Diagnostic Pitfall in Cardiac Angiosarcoma: Initial Misdiagnosis as Masson Tumor Due to Sampling of Necrotic Tissue

Background and Clinical Significance: Cardiac and mediastinal angiosarcomas are rare, aggressive malignancies that often present with nonspecific symptoms and pose significant diagnostic challenges. Tumor heterogeneity and necrosis may lead to false-negative biopsy results; Case Presentation: We report a 64-year-old man who initially presented with cardiac tamponade of unclear etiology. Despite an extensive workup, the patient remained asymptomatic for five months before re-presenting with dyspnea and a large mediastinal mass compressing the right heart, along with a lytic rib lesion. Initial ultrasound-guided biopsy of the rib lesion demonstrated a benign vascular proliferation consistent with Masson tumor (intravascular papillary endothelial hyperplasia), which was discordant with aggressive imaging findings. Further evaluation with positron emission tomography–computed tomography (PET-CT) revealed peripheral metabolic activity, and cardiac magnetic resonance imaging (MRI) demonstrated a heterogeneous mass with central necrosis and peripheral enhancement. A repeat CT-guided biopsy targeting the metabolically active region confirmed angiosarcoma, with immunohistochemical staining demonstrating diffuse positivity for ERG, CD31, and CD34. The patient was treated with palliative radiation and paclitaxel-based chemotherapy but experienced rapid clinical decline and transitioned to comfort-focused care; Conclusions: This case highlights the importance of correlating imaging with pathology and emphasizes the risk of sampling error in necrotic tumors. PET-guided biopsy targeting viable tumor regions is essential in cases with discordant findings.

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Publication Details

Journal
Reports — Medical Cases Images and Videos
Published
2026-06-25
DOI
https://doi.org/10.3390/reports9030201
Primary Topic
Vascular Tumors and Angiosarcomas
Type
article
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article

Diagnostic Pitfall in Cardiac Angiosarcoma: Initial Misdiagnosis as Masson Tumor Due to Sampling of Necrotic Tissue

Hibba tul Rehman, Mahyar Toofantabrizi, Hasan Obeidat, Sarah J. Silva et al.
Reports — Medical Cases Images and Videos
Vascular Tumors and Angiosarcomas
article

Diagnostic Pitfall in Cardiac Angiosarcoma: Initial Misdiagnosis as Masson Tumor Due to Sampling of Necrotic Tissue

Hibba tul Rehman, Mahyar Toofantabrizi, Hasan Obeidat, Sarah J. Silva, Katie Li
article en

Abstract

Background and Clinical Significance: Cardiac and mediastinal angiosarcomas are rare, aggressive malignancies that often present with nonspecific symptoms and pose significant diagnostic challenges. Tumor heterogeneity and necrosis may lead to false-negative biopsy results; Case Presentation: We report a 64-year-old man who initially presented with cardiac tamponade of unclear etiology. Despite an extensive workup, the patient remained asymptomatic for five months before re-presenting with dyspnea and a large mediastinal mass compressing the right heart, along with a lytic rib lesion. Initial ultrasound-guided biopsy of the rib lesion demonstrated a benign vascular proliferation consistent with Masson tumor (intravascular papillary endothelial hyperplasia), which was discordant with aggressive imaging findings. Further evaluation with positron emission tomography–computed tomography (PET-CT) revealed peripheral metabolic activity, and cardiac magnetic resonance imaging (MRI) demonstrated a heterogeneous mass with central necrosis and peripheral enhancement. A repeat CT-guided biopsy targeting the metabolically active region confirmed angiosarcoma, with immunohistochemical staining demonstrating diffuse positivity for ERG, CD31, and CD34. The patient was treated with palliative radiation and paclitaxel-based chemotherapy but experienced rapid clinical decline and transitioned to comfort-focused care; Conclusions: This case highlights the importance of correlating imaging with pathology and emphasizes the risk of sampling error in necrotic tumors. PET-guided biopsy targeting viable tumor regions is essential in cases with discordant findings.

Reports — Medical Cases Images and VideosVol. 9(3)
University of Maryland, Baltimore (US), University of Maryland Medical Center (US), Georgetown University (US), MedStar Franklin Square Medical Center (US), MedStar Georgetown University Hospital (US), MedStar Health (US)
Good health and well-being
Openalex Percentile: Top 8%
Vascular Tumors and Angiosarcomas
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