Leiomyosarcoma of the inferior vena cava: a case report and review of the literature

Reddy, Venkataprasanth P.; Vanveldhuizen, Peter J.; Muehlebach, Gregory; Dusing, Reginald W; Birkbeck, James P; Williamson, Stephen K.; Krishnan, Leela; Meyers, David G.

doi:10.1186/1757-1626-3-71

Cited by 26 publications

(36 citation statements)

References 8 publications

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“…Histopathology of leiomyosarcoma reveals spindle tumor cells, which are positive for markers of smooth muscle activity includingvimentin, muscle actin, alpha-smooth muscle actin, and desmin [11].…”

Section: Discussionmentioning

confidence: 99%

A Rare Retroperitoneal Mass: Leiomyosarcoma of the Inferior Vena Cava

Baram¹,

Tayeb²,

Bakir³

2014

WJCS

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Leiomyosarcoma of the inferior vena cava (IVC) is a rare soft tissue tumor, mesenchymal in origin that arises from smooth muscles of tunica media; it accounts for about 0.5% of all soft tissue sarcomas, and it is the commonest vascular leiomyosarcoma. The tumor progression is slow, and it is asymptomatic until advanced stage in which involvement of surrounding structures even when the symptoms present are nonspecific. Presentation of Case: A 60 years old lady presented with upper abdominal pain for 3 months duration. Past surgical history was significant for Hysterectomy 15 years ago. On examination: soft abdomen, palpable non pulsating right hypochondrial mass. Ultrasound of the abdomen showed tumor of the head of pancreas. CT scan showed large retroperitoneal tumor extending from the head of pancreas to IVC. Trans abdominal CT guided FNAC showed retroperitoneal sarcoma while Immunohistochemistry (IHC) was proved to be Leiomyosarcoma of the IVC. Discussion: Leiomyosarcoma of inferior vena cava (IVC) is a rare soft tissue tumor, mesenchymal in origin that arises from smooth muscles of tunica media; it accounts for about 0.5% of all soft tissue sarcomas, and it is the commonest vascular leiomyosarcoma. The type of surgical management is a matter of debate and includes resection alone, primary repair/cavoplasty, or replacement with a graft. Reconstruction of the IVC is not always required especially in chronic occlusions. Conclusion: Despite all the advanced modalities, surgery remains the most effective method for treatment of Leiomyosarcoma.

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Section: Discussionmentioning

confidence: 99%

A Rare Retroperitoneal Mass: Leiomyosarcoma of the Inferior Vena Cava

Baram¹,

Tayeb²,

Bakir³

2014

WJCS

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“…Nowadays with the development of image techniques, such as positron emission tomography (PET), the diagnosis of retroperitoneal tumors as well as staging and treatment planning appear to be more accurate [5]. Despite all these advanced technologies, the histopatology is still crucial for diagnosis [5]. Histopathology of leiomyosarcoma reveals spindle tumor cells, which are positive for markers of smooth muscle activity including vimentin, muscle actin, alpha-smooth muscle actin and desmin [5].…”

Section: Discussionmentioning

confidence: 99%

“…Budd-Chiari syndrome is a rare presentation of tumors that invade IVC above hepatic veins [13]. If the IVC tumor invades, right atrium arrhythmias, dyspnea, new heart murmurs and heart failure could be observed [3,5]. Intravascular leiomyosarcoma may also be accompanied by symptoms of venous thrombosis [14].…”

Section: Discussionmentioning

confidence: 99%

“…CT and MRI are also helpful in assessment of its spreading, localization among other organs and possible distant metastases [14]. Nowadays with the development of image techniques, such as positron emission tomography (PET), the diagnosis of retroperitoneal tumors as well as staging and treatment planning appear to be more accurate [5]. Despite all these advanced technologies, the histopatology is still crucial for diagnosis [5].…”

Section: Discussionmentioning

confidence: 99%

“…It appears more often in women, between the 5 th and 6 th decade of life [4]. Clinical symptoms of the disease are not characteristic and can appear several years before diagnosis [5]. Here we present a case of a patient with leiomyosarcoma of the inferior vena cava complicated by Budd-Chiari syndrome and disseminated intravascular coagulation (DIC).…”

Section: Introductionmentioning

confidence: 99%

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Leiomyosarcoma of inferior vena cava complicated by Budd-Chiari syndrome and disseminated intravascular coagulation — case report

et al. 2014

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AbstractLeiomyosarcoma of inferior vena cava is a rare malignant mesenchymal tumor of the venous system that typically occurs in adulthood. Correct and early recognition of leiomyosarcoma is very important, because a complete resection of the tumor (with occasionally chemio-or radiotherapy) can lead to prolonged survival. We report a case of a 54-year-old man suffering from the leiomyosarcoma of inferior vena cava with infiltration of retroperitoneum and right adrenal gland.

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