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Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 686-688
Inferior vena cava leiomyosarcoma with liver metastasis at presentation in a young male: A challenging diagnostic quandary

1 Department of Pathology, National Cancer Institute, Nagpur, Maharashtra, India
2 Department of Radiology, National Cancer Institute, Nagpur, Maharashtra, India

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Date of Submission21-Mar-2021
Date of Decision16-Nov-2021
Date of Acceptance22-Nov-2021
Date of Web Publication21-Jul-2022


Leiomyosarcomas of vascular origin are very rare tumors, predominantly affecting the inferior vena cava (IVC). Although vascular leiomyosarcomas are slow-growing, their non-specific and late presentation results in delayed diagnosis which portends a very poor prognosis. Here we report a case of a 24-year-old man who presented with abdominal pain since 15 days and was found to have unresectable metastatic leiomyosarcoma of the inferior vena cava at initial diagnosis.

Keywords: Leiomyosarcoma, leiomyosarcoma of the inferior vena cava, sarcoma

How to cite this article:
Devi Y, Pangarkar M, Pagey R, Juvekar SL. Inferior vena cava leiomyosarcoma with liver metastasis at presentation in a young male: A challenging diagnostic quandary. Indian J Pathol Microbiol 2022;65:686-8

How to cite this URL:
Devi Y, Pangarkar M, Pagey R, Juvekar SL. Inferior vena cava leiomyosarcoma with liver metastasis at presentation in a young male: A challenging diagnostic quandary. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Aug 15];65:686-8. Available from: https://www.ijpmonline.org/text.asp?2022/65/3/686/351598

   Introduction Top

Sarcomas are rare malignant tumors of mesenchymal origin that comprise approximately 1% of all adult malignancies. Leiomyosarcomas (LMS) are sarcomas arising from smooth muscle cells and can originate at various locations. Vascular leiomyosarcomas account for 2% of all LMS and are difficult to diagnose due to its non-specific symptoms. Treatment includes complete surgical resection with or without chemotherapy and radiotherapy. However, prognosis for vascular leiomyosarcomas are poor and outcomes are quite dismal.

   Case Report Top

A 24-year-old man presented to our hospital with complaints of pain in the abdomen since 15 days which was insidious in onset and gradually progressive. The patient also complained of weight loss and generalized weakness. He had a past medical history of renal calculi for which he had received treatment one year back. The patient had no co-morbidities and no significant family history.

Physical examination did not reveal significant findings. Hematological investigations were normal with Carcino Embryonic Antigen (CEA) level 0.91 ng/ml (normal range, 0-5.00 ng/ml) and CA 19.9 level 6.50 U/ml (normal range, 0-37 U/ml). Liver function tests were deranged with elevated SGOT (1481 U/L), SGPT (1561 U/L) and total bilirubin (1.8 mg/dl). Serum alkaline phosphatase was within normal range (23 U/L). Serum creatinine was slightly elevated (1.59 mg/dL).

Contrast-enhanced computed tomography (CECT) of abdomen and pelvis revealed IVC to be dilated and shows a heterogeneously enhancing filling defect measuring 16 cm × 5 cm × 5 cm which is seen extending superiorly up to the level of right atrium and inferiorly up to the renal level [Figure 1]. A large conglomerating nodal mass in the aortocaval region was also seen which was inseparable from the primary tumor. Few discrete para-aortic, gastrohepatic and peri-portal lymph nodes were seen suggestive of metastasis. Multiple peripherally enhancing soft tissue lesions in both lobes of liver were also noted consistent with metastasis. CECT was reported as large IVC tumor thrombus with large aorto-caval nodal mass and metastatic lesions in liver.
Figure 1: CECT of abdomen and pelvis shows dilated IVC with a heterogenously enhancing filling defect measuring 16 × 5 × 5 cm extending superiorly upto the level of right atrium and inferiorly upto the renal level (a). Peripherally enhancing soft tissue lesions in liver consistent with metastasis (b)

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The differential diagnoses for a solid retroperitoneal mass in an adult male without its origin from adjacent solid organs (kidney, adrenal, pancreas and bowel) are lymphoproliferative disorder, germ cell tumor, and mesenchymal tumors.

The patient underwent CT-guided fine-needle aspiration cytology (FNAC) and biopsy from the hepatic lesion. FNA cytosmears were paucicellular and showed only an occasional group of benign hepatocytes. Cytology was reported as inadequate for opinion. Histopathological examination of core needle biopsy showed multiple linear cores of hepatic parenchyma infiltrated by poorly differentiated malignant cells showing spindle cell morphology. There was extensive nuclear pleomorphism with tumor cells showing multilobated nuclei. Stroma was edematous and showed myxoid change at places. Frequent mitotic figures were noted but there was no necrosis. Histopathology was reported as metastatic poorly differentiated malignancy with spindle cell morphology [Figure 2].
Figure 2: Histopathological examination showed multiple linear cores of hepatic parenchyma infiltrated by poorly differentiated malignant cells showing spindle cell morphology (a) with extensive nuclear pleomorphism and multilobated nuclei. Frequent mitotic figures noted but necrosis is not seen (b)

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Based on the radiologic and microscopic evaluation, the diagnosis of a malignant mesenchymal tumor was considered. Immunohistochemistry (IHC) was done which showed that the tumor cells were strongly and diffusely immunoreactive to Desmin, Smooth muscle actin (SMA), H- caldesmon while were immunonegative to Myo-D1, CD-31, CD-34, CK 7, CK 20 and Heppar-1 [Figure 3]. IHC was reported as leiomyosarcoma metastatic to liver.
Figure 3: Immunohistochemistry showed diffuse cytoplasmic immunoreactivity to Desmin (a), SMA (b), H- caldesmon (c) while were immunonegative to Myo-D1 (d), CD-34 (e) and Heppar-1 (f)

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Due to multiple metastatic lesions in liver at diagnosis, the patient was considered inoperable. Unfortunately, the patient's clinical condition deteriorated rapidly and he expired within a week of diagnosis.

   Discussion Top

Sarcomas are rare malignant tumors of mesenchymal origin that comprise about 1% of all adult malignancies.[1] Leiomyosarcomas are of smooth muscle cell origin and vascular leiomyosarcomas account for 1-2% of all LMS, rarely affecting the Inferior vena cava (IVC).[2] Vascular LMS are usually seen in the sixth decade of life with females being more commonly affected (M:F = 1:4).[3] LMS of vascular origin forms in the tunica media and its growth pattern can be extaluminal, intraluminal or combined.

LMS can be divided into three types based on the segment of IVC affected. Lower segment involves the infra-renal area (34%), middle segment involves the region between hepatic and renal veins (42%) and upper segment involves the region from the hepatic vein to the right atrium (24%).[4] The tumor can involve more than one segment, like our case which involved the upper and middle segment and such large tumors are usually unresectable due to its extent and invasion into adjacent organs.

The most common presentation of IVC leiomyosarcoma is pain or discomfort in the abdomen. Other symptoms include lower extremity edema, weight loss and dyspnea.[5] Due to its non-specific symptoms, diagnosis is often delayed. Distant metastasis occurs in about 50% of the cases usually in the late stages involving liver, lung, lymph nodes or bone.

Computed tomography (CT) and magnetic resonance imaging (MRI) is the imaging modalities of choice that aid in the diagnosis determines the extent of disease and potential for surgical resectability.

Complete surgical resection is currently the only potential curative treatment.[6] Despite complete surgical resection, local recurrences are quite common. Surgical options available include complete resection and placement of graft, partial resection and cavoplasty and ligation of IVC. The five-year survival rate is 53% in leiomyosarcoma of IVC.[7] The efficacy of chemotherapy and radiotherapy is limited.[8] For patients who have an unresectable tumor, prognosis is very poor and survival time is measured in months as was the condition in our case. Involvement of upper segment IVC, right atrium, intraluminal growth and residual post-surgical macroscopic disease are adverse prognostic factors that result in early death, usually within 2 years.[9]

   Conclusion Top

We have reported this case due to its unusual presentation in a male patient at a very young age who had metastatic disease at presentation. As surgical resection was not possible, these tumors portend a very poor prognosis. The role of chemotherapy and radiotherapy in IVC leiomyosarcoma is for palliation alone and treatment modalities for such inoperable patients' needs to be evaluated further.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Zavareh RH, Beni HR, Iranpour A, Samimi MA, Sadeghipour A, Alavi Niakou SN. Leiomyosarcoma of inferior vena cava and right atrium with ascites and jaundice: A case report. Int J Hematol Oncol Stem Cell Res 2016;10:232-5.  Back to cited text no. 1
Cortecero JM, Guirau Rubio MD, Roma AP. Leiomyosarcoma of the inferior vena cava: AIRP best cases in radiologic-pathologic correlation. RadioGraphics 2015;35:616-20.  Back to cited text no. 2
Kapoor R, Bansal A, Sharma SC. Leiomyosarcoma of inferior vena cava: Case series of four patients. J Cancer Res Ther 2015;11:650.  Back to cited text no. 3
Ceyhan M, Danaci M, Elmali M, Ozmen Z. Leiomyosarcoma of the inferior vena cava. Diagn Interv Radiol 2007;13:140-3.  Back to cited text no. 4
Fujita S, Takahashi H, Kanzaki Y, Fujisaka T, Takeda Y, Ozawa H, et al. Primary leiomyosarcoma in the inferior vena cava extended to the right atrium: A case report and review of the literature. Case Rep Oncol 2016;9:599-609.  Back to cited text no. 5
Tsuchiya Y, Ito T, Sakurada M, Kushida T, Orita H, Maekawa H, et al. A case of giant leiomyosarcoma of the inferior vena cava with liver metastases: A surgical challenge. J Case Rep Images Surg 2016;2:76-9.  Back to cited text no. 6
Hines OJ, Nelson S, Quinones-Baldrich WJ, Eilber FR. Leiomyosarcoma of the inferior vena cava: Prognosis and comparison with leiomyosarcoma of other anatomic sites. Cancer 1999;85:1077-83.  Back to cited text no. 7
Ito H, Hornick JL, Bertagnolli MM, George S, Morgan JA, Baldini EH, et al. Leiomyosarcoma of the inferior vena cava: Survival after aggressive management. Ann Surg Oncol 2007;14:3534-41.  Back to cited text no. 8
Laskin WB, Fanburg-Smith JC, Burke AP, Kraszewska E, Fetsch JF, Miettinen M. Leiomyosarcoma of the inferior vena cava: Clinicopathologic study of 40 cases. Am J Surg Pathol 2010;34:873-81.  Back to cited text no. 9

Correspondence Address:
Yogita Devi
36, Ganguly Layout, Jaiprakashnagar, Somalwada, Nagpur - 440 025, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_296_21

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