|Year : 2014 | Volume
| Issue : 1 | Page : 48-50
Clinical, radiologic, and pathologic analysis of penile epithelioid hemangioendothelioma: A diagnostic and management dilemma
Darshan P Patel1, Jeffrey D Redshaw1, Ting Liu2, Jeremy B Myers1, James M Hotaling1, William O Brant1
1 The Center for Reconstructive Urology and Men's Health, Division of Urology, Utah 84132, USA
2 Surgical Pathology, Department of Pathology, University of Utah, Utah 84132, USA
|Date of Web Publication||25-Jul-2014|
Darshan P Patel
Department of Surgery, Division of Urology, University of Utah Health Care 30 N 1900 E, RM 2B208B, Salt Lake City, UT 84132
Source of Support: None, Conflict of Interest: None
Epithelioid hemangioendotheliomas (EHE) are rare soft tissue tumors and only 17 cases involving the penis have been reported in the literature. These tumors are often incorrectly diagnosed as Peyronie's disease or superficial penile thrombosis. We present the case of a 59-year-old male referred to our institution with EHE after being incorrectly diagnosed with Peyronie's disease. A color Doppler ultrasound of the penis showed a highly vascular mass superficial to the tunica albuginea and a subsequent biopsy demonstrated EHE with low-grade features. Complete local excision was performed and given the patient's low-risk pathological features, he has been followed with only close surveillance. Early recognition of epithelioid vascular tumors is important and using adequate imaging modalities is critical to avoid prolonged periods of misdiagnosis.
Keywords: Epithelioid hemangioendothelioma, penis, vascular neoplasms
|How to cite this article:|
Patel DP, Redshaw JD, Liu T, Myers JB, Hotaling JM, Brant WO. Clinical, radiologic, and pathologic analysis of penile epithelioid hemangioendothelioma: A diagnostic and management dilemma. J Integr Nephrol Androl 2014;1:48-50
|How to cite this URL:|
Patel DP, Redshaw JD, Liu T, Myers JB, Hotaling JM, Brant WO. Clinical, radiologic, and pathologic analysis of penile epithelioid hemangioendothelioma: A diagnostic and management dilemma. J Integr Nephrol Androl [serial online] 2014 [cited 2019 Jul 15];1:48-50. Available from: http://www.journal-ina.com/text.asp?2014/1/1/48/137555
| Introduction|| |
Epithelioid vascular tumors are uncommon vascular neoplasms, mostly occurring in the soft tissues of the head and neck. Histological features of the tumor's neoplastic endothelial cells are used to characterize these lesions into three groups: Epithelioid hemangioma, epithelioid hemangioendothelioma (EHE), and epithelioid angiosarcoma. Less than 17 cases of EHE of the penis have been previously reported in the literature. These cases often have a prolonged history of missed or incorrect diagnoses.  We present a case of a 59-year-old male with EHE of the penis who was initially referred to our institution after a preliminary diagnosis of penile vein thrombosis versus atypical Peyronie's disease.
| Case report|| |
We report a case of a 59-year-old male with a painful, enlarging lump on the base of his penis for 5 months. The patient initially was seen by another urologist and diagnosed with penile vein thrombosis versus atypical Peyronie's disease. He was treated with antibiotics and ibuprofen for 2 weeks, but the mass continued to enlarge. He was then referred to our institution. On physical exam a firm, mobile, 1.0 cm × 1.0 cm mass at the base of his penis was noted. Color flow Doppler demonstrated a 1.25 cm × 0.3 cm vascular mass superficial to the tunica of the dorsal penis [Figure 1]. Given the concern for malignancy because of the abundant blood supply, excisional biopsy was performed [Figure 2]. A 1.3 cm × 0.5 cm mass was found at the base of the penis with one branch of the deep dorsal arteries supplying the mass. The mass was not adherent to surrounding structures and was completely resected.
|Figure 1: Ultrasound and echo color Doppler demonstrates a discrete highly vascular lesion superficial to the tunica|
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|Figure 2: Intra-operative picture showing vascular mass at the base of the penis|
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Histological examination of the mass revealed a highly cellular, vascular lesion. It was composed of a central thick-walled feeding vessel surrounded by sheets of epithelioid endothelial neoplastic cells, with abundant eosinophilic cytoplasm, plump vesicular nuclei, and inconspicuous nucleoli embedded in a sclerotic stroma. Intracellular red blood cells were also seen. There was no significant cytologic atyptia, increased mitotic activity, or tumor necrosis [Figure 3]. Areas of extravasated red cell and hemosiderin-filled macrophages were noted. Immunohistochemical staining demonstrated the epithelioid neoplastic cells to be strongly and diffusely positive for CD31 (Dako© , Carpinteria, CA, USA) and focally positive for FLI-1 (Santa Cruz Biotechnology© , Santa Cruz, CA, USA), but negative for CD34 (Ventana© , Tucson, AZ, USA) and D2-40 (Dako© , Carpinteria, CA, USA).
|Figure 3: (a) Anastomosing clusters of with mild pleomorphism without evidence of mitotic figures or necrosis. (b) Immuno-histochemical staining diffusely positive for CD31 consistent with an epithelioid vascular tumor|
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The mass was EHE based upon these morphologic features. The other possible diagnosis was epithelioid angiosarcoma, but there were no mitotic figures, or significant nuclear pleomorphism/atypia, which would be expected with this tumor. A computed tomography (CT) chest, CT abdomen/pelvis, magnetic resonance imaging (MRI) pelvis were done, which did not show any local residual tumor, intrapelvic lymph node involvement, or any distant metastatic disease. MRI of the pelvis at 3 months and 9 months did not demonstrate recurrent or metastatic disease.
| Discussion|| |
Epithelioid hemangioendothelioma of the penis was first described by Weiss and Enzinger in 1982. Less than 17 cases have been reported in the English literature.  EHE is an intermediate grade of the epithelioid vascular neoplasms based on its variable malignant potential. Vascular neoplasms have been sub-classified as epithelioid hemangioma, EHE, and epithelioid angiosarcoma based on histological features. Epithelioid hemangiomas consist of well-formed vascular channels in a nodular architecture lined by uniform epithelioid cells. These tumors do not show mitotic activity and necrosis, often seen with more aggressive epithelioid vascular tumors. Given the slow, local growth and a rare chance of reoccurrence and metastasis, local excision is the recommended treatment. At the other end of the spectrum is epithelioid angiosarcoma, characterized by the neoplastic cells arranged in sheets or solid growth pattern with considerable nuclear pleomorphism, atypia, mitotic figures, and tumor necrosis. These aggressive tumors have 85% local reoccurrence rate and 20-50% rate of metastasis, requiring wide local excision, lymph node dissection, adjuvant chemotherapy, and routine follow-up imaging monitoring for reoccurrence or metastasis.  Doxorubicin based chemotherapy has been successful at treating patients with epithelioid angiosarcoma.  Adjuvant radiation therapy has also been successful for control of residual local disease or to treat high-risk tumors after complete resection.
The patient in our case was diagnosed with EHE. This diagnosis is the rarest among the three epithelioid vascular tumors. Histologically, EHE consists of isolated aggregates of cells with minimal or mild pleomorphism and mitotic activity set within a hyaline matrix.  Although EHE has a 20-30% risk of metastasis, most previous reports have noted adequate tumor control with just local excision.  In cases of high-grade pathologic findings or metastasis, treatment with doxorubicin chemotherapy and/or adjuvant radiation may be needed.  In EHE, there is a 10% risk of local reoccurrence so patients must have very close surveillance after primary treatment.
Making the correct diagnosis of EHE is a challenge, as it can be incorrectly diagnosed as a more common cause of painful penile lesions. It is critical to diagnose EHE early because it can be a malignant tumor with potential for metastasis. The potential for incorrectly diagnosing these tumors is demonstrated in the literature. Previously, Gharajeh et al. described a case of EHE in a 42-year-old male, which clinically resembled a superficial vein thrombosis treated with wide local excision.  Natali et al. have reported a case of EHE in a 42-year-old man who initially was diagnosed and treated for Peyronie's disease by simple penile ultrasound. Continued symptomatology prompted color flow Doppler suggesting presence of a highly vascular lesion, that after local excision, was diagnosed as EHE. 
These cases highlight the importance of adequate diagnostic imaging when evaluating painful penile lesions. Color flow Doppler ultrasound was critical in uncovering the associated vascularity of the mass and increasing our suspicion of malignancy. In a previous report when diagnosis was delayed, the patient was treated for Peyronie's disease for 4 years, until a color flow Doppler ultrasound was performed, and the correct diagnosis of an epithelioid vascular tumor was made.  Clinicians evaluating painful penile lesions should always consider epithelioid vascular tumors in their differential diagnosis. Although these tumors are extremely rare, incorrectly diagnosing the tumors can have devastating consequences, and routine color flow Doppler ultrasound should be considered for evaluation.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]