A unique presentation of pericardial epithelioid angiosarcoma with multifaceted complications

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Pericardial epithelioid angiosarcoma (n=1). Intervention: Paclitaxel followed by doxorubicin-ifosfamide chemotherapy, pericardiocentesis, and bilateral pleural drainage.

Q: What does this research mean for the field?

Pericardial epithelioid angiosarcoma is an aggressive malignancy that can present in young adults with high-output chylothorax and cardiac tamponade, showing rapid progression to distant metastases despite paclitaxel-based chemotherapy. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Moied Al Sakan; Mtanyous Chihab; Ouwais Alkhateb; Omar Fakhreddine; Arafat Tfayli

doi:10.1097/ms9.0000000000002521

August 30, 2024Annals of Medicine and SurgeryOpen Access

A unique presentation of pericardial epithelioid angiosarcoma with multifaceted complications

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Key result

Pericardial epithelioid angiosarcoma rapidly metastasizes despite paclitaxel, causing high-volume chylothorax and 7-month survival.

n=1

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Why the study?

Pericardial epithelioid angiosarcoma is rarely reported, associated with poor prognosis, and lacks a definitive proven treatment regimen.

Key Points

Document a rare case of aggressive metastatic pericardial epithelioid angiosarcoma presenting with severe cardiopulmonary complications in a young adult.
Evaluated a 25-year-old female presenting with dyspnea, palpitations, bilateral pleural effusions, and cardiac tamponade using pericardiocentesis, thoracentesis, and tissue biopsy.
Staged disease using fluorodeoxyglucose-positron emission tomography (FDG-PET) and mediastinoscopy.
Administered systemic chemotherapy comprising six weekly cycles of paclitaxel followed by Adriamycin-Ifosfamide.
Mediastinal biopsy confirmed high-grade pericardial epithelioid angiosarcoma with vaso-formation, and FDG-PET revealed intense uptake across anterior mediastinal, perivascular, paratracheal, subcarinal, and pleural lymph nodes with maximum SUV up to 8.
Imaging revealed disease progression with liver and spine metastases following six cycles of paclitaxel, alongside multifaceted complications including chylothorax draining 2–5 l daily, left atrial thrombus, and septic shock requiring intensive care.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

1 previously healthy 25-year-old female with high-grade metastatic pericardial epithelioid angiosarcoma presenting with cardiac tamponade and massive bilateral pleural effusions, followed for 7 months.

I

Intervention

Pericardiocentesis, diagnostic and therapeutic thoracentesis with bilateral chest tube/pigtail catheter insertion, mediastinoscopy with biopsy, anticoagulation for left atrial thrombus, proton pump inhibitors for melena/esophageal ulcer, followed by six cycles of weekly paclitaxel and subsequently switched to doxorubicin-ifosfamide (one cycle received).

O

Outcome

Clinical course, disease progression, complications (high-output pleural effusions/chylothorax of 2-5 L/day, bilateral hemopneumothoraxes, left atrial thrombus formation, septic shock, respiratory failure), and survival at 7 months.

This case report highlights an exceptionally rare presentation of metastatic pericardial epithelioid angiosarcoma in a young adult complicated by cardiac tamponade, left atrial thrombus, and massive high-output chylothorax, underscoring its aggressive progression despite taxane and anthracycline-based chemotherapy.

Limitations

Rarity of pericardial epithelioid angiosarcoma means treatment decisions are based on limited evidence and absence of prospective trials
Opting for taxane therapy alone limits the ability to add to emerging evidence on anti-VEGF therapy
Limited immunohistochemical reporting in prior literature (such as ERG, vimentin, and CD31) restricts comparison across cases
Unclear at what stage of tumor invasion the entity exhibits high-output chylo-pneumothorax
Treatment decisions were based on limited evidence due to the rarity of pericardial epithelioid angiosarcoma and the absence of prospective trials.
Opting for taxane therapy alone limits the case report's value in adding to the emerging evidence of anti-VEGF therapy on epithelioid angiosarcomas.
Limited immunohistochemical comparison with previously published cases due to unreported staining markers (ERG, vimentin, CD31) in the scarce literature.
It remains unclear at what stage of tumor invasion the entity exhibits the reported high-output chylo-pneumothorax.

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Implication

Alerts clinicians to rare pericardial angiosarcoma in young adults with refractory effusions; extends sparse data on its rapid progression despite paclitaxel.

Abstract

Introduction and importance: Angiosarcomas are rare tumors of endothelial origin and may arise in any organ. Epithelioid angiosarcomas are a subtype of angiosarcoma that are rapidly progressive and typically fatal. Case presentation: The authors report a case of a 25-year-old previously healthy female who presented initially for dyspnea and palpitations, on further evaluation she was found to have large bilateral pleural effusions and cardiac tamponade. Clinical discussion: Pericardiocentesis and thoracentesis were performed alongside biopsies that revealed atypical cellular proliferation.Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed avid uptake in the anterior mediastinum, perivascular, paratracheal, subcarinal and pleural lymph nodes with large FDG uptake in the bilateral pleural effusion.Mediastinoscopy was done and biopsies showed an overtly malignant, epithelioid neoplasm with foci of vaso-formation; Keeping with high-grade epithelioid angiosarcoma of the pericardium.She received six cycles of weekly paclitaxel, but imaging for abdominal pain incidentally showed evidence of metastasis to the liver and spine so she was switched to Adriamycin-Ifosfamide for which she received one cycle so far.Her hospital course was complicated by high-output pleural effusions, chylothorax, left atrial thrombus formation and an intensive care unit stay for septic shock. Conclusion: Pericardial epithelioid angiosarcoma has been reported rarely in the literature. The authors aim to report a case of extensive metastatic pericardial epithelioid angiosarcoma in a young patient; which we believe can be an addition to the literature of a malignancy associated with poor prognosis and no definitive proven treatment regimen.

Discussion

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Cite This Study

Sakan et al. (2024) conducted a case report in Pericardial epithelioid angiosarcoma (n=1). Paclitaxel followed by doxorubicin-ifosfamide chemotherapy, pericardiocentesis, and bilateral pleural drainage was evaluated. Pericardial epithelioid angiosarcoma in a 25-year-old female progressed to liver and spine metastases despite 6 cycles of weekly paclitaxel, with 2-5 L/day chylothorax and survival to 7 months.

synapsesocial.com/papers/6a8090b56a1c77b118d492e9 https://doi.org/10.1097/ms9.0000000000002521

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A unique presentation of pericardial epithelioid angiosarcoma with multifaceted complications

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

A unique presentation of pericardial epithelioid angiosarcoma with multifaceted complications

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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