Case Reports

Pseudomyogenic Hemangioendothelioma

Author and Disclosure Information

Pseudomyogenic hemangioendothelioma (PMHE) is a rare vascular tumor that was added to the World Health Organization classification of soft tissue tumors. These tumors have a unique clinical presentation and microscopic appearance as compared to other vascular tumors in the differential diagnosis. Unlike its microscopic mimicker epithelioid sarcoma, PMHE rarely metastasizes and long-term survival in affected patients is excellent. In this report, we present a patient with PMHE and review the current literature on clinical presentation and histologic differentiation of this unique tumor, comparing findings to its mimickers.

Practice Points

  • Pseudomyogenic hemangioendothelioma (PMHE) is an uncommon vascular tumor that most often presents as multiple distinct nodules on the legs in young men.
  • Pseudomyogenic hemangioendothelioma has an unusual immunohistochemistry staining pattern, with positive staining for cytokeratin AE1/AE3, CD31, and ERG but negative for CD34.
  • Although local reoccurrence is common, PMHE metastasis is very uncommon.



Pseudomyogenic hemangioendothelioma (PMHE), also referred to as epithelioid sarcoma–like hemangioendothelioma,1 is a rare soft tissue tumor that was described in 1992 by Mirra et al2 as a fibromalike variant of epithelioid sarcoma. It predominantly affects males between the second and fifth decades of life and most commonly presents as multiple nodules that may involve either the superficial or deep soft tissues of the legs and less often the arms. It also can arise on the trunk. We present a case of PMHE occurring in a young man and briefly review the literature on clinical presentation and histologic differentiation of this unique tumor, comparing these findings to its mimickers.

Case Report

A 20-year-old man presented with skin lesions on the left leg that had been present for 1 year. The patient described the lesions as tender pimples that would drain yellow discharge on occasion but had now transformed into large brown plaques. Physical examination showed 4 verrucous plaques ranging in size from 1 to 3 cm with hyperpigmentation and a central crust (Figure 1). Initially, the patient thought the lesions appeared due to shaving his legs for sports. He presented to the emergency department multiple times over the past year; pain control was provided and local skin care was recommended. Culture of the discharge had been performed 6 months prior to biopsy with negative results. No biopsy was performed on initial presentation and the lesions were diagnosed in the emergency department clinically as boils.

Figure 1. Verrucous plaques involving the anterior and medial area of the left knee.

After failing to improve, the patient was seen by an outside dermatologist and the clinical differential diagnosis included deep fungal infection, atypical mycobacterial infection, and keloids. A 4-mm punch biopsy was taken from the periphery of one of the lesions and demonstrated hyperkeratosis, papillomatosis, and acanthosis (Figure 2). Within the superficial and deep dermis and focally extending into the subcutaneous tissue, there were sheets of spindled to epithelioid-appearing cells with moderate cytologic atypia (Figure 3). The tumor showed infiltrative margins. There was moderate cellularity. The individual cells had a rhabdoid appearance with large eccentric vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm (Figure 4). No definitive evidence of glandular, squamous, or vascular differentiation was present. There was an associated moderate inflammatory host response composed of neutrophils and lymphocytes. Occasional extravasated red blood cells were present. Immunohistochemistry staining was performed and the atypical cells demonstrated diffuse positive staining for friend leukemia integration 1 transcription factor (FLI-1), erythroblastosis virus E26 transforming sequence-related gene (ERG)(Figure 5), CD31, and CD68. There was patchy positive staining for cytokeratin AE1/AE3, CD10, and factor VIII. There was no remarkable staining for human herpesvirus 8, epithelial membrane antigen, S-100, CD34, cytokeratin 903, and desmin. Overall, the histologic features in conjunction with the immunohistochemistry staining were consistent with a diagnosis of PMHE.

Figure 2. The epidermis demonstrated hyperkeratosis, papillomatosis, and acanthosis. Within the dermis there was a moderately cellular proliferation of tumor cells (H&E, original magnification ×20).

Figure 3. Moderately cellular proliferation of spindled to epithelioid-appearing cells within a fibrous stroma (H&E, original magnification ×40).

Figure 4. The tumor cells had moderate cytologic atypia with vesicular nuclei and small nucleoli. There was an associated inflammatory host response (H&E, original magnification ×100).

Figure 5. Diffuse positive immunoperoxidase staining for erythroblastosis virus E26 transforming sequence-related gene, ERG, supported the vascular origin of the tumor (original magnification ×40).

Magnetic resonance imaging was then performed to evaluate the depth and extent of the lesions for surgical excision planning (Figure 6), which showed 5 nodular lesions within the dermis and subcutis adjacent to the proximal aspect of the left tibia and medial aspect of the left knee. An additional lesion was noted between the sartorius and semimembranosus muscles, which was thought to represent either a lymph node or an additional neoplastic lesion. Chest computed tomography also displayed indeterminate lesions in the lungs.

Figure 6. Magnetic resonance imaging showed 1 isointense to muscle lesions (red arrow) in the skin and subcutaneous tissue. Additional lesions were present in different sections.

Excision of the superficial lesions was performed. All of the lesions demonstrated similar histologic changes to the previously described biopsy specimen. The tumor was limited to the dermis and subcutaneous tissue. The patient was lost to follow-up and the etiology of the lung lesions was unknown.


Next Article: