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

Abstract

Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent, endothelial neoplasm of low‐grade malignancy, often mimicking myoid and epithelioid tumors histologically. It is more frequent in young adult males and it usually presents with multiple cutaneous nodules, mostly localized at the extremities. It traverses several tissue planes simultaneously and can involve dermis, subcutis, skeletal muscle, and bone. Histologically, it is characterized by plump spindle cells with eosinophilic cytoplasm, often arranged in fascicles and epithelioid cells with “pseudomyogenic” morphology. Immunohistochemically, PMH is positive for Factor VIII, FLI‐1, INI‐1, vimentin, MDM2, CDK4, CD31, AE1/AE3, EMA, and P63. The efficacy of treatments is only partially known. Because of the frequent multifocal aspect of PMH, which contraindicates surgery, systemic treatments, such as gemcitabine, sirolimus, and everolimus are used. Based on our observation of multifocal PMH of the foot in a 17‐year‐old male patient, treated with gemcitabine with complete cutaneous response in a 2‐year follow‐up, we decided to discuss this rare tumor and underline its progression and therapeutic approaches. Thanks to a correct diagnosis, it is possible to avoid aggressive therapeutic approaches, which would be necessary for nonindolent diseases, such as sarcoma, which often needs amputation.

Pseudomyogenic hemangioendothelioma – A rare vascular tumor

“…1 It has a proclivity for younger males and lower limbs but can be found in the upper limbs, trunk, head or neck. 2 It usually displays indolent behavior but, like our case, aggressive metastases are reported. 3 Pseudomyogenic hemangioendothelioma can be diagnostically challenging and confused with sarcomatous tumors or other disorders, as noted in our patient.…”

Childhood Vascular Tumors

“…Histologically, the tumor is composed of sheets and cords of spindled cells with eosinophilic cytoplasm. Despite the resemblance of myoid cells, the tumor cells stain negative for desmin and positive for endothelial markers (45,49,50). Treatment of PHE is often determined by the size and location of the tumor; surgical excision is usually the treatment of choice, but given the propensity for PHEs to be multifocal, surgery may not be an option.…”

“…Additionally, one third of patients have recurrence after surgical excision (51). In such cases, medical management with gemcitabine, sirolimus, and everolimus have been used successfully (50)(51)(52). Given the rarity and recent discovery of PHE, clinical trials have not yet been conducted, so further research is needed in medical management treatment options.…”

A rare case of pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) of the breast first misdiagnosed as metaplastic carcinoma by FNAB and review of the literature

“…Different systemic therapies (Table 1) have been described in case reports in the literature. Among them, inhibitors of mammalian target of rapamycin (mTOR) show major efficiency [1, 11–13, 20, 21] in cases of progressive metastatic and relapsing multifocal PHE/ESH resistant to multiagent chemotherapy. mTOR, a serine/threonine kinase regulated by phosphoinositide-3-kinase (PI3K), acts as a master switch for numerous cellular processes, such as cellular catabolism and anabolism, motility, angiogenesis and growth.…”

“…In 2013, Pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) has been accepted as a new vascular fumor entity by WHO. PHE/ESH is a distinct, uncommon tumor with an endothelial phenotype that usually arises in soft tissue, and its biological behavior is intermediate between a benign hemangioma and a fully malignant angiosarcoma [1]. Histologically, PHE/ESH is characterized by ill-defined nodules of plump spindle and epithelioid cells with abundant densely eosinophilic cytoplasm that grow in sheets and fascicles [2].…”