Cutaneous Epithelioid Sarcomalike (Pseudomyogenic) Hemangioendothelioma

Abstract

We recommend adding the low-grade neoplasm ES-H to the large list of cutaneous vascular proliferations. Dermatologists should be aware of this low-grade cutaneous vascular tumor.

FOSB immunoreactivity in endothelia of epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia)

“…It must be pointed out that FOSB immunoreactivity is not specific for EH. As stated above, PHE is defined by a recurrent Positive for either CAMTA1 27,42 or TFE3 35,42,43 ; negative for FOSB 29 Often positive for D2-40/podoplanin 43 ; MYC-negative; INI1 preserved 43,44 Angiosarcoma (AS) Negative for FOSB 29 , CAMTA1 27,42 Often co-expressing D2-40/podoplanin and c-MYC 38,39 Epithelioid angiosarcoma (EAS) Negative for FOSB, 27,29 CAMTA1 27,42 Often (focal) co-expression of cytokeratin and EMA 45,46 ; ERG-positive 47 Pseudomyogenic/epithelioid sarcoma-like hemangioendothelioma (PHE) 36 Co-expression of FOSB 27,29 and AE1/AE3 27,48 Negative for pan-cytokeratin MNF116 49 ; INI1 preserved 48,49 Epithelioid sarcoma (ES) INI1 (SMARCB3)-negative 44,50 Mostly ERG-negative 47 (ERG-expression extremely rare); negative for CAMTA1 27,42 , FOSB 27,29 Cutaneous epithelioid angiomatous nodule Negative for FOSB 29 This study adds to previous evidence of the oncogenic role of FOSB in the pathogenesis of endothelial neoplasms, such as EH and PHE.…”

The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature

“…On the other hand, there is not expression of CD34, S‐100, smooth muscle actin, desmin, MyoD1, and HMB45 (Fan et al, ; Rawal, Anderson, & Dodson, ; Requena et al, ).…”

“…Immunohistochemical staining of the tumor indicated positivity for vimentine and cytokeratin (Figure d). Most importantly, it showed a characteristic expression of INI‐1 (Requena, Santonja, Martinez‐Amo, Saus, & Kutzner, ). It resulted negative for desmin and s100, with no myogenic differentiation.…”

“…Nuclear atypia can be mild to moderate. In some cases a neutrophilic inflammatory infiltrate can be observed.Immunohistochemically, tumor cells show diffuse strong expression of Factor VIII, FLI-1, INI-1, vimentin, MDM2, CDK4, local expression of CD31, cytokeratin AE1/AE3, EMA, and P63.On the other hand, there is not expression of CD34, S-100, smooth muscle actin, desmin, MyoD1, and HMB45(Fan et al, 2015;Rawal, Anderson, & Dodson, 2017;Requena et al, 2013).Vimentin has a low-grade specificity and for this reason is no longer relevant(Rawal et al, 2017).Diagnosis of PMH is based on the peculiar clinical presentation, histological examination, and demonstration of the expression of both keratins and endothelial markers by immunohistochemistry.Molecular genetics of PMH is unknown: Trombetta et al described a balanced translocation between Chromosomes 7 and 19 [t(7;19) (q22;q13)] as a unique clonal change(Trombetta et al, 2011). In a recent study, Hung et al describes a new useful diagnostic marker for PMH.…”

Diagnostically Challenging Epithelioid Vascular Tumors

“…[51][52][53] Surgical resection is the treatment of choice. However, in some cases, the multifocal nature of the tumor may be quite widespread.…”