Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 4  |  Page : 315-319

A rare case of primary leiomyosarcoma of the inferior vena cava with an intra- and extravascular component


1 Department of Vascular and Endovascular Surgery, Yashoda Hospital, Somajiguda, Hyderabad, Telangana, India
2 Department of G.I Surgery, Yashoda Hospital, Somajiguda, Hyderabad, Telangana, India

Date of Submission21-Mar-2019
Date of Acceptance21-Oct-2019
Date of Web Publication20-Dec-2019

Correspondence Address:
Dr. Devender Singh
Department of Vascular and Endovascular Surgery, Yashoda Hospital, Somajiguda, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_18_19

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  Abstract 


Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare vascular tumor. It is frequently seen in the sixth decade of life with a female predominance. The diagnosis is often challenging as patients present with nonspecific complaints such as dyspnea, abdominal discomfort, or back pain. Computed tomography (CT) and magnetic resonance imaging individually or in combination with cavography, ultrasonography, and echocardiography allow an early preoperative diagnosis. Herein, we present a rare case report of primary leiomyosarcoma of the IVC in a 65-year-old female patient who presented with vague abdominal discomfort. Ultrasound of the abdomen detected a mass in the retroperitoneum with mixed echogenicity. CT scan revealed a 10.5 cm × 8.5 cm × 6.3 cm lobulated, heterogeneously enhancing mass with areas of necrosis and involving IVC. The diagnosis of a retroperitoneal mass involving IVC with a possibility of an IVC tumor was made. En bloc resection of the tumor with primary closure of the IVC was performed after careful dissecting from the surrounding structures. Specimen grossly revealed a soft pedunculated tissue mass projecting into the IVC and a large lobulated mass of 13 cm × 12 cm × 10 cm adherent to the surrounding structures. Histopathological report confirmed the diagnosis of primary leiomyosarcoma of the IVC.

Keywords: Inferior vena cava, primary leiomyosarcoma, vascular tumour


How to cite this article:
Singh D, Sarathy G P, Moodadla S. A rare case of primary leiomyosarcoma of the inferior vena cava with an intra- and extravascular component. Indian J Vasc Endovasc Surg 2019;6:315-9

How to cite this URL:
Singh D, Sarathy G P, Moodadla S. A rare case of primary leiomyosarcoma of the inferior vena cava with an intra- and extravascular component. Indian J Vasc Endovasc Surg [serial online] 2019 [cited 2020 Jan 22];6:315-9. Available from: http://www.indjvascsurg.org/text.asp?2019/6/4/315/273587




  Introduction Top


Leiomyosarcoma of vascular origin is a rare tumor, with its common origin from smooth muscle cells of the inferior vena cava (IVC). Since 1871, <400 cases have been reported worldwide.[1] Leiomyosarcomas are most frequently encountered in the sixth decade with a female predominance. The diagnosis is often challenging as patients present with nonspecific complaints such as dyspnea, abdominal discomfort, or back pain. Computed tomography (CT) and magnetic resonance imaging (MRI) individually or in combination with cavography, ultrasonography (US), and echocardiography allow an early preoperative diagnosis. Histopathological and immunohistochemistry (IHC) examination further confirms the diagnosis of leiomyosarcoma. Herein, we present a rare case of primary leiomyosarcoma of the IVC with an intravascular and extravascular component, managed surgically.


  Case Report Top


A 65-year-old lady presented with a history of vague abdominal discomfort for 1 year. She had no other significant medical and surgical history. Ultrasound of the abdomen detected a mass in the retroperitoneum with mixed echogenicity. Contrast-enhanced CT scan of the abdomen showed an infiltrative predominantly necrotic heterogeneously enhancing large mass lesion seen on the right side of the retroperitoneum in the subhepatic location. The lesion measured approximately 10.5 cm × 8.5 cm × 6.3 cm in size (craniocaudal × transverse × anteroposterior). Laterally, the lesion showed focal parenchymal infiltration into the liver with an extrinsic indentation on the adjacent gallbladder. Medially, the lesion showed broad-based attachment to the inferior second part of the duodenum. Posteromedially, the lesion infiltrated into the IVC for a short segment with associated tumoral thrombus in the infrarenal segment. Inferolaterally, the lesion lay in close proximity to hepatic flexure with focal loss of fat plane [Figure 1] and [Figure 2]. No obvious retroperitoneal lymphadenopathy was noted. The diagnosis of primary vena caval tumor was made with differentials included leiomyosarcoma and angiosarcoma. Surgical excision of the tumor was planned. On exploration, a 12 cm × 10 cm mass was palpated in the retroperitoneum that struck to the duodenum, right kidney, ureter, and IVC. En bloc resection of the tumor was done after carefully dissecting out from the kidney, ureter, duodenum, and liver. One centimeter of cuff of the liver was also excised as the tumor was densely struck to it. Intravascular part of about 2-cm tumor was removed after excision of small cuff of IVC [Figure 3]. The IVC was closed primarily without a significant narrowing [Figure 4]. The patient tolerated the procedure well, and postoperative period was uneventful. Surgical specimen was sent for histopathological examination [Figure 5]. Grossly, it revealed a soft-tissue mass adherent to vessel measuring 13 cm × 12 cm × 10 cm [Figure 6]. The external surface was smooth and bosselated. The cut section was nodular, gray–white, and firm in consistency. A small piece of the liver was seen attached to one end, measuring 2 cm × 2 cm × 1.1 cm which was grossly unremarkable. Microscopically, sections showed a vessel wall with attached tumor mass. The tumor mass was composed of spindle-shaped cells with eosinophilic cytoplasm arranged in fascicles and sheets. Tumor cells displayed nuclear atypia with hyperchromasia, increased nuclear-cytoplasmic ratio (N/C ratio), and significant mitotic activity of 10–15 mitotic figures/10 high-power field in the cellular area) [Figure 7]. The focal area also showed osteoclastic type of giant cells. Sections from the liver showed no evidence of tumor tissue. The diagnosis of leiomyosarcoma was made. IHC study revealed positivity for smooth muscle actin and desmin and Ki-67 positivity in 12%–15% of tumor cells and was consistent with the diagnosis of leiomyosarcoma [Figure 8].
Figure 1: Contrast-enhanced computed tomography of the abdomen: A large infilterative mass lesion in the right side of the retroperitoneum in the subhepatic location

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Figure 2: Contrast-enhanced computed tomography abdomen showing a lesion infiltrates into the inferior vena cava for a short segment with associated tumoral thrombus in the infrarenal segment

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Figure 3: Exicising intravascular part of the tumor with a cuff of the inferior vena cava

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Figure 4: Primary repair of the inferior vena cava

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Figure 5: Surgical specimen of the tumor

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Figure 6: Surgical specimen of both intra- and extravascular part of the inferior vena cava tumor

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Figure 7: Histopathological examination showing spindle-shaped cells with eosinophilic cytoplasm arranged in fascicles and sheets with nuclear atypia and hyperchromasia

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Figure 8: Immunohistochemistry study showing positivity for smooth muscle actin and desmin

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  Discussion Top


Leiomyosarcoma of the IVC is a rare clinical entity that has been described in fewer than 400 patients in the literature.[1] These tumors originate from smooth muscle cells. Leiomyosarcoma is the most common primary tumor of the IVC and the second most frequent retroperitoneal neoplasm in adults.[2],[3] These tumors exhibit three main growth patterns: extraluminal (62% of cases), intraluminal (5%), and combined extra- and intraluminal (33%).[3]

Two-thirds to three-fourths of vascular leiomyosarcomas occur in women, and most develop in the fifth or sixth decade of life.[4] A vascular leiomyosarcoma can develop in any vein but is usually found in the IVC. The tumor is relatively slow growing and tends to expand along the least-resistant tissue planes rather than invades adjacent organs.[5]

Leiomyosarcomas are classified into three groups according to their localization in the IVC. Upper-segment involvement indicates localization from the hepatic vein to the right atrium (24% of the cases), middle-segment involvement spans the hepatic to renal veins (42%), and lower-segment involvement includes the infrarenal area (34%).[2] The tumors are most frequently located between the diaphragm and renal veins, although in 10%–17% of cases, the entire caval length is involved.[3] The tumor may extend into the right side of the heart and rarely into the pulmonary artery. The clinical manifestation depends on the involved part of the IVC and the dimensions and growth pattern of the tumor. Upper-segment involvement may manifest as Budd–Chiari syndrome, middle-segment involvement may manifest as nephrotic syndrome, and infrarenal involvement may manifest as lower extremity edema.[2],[6],[7]

The most common patient presentation includes nonspecific symptoms such as exhaustion, abdominal pain, weight loss, an abdominal mass or swelling, nausea, and vomiting.[2],[3] Some patients with large tumors experience weight gain. Late-stage symptoms include abdominal distention, changes in defecation habits, leg edema, back or radicular pain, and frequent urination. Atypical signs include hemoperitoneum, gastrointestinal bleeding, dystonia, and paraplegia. A leiomyosarcoma with an intraluminal component is more likely to produce symptoms at an earlier stage than is a completely extraluminal tumor.[3],[5] Uncommon complications of leiomyosarcoma include lung emboli, abnormal red blood cell morphology, consumption coagulopathy, and cardiac arrhythmias.[3]

At the macroscopic evaluation, a leiomyosarcoma typically appears as a gray-to-white fleshy mass. A somewhat whorled appearance may be evident. Larger tumors often exhibit hemorrhage, necrosis, or cystic changes. The tumor border frequently appears to be well circumscribed. Entirely intraluminal leiomyosarcomas manifest as polypoid or nodular masses that are firmly attached to the vessel wall.[3]

At the microscopic evaluation, a leiomyosarcoma exhibits an atypical interlaced pattern, with sweeping bundles of spindle-shaped cells with elongated, occasionally truncated, blunt-ended nuclei. Mitoses are easily identified but may not correlate with biologic behavior.

Metastases to the liver, lungs, and lymph nodes usually occur at the late stages of the disease. Recurrences are common, and subsequent tumors may occasionally exhibit more aggressive histologic characteristics than the original neoplasm.[3]

Imaging plays an important role in the preoperative staging of leiomyosarcoma. CT is useful for identifying, diagnosing, and following up intravascular leiomyosarcomas. The most typical imaging appearance of these tumors includes vein dilatation and an intermediate-attenuation mass with irregular enhancement and total or near-complete vein obstruction. The tumor is lobulated, well-defined, and heterogeneous and exhibits signs of hemorrhage and necrosis. Infrequently, a predominantly cystic mass representing extensive necrosis may be evident. Calcification is unusual.[3] An intraluminal leiomyosarcoma is extremely difficult to detect at CT without the use of contrast material.[3],[8],[9]

Intraluminal leiomyosarcomas can be readily depicted at MRI. On images obtained with spin-echo sequences and presaturation pulses, the patent blood vessels appear dark and contrast with the solid tumor. On gradient-echo MR images obtained during breath holding, the patent vessels are hyperintense relative to the less intense tumor. These pulse sequences can be used to create MR angiograms that depict the arterial or venous anatomy before surgery. It has been suggested that tumor thrombosis could be distinguished from blood thrombosis on the basis of the differences in signal intensity seen on MR images.[3],[5]

Isointense tumors can be seen on T1-weighted MR images, and iso- to hyperintense tumors can be seen on T2-weighted MR images. Hemorrhagic regions appear as hyperintense areas on T1-weighted images. The tumors usually exhibit marked enhancement.[5],[8] Given the complex appearances of these tumors, both CT and MRI are useful for differentiating a neoplasm from a simple thrombus. Leiomyosarcomas with extensive extravascular components are much more difficult to differentiate from retroperitoneal tumors that compress or invade the IVC.[5],[8] Such cases require differentiation from tumors that affect the hepatic, adrenal, or other retroperitoneal structures. The three-dimensional capability of MRI permits intra- and extraluminal staging and determination of adjacent organ involvement.[5]

Although the imaging findings may strongly suggest a primary tumor of the IVC, biopsy may help, and US-guided biopsy is the modality of choice for obtaining tissues for histologic analysis.[5]

The tumor location and the extent of local invasion are especially important factors in treatment decision-making. Surgical resection offers the only prospect of cure. In cases with involvement of the middle and lower IVC, radical excision can be attempted. In cases with adequate development of venous collateral pathways, the IVC can be ligated without resulting in appreciable edema in the lower extremities.[3] When the middle third of the IVC is removed, although the right kidney must be resected or autotransplanted to the iliac fossa, the left kidney contains sufficient collateral vessels for patient survival. There is no acceptable surgical therapy for tumors in the upper IVC with hepatic vein involvement. Radiation therapy and chemotherapy are often used, but their exact roles have not been well established.[3]

The most important factor affecting survival in patients with any type of leiomyosarcoma appears to be the adequacy of surgical resection. Although complete resection is often impossible, patients with completely resected tumors have a longer median survival than do those with incomplete resection. Patients with inoperable tumors have a poor prognosis. Most patients who survive the primary tumor will eventually develop metastases; distant metastases are present at diagnosis in approximately 40% of cases. In general, hematologic metastasis is more common than lymphatic dissemination. The most common sites of metastasis are the liver, lungs, brain, and peritoneum; less common sites include the skin, soft tissues, bones, kidneys, and omentum. As metastases increase in size, they often exhibit cystic necrosis. Even when complete excision is believed to have been accomplished, local recurrence is common and is observed in 40%–77% of the cases.[3]

Our patient recovered well after the surgery and was advised to undergoe regular follow-up imaging to monitor for recurrences.

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.
Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari N. International registry of inferior vena cava leiomyosarcoma: Analysis of a world series on 218 patients. Anticancer Res 1996;16:3201-5.  Back to cited text no. 1
    
2.
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. 2
    
3.
Hartman DS, Hayes WS, Choyke PL, Tibbetts GP. From the archives of the AFIP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: Radiologic-pathologic correlation. Radiographics 1992;12:1203-20.  Back to cited text no. 3
    
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Mingoli A, Feldhaus RJ, Cavallaro A, Stipa S. Leiomyosarcoma of the inferior vena cava: Analysis and search of world literature on 141 patients and report of three new cases. J Vasc Surg 1991;14:688-99.  Back to cited text no. 4
    
5.
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. 5
    
6.
Mönig SP, Gawenda M, Erasmi H, Zieren J, Pichlmaier H. Diagnosis, treatment and prognosis of the leiomyosarcoma of the inferior vena cava. Three cases and summary of published reports. Eur J Surg 1995;161:231-5.  Back to cited text no. 6
    
7.
Young R, Friedman AC, Hartman DS. Computed tomography of leiomyosarcoma of the inferior vena cava. Radiology1982;145:99-103.  Back to cited text no. 7
    
8.
Blum U, Wildanger G, Windfuhr M, Laubenberger J, Freudenberg N, Munzar T. Preoperative CT and MR imaging of inferior vena cava leiomyosarcoma. Eur J Radiol 1995;20:23-7.  Back to cited text no. 8
    
9.
Ahluwalia A, Saggar K, Sandhu P, Gupta K. Primary leiomyosarcoma of inferior vena cava: An unusual entity. Indian J Radiol Imaging 2002;12:515-6.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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