Vol 16 No 01 January-February 2019 89 CASE REPORT Female Urethral Cavernous Hemangioma, A Rare Entity: Two Case Reports and Review of The Literature Farzaneh Sharifiaghdas1, Nastaran Mahmoudnejad2* ,Niloofar Rostaminezhad3, Mahmoud Parvin4 Genitourinary hemangiomas are very rare. To our knowledge few cases of female urethral hemangiomas have been reported and presenting cases are the first reports in Iran. They should be considered as differential diagnosis of any female patient with microscopic or gross hematuria or bloody urethral discharge, especially when other parts of urinary system are radiologically intact. Thorough phys- ical examination of genital area is highly recommended in order not to miss any urethral lesions. Herein we report two cases of female urethral cavernous hemangioma, their management and a review of literature. Keywords: urethral hemangioma; hematuria; urethral mass; cavernous hemangioma; female urethral mass INTRODUCTION Hemangioma of the urinary tract is an unusual entity and a few cases have been reported (Table 1). They may occur in all parts of the urinary tract but kidneys seem to be the most frequently affected followed by 1Professor of Urology, Shahid Labbafinejad Medical Center, Urology and Nephrology Research Center (UNRC), Shahid Beheshti University of Medical Sciences, Tehran, Iran. 2Fellowship of Female Urology, Shahid Labbafinejad Medical Center, Urology and Nephrology Research Center (UNRC), Shahid Beheshti University of Medical Sciences, Tehran, Iran. 3Resident of urology, Shahid Labbafinejad Medical Center, Urology and Nephrology Research Center (UNRC), Shahid Beheshti University of Medical Sciences, Tehran, Iran. 4Associate professor of pathology, , Shahid Labbafinejad Medical Center, Urology and Nephrology Research Center (UNRC), Shahid Beheshti University of Medical Sciences, Tehran, Iran. *Correspondence: Shahid Labbafinejad Medical Center, 9th Boustan St., Pasdaran Ave. Tehran, Postal code: 16666 Iran Tel/Fax: +98-21-22588016. Email: nastaran.mahmoudnejad@gmail.com. Received February 2017 & Accepted June 2018 Figure 1. a: 1x1 cm reddish urethral mass. b&c: Section show polypoid fragments of urothelial lined mucosa with presence of dilated cavernous vessels filled with blood and thrombus in lamina propria. Lymphocytic infiltration around vessels is prominent. the bladder. They are generally seen as isolated lesions but they may be multiple and associated with congen- ital disorders such as klipple- Trenaunay syndrome , Sturge-Weber and systemic angiomatosis(1). Herein we report two cases of cavernous hemangioma of the ure- thra in female patients for the first time in Iran. To our best knowledge, there are less than ten documented case reports of female urethral cavernous hemangioma in the literature(2-7). CASE REPORT Case one: A 38 year old woman presented with painless urethral mass since 6 months ago. There was no complaint of gross hematuria or any other urinary symptoms. She mentioned episodes of mild bleeding from the mass in last few days. In physical examination a 1x1 cm round, reddish mass was seen in urethral meatus (Figure 1, a). The gross appearance was similar to a urethral caruncle. Urine analysis revealed microscopic hematuria. Ultra- sound of genitourinary system and urine cytology were normal. In cysto-urethroscopic evaluation, the origin of the mass seemed to be in distal urethra. Other parts of the urethra and urinary bladder were normal. Written informed consent was obtained from the patient and excisional biopsy of the mass was done at the same ses- sion. The mass had a broad base and was bigger than its initial view. The base of the mass was repaired with separate absorbable sutures. The pathology report re- vealed a cavernous hemangioma (Figure1, b&c). Case two: A 65 year old female presented with a relatively large urethral mass since 20 days ago following constipation and straining. There was no dysuria, hematuria or oth- er irritative urinary symptoms however she mentioned difficulty in voiding in last few days. In physical ex- amination, a large peri-urethral mass with diffuse areas of thrombosis in its surface was noticed (Figure 2, a). In order to ameliorate obstructive urinary symptoms, a 16 French foley catheter was inserted in urethra and conservative management was initiated. Meanwhile, further evaluations including ultrasonography of geni- tourinary tract, urine analysis and urine cytology were performed. After 10 days of medical and conservative treatment including topical estrogen and oral antibiotic, the mass seemed to be smaller in size however it was Case First Author Journal Year Age of Patient 1 Uchida K. J Urol. 2001 59 2 Tabibian L. J Urol. 2003 81 3 Rao.A.R. Urology 2005 26 4 Rohan VS. Saudi J Kidney Dis Transpl. 2006 60 5 De Silva- Gutierrez A. Rev Mex Urol. 2013 84 6 Ongun S. Urol J. 2014 68 7 Bolat MS. Pan Afr Med J. 2015 51 8 Regragui S. Pan Afr Med J. 2016 61 9 Chiao-Ching Li. Medicine 2017 8 10 (Present case1) Sharifiaghdas F Urol J. 2018 38 11 (present case2) Sharifiaghdas F Urol J. 2018 65 Table 1. Reports of female urethral hemangioma in the literature. Figure 2. a: a large peri-urethral mass with diffuse areas of thrombosis in its surface. b: The same mass after conservative treatment. Female urethral cavernous hemangioma-Sharifiaghdas et al. Case Report 90 Vol 16 No 01 January-February 2019 91 not completely resolved (Figure 2, b). All para-clinical tests found to be normal except for microscopic hema- turia. Cysto-urethroscopic evaluation under spinal an- esthesia showed normal blabber but a mass originating from distal urethra. Excisional biopsy of the mass was performed at the same session using electro-cautery. In order to prevent subsequent meatal stenosis, eversion of urethral mucosa with simple 4-0 absorbable sutures was done properly. Foley catheter remained for another 7 days. The pathology report revealed a cavernous he- mangioma with multiple focies of thrombosis. In one year follow up of two cases, neither of them had any complications or recurrence of the mass. DISCUSSION The origin of hemangiomas is controversial but may arise from embryonic nests of unipotent angioblastic cells, which fail to develop into normal blood vessels (8). The lesions are composed of a mixture of endotheli- um lined spaces containing erythrocytes and organized thrombi. They grow slowly and do not communicate with the surrounding vessels therefore are not truly in- vasive(1). Mean age of presentation is 22 years, while age ranges from 3 to 68 years(3). Urethral hemangiomas are the least common genitourinary hemangiomas(9). Female urethral hemangiomas are very rare and ninety percent of urethral hemangiomas are seen in men. The most common symptom of urethral hemangiomas is he- maturia either microscopic or gross(3). Sometimes the bleeding can be very intense and cause anemia(6). Other clinical presentations are: intermittent urethral bleeding and discharge, meatal mass, urinary retention, and low- er urinary tract symptoms(3,9,10). Differential diagnosis for female urethral mass includes: urethral caruncle, urethral prolapse, peri-urethral abscess, warts, urethral polyp, granuloma gravidarum, leiomyoma, malignan- cies like Squamous cell carcinoma, Transitional cell carcinoma, Sarcoma, and Melanoma(6). Urethroscopy is the diagnostic method of choice and will define the site and extent of the lesions(3). MRI is another useful diagnostic modality(2,4). Accurate diagnosis requires pathologic confirmation. Treatment options depend on location and size of the mass and management should be individualized. Asymptomatic lesions do not require any treatment(1). Hemangiomas are often underestimat- ed. Even small lesions may extend further than is imme- diately obvious(1). Small isolated lesions are treated endoscopically by electrocoagulation, fulguration or Nd:YAG, Argon or KTP laser ablation(8,9). Electro fulguration carries the risk of urethral scarring(1). Other treatment options in- clude: local resection, topical as well as oral steroids, sclerotherapy, and cryoablation(5,8,10). In case of local recurrence after endoscopic ablation, open exploration and wide excision is recommended(1). Although urethral hemangiomas are very rare, they should be considered as differential diagnosis of any female patient with microscopic or gross hematuria or bloody urethral discharge. We believe that in "large masses" surgery is the treatment of choice and local medical treatment will change the gross appearance of the mass in a way that diagnosis would be very difficult with no benefit for the patient . ACKNOWLEDGEMENT The authors gratefully acknowledge Dr. Niloufar Mahmoudnejad for her exemplary guidance and editing assistance. REFERENCES 1. Jahn H, Nissen HM. Haemangioma of the urinary tract: review of the literature. Br J Urol. 1991;68:113-7. 2. Uchida K, Fukuta F, Ando M, Miyake M. Female urethral hemangioma. J Urol. 2001;166:1008. 3. Tabibian L, Ginsberg DA. Thrombosed urethral hemangioma. J Urol. 2003;170:1942. 4. Rohan VS, Hanji AM, Patel JJ, Tankshali RA. Female urethral hemangioma. Saudi J Kidney Dis Transpl. 2008;19:647-8. 5. Ongun S, Celik S, Aslan G, Yorukoglu K, Esen A. Cavernous hemangioma of the female urethra: a rare case report. Urol J. 2014;11:1521-3. 6. Regragui S, Slaoui A, Karmouni T, El Khader K, Koutani A, Attya AI. Urethral hemangioma: case report and review of the literature. Pan Afr Med J. 2016;23:96. 7. Ahuja A, Sen AK, Bhardwaj M. Cavernous hemangioma of anterior urethra: An unusual cause of vaginal bleeding. Indian J Pathol Microbiol. 2016;59:245-6. 8. Khaitan A, Hemal AK. Urethral hemangioma: laser treatment. Int Urol Nephrol. 2000;32:285- 6. 9. Parshad S, Yadav SP, Arora B. Urethral hemangioma. An unusual cause of hematuria. Urol Int. 2001;66:43-5. 10. Hayashi T, Igarashi K, Sekine H. Urethral hemangioma: case report. J Urol. 1997;158:539-40. Female urethral cavernous hemangioma-Sharifiaghdas et al.