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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 256-258
Leiomyosarcoma of inferior vena cava with intracardiac extension presenting as Budd-Chiari syndrome: Report of a rare case

Department of Pathology, Postgraduate Institute of Medical Education and Research, Dr. Ram Manohar Lohia Hospital, New Delhi, India

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Date of Web Publication19-Jun-2017


Primary leiomyosarcoma of vascular origin is a rare malignant smooth muscle tumor. This report describes a case of primary leiomyosarcoma of the inferior vena cava (IVC) in a 52-year-old male who presented with complaints of abdominal pain, chest pain, and bilateral lower limb swelling for 4 months. Imaging of the chest and abdomen revealed a mass in the IVC extending into right atrium, heterogeneous enhancement of liver, hepatosplenomegaly, and ascites. Histopathological examination showed a malignant spindle cell tumor with cells arranged in fascicular and hemangiopericytomatous pattern. Immunohistochemistry for smooth muscle actin and desmin confirmed smooth muscle origin of the tumor. Recognition of this tumor in this rare location and Budd-Chiari syndrome-like presentation is imperative for proper management of the patient.

Keywords: Budd-Chiari syndrome, inferior vena cava, leiomyosarcoma, vascular tumor

How to cite this article:
Nambiyar K, Ahuja A, Bhardwaj M. Leiomyosarcoma of inferior vena cava with intracardiac extension presenting as Budd-Chiari syndrome: Report of a rare case. Indian J Pathol Microbiol 2017;60:256-8

How to cite this URL:
Nambiyar K, Ahuja A, Bhardwaj M. Leiomyosarcoma of inferior vena cava with intracardiac extension presenting as Budd-Chiari syndrome: Report of a rare case. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 Jan 27];60:256-8. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/256/208412

   Introduction Top

Leiomyosarcoma is a malignant tumor of smooth muscle origin and accounts for <1% of all malignant vascular tumors.[1] Primary leiomyosarcoma of vascular origin is very rare, and it is most commonly seen in the inferior vena cava (IVC).[1] The diagnosis is usually delayed due to nonspecific complaints by the patients, and in spite of modern imaging techniques, preoperative diagnosis is difficult to establish. We report this rare case in a patient who presented with nonspecific complaints and sign's like Budd-Chiari syndrome on clinical examination. Later, on histopathological examination, it turned out to be unusual presentation of IVC leiomyosarcoma.

   Case Report Top

A 52-year-old male was admitted with complaints of abdominal pain, chest pain, and bilateral lower limb swelling for 4 months. On examination, he had bilateral pedal edema, ascites, and hepatosplenomegaly. Routine blood and urine examinations were normal. Contrast-enhanced computed tomography abdomen and cardiac magnetic resonance imaging revealed a mass in IVC extending into right atrium (RA) measuring approximately 3.75 cm × 5.3 cm with markedly distended IVC [Figure 1]. There was heterogeneous enhancement of liver without any mass lesion along with hepatosplenomegaly and ascites suggestive of hepatic venous congestion. He was taken for surgery and intraoperatively a mass completely filling IVC and most of the RA was found. The mass was adherent to the IVC and RA junction. Due to the high location of the tumor, the surgeon was unable to do complete resection. Debulking of the tumor was done and the mass was sent for histopathological examination.
Figure 1: Cardiac magnetic resonance imaging showing large lobulated mass lesion approximately 3.75 cm × 5.3 cm with epicenter in the right atrium, extending into inferior vena cava (arrow) and markedly distending its caliber

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On gross, multiple irregular, gray white mass ranging from 0.5 cm to 7 cm × 6 cm × 3.5 cm was received. Cross-section of mass was gray white to greenish in color with cleft-like spaces filled with mucinous to hemorrhagic material.

Histopathological examination of the mass showed a cellular tumor composed of spindle cells arranged in fascicular and hemangiopericytomatous pattern [Figure 2]. The tumor cell nuclei were elongated, blunt ended with coarse chromatin, prominent nucleoli, and exhibited abundant eosinophilic cytoplasm with ill-defined margins. Focal pleomorphism, few bizarre giant cells, and 4–5 mitosis/50 high-power field were also seen [Figure 2] inset]. Focal areas showed hyalinization and necrosis. Histopathological grading of the tumor according to the Federation Nationale des Centres de Lutte Contre le Cancer grading system was Grade 1. On immunohistochemical staining, tumor cells were diffusely positive for smooth muscle antigen and focally positive for desmin [Figure 3]. Based on characteristic morphological and immunohistochemical features, a diagnosis of primary leiomyosarcoma of IVC was made.
Figure 2: Photomicrograph showing fascicles of spindle cells

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Figure 3: (a) Smooth muscle antigen immunopositivity in spindle cells, (b) Desmin immunopositivity in spindle cells (IHC, Original magnification ×200)

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   Discussion Top

Leiomyosarcoma originating in IVC is a rare, malignant, slow-growing tumor with poor prognosis.[2] Leiomyosarcoma of IVC was first described by Perl in 1871.[1] To the best of our knowledge, only over 200 cases of leiomyosarcoma of IVC have been reported in English literature and International Registry so far.[1] IVC leiomyosarcoma is most frequently seen in the sixth decade with a female predominance.[3] The diagnosis is often delayed as the patients usually present with nonspecific complaints such as dyspnea, abdominal pain, distention, back pain, weight loss, or malaise. Tumor location is important as it determines the presenting symptoms, surgical resectability, and prognosis. According to its location, the tumor is classified into segment I (below renal vein), segment II (between hepatic vein and renal vein), and segment III (above hepatic vein). Most tumors arise in segment II and III which constitute about 50.8% and 44.2%, respectively. Segment III constitutes only 4.2% of tumor.[4] Growth pattern of the tumor varies from intraluminal to extraluminal or both. About 62% of cases have extraluminal, 33% have both, and only 5% of cases have intraluminal growth pattern.[4]

In our case, the patient had suprahepatic tumor with intracardiac extension; therefore, he had features of a Budd-Chiari syndrome in the form of passive venous congestion, hepatomegaly, and ascites. The likelihood of the origin of tumor from RA and its progression into IVC is unlikely because the blood flow is toward RA. In one of the largest series of leiomyosarcoma of IVC by Kieffer et al., only two patients of 22 cases presented with Budd-Chiari syndrome and intracardiac extension of the tumor.[5]

The diagnosis is often challenging due to rarity of the tumor and nonspecific complaints. Five and 10 years survival rates are 49.4% and 29.5%, respectively, reflecting the aggressive nature of these tumors.[5] The optimal treatment for IVC leiomyosarcoma is still not known as few cases have been reported. Combined use of imaging modalities along with early excision and histopathological examination is required for the diagnosis of this tumor in this rare location, thereby helping in adequate management of the patient. Complete wide resection of the tumor with adjunctive radiotherapy or chemotherapy is the most preferred treatment.[5]

This case was quite rare and unusual with respect to sex (male), location (suprahepatic) of tumor with intracardiac extension, Budd-Chiari syndrome-like presentation, and intraluminal tumor growth pattern.

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There are no conflicts of interest.

   References Top

Jenkins S, Marshall GB, Gray R. Leiomyosarcoma of the inferior vena cava. Can J Surg 2005;48:252-3.  Back to cited text no. 1
Ueda J, Yoshida H, Mamada Y, Taniai N, Yoshioka M, Kawano Y, et al. Surgical resection of a leiomyosarcoma of the inferior vena cava mimicking hepatic tumor. Case Rep Med 2013;2013:235698.  Back to cited text no. 2
Hemant D, Krantikumar R, Amita J, Chawla A, Ranjeet N. Primary leiomyosarcoma of inferior vena cava, a rare entity: Imaging features. Australas Radiol 2001;45:448-51.  Back to cited text no. 3
Huang J, Liu Q, Lu JP, Wang F, Wang L, Jin AG. Primary intraluminal leiomyosarcoma of the inferior vena cava: Value of MRI with contrast-enhanced MR venography in diagnosis and treatment. Abdom Imaging 2011;36:337-41.  Back to cited text no. 4
Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L. Leiomyosarcoma of the inferior vena cava: Experience in 22 cases. Ann Surg 2006;244:289-95.  Back to cited text no. 5

Correspondence Address:
Arvind Ahuja
Department of Pathology, Postgraduate Institute of Medical Education and Research, Dr. RML Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_782_15

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