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A 26-year-old female patient presented with a 2 year history of asymptomatic erythematous nodules on the left side of the back. She denied having fever, fatigue, chills or weight loss. Physical examination demonstrated multiple reddish nodules coalesced into an irregular plaque and several satellite nodules on the periphery involving the surface of her back (Fig. 1). The findings of systemic reviews were unremarkable, and there was no lymphadenopathy. The complete blood cell count revealed a normal white blood cell count. The biopsy from an erythematous nodule showed vascular structures in a retiform pattern involving the superficial and deep dermis, resembling rete testis. The vascular channels were lined by monomorphic cells with hobnail endothelial cells. No obvious nuclear atypia was present nor were mitotic figures. The tumour also showed solid areas composed of spindle cells and epithelioid cells (Fig. 2A, B). The immunohistochemistry revealed the tumour cells were positive for CD31 and ERG (Fig. 3A, B), and negative for S-100, SOX10, SMA, CK-pan, CK8/18, Desmin, HHV8, CD34 and D2-40. The Ki-67 labeling index was 15%.
Fig. 2. The pathological slice of the nodule showed that vascular structures in a retiform pattern involving the superficial and deep dermis, resembling rete testis.
The vascular channels were lined by monomorphic cells with hobnail endothelial cells. The tumour also showed solid areas composed of spindle cells and epithelioid cells (HE staining, A, B:200×).
Retiform hemangioendothelioma (RH) is an exceptionally rare, low-grade malignant vascular neoplasm first characterized by Calonje et al. (1). With biologic behaviour intermediate between benign hemangiomas and aggressive angiosarcomas, RH exhibits a propensity for local recurrence but seldom metastasizes. Clinically, RH most often presents as a slow-growing, exophytic dermal or subcutaneous tumour appearing as plaques, nodules or masses – predominantly affecting the extremities, with a distinct predilection for the lower limbs (1–4). A bruise-like appearance may be observed (1, 2). While predominantly affecting young to middle-aged women (5) as solitary tumours, our case underscores the documented occurrence of multiple lesions (6–9).
Histologically, RH features a distinctive retiform network of arborizing vessels lined by monotonous hobnail endothelial cells devoid of atypia or mitoses. Immunohistochemically, CD31 remains the most reliable immunohistochemical marker. Our case showed the tumour cells were positive for CD31 and ERG, aligning with prior reports (10).
Clinicopathological differential diagnosis of RH includes angiosarcoma, Dabska tumour and Kaposi hemangioendothelioma (1, 3, 9). Angiosarcoma is charactered by obvious nuclear atypia and mitotic figures. Dabska tumour often occurs in children and consists of papillary endothelial projections. Kaposi hemangioendothelioma has glomeruloid structures alternating with slit-like spaces containing erythrocytes. The recommended treatment for RH includes surgical excision with tumour-free margins (11). This patient received Mohs micrographic surgery. Besides, adjuvant radiotherapy has been effectively chosen in RH that had been deemed unresectable or those having regional lymph node metastasis (5).
This case illustrates the diagnostic challenges posed by multifocal RH presenting on a young female’s back – an unusual anatomical location. These rare presentations require meticulous clinical evaluation to prevent misclassification of such infrequent tumours in atypical sites.
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