Dyspnea due to an uncommon vascular tumor: leiomyosarcoma of the infrahepatic vena cava inferior

Abstract

Background Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare malignancy that originated from the smooth muscle tissue of the vascular wall. Diagnoses, as well as, treatment of the disease are still challenging and to date, a radical surgical resection of the tumor is the only curative approach. Case report We report on the case of a 49-year old male patient who presented with suddenly experienced dyspnea. Besides bilateral pulmonary arterial embolism, a lesion close to the head of the pancreas was found using CT scan, infiltrating the infrahepatic IVC. Percutaneous ultrasound-guided biopsy revealed a low-grade LMS. Intraoperatively, a tumor of the IVC was observed without infiltration of surrounding organs or distant metastases. Consequently, the tumor was removed successfully, by en-bloc resection including prosthetic graft placement of the IVC. Histological workup revealed a completely resected (R0) moderately differentiated LMS of the IVC. Conclusion LMS of the infrahepatic IVC is an uncommon tumor, which may present with dyspnea as its first clinical sign. Patients benefit from radical tumor resection. However, due to the poor prognosis of vascular LMS, a careful follow-up is mandatory.

Leiomyosarcoma of the inferior vena cava presenting with bilateral lower extremity edema with comorbid sarcoidosis: A case report

“…While LMS is an exceedingly rare tumor, it is the most common primary tumor associated with the IVC [5] , typically presenting in females around fifty- to sixty-years of age [6] . There are only a few hundred reported cases of IVC LMS in the literature [4] and little is known in regard to prognosis and treatment for these tumors due to the scarcity of data; however, a study with a pooled data analysis of 377 cases revealed a very high likelihood of relapse though long-term survival is possible [9] .…”

Incidentally Diagnosed Extraluminal Leiomyosarcoma of Infrarenal Inferior Vena Cava: A Case Report and Literature Review from a Radiologist’s Perspective

“…Tumors originating from the suprahepatic segment (segment 3) of IVC commonly have a predominantly intraluminal component and show high prevalence of intracardiac extension [ 5 , 13 , 18 ]. Gafarli S et al reported an intraluminal tumor in segment 1 with transluminal extension into the right atrium, presenting with pulmonary thromboembolism [ 21 ]. Generally, intraluminal tumors from segment 1 show distal extension in to the iliac veins [ 5 , 15 ].…”

“…On Positron emission tomography (PET), these tumors show mild to intense uptake of fluorodeoxyglucose (FDG) [ 21 , 28 , 29 ]. Singh N et al [ 28 ] presented a case of intraluminal LMS showing intense FDG uptake with SUV max value of 18.…”

Incidentally Diagnosed Extraluminal Leiomyosarcoma of Infra Renal Inferior Vena Cava: A Case Report and Literature Review From a Radiologist’s Perspective

“…Tumors originating from the suprahepatic segment (segment 3) of IVC commonly have a predominantly intraluminal component and show high prevalence of intracardiac extension (5, 13, 18). Gafarli S et al reported an intraluminal tumor in segment 1 with transluminal extension into the right atrium, presenting with pulmonary thromboembolism (21). Generally, intraluminal tumors from segment 1…”

“…On Positron emission tomography (PET), these tumors show mild to intense uptake of uorodeoxyglucose (FDG) (21,28,29). Singh N et al (28) presented a case of intraluminal LMS showing intense FDG uptake with SUV max value of 18.…”