Key result
Cabozantinib plus nivolumab with pericardiectomy yields transient clinical improvement in metastatic clear cell sarcoma.
Why the study?
Cardiac involvement in clear cell sarcoma is exceptionally rare and sparsely documented, posing diagnostic and therapeutic challenges.
Case Report (n=1)
This case report highlights that pericardial metastasis from clear cell sarcoma is a rare, life-threatening manifestation that can evade initial pathological confirmation and cause fatal diastolic dysfunction despite clinical response to cabozantinib plus nivolumab.
Raises awareness of rare cardiac metastases in clear cell sarcoma; extends documented sites but remains hypothesis-generating.
Background: Clear cell sarcoma is a rare yet aggressive malignancy of the soft tissue, driven by EWSR1 mutations, with a high tendency for metastasis. Cardiac involvement is exceptionally rare and sparsely documented, posing many diagnostic and therapeutic challenges. Case presentation: A 51-year-old gentleman with primary CCS of the left thigh initially treated with local excision and adjuvant radiation therapy, returned 2 years later after failing to follow up with diffuse metastasis to the lungs, liver, lymph nodes and bones. First line combination chemotherapy achieved a major response but was complicated by pulmonary embolism, and large symptomatic pericardial effusion. A pericardial window was performed, and revealed visible pericardial tumor implants, only for the pathology to report fibrotic tissue solely with no confirmed malignancy. Despite further response to second line of cabozantinib plus nivolumab combination therapy and significant clinical improvement, pericardial effusion recurred requiring surgical pericardiectomy. Unfortunately, the patient passed away from severe diastolic dysfunction from a fibrotic pericardium and urosepsis. Discussion: Cardiac metastasis from CCS is rare and sometimes even evades pathological confirmation despite strong clinical and intraoperative suspicion. This case underscores the complexity of diagnosing thoracic spread of the disease and its treatment. In such cases, the combined use of multimodal imaging, molecular analysis and repeat tissue sampling is essential to appropriate management. Targeted therapies such as cabozantinib in combination with immunotherapy may result in significant disease control in refractory metastatic CCS cases. Conclusion: Cardiac involvement in CCS is rare but present as a life-threatening manifestation of the disease that requires high clinical vigilance. Recognizing the pattern of metastasis and holding multidisciplinary evaluations with consideration to novel systemic therapies is essential to optimizing outcomes. Continuous reporting of these cases is very important in improving diagnostic accuracy and guiding management strategies.
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Mansour et al. (2026) conducted a case report in Clear cell sarcoma with pericardial metastasis (n=1). Combination chemotherapy, second-line cabozantinib plus nivolumab, pericardial window, and surgical pericardiectomy was evaluated on Clinical response and survival. Cabozantinib plus nivolumab and pericardiectomy in a 51-year-old man (n=1) with metastatic clear cell sarcoma yielded clinical improvement before death from diastolic dysfunction and urosepsis.
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