# A case report of recurrent primary posterior mediastinal perivascular epithelioid cell tumour compressing the right inferior pulmonary vein, atria, and inferior vena cava

## TL;DR

A 36-year-old man with a history of a rare chest tumor experienced a recurrence that compressed vital heart and lung structures, requiring a multidisciplinary approach for diagnosis and treatment.

## Contribution

This case highlights the rare recurrence of mediastinal PEComa and the importance of multimodal imaging and collaboration in managing such complex tumors.

## Key findings

- A recurrent mediastinal PEComa was identified using TEE and cMRI, showing its size and proximity to critical structures.
- The case emphasizes the need for a multidisciplinary team to manage rare and complex tumor recurrences.
- Surgical resection was recommended, followed by potential chemotherapy and radiation.

## Abstract

Perivascular epithelioid cell tumours (PEComas) are rare soft tissue neoplasms that commonly occur in the uterus, skin, and liver and less commonly in the retroperitoneum, colon, and mediastinum.

A 36-year-old male patient with a history of mediastinal PEComa status post resection, essential hypertension, and atrial fibrillation status post appendage ligation currently not on anticoagulation presented with a 1-week history of fevers, chills, productive cough, chest pain, dyspnoea on exertion, loss of appetite, and general weakness. Vital signs, physical exam, laboratory data, electrocardiogram, and chest radiograph were grossly unremarkable. A multimodality imaging approach utilizing transthoracic echocardiogram, transoesophageal echocardiogram (TEE), cardiac magnetic resonance imaging (cMRI), and computed tomography angiography of the chest, abdomen, and pelvis revealed a local 40 mm × 53 mm globular bilobed vascularized scar-free posterior mediastinal mass arising from the roof of the left and right atria and extending superiorly to the main pulmonary artery and inferiorly to the inferior vena cava. Based on the mass’ size and proximity to vital structures and tumour recurrence, the case was presented during tumour board rounds, and the outcome was to surgically resect the mass and then have the patient follow up with medical oncology and radiation oncology for possible chemotherapy and radiation, respectively.

Perivascular epithelioid cell tumours are rare, and mediastinal PEComas are even rarer, warranting a multimodality imaging approach involving TEE and cMRI and a multidisciplinary approach involving anaesthesiologists, cardiologists, cardiothoracic surgeons, medical oncologists, pathologists, radiologists, and radiation oncologists.