Suction thrombectomy of a uterine carcinosarcoma tumor thrombus extending into the IVC and right atrium

Abstract

Uterine carcinosarcoma is a rare, aggressive tumor with several cases in the literature reporting cardiac tumor thrombus involvement. In this case report, we describe a 72-year-old female with a history of uterine carcinosarcoma, who presented with extensive thrombus in the Inferior Vena Cava (IVC) and right atrium. The patient underwent an aspiration thrombectomy which aided in intravascular debulking of the thrombus. Histopathological analysis of the thrombus revealed tumor thrombus. In cryptic cases of tumor thrombus, thrombectomy with histopathological analysis can help confirm the diagnosis of metastatic disease and help guide oncologic staging and further therapy.

Introduction

Uterine carcinosarcoma is a rare, aggressive tumor which comprises about 2–3% of uterine cancers but carries a high risk of disease recurrence and mortality [1]. It typically presents as a malignant mixed Mullerian tumor with both epithelial and mesenchymal cell components. Primary treatment for uterine carcinosarcoma involves surgical debulking followed by adjuvant therapy with cytotoxic chemotherapy or radiation [2]. Cardiac metastasis is a rare presentation of this disease and only a few cases have been described in the literature [1, 3]. Cardiac metastasis with tumor thrombus is an uncommon cause of cardiac thrombus but has been reported throughout the literature with most cases occurring from hepatocellular, ovarian, and pancreatic cancers [4].

Case

A 72-year-old female presented with worsening abdominal pain and intolerable nausea and vomiting for one week. Her past medical history included Non-Hodgkin’s Lymphoma (diagnosed at age 46) s/p chemotherapy and allogenic transplant with total body irradiation, uterine carcinosarcoma, and congestive heart failure with an ejection fraction of 30%. She was diagnosed with stage 1A uterine carcinosarcoma at age 67 s/p total abdominal hysterectomy with bilateral salpingo-oophorectomy and underwent 6 cycles of carboplatin and paclitaxel followed with external beam radiation to the pelvis. On admission, the patient was afebrile (36.9 °C) and hypotensive (blood pressure of 113/57 mm Hg requiring levophed, vasopressin, and stress dose steroids). Rest of vitals were stable with respiratory rate of 20 breaths per minute, pulse of 100 beats per minute, and oxygen saturation of 97%. She had a mild leukocytosis (10,800 white blood cells/microliter), elevated AST/ALT (181 and 100 units/liter respectively), hyperbilirubinemia (Tbili 12.5 µmol/liter), and elevated alkaline phosphatase (1242 units/liter). Imaging revealed the presence of left pleural effusion, multiple upper lobe pulmonary nodules, and a large inferior vena cava thrombus with proximal left renal vein involvement (Fig. 1A). The thrombus progressed from the infrahepatic inferior vena cava into the right atrium. Axial imaging revealed an enlarged and centrally necrotic para-aortic lymph node, which led to concern of the thrombus being tumor thrombus; given the patients history of uterine carcinosarcoma (Fig. 1B). Due to her worsening abdominal pain with no other clear etiology the decision was made to attempt to debulk the thrombus with aspiration thrombectomy.

Fig. 1.

A Coronal abdominal CT scan showing the presence of a hyperdense tumor thrombus within the IVC. B Axial scan demonstrating the metastatic/residual disease with para-aortic lymphadenopathy

Procedure

Right common femoral vein and right internal jugular vein access sheaths were placed. An initial venogram of the IVC with a flush catheter revealed an intraluminal filling defect that spanned from the level of the left renal vein to the suprahepatic IVC (Fig. 2). An IVUS catheter (Philips Cambridge, MA) was inserted through the femoral vein sheath to both confirm the findings of venography, as well as guide clot engagement with the thrombectomy device. A Gore Dry Seal Sheath (Gore, Flagstaff, AZ) was advanced into the suprahepatic inferior vena cava via the right internal jugular vein approach. The F22 Alphavac device (AngioDynamics, Latham, NY) with a 20 degree curve was the thrombectomy device utilized.

Fig. 2.

IR Angiogram of the tumor thrombus prior to intervention showing multiple filling defects in the inferior vena cava extending into the right atrium

The thrombus extending into the right atrium was then engaged with the 42 French nose cone of the Alphavac system, with the aid of real time intravascular ultrasound (IVUS) visualization (Fig. 3). During the initial few minutes of thrombectomy the patient became hypotensive, requiring vasopressor support from the anesthesia team. A pulmonary angiogram showed no evidence of acute PE. Following hemodynamic stabilization, thrombectomy was continued. Final angiography showed thrombus debulking (Fig. 4). The thrombus underwent histopathological analysis revealing high-grade malignant cells with carcinomatous and rhabdoid morphologies (Fig. 5). This shared a similar histomorphology to the patient’s pathology at the time of prior hysterectomy.

Fig. 3.

Initial intravascular ultrasound showing the thrombus extending into the inferior vena cava

Fig. 4.

Post-operative IR angiogram revealing reduced clot burden with improved flow in the IVC

Fig. 5.

Image on the left is histopathology showing sarcomatous and epithelioid components of the uterine carcinosarcoma. This histomorphology mirrored the patient’s prior hysterectomy and primary tumor analysis from 2018. Image on the right shows the histopathology of fibrin and clot deposition around the sarcomatous portion of the tumor

Following the procedure, the patient was found to be septic in the setting of ascending cholangitis. They were not responsive to 2-L fluid bolus and 200 mcg phenylepinephrine with blood pressure ranging from 67–93/35–52 mm Hg. A percutaneous-transhepatic drainage was placed and the next day the patient was only arousable to sternal chest rub and her code status was changed to “do not resuscitate” due to worsening sepsis requiring the need for pressors overnight. The patient’s family decided to transition to comfort care, so the patient was discharged to hospice.

Discussion

Endometrial malignancy is rarely reported in the literature as a source of primary cardiac metastasis. A report found that out of 1100 gynecological cancer cases, only 6 had metastasis to the heart [5]. Uterine carcinosarcoma may enter the heart through spread from the lymphatic or hematogenous route. The majority of metastasized uterine cancers are squamous cell carcinoma (50%) while adenocarcinoma only comprises 17% of metastases cases [6]. Given that carcinosarcoma is a rare and insidious form of adenocarcinoma, this makes such a presentation even less likely. Uterine carcinosarcoma has a poor prognosis, with a 5-year survival ranging from 30–40% with a local recurrence rate for early-stage disease ranging between 40–60% [7]. The highly aggressive nature of this condition makes treatment options limited.

In this case, suction thrombectomy was able to confirm the diagnosis of tumor thrombus from uterine carcinosarcoma intravascular spread. Aspiration thrombectomy can percutaneously remove intravascular substances including foreign bodies, thrombus, and tumor thrombus. Surgical embolectomy and thrombolytic therapies may also be used to treat cardiac bland thrombus, but they are both associated with significant mortality, and may not allow for the en bloc removal of intravascular material which can be further analyzed through pathology [8]. AngioVac (Angiodynamics, Latham, NY) is a vacuum assisted thrombectomy system that has been used in the past, since it has an extracorporeal bypass circuit which allows for the reperfusion of filtered blood back into the body. The AlphaVac has emerged as an aspiration thrombectomy device that has the benefits of the AngioVac system (such as a 42 Fr nose cone to engage intravascular material) without requiring the use of a perfusionist of venous bypass machinery [9]. The AlphaVac system does not require a reinfusion cannula which allows for faster setup, however, it may be associated with increased procedural blood loss during aspiration.

There are very few case reports of aspiration thrombectomy involving the AlphaVac system, with most studies primarily focusing on its applications in pulmonary embolism and right atrial thrombus. The AngioVac device has been studied more extensively, with one case series reporting a high rate of success for acute right atrial and iliocaval thrombosis removal by mechanical thrombectomy [10]. Out of 48 cases, the use of AngioVac resulted in 83.3% success rate of clot removal with favorable outcomes and survival to hospital discharge. Another investigation showed that patients who presented with symptomatic iliocaval thrombosis that failed medical management were able to be successfully aspirated with AngioVac despite having a more chronic thrombosis [11]. In cases where surgical intervention may be contraindicated, mechanical aspiration thrombectomy remains an effective tool to provide symptomatic relief for patients with venous thrombotic disease.

Another case study reported similar results for a 59-year-old female patient with endometrial carcinosarcoma who developed metastasis of a tumor thrombus into the right atrium and ventricle. AngioVac aspiration led to no damage of the tricuspid valve and removal of the thrombus, allowing for improvement of the patient’s dyspnea, along with pathologic confirmation of a metastatic endometrial carcinosarcoma [1]. The patient was found to have metastasis to spine a few months later and elected for hospice care as well due to deterioration of her condition. Cardiac metastases have been reported to occur in as little as 1.5% of cancer patients with even fewer being from gynecological malignancies. Aggressive treatment with open surgical excision, chemotherapy, and radiation therapy have been shown to increase lifespan by 3–13 months at most. Our case also successfully reduced clot burden and confirmed through histopathology the presence of uterine carcinosarcoma using the new AlphaVac device. The removal of a thrombus using this method can allow for histopathological analysis of thrombus to determine the presence of neoplastic cells. Implementing mechanical thrombectomy helped alleviate the 59-year-old patient’s distressing symptoms of dyspnea and our patient’s abdominal pain and severe nausea/vomiting. This case highlights the novelty and utility of using mechanical thrombectomy as both a therapeutic and diagnostic tool in patients with tumor thrombus of unclear etiology.

Funding

None.

Declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. IRB approval was not required for the creation of this manuscript.

Informed consent

Informed consent was obtained from all individual participants included in the study.