Epithelioid Hemangioendothelioma of Skin and Soft Tissues: Clinicopathologic and Immunohistochemical Study of 30 Cases

Abstract

Epithelioid hemangioendothelioma of soft tissues (EHE) represents a distinct entity with an unpredictable clinical course. We analyzed the clinicopathologic and immunohistochemical features in a series of 30 patients. Patient age range was 16-74 years (median 50); 18 of 30 patients were female. Eight tumors arose in the lower and two in the upper extremities, seven on the trunk, five each in the head/ neck and anogenital regions, two in the mediastinum, and one in the abdomen. Seventeen neoplasms were located in deep soft tissues, nine were subcutaneous or perifascial, and four were dermal; size ranged from 0.4 to 10 cm; in 11 cases the tumor was > 5 cm. Tumors with an infiltrative growth pattern were more common than entirely circumscribed lesions. The tumors were composed histologically of short strands, cords, or small clusters of epithelioid, round, to slightly spindled endothelial cells that formed at least focally, intracellular lumina and were set in a frequently myxohyaline stroma. Thirteen of 30 lesions showed angiocentric growth, which was occlusive in many cases. Immunohistochemically, all cases tested were positive for at least one endothelial marker (CD31, CD34, factor VIII, Ulex europaeus), six of 23 (26%) were positive for cytokeratin, and five of 11 (45%) were positive for alpha-smooth muscle actin. Median follow-up of 36 months (range 2-96) in 24 cases showed local recurrence in three cases and systemic metastases in five cases (21%); four patients (17%) died of tumor. Although more aggressive histologic features (striking nuclear atypia in eight cases, numerous spindled cells in 10, more than two mitoses per 10 high-power fields in nine, and small, more solid angiosarcomalike foci in four cases) tended to be related to poor clinical outcome, there was no clear correlation. Two metastasizing cases showed no histologically atypical features whatever. We suggest that EHE of soft tissue is better regarded as a fully malignant, rather than borderline, vascular neoplasm, albeit the prognosis is better than in conventional angiosarcoma.

Malignant vascular tumors—an update

“…However, 20-30% of tumors metastasize, and about 15% of patients die of their disease. 4,15 This contrasts with the behavior of most angiosarcomas. Overtly malignant lesions typically pursue an aggressive course.…”

Epithelioid Hemangioendothelioma of the Maxillary Sinus

“…1 Axial, non-contrast, CT scan demonstrating a complex mass filling the right maxillary sinus with erosion of the medial (arrow) and posterior maxillary sinus walls (asterisk) multi-organ disease [11,12]. Mentzel et al [12] noted 20 % of tumors metastasized and 17 % of patients died in their study. In our patient, the clinical history of a 1-year period of symptoms and radiographic findings of bony erosion and remodeling without soft tissue extension beyond the maxillary sinus suggests a low grade, slowly evolving process.…”

“…This results in an unpredictable, yet oftentimes, indolent clinical course, and patients can survive for decades even with Fig. 1 Axial, non-contrast, CT scan demonstrating a complex mass filling the right maxillary sinus with erosion of the medial (arrow) and posterior maxillary sinus walls (asterisk) multi-organ disease [11,12]. Mentzel et al [12] noted 20 % of tumors metastasized and 17 % of patients died in their study.…”

Myopericytoma of the Distal Forearm: A Case Report

“…Considering the location of the mass, we selected incisional biopsy to avoid potential nerve and vessel injury. The pathological differential diagnos i s o f m y o p e r i c y t o m a i n c l u d e s m y o f i b r o m a , angioleiomyoma, and glomus tumor because of histologic overlap [13]. On microscopic examination, myopericytomas contain elongated and ectatic blood vessels concentrically surrounded by spindle myoid tumor cells with eosinophilic cytoplasm.…”