Retiform hemangioendothelioma presenting as bruise‐like plaque in an adult woman

Abstract

A 55-year-old woman presented with a 9-week history of a 0.9 × 1.2-cm symptomless, solitary, slowly enlarging bruiselike plaque located on her left middle finger. One week later, the skin over the affected area became centrally eroded and thus attributed to mechanic trauma (Fig. 1). Her previous medical history included arterial hypertension and resection of nonfunctioning adrenocortical neoplasm (incidentaloma) since 5 months. Laboratory examinations, including blood cell counts, urinalysis, routine blood chemistry, immunologic investigations and endocrine screening, were within normal range or negative. X-rays of the left hand were normal. There was no clinical evidence of regional lymph node involvement. Because of the history of adrenocortical neoplasm, computed tomography (CT) scans of the chest and abdomen showed no evidence of metastatic disease. A presumptive clinical diagnosis of cutaneous angiosarcoma was suspected.Histologic examination of the biopsy specimen revealed vascular proliferation within the dermis, composed of arborizing vessels lined by monomorphic, protuberant, hobnail-like endothelial cells (Fig. 2). Focal solid areas consisting of bland, mostly spindle, cells sometimes investing minute papillae with hyaline-like collagenous cores were also seen. Cellular atypia and mitotic activity were unremarkable. Immunostaining against CD31 decorated most endothelial cells, as well as a large number of those within the solid areas (Fig. 3). Histological diagnosis of retiform hemangioendothelioma was established. A subsequent surgical excision with histologically tumor-free margins was achieved. The patient has been followed up closely for 4 years with no recurrence or metastases of both neoplasms.

Medial canthus retiform hemangioendothelioma

“…[1] RH also stains for factor VIII-related antigen and bound UEA. [9] The lymphocytes within the stroma are positive for pan-T cell markers and a minority of lymphocytes stain for CD20 (B cell marker). [1]…”

Retiform hemangioendothelioma in the infratemporal fossa and buccal area: a case report and literature review

“…To date, 29 papers and 48 RH cases have been reported, including this case. (Table 1)12345678910111213141516171819202122232425262728 There has been a female predominance (female:male, 32:16), and the patients were predominantly young and middle-aged adults in 2005. However, in this review, patients under 20 years represented nearly 30% of the sample, and patients over 50 years accounted for nearly 20%.…”

Unresectable Retiform Hemangioendothelioma Treated with External Beam Radiation Therapy and Chemotherapy: A Case Report and Review of the Literature

“…Recurrences are observed ranging from months to several years after original presentation. Etiology is unknown; however, an association between RH and human herpes virus type 8 [6], lymphedema [1], previous radiotherapy [1, 6], and noncutaneous malignant neoplasms [1, 6, 7, 11] has been reported but not clearly established. …”

“…Some areas of the tumor are solid and are composed of epitheloid or spindle cells and dilated vascular channels sometimes with intraluminal papillary projections. Cytologic atypia is minimal and few or no mitotic figures are seen [1, 7]. Immunohistochemically, neoplastic cells are positive for CD 31, CD 34, factor VIII-related antigen, and bound Ulex earopaeus agglutinin (UEA) (7).…”

“…Other vascular tumors that morphologically can present similarly to RH and need to be differentiated from RH include Dabska's tumor, lymphoma, dermatofibrosarcoma protuberans, hemangioma, bacillary angiomatosis, cutaneous metastases, blue-rubber bleb nevus syndrome, Kaposi's sarcoma, targetoid hemosiderotic hemangioma, hobnail hemangioma, epitheloid hemangioendothelioma, and polymorphous hemangioendothelioma of lymph node [1, 2, 7, 9]. Even though some histological overlap exists, each of the neoplasms has their unique features [1, 2, 8].…”