Leiomyosarcoma of the inferior vena cava presenting as a cardiac mass

Introduction Primary intravenous leiomyosarcomas are rare vascular tumors with aggressive disease biology. The diagnosis and management have been challenging as little data exist from large databases. Methods A literature search was done to identify all cases of primary leiomyosarcomas in the last five years. Clinicopathological features and management strategies were evaluated. Results The median age was 53 years, predominantly females (2.5:1), presenting as metastases in up to 12.1% cases. Most tumors were locally advanced with a median size of 10cm. Inferior vena cava involvement from renal veins to infrahepatic veins remains the most frequent site (57.1%cases) while nearly half (52.8%) proceeded for surgery without histological proof. Most patients could undergo upfront resection (88.0%) with few patients receiving neoadjuvant chemotherapy (4.3%) or neoadjuvant radiotherapy (2.2%). Significant multivisceral resections included right nephrectomy (41.3%), liver resection (25.7%) and left nephrectomy (2.2%). Most patients (91.8%) needed an inferior vena cava graft placement with remarkable microscopically negative margins (85.5% cases). Doxorubicin and ifosfamide were the most frequently used combination chemotherapy regimens in both pre and postoperative settings with partial responses. The median overall and disease free survival among operated patients was 60 months and 28 months respectively. In multivariate analysis large tumor, extensive inferior vena cava involvement, and need for adjuvant chemotherapy appeared significant predictors for overall survival. Conclusions Aggressive upfront surgical resection with clear margin remains the key for long-term survival. Doxorubicin-based regimens were preferred as neoadjuvant chemotherapy while adjuvant treatment with chemotherapy, radiotherapy, or both may be considered in high-risk patients.

show abstract

“…11,12 IHC makers frequently reported positive were smooth muscle actin, desmin, vimentin, and caldesmon. 13,49…”

Section: Resultsmentioning

confidence: 99%

“…11,12 IHC makers frequently reported positive were smooth muscle actin, desmin, vimentin, and caldesmon. 13,49 Management Surgery. Most patients (n = 81) underwent upfront resection (88.0%), while four others were operated following neoadjuvant treatment (Table 2).…”

Section: Clinical Features and Diagnostic Modalities (Imaging And Tis...mentioning

confidence: 99%

A systematic review of the current management approaches in leiomyosarcoma of inferior vena cava—Results from analysis of 118 cases

Saikia

Rastogi

Barwad

et al. 2021

Asian Cardiovasc Thorac Ann

View full text Add to dashboard Cite

show abstract

“…The largest case series by Perhavec et al showed the most common reasons for technical unresectability to be the involvement of the superior mesenteric vessels, followed by the involvement of bone, portal vein, or hepatic axis (8). When managing an intracardiac tumor thrombus, wide resection is still preferred and intra-operative cardiopulmonary bypass should be considered to facilitate resection and maintain organ perfusion (20)(21)(22) Their rarity poses a great oncologic and surgical challenge, as the bene ts of systemic treatment are unde ned, and surgical management remains controversial. Neoadjuvant therapy can be used to reduce tumor size and improve resectability.…”

Section: Discussionmentioning

confidence: 99%

Unresectable Leiomyosarcoma Of The Inferior Vena Cava With Right Atrium Tumor Thrombus: When To Deem This Tumor Inoperable? A Case Report And Literature Review.

Castellanos,

Tabbara,

Livingstone

et al. 2023

Preprint

View full text Add to dashboard Cite

Background Leiomyosarcomas (LMS) of the inferior vena cava (IVC) are a rare form of retroperitoneal malignancy and their venous extension to the right atrium is an even rarer event. These tumors pose a unique surgical challenge and often require a multidisciplinary team-based approach for their surgical treatment. Case presentation: We present a case of a 68-year-old male with primary LMS of the IVC with a tumor thrombus extending into the right atrium that was initially deemed inoperable. Conclusions After extensive neoadjuvant chemo-radiation with minimal tumor effect, the patient underwent en-bloc surgical resection of the tumor along with removal of the infrarenal IVC and right kidney and adrenal without the need for cardiopulmonary bypass.

show abstract

“…La radioterapia y la quimioterapia adyuvantes han demostrado prolongar la supervivencia y mejorar el control de la enfermedad en algunos casos 19,20 . La doxorrubicina, la ifosfamida, la vincristina, el etopósido, el uracilo, el cisplatino, la ciclofosfamida y la mitomicina se encuentran entre los agentes quimioterapéuticos que se han probado, con resultados muy variados 10,21,22 . Los factores que determinan la supervivencia incluyen el índice mitótico del tumor, la presencia de metástasis, la necrosis en la sección del tumor y la localización del tumor en el lado izquierdo del corazón 20 .…”

Section: Discussionunclassified

Sarcoma de la vena pulmonar

Martínez-Montalvo¹,

Conde-Camacho²,

Conrado-Jiménez³

et al. 2022

RCCAR

View full text Add to dashboard Cite

Los sarcomas son neoplasias primarias, cuya ubicación es rara en grandes vasos y excepcional en las venas pulmonares. Los dos tipos más frecuentes en las venas pulmonares son el leiomiosarcoma y el fibrosarcoma, con una distribución alrededor de la cuarta a la quinta décadas de vida, un pronóstico de 23 meses y 60% de metástasis al momento del diagnóstico. La presentación clínica es inespecífica ya que simula situaciones como embolia pulmonar, falla cardiaca descompensada y masa en la aurícula izquierda. Para su diagnóstico se cuenta con diferentes herramientas, como la ecocardiografía, la tomografía computarizada, la angiografía coronaria, la resonancia magnética y la tomografía por emisión de positrones (PET TC). El tratamiento incluye resección quirúrgica radical, manejo adyuvante con quimioterapia y radioterapia, e incluso, en casos seleccionados, trasplante de corazón. Se presenta el caso de una paciente con diagnóstico inicial de embolia pulmonar, con un episodio de edema pulmonar secundario a masa en la aurícula izquierda y extensión de un sarcoma de vena pulmonar derecha, con desenlace fatal. Se aporta a la literatura con el caso y la revisión de tema.

show abstract

Leiomyosarcoma of the inferior vena cava presenting as a cardiac mass

Cited by 9 publications

References 15 publications

A systematic review of the current management approaches in leiomyosarcoma of inferior vena cava—Results from analysis of 118 cases

A systematic review of the current management approaches in leiomyosarcoma of inferior vena cava—Results from analysis of 118 cases

Unresectable Leiomyosarcoma Of The Inferior Vena Cava With Right Atrium Tumor Thrombus: When To Deem This Tumor Inoperable? A Case Report And Literature Review.

Sarcoma de la vena pulmonar

Contact Info

Product

Resources

About