune, Maharashtra, India
2 Medical Oncology, Ruby Hall Clinic, Pune, Maharashtra, India
3 Histopathology, Ruby Hall Clinic, Pune, Maharashtra, India

Date of Submission06-Aug-2019
Date of Decision22-Dec-2019
Date of Acceptance04-Jul-2020
Date of Web Publication27-Jan-2021

Correspondence Address:
Sanjay P Deshmukh
Surgical Oncology, Ruby Hall Clinic, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijc.IJC_705_19

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 » Abstract 


We herein describe a case of primary leiomyosarcoma of descending mesocolon mesentery in a pregnant woman. A 31-year-old woman was referred to our clinic for the presence of a suspicious mass (solid heterogenous lesion with lobulated margins) detected during routine obstetric ultrasonography (USG), growing throughout her term. Imaging in her third trimester showed a considerable increase in the size of the mass and was suspected to be malignancy of uterine origin. Tru-cut biopsy performed post-partum indicated leiomyosarcoma. She underwent neoadjuvant chemotherapy with six cycles of dacarbazine and doxorubicin with partial response. Subsequently, she underwent surgery, and the tumor was found to be present in sigmoid colon mesentery extending in retroperitoneum involving 5 cm of ureter. The mass was resected along with part of the colon and ureter that was involved by disease. Patient had uneventful recovery post-surgery. Considering moderate response to chemotherapy and discussion in tumor board, she was not given adjuvant chemotherapy. At follow-up of 15 months, the patient is disease-free with a normal, healthy baby.


Keywords: Leiomyosarcoma, mesocolon, pregnancy


How to cite this article:
Deshmukh SP, Jain M, Chharchhodawala T. Mesenteric leiomyosarcoma in a pregnant woman: A case report and review of literature. Indian J Cancer 2021;58:267-72

How to cite this URL:
Deshmukh SP, Jain M, Chharchhodawala T. Mesenteric leiomyosarcoma in a pregnant woman: A case report and review of literature. Indian J Cancer [serial online] 2021 [cited 2021 Jun 15];58:267-72. Available from: https://www.indianjcancer.com/text.asp?2021/58/2/267/308051





 » Background Top


Incidence of cancer in pregnancy is one in 1000 with most common malignancies being breast and cervical cancers, melanoma, lymphomas, and leukemias.[1],[2] Adnexal masses are also common in pregnancy and have been reported in up to 8.8% of patients, wherein most are asymptomatic and resolve spontaneously. Most which persist carry low malignant risk. Borderline epithelial ovarian cancer and germ cell tumor are common cancers seen in adnexal masses during pregnancy.[3] Soft-tissue sarcomas are rare cancers seen in pregnancy. Osteosarcoma and Ewing's sarcoma are reported in pregnant women and also are seen in women of childbearing age.[4] Soft tissue malignancies in mesentery are infrequent lesions ranging from benign cysts to aggressive malignancies and often present a diagnostic and therapeutic challenge as mesentery receives metastases from the gastrointestinal tract, pancreas, and biliary cancers etc.[5] Mesenteric leiomyosarcoma is uncommon disease which represents 10–20% of soft tissue sarcomas, is aggressive and presents with poor prognosis.[6] Imaging techniques, which usually lead to diagnosis of this malignancy, is compromised during pregnancy with symptoms masked by the fact that growth of fetus causes physiological changes in the patient's body.

The case being reported is one such rare presentation of a pregnant woman with leiomyosarcoma possibly originating in descending mesocolon. The peculiarity of this case comes from the fact that she remained undiagnosed for 5 months despite routine sonograms and blood tests as she was pregnant, and her lesions were not clearly visible and mostly regarded as “uterine fibroids”. In her routine ultrasonography (USGs) the lesions could be seen growing significantly in size, which is when the case was referred to our clinic. Post-partum imaging revealed a huge mass in the peritoneum and surrounding organs. The patient had also developed metastasis to both liver and lungs.

Case presentation

A 31-year-old woman was referred to our clinic for the presence of a solid heterogenous mass detected during routine obstetric examinations. During her first trimester of pregnancy, an adnexal mass was seen in the left parametrial area of size 8 x 10 cm. It was seen, on regular obstetric sonography, over a period of 6 months to progressively increase and to reach a size of 18 x 22 cm. The lesion was an incidental finding during routine USG, wherein it was previously believed to be an adnexal mass (fibroid) but found to be growing aggressively within a course of 4 months. There were no associated bowel or bladder complaints and no history of any long-term illness. She sometimes used to experience abdominal pain on left side with occasional back pain before and during her pregnancy. The woman was in her 3rd trimester and due to deliver in a month. Blood counts, renal function tests, and liver function tests were performed, and all the values were within normal limits except for slight anemia. A magnetic resonance imaging (MRI) was ordered to evaluate the disease and understand diagnosis, which was refused by the patient. Fetal monitoring and ultrasonography evaluations showed no fetal stress; hence the patient was advised to come for further tests immediately post-delivery. Cesarean section was planned for 15 days after the initial consultation and a healthy baby was delivered at 35 weeks. During surgery, large mass was noted near the left parametrium attached to gravid uterus.

On her post-partum abdominal examination, a soft to firm midline mass was palpable of 32 weeks size of gravid uterus and had smooth surface. The mass had restricted mobility from side to side, with no tenderness or any changes in the overlying skin. USG examination showed solid well defined non-tender mass with minimal vascularity. positron emission tomography – computed tomography (PET-CT) scan of abdomen and pelvis revealed a huge mass of standardized uptake value (SUV) 10.85 and size 21.5 cm × 15.4 cm × 18.2 cm extending superiorly up to pancreas and inferiorly up to uterus. It was seen reaching up to the anterior abdominal wall with indistinct fat planes with the anterior abdominal wall in left lumbar region and iliac fossa. The mass showed large areas of necrosis within. Enlarged preaortic, paraaortic, bilateral common iliac, external iliac nodes were seen, some of them showing fluorodeoxyglucose (FDG) avidity. An FDG avid hypodense lesion measuring 1.3 X 0.9 cm was seen in segment IV of liver [Figure 1]. Tru-cut biopsy was performed to make a definitive diagnosis. The cut section showed spindle cell tumor with intermixed myxoid areas raising the differentials of gastrointestinal stromal tumor (GIST) and leiomyosarcoma (LMS). Immunoreactivity for desmin and h-caldesmon and negative for c-kit ruled out GIST establishing the diagnosis as LMS. Origin of the LMS was believed to be uterus. After multidisciplinary assessment, we initially decided to administer 3 cycles of chemotherapy to the patient with doxorubicin (50 mg/m2, dacarbazine (150 mg/m2) with granulocyte colony stimulating factor (G-CSF) given at 3 weeks interval. After 6 cycles of chemotherapy, the lesion got regressed and the 10% reduction in size came with symptomatic relief for the patient. Three more cycles of chemotherapy were administered, following which patient was scheduled for surgery for excision of mass. A week later the patient was operated on to carry out resection. The tumor could be seen arising from the mesentery in close vicinity to descending colon as opposed to uterus as seen in PET-CT. Mass was excised in toto along with large part of colon (30 cm) and a part of ureter (5 cm). The patient was discharged 4 days later and kept on follow-up.
Figure 1: FDG positron emission tomography/CT fusion imaging revealed a large hypermetabolic heterogeneously enhancing lobulated mass in pelvis and abdomen with significantly increased FDG uptake (maximum standardized uptake value of 10.85). The lesion encased left mid ureter with proximal hydroureteronephrosis and caused displacement of small bowel loops towards right side (Shown with arrow). Mildly enlarged bilateral common iliac, external iliac, and preaortic nodes were seen with mild FDG activity

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Histopathological analysis of the mass

On gross examination, the tumor measured 24 x 36 x 12 cm and weighed 1.4 kg. Surface of the mass showed a variegated appearance with solid, cystic areas, regions of hemorrhage and large areas of necrosis. Histologically, the tumor consisted of spindle cells with eosinophilic cytoplasm and large number of bizarre giant cells, a high mitotic activity 9 mitoses per 10 per high power field, and more than 50% necrosis. The tumor infiltrated into sub-serosal fat of colon. On morphology, diagnosis of high-grade sarcoma was considered with differential diagnosis of leiomyosarcoma, GIST, malignant peripheral nerve sheath tumor (MPNST), dedifferentiated liposarcoma and pleomorphic sarcoma. Immunohistochemical findings revealed that the tumor was positive for smooth muscle actin (SMA) and desmin, and negative for CD117 (c-kit), DOG1 and S100 [Figure 2]. Due to morphological features and immunohistochemistry findings, diagnosis of high grade leiomyosarcoma was made.
Figure 2: Histological examination revealed viable tumor cells with spindle morphology, elongated cytoplasm and few bizarre tumor giant cells (a and b). On immunohistochemistry, the tumor cells stained positive for (c) α smooth muscle actin and (d) desmin, but negative for CD117 and S100 and DOG-1 (not shown)

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The patient showed good response during postoperative recovery. She was on regular follow-up for clinical and scanning surveillances for every 3 months to monitor any signs of recurrence or metastasis. At 15 months follow up, she remains free of disease with good quality of life and a healthy baby.


 » Discussion Top


Adnexal masses are common occurrences in women of any age and pregnancy is no exception.[3] A mass observed during routine USG with no associated complaints is most likely to be considered as a benign formation. A close monitoring by imaging using USG to stratify the risk of malignancy is the only method to ensure timely diagnosis. Physiological changes happening during pregnancy often mask the signs and symptoms of malignancy, making the diagnosis difficult. The patient had observed abdominal distension and discomfort, which are generally the common presenting symptoms, but had ignored them because of pregnancy. The patient was initially asymptomatic with no lesions observed in imaging in first trimester. Increase in the size of lesion along with onset of symptoms like feeling of fullness, nausea, and tiredness in her second trimester and third trimester were possible signs of aggressive growth.

The present case demonstrates a diagnostic challenge, where malignancy initially considered to be adnexal mass, coexisted with the fetus, grew aggressively throughout the term. Pregnancy-associated cancers are always difficult to manage as malignancy influences therapeutic approaches. Standard of care for the underlying disease has to be taken into account while considering the fetal safety. In the case discussed, the woman presented in her last trimester due to deliver in a month's time. Careful analysis envisaged no immediate harm to fetus and a preponed delivery via caesarean section ensured timely treatment for the patient with no harm to the neonate. Most commonly sarcomas are diagnosed in third trimester of pregnancy and present in advanced stage of cancer. As demonstrated in a case series of sarcomas, all the presenting patients were in their third trimester wherein 3 women with more than 32 weeks were delivered before treatment and five patients in early part of third trimester received doxorubicin based chemotherapy during pregnancy.[4] What makes the present case more intriguing, is the fact that the patient had a malignancy in mesenteric cavity which in itself poses a diagnostic and therapeutic challenge.

Most of the mesenteric leiomyosarcomas tend to be asymptomatic and grow to a large size by the time of diagnosis since the mobility and elasticity of the mesentery allow the tumor to occupy a large intraperitoneal space without causing any obstructive symptoms.[7] Owing to the fact that tumors in mesentery are difficult to diagnose and have similarity to GIST, lipoma, liposarcoma etc. it becomes very important to understand the differentials to achieve best possible outcome. As mentioned in [Table 1], there have been cases of mesenteric leiomyosarcomas with distinct histopathological signatures.[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] In the absence of clear guidelines, the line of treatment varies from case to case, surgery being the cornerstone of treatment and only therapeutic modality proven to provide cure.[14] Complete surgical resection is critical to achieve the best outcome but is sometimes difficult due to large tumor size. For advanced metastatic disease, surgery should not be the only treatment of choice. Chemotherapy can be considered in selected cases where the numbers of metastatic foci are amenable to intervention. Our patient showed good response to chemotherapy as shown by decrease in liver and lung lesions, indicating that micro-metastases may have been eradicated by the chemotherapy.
Table 1: Clinical and histopathological characteristics of mesenteric leiomyosarcoma

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The prognosis of leiomyosarcoma remains poor and standardized chemotherapy for this rare disease has not yet been established. Early diagnosis and surgical removal of the tumor is the only potentially curative option for leiomyosarcomas in mesentery. It is important to accumulate evidence of such cases in order to develop guidelines for management and enabling early diagnosis of such cases. Interestingly, frequent USG imaging in the pregnancy provided a boon in this case, but effective diagnosis needs to be established to improve outcomes. Clinicopathological and literature review of this rare leiomyosarcoma of mesocolon has been discussed in our case presentation.

We consider that the management of such malignancies during the third trimester of pregnancy should include an adapted chemotherapy regimen tailored to the pharmacological specificities of pregnant patients. The diagnostic approach should be tailored specifically in every pregnant woman in whom sarcoma is suspected.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names 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.
Zagouri F, Dimitrakakis C, Marinopoulos S, Tsigginou A, Dimopoulos MA. Cancer in pregnancy: Disentangling treatment modalities. ESMO Open 2016;1:e000016.  Back to cited text no. 1
    
2.
Hepner A, Negrini D, Hase EA, Exman P, Testa L, Trinconi AF, et al. Cancer during pregnancy: The oncologist overview. World J Oncol 2019;10:28-34.  Back to cited text no. 2
    
3.
Cavaco-Gomes J, Jorge Moreira C, Rocha A, Mota R, Paiva V, Costa A. Investigation and management of adnexal masses in pregnancy. Scientifica 2016;2016;3012802.   Back to cited text no. 3
    
4.
Mir O, Berrada N, Domont J, Cioffi A, Boulet Be, Terrier P, et al. Doxorubicin and ifosfamide for high-grade sarcoma during pregnancy. Cancer Chemother Pharmacol 2012;69:357–67.  Back to cited text no. 4
    
5.
Weiss SW, Goldblum JR. Leiomyosarcoma. In: Weiss SW, Goldblum JR, editors. Enzinger and Weiss's Soft Tissue Tumors. 6th ed. Philadelphia; 2013. p. 769-88.  Back to cited text no. 5
    
6.
Serrano C, George S. Leiomyosarcoma. Hematol Oncol Clin North Am 2013;27:957–74.  Back to cited text no. 6
    
7.
Harada N, Shimada Y, Imamura M, Yamabe H, Furuyama H, Fujisawa I. Huge leiomyosarcoma of the mesentry: A case report. Nihon Geka Hokan 1994;63:155-60.  Back to cited text no. 7
    
8.
Iwasaki M, Kitaguchi K, Kobayashi H. Mesenteric leiomyosarcoma in a 13-year-old boy. J Pediatr Surg 2010;45:1893-5.  Back to cited text no. 8
    
9.
Hamai Y, Hihara J, Emi M, Aoki Y, Kushitani K, Tanabe K, et al. Leiomyosarcoma of the sigmoid colon with multiple liver metastases and gastric cancer: A case report. BMC Gastroenterol 2012;12:98.  Back to cited text no. 9
    
10.
Koczkowska M, Lipska BS, Grzeszewska J, Limoni J, Biernat W, Jassesm J. Primary leiomyosarcoma of the mesentery in two sisters: Clinical and molecular characteristics. Pol J Pathol 2013;64:5963.  Back to cited text no. 10
    
11.
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. 11
    
12.
Ilias AS, Yaacob H, Wan Zain WZ, Zakaria AD. Rare case of primary leiomyosarcoma of sigmoid mesocolon. BMJ Case 2017;2017:bcr2017219826.  Back to cited text no. 12
    
13.
Kluger Y, Ben-Ishay O. Leiomyosarcoma of the mesenteric root: A strategic location of a rare tumor. Case Rep Oncol 2015;8:359-62.  Back to cited text no. 13
    
14.
Varghese M, Bruland O, Wiedswang AM, Lobmaier I, Rosok B, Benjamin RS, et al. Metastatic mesenteric dedifferentiated leiomyosarcoma: A case report and a review of literature. Clin Sarcoma Res 2016;6:2.  Back to cited text no. 14
    
15.
Dasgupta S, Chakrabarti S, Ghosh S, Das S. Ileal Mesenteric leiomyosarcoma-Report of a rare neoplasm. J Gastrointest Cancer 2016;47:114-7.  Back to cited text no. 15
    
16.
Dalal V, Kaur M, Menia R, Siraj F, Bhatnagar A, Agrawal U. Primary leiomyosarcoma of the mesentery: A case report. Iran J Med Sci 2017;42:505–8.  Back to cited text no. 16
    
17.
Garg R, Al Rajjal A, Berri R, Barawi M. Primary gastric leiomyosarcoma: A case report and review of the literature. J Gastrointest Canc 2020;51:335–40.  Back to cited text no. 17
    
18.
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;5:53-56.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2]
 
 
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