Pseudomyogenic hemangioendothelioma/epithelioid sarcoma-like hemangioendothelioma of the lower limb: report of a rare case

Abstract

Pseudomyogenic hemangioendothelioma is an extremely rare soft tissue tumor, also named as epithelioid sarcoma-like hemangioendothelioma, which occurs more frequently in young adult males. It was originally recognized as a variant of epitheloid sarcoma, however it is now concluded as a distinctive, rarely metastasizing endothelial neoplasm. We present a case of pseudomyogenic hemangioendothelioma in the lower limb in a 49-year-old female who has a long course of disease and suffered from twice local recurrences and lymph node affection of the tumor. The mass was subcutaneous and the margins were ill-defined. Morphologically, the tumor cells show diversity, composed of large spindle cells and round cells, both with abundant eocinophilic cytoplasm, mimicking rhybdomyoplasts and epitheloid cells respectively. The tumor cells show diffuse strong expression of Factor VIII, Fli-1, INI-1, vimentin, MDM2, and CDK4, local expression of CD31, AE1/AE3, EMA and P63, and no expression of CD34, S-100, actin-sm, desmin, MyoD1, and HMB45. Based on these information, this case is diagnosed as pseudomyogenic hemangioendothelioma after ruling out the main differential diagnosises including epithelioid sarcoma, malignant peripheral nerve sheath tumor and rhabdomyosarcoma. From this case we suggest that pseudomyogenic hemangioendothelioma may be confused with a variety of soft tissue neoplasm histologically. The clinical feature of the case of a long course of disease with twice local recurrences and final lymph node involvement 10 years after excision of the primary tumor indicates a relative indolent behavior of this tumor.

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

“…PMH has been included in 2013 in the WHO classification of soft tissue and bone tumors (Fletcher, Bridge, Hogendoorn, & Mertens, ). It occurs mainly in patients between 20 and 50 years of age, more frequently in male sex and it is usually localized at the extremities (Fan et al, ). As reported by Hornick and Fletcher, in their 50 cases the 82% of patients were under the age of 40 years and there was a 4.6:1 male predominance.…”

“…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, ).…”

“…It shows a predilection for young male patients and it is usually localized at the distal extremities (Fan, Yang, Lin, & Wang, ; Hornick & Fletcher, ).…”

“…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.…”

Long period of relative quiescence in distal-type epithelioid sarcoma of the forearm with recurrence after surgery

“…The tumor samples were examined via hematoxylin-eosin (HE) and immunohistochemistry staining as described previously. [ 9 ] Figure 2 shows the histopathological features of the tumor. The tumor cells of the samples obtained during the first surgery were arranged in irregularly cell nests (Fig.…”