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| CASE REPORT |
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| Year : 2015 | Volume
: 2
| Issue : 1 | Page : 38-40 |
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Penile epithelioid hemangioma: A case report
Archana Rajender, Elizabeth Phillips, Ricardo Munarriz
Department of Urology, Boston University Medical Campus, Boston, MA 02118, USA
| Date of Web Publication | 23-Jan-2015 |
Correspondence Address:
Archana Rajender
Department of Urology, Boston University Medical Campus, 725 Albany St., Suite 3B, Boston, MA 02118
USA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2225-1243.150011
Penile epithelioid hemangioma is a very rare benign vascular neoplasm of unclear etiology. To the best of our knowledge, only 28 cases in the literature have been reported to date. We report a case of epithelioid hemangioma of the penis in a 41-year-old man with a painful penile mass. The presented case exemplifies the importance of establishing a correct diagnosis through appropriate histologic analysis, as epithelioid hemangiomas are often misdiagnosed as two highly malignant tumors, epithelioid hemangioendothelioma and epithelioid angiosarcoma. Establishing benignancy is crucial in order to avoid inappropriate and overly aggressive treatment. Keywords: Epithelioid hemangioma, genitourinary malignancies, immunohistochemistry, penile mass, vascular tumors
How to cite this article:
Rajender A, Phillips E, Munarriz R. Penile epithelioid hemangioma: A case report. J Integr Nephrol Androl 2015;2:38-40 |
| Introduction | |  |
Epithelioid hemangioma, previously termed "angiolymphoid hyperplasia with eosinophilia," is a rare vascular neoplasm first described by Wells and Whimster in 1969. [1] While these tumors have been well documented in other anatomic sites (head, neck, and distal extremities), involvement of the penis is extremely uncommon. [2] Only 28 cases of penile epithelioid hemangiomas have been reported in the literature to date. [2],[3],[4],[5],[6],[7],[8] Here we report a case of penile epithelioid hemangioma in a 41-year-old man. This report adds to the documented cases in the literature and helps delineate the salient features of this rare, benign vascular tumor.
| Case report | | |
A 41-year-old otherwise healthy man presented with a painful, dorsal lesion of the penile shaft, which had been present for 2 years. The pain was particularly severe during intercourse with a pain scale rating of 7/10. The quality of the pain was described as "sharp" or "electric-like." He had no history of penile trauma in the erect state and denied blunt perineal trauma. Physical examination revealed a well-circumscribed, tender lesion on the proximal shaft of the penis [Figure 1]a. Penile duplex Doppler ultrasound confirmed an 8.7 mm, round, midline, solid nodule superficial to the tunica [Figure 1]b, without flow. Despite several months of conservative management, his pain progressed without obvious increase in nodular size. Decision was made to proceed with exploration and an excisional biopsy of the lesion. Through a 4 cm, longitudinal incision, the dorsum of the penis was explored, revealing a solid, vascular tumor of the deep dorsal vein [Figure 1]c. The vessel was ligated proximal and distal to the lesion and the specimen was sent to pathology as a vascular malformation. At 1 month after surgery, the patient noted complete resolution of symptoms and physical exam noted no signs of recurrence. | Figure 1: (a) Well-circumscribed, tender lesion on the proximal shaft of the penis. (b) Penile duplex Doppler ultrasound. (c) Surgical view of vascular lesion.
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The surgical specimen consisted of a single piece of well-encapsulated, soft, hemorrhagic pink tissue measuring 0.7 × 0.5 × 0.4 centimeters. Histologic staining revealed a solid growth pattern of plump epithelioid cells with large nuclei, an open chromatin pattern and centrally defined nucleoli [Figure 2]a. The epithelioid cells formed immature vessels as well as solid regions arranged in nests and cords. Moderate inflammatory cells (lymphocytes) were noted in a minimally developed stroma. Immunohistochemical staining revealed that the epithelioid cells were positive for CD31 [Figure 2]b, CD34 (focally positive), Keratin AE 1/3, KI-67 (Focally positive, 10%) and MSA (HHF-35) [Figure 2]c. The pathologic diagnosis was epithelioid hemangioma, atypical variant. | Figure 2: (a) High-power view of the vascular lesion showing immature vessels (arrow head) lined by plump epithelioid cells (arrows). An indeterminate, solid, growth pattern of epithelioid cells is present, which is common to both malignant tumors and atypical benign lesions. (b) Immunohistochemical staining, positive for MSA (HHF-35) which identifies a well formed myopericytic layer around canalized blood vessels, a good marker of lesional maturity, a feature of benignancy. (c) Immunohistochemical staining positive for CD31, a marker of epithelioid endothelial cell proliferation.
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| Discussion | | |
Penile epithelioid hemangioma is a benign vascular tumor that is often initially misdiagnosed as other, more common penile nodular diseases such as a penile cyst, Peyronie's disease or penile phlebothrombosis. Duplex Doppler ultrasound plays an important role in differentiating these etiologies. [3] However, when the diagnosis remains unclear, local excision and pathologic evaluation should be performed. Epithelioid hemangioma has two histologic variants: A "typical" form, and an immature "atypical/exuberant" form. The "atypical" form is histologically similar to the malignant vascular tumors, epithelioid hemangioendothelioma and epithelioid angiosarcoma, as they all demonstrate a largely indeterminate growth pattern of endothelial cells. [4],[9] Due to the similarities in histologic presentations, the "atypical" variant of epithelioid hemangioma is frequently confused with these malignant epithelioid tumors. [3],[4],[5] Fetsch et al., noted in their case series, that 60% of the 'typical' variant and 80% of the "atypical" variant cases were initially diagnosed by at least one pathologist as a malignant vascular tumor. [4] The key feature that differentiates benign lesions from malignant ones is the immunohistochemical presence of muscle-specific actin (HHF-35). Decreased keratin reactivity, and presence of CD31 and factor VIIIrAg also suggest a non-malignant pathology. These immunohistochemical stains are crucial, so as to avoid overly aggressive treatment required for malignant vascular tumors. [4],[9]
Complete surgical excision of "typical" lesions is appropriate, as follow-up data suggests no recurrences or unexpected clinical findings. [4],[5] Similarly, for atypical variants, local excision is suited. However, the excision should contain wide margins, as few cases of regional recurrences have been reported. [4] Frequent clinical follow-up after excision is not indicated for epithelioid hemangiomas.
This case report exemplifies the difficulty in correctly diagnosing vascular tumors of the penis. Although rare, epithelioid hemangiomas should be considered in the differential diagnosis of a painful penile mass. A thorough clinical and immunohistological evaluation is crucial to avoid misdiagnosis with other highly malignant vascular neoplasms.
| Acknowledgement | | |
We thank the Department of Pathology at Boston Medical Center for their assistance in the preparation of the histological slides in [Figure 2].
| References | | |
| 1. | Wells GC, Whimster IW. Subcutaneous Angiolymphoid Hyperplasia with Eosinophilia. Br J Dermatol 1969;81:1-14.  |
| 2. | Olsen TG, Helwig EB. Angiolymphoid Hyperplasia with Eosinophilia: A clinicopathologic study of 116 patients. J Am Acad Dermatol 1985;12:781-96. |
| 3. | Natali A, Nesi G, Vittori G, Minervini A, Franchi A, Agostini S, et al. Rare case of atypical epithelioid hemangioma of penis initially misdiagnosed as Peyronie′s Disease: Report with clinical, radiologic and immunohistochemical analysis. Urology 2009;73:210e7-10. |
| 4. | Fetsch JF, Sesterhenn IA, Miettinen M, Davis CJ Jr. Epithelioid Hemangioma of the Penis - A. Clinicopathologic and Immunohistochemical Analysis of 19 Cases, with Special Reference to Exuberant Examples often Confused with Epithelioid Hemangioendothelioma and Epithelioid Angiosarcoma. Am J Surg Pathol 2004;28:523-33. |
| 5. | Ismail M, Damato S, Freeman A, Nigam R. Epithelioid hemangioma of the penis: Case report and review of literature. J Med Case Rep 2011;5:260. |
| 6. | Park JS, Lee MJ. A Case of Angiolymphoid Hyperplasia with Eosinophilia (ALHE) in the Genital Area Accompanied by Varicocele. Int J Dermatol 2009;48:1262-70. |
| 7. | Srigley JR, Ayala AG, Ordonez NG, van Nostrand AW. Epithelioid Hemangioma of the penis: A rare and distinctive vascular lesion. Arch Pathol Lab Med 1985;109:51-4. |
| 8. | Fetsch JF, Weiss SW. Observations concerning the pathogenesis of Epithelioid Hemangioma (angiolymphoid hyperplasia). Mod Pathol 1991;4:449-55. |
| 9. | 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.  |
[Figure 1], [Figure 2]
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