Cutaneous Vascular Neoplasms of Uncertain Biological Behavior

Abstract

Neoplasms of uncertain biological behavior present physicians with a genuine conundrum in practice. Cutaneous vascular neoplasms within this category are exceedingly rare, possessing significant gaps and uncertainty in many facets of clinical practice. Firstly, lesions were selected for review based on their categorization as indeterminate behavior, indicating the potential for local recurrence and rarely metastasize. After identification of the target lesions, a comprehensive review of the literature using national databases produced several landmark studies and case series regarding these neoplasms. Limiting the review to only cutaneous limited tumors narrowed the pool of studies; however, quite a large sum of papers remained. Examination of each paper yielded beneficial results on diagnosing, effective treatments, follow-up findings, and prognosis for each indeterminate lesion discussed. Overall, the literature search combined the molecular, histologic, immunohistochemical, surgical strategies to develop an up-to-date and comprehensive framework to guide physicians when encountering such lesions. The tumors reviewed include: kaposiform hemangioendothelioma, endovascular papillary angioendothelioma, pseudomyogenic hemangioendothelioma, retiform hemangioendothelioma, epithelioid hemangioendothelioma, and composite hemangioendothelioma.

Multiply Recurrent Composite Hemangioendothelioma of Penis with Histologic Progression to High-Grade Features

“…Therefore, the patients must be followed closely. The first-line treatment is wide local surgical excision [ 10 ].…”

“…Similar to CHE, RHE shows reactivity with CD31, CD34, FLI-1, EGR, and podoplanin (D2–40) immunohistochemistry, and it does not express GLUT1 and HHV8. RHE also has a very low risk of metastasis, but it has a high rate of local recurrence [ 10 ].…”

“…In contrast, the patients with AS and EHE receive radiation and/or chemotherapy in addition to the resection. Differentiating CHE from AS can be challenging as CHE very often contains angiosarcomatous and/or benign angiomatous components [ 1 , 10 ]. Shon et al reported 23/38 cases of primary cutaneous AS expressing cMYC by IHC and showing a cMYC gene rearrangement by FISH [ 11 ].…”

Hemangioendotelioma Retiforme en Tórax: reporte de un caso

“…Generalmente mal delimitada, puede tener una base subcutánea o dérmica. Son generalmente asintomáticos, tiene una recurrencia local frecuente de hasta un 60%, rara vez hace metástasis 7,8 . Se ha reportado metástasis hacia ganglios linfáticos, donde el tumor se encontraba presente en patrón bifásico y solo el componente en forma de huso hizo metástasis.…”

“…El diagnóstico diferencial se realiza con: Tumor Dabska (angioendotelimoa papilar endovascular) donde es más difícil de distinguir y el diagnóstico se basa en la edad de presentación donde el tumor de Dabska es más frecuente en niños y el hemangioendotelioma retiforme en adultos 3,7 .…”