Leiomyosarcoma of the Inferior Vena Cava in an HIV-Positive Adult Patient: A Case Report and Review of the Literature

Abstract

Patient: Female, 64Final Diagnosis: IVC leiomyosarcomaSymptoms: Back pain • leg pain • leg swellingMedication: —Clinical Procedure: IVC filter placement • CT-guided IVC mass biopsySpecialty: OncologyObjective:Rare diseaseBackground:Leiomyosarcoma is the most common primary malignancy of the inferior vena cava (IVC), and represents approximately 10% of primary retroperitoneal sarcomas. Leiomyosarcoma presents with non-specific symptoms, including abdominal pain or back pain. There is an increased incidence in immunosuppressed individuals.Case Report:An unusual presentation of IVC leiomyosarcoma is reported in a 46-year-old female patient infected with human immunodeficiency virus (HIV) who was on highly active antiretroviral therapy (HAART) and who had a normal CD4 count of 934, who presented with back pain. Magnetic resonance imaging (MRI) of the lumbar spine showed a mass of the IVC. Initial computed tomography (CT)-guided biopsy of the IVC mass was non-diagnostic. An IVC filter was inserted, and the patient was discharged home, but 20 days later, she returned to the hospital with worsening right flank pain. Laboratory tests showed acute renal failure, and a repeat CT scan showed IVC thrombus extending 5 cm superiorly. When compared with the previous CT, there was an extension of thrombus into both renal veins. Histopathology of a transjugular needle core biopsy showed a moderately differentiated leiomyosarcoma. The patient was transferred to a multidisciplinary sarcoma center for surgical resection, chemotherapy, and radiation therapy.Conclusions:This report is of a rare case of IVC leiomyosarcoma in a middle-aged HIV-positive woman with a normal CD4 count. Leiomyosarcoma of the IVC is extremely rare, is often detected when advanced, and has a poor prognosis. This case report describes the clinical, imaging, surgical and histopathological findings of leiomyosarcoma of the IVC.

Primary external iliac vein leiomyosarcoma in a young female diagnosed by contrast-enhanced ultrasound

“…[ 14 ] The etiology remains unclear, although it has been reported that LMS is highly probable in immunosuppressed patients (HIV-positive patients and organ transplant recipients). [ 15 ] In this case, the patient tested negative for HIV serology, and there was no significant medical evidence that indicated the possibility of immunosuppression. The histological variant of sarcoma does not provide sufficient information for planning therapy and predicting prognosis.…”

Treatment of a Double Cancer Patient With Primary Inferior Vena Cava Sarcoma and Lung Adenocarcinoma: A Case Report and Literature Review

“…Because of the lack of specificity, most of these techniques are considered non-diagnostic. The diagnosis of the IVC sarcoma is difficult, and due to the insidious course of the disease, and most cases are diagnosed at a late stage ( 16 ). The final diagnosis of the IVC leiomyosarcoma is based on pathology.…”

“…At present, radical surgical resection with free margins is believed to be the best curative therapy for the IVC leiomyosarcoma ( 14 , 16 , 19 ). The surgical approach differs depending on the tumor invasiveness and location, including simple tumor excision with IVC repair and tumor excision with graft replacement.…”

Concomitant Aorto-Caval Reconstruction for Inferior Vena Cava Leiomyosarcoma

“…7,8 Since then, early aggressive surgical intervention has been the treatment of choice with roughly 600 reported cases globally as of 2017. 9 In the largest pooled analysis to date, Wachtel et al evaluated resection outcomes in 377 patients with surgical resection finding a low 30-day mortality of 1.9%, but 24.7% complication rate including lower extremity edema and acute renal failure but also bowel injury and hemorrhage. 4 In this cohort, disease-free survival and overall survival were 57% and 92% at one year and 6% and 55% at 5 years, respectively; however, the median overall survival was only 23 months.…”