Successful surgical resection of a metastatic clear cell sarcoma in the heart: a case report

TL;DR

A rare case of clear cell sarcoma in the heart was successfully treated with surgical removal, marking the first known instance of this treatment approach.

Contribution

This is the first reported case of surgical resection for metastatic clear cell sarcoma in the heart.

Findings

PET-CT identified a metastatic tumor in the left ventricle of the heart four years after initial amputation for CCS.

Surgical resection of the cardiac tumor was performed and confirmed as clear cell sarcoma via pathology.

The case highlights the importance of multimodal imaging and surgical intervention for rare cardiac tumors.

Abstract

Clear cell sarcoma (CCS) is a very rare disease and one with a very poor prognosis. Furthermore, its occurrence in the heart is very rare and past reports are scarce.

A 33-year-old man who had undergone left arm amputation due to CCS came to the hospital because a positron emission tomography computed tomography (PET-CT) four years post-amputation showed an accumulation in the heart. The PET-CT with glucose suppression treatment showed fluorodeoxyglucose accumulation in the myocardium between the middle of the anterolateral wall and the papillary muscle of the posterior lateral wall of the left ventricle (LV). Based on the course of the disease up to now, it was considered that the accumulation was most likely metastasis of CCS. Observation of the heart after a median sternotomy revealed a white tone, well-defined lesion in the middle of the anterolateral wall of LV. The tumour on the posterolateral side of LV was not exposed on the surface, but it was palpated and was still recognizable as a firm neoplastic lesion. Because the mass was identified as a sarcoma on intraoperative rapid pathology, we decide to perform a total resection. Both lesions were excised, and pathology revealed a diagnosis of CCS.

Clear cell sarcoma is a very rare disease that accounts for <1% of all soft tissue sarcomas, and its occurrence in the heart is even rarer. It requires a combination of many imaging modalities. To our knowledge, this is the first case of CCS in the heart treated with surgical resection.

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