Tumor > Malignant > Epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (Intravascular bronchoalveolar tumor)

Clinical:

Epithelioid hemangioendothelioma is a rare neoplasm. All age groups are affected, but 40% of patients are under 30 years of age [1]. Patients are usually asymptomatic or they may present with cough, chest pain, or weight loss [1]. Many tumors will behave in a benign fashion, whereas others are highly malignant. A poor prognosis is associated with airway, vascular, and pleural involvement [1].

The most characteristic and common finding is the presence of multiple, small (less than 2-3 cm in diameter), nodules in both lungs in a peribronchovascular distribution [1,2]. The nodules are usually well-circumscribed or slightly ill-defined [1]. This pattern is associated with the best prognosis [2].

Other patterns of involvement include a reticulonodular pattern, a parenchymal tumor with pleural invasion, and diffuse pleural thickening [3]. The reticulonodular pattern is characterized by multiple reticulonodular opacities associated with interlobular septal thickening and ground-glass opacities and this pattern can mimic lymphangitic metastatic disease or interstitial lung disease [3]. On histopathologic evaluation, the findings are due to infiltrating nodular proliferation of neoplastic cells within the lumens of small blood vessels and lymphatic vessels [3].

The parenchymal tumor (solitary or multiple) with pleural invasion is a less common manifestation, but it is associated with the worst prognosis [3].

The diffuse pleural thickening pattern is uncommon [3]. The thickening can extend along the fissures, be associated with a pleural effusion, and mimic mesothelioma or pleural metastatic disease [3].

Tumor wedge resection can be performed for patients with amenable tumor burdens [2]. Patients with slowly progressive disease may benefit from treatment with celecoxib because of it's antiangiogenic properties [2]. Sirolimus, a drug which targets the mammalian target of rapamycin, has also been used with some success [2].

REFERENCES:

(1) Radiographics 2002; Gimenez A, et al. Unusual primary lung tumors: a radiologic-pathologic overview. 22: 601-619

(2) AJR 2020; Jang JK, et al. A review of the spectrum of the imaging manifestations of epithelioid hemangioendothelioma. 215: 1290-1298

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