AME case reports · 2025
Pulmonary epithelioid hemangioendothelioma: a case report and review of literature.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
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Atuiri Clifford, Li Xiao, Abdul-Nabi Anmaar, Rana Meera
Аннотация
BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a vascular tumor of unpredictable clinical behavior, representing a diagnostic and therapeutic challenge due to its rarity and complex presentation. This case report highlights a unique presentation of pulmonary EHE in a 69-year-old female and provides a comprehensive review of the literature to enhance awareness and understanding of this condition.
CASE DESCRIPTION: A 69-year-old Caucasian female with a history of type 2 diabetes and hypertension presented with progressive chest pain and right-sided neck and shoulder discomfort. Imaging revealed a significant right pleural effusion and a superior mediastinal mass, raising suspicion for malignancy. Histopathological analysis confirmed EHE, with immunohistochemical positivity for CD31 and negative staining for CD34, supporting the diagnosis. The patient underwent surgical resection and vascular stenting, followed by targeted therapy with trametinib as per SARC033 trial protocols. A 4-month follow-up showed no evidence of disease progression.
CONCLUSIONS: The clinical ambiguity of EHE necessitates awareness and prompt action due to its potential for aggressive behavior and varied therapeutic responses. Early recognition, histopathological confirmation, and a multidisciplinary approach are essential for optimal management. While surgical resection is preferred for localized disease, targeted therapies show promise in advanced cases. Continued research is needed to refine treatment protocols and improve patient outcomes.
CASE DESCRIPTION: A 69-year-old Caucasian female with a history of type 2 diabetes and hypertension presented with progressive chest pain and right-sided neck and shoulder discomfort. Imaging revealed a significant right pleural effusion and a superior mediastinal mass, raising suspicion for malignancy. Histopathological analysis confirmed EHE, with immunohistochemical positivity for CD31 and negative staining for CD34, supporting the diagnosis. The patient underwent surgical resection and vascular stenting, followed by targeted therapy with trametinib as per SARC033 trial protocols. A 4-month follow-up showed no evidence of disease progression.
CONCLUSIONS: The clinical ambiguity of EHE necessitates awareness and prompt action due to its potential for aggressive behavior and varied therapeutic responses. Early recognition, histopathological confirmation, and a multidisciplinary approach are essential for optimal management. While surgical resection is preferred for localized disease, targeted therapies show promise in advanced cases. Continued research is needed to refine treatment protocols and improve patient outcomes.