Rapidly Progressive and Debilitating Epithelioid Hemangioendothelioma: An Atypical Presentation of a Rare Malignant Cutaneous Vascular Neoplasm.

ABSTRACT: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor that comprises less than 1% of all vascular tumors. Cutaneous involvement in EHE can occur either by spreading from underlying bone or rarely could be limited to the skin and mostly presents as solitary well-circumscribed mass to an ill-defined infiltrative lesion. We present a case of rapidly progressive and debilitating EHE presenting multiple vascular papules and nodules. Histopathology showed an ill-circumscribed nodular proliferation of epithelioid and spindled cells in the dermis that extended into the subcutaneous tissue. The tumor cells had moderate eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. In addition, they showed evidence of lumen formation and intracytoplasmic vacuoles. Brisk mitosis was noted. On immunohistochemistry, the cells were strongly positive for CD31, CD34, and ERG (ETS [erythroblast transformation-specific]-related gene). MIB-1 labeling index was more than 75% in the highest proliferating areas. A high degree of clinical suspicion and immunopathological examination is recommended for early diagnosis of this rare condition before it becomes function or life-threatening.

Dermoscopic Analysis of Vascular Malformations and Tumors Based Upon Dominant Vascular Dermoscopic Features: A Retrospective Analysis From a Tertiary Care Center of East India.

Background Cutaneous vascular malformations and tumors comprise a vast group of conditions with variable clinical presentations. It is imperative to differentiate them from nonvascular lesions and from each other as their management and prognosis differ significantly. There is only sparse literature on dermoscopic features of various vascular malformations and tumors, especially from India. Aim We aimed to retrospectively study the dermoscopic findings of various vascular malformations and tumors based on their dominant vascular dermoscopic feature. Method All the vascular malformations and tumors for which clinical details and clinical and dermoscopic images were available were included in the analysis. The dominant vascular feature(s) was defined as a single or combination of two or more vascular features (in case a single vascular feature does not satisfy the criteria) that constitute more than 75% of the lesions' vascular features. These included red, purple, blue, black (or any combination) dots, globules, lacunae, structureless area, linear, linear irregular, hairpin, comma, and arborizing vessels. Results A total of 52 patients with 68 vascular lesions (22 vascular malformations and 46 vascular tumors) were analyzed. Port-wine stain showed linear irregular vessels with sharp border with or without intervening white structureless area; unilateral nevoid telangiectasia had red dots and globules; angiokeratoma displayed red, reddish-purple to brown lacunae; blue color was seen in venous and glomuvenous malformation and venous lake; a mixed pattern was noted in infantile hemangioma and verrucous hemangioma; a red to reddish-white structureless area was observed in pyogenic granuloma and cherry angioma, and a subungual ill-defined pink structureless area was spotted in subungual glomus tumor. Conclusion The dermoscopic features observed in various vascular lesions may overlap; however, the dominant dermoscopic feature along with its color may point to the diagnosis.