only less than 5 cases of primary urethral angioleiomyomas are reported in literature mostly in females . angioleiomyomas are benign smooth muscle tumors that are commonly seen in subcutaneous location of the extremities . herein , we present a rare case of primary angioleiomyoma of urethra in a 72 years old male who presented with hematuria . a 72 year old male who is a known case of diabetes mellitus presented with gross hematuria of two day duration . his physical examination was unremarkable apart from markedly enlarged prostate that was identified on rectal examination . due to the persistent gross hematuria and passage of blood clots and presence of atypical cells in urine , clinical decision was made to do cystoscopy and at the same time do transurethral resection of prostate ( turp ) since the patient had a markedly enlarged prostate . cystoscopy showed an incidental urethral polypoid lesion that was excised in addition to random biopsies that were taken from the urinary bladder . the prostatic urethral polyp showed a well circumscribed nodule exhibiting scattered thick walled blood vessels that were surrounded by spindle cell proliferation ( fig . the cells were elongated , had eosinophilic cytoplasm , bland appearing nuclei , and lacked any cytologic atypia or increased mitotic activity ( fig . the smooth muscle walls of the blood vessels were strongly positive for actin ( fig . the patient had an uneventful postoperative course and 12 years later he had no recurrence of hematuria , atypical cells in urine , or evidence of recurrent urethral lesion . most urethral lesions encompass urethral caruncle , diverticulum , mucosal prolapse , skene duct cyst / abscess , cowper gland duct cyst , fibroepithelial polyp , and prostatic - type poly . marshall et al . reported a study of 394 lesions of female urethra revealing 372 benign lesions and only 22 malignant tumors . caruncle was found to be the most commonly encountered lesion accounting for 96% and no leiomyoma or angioleiomyoma was reported in that study . subsequently , few leiomyomas were reported in literature with only rare cases of angioleiomyomas that arose from urethra [ 37 ] . most of these angioleiomyomas were associated with obstructive symptoms as is the case with many other urethral lesions , however our patient presented only with gross hematuria and no symptoms of obstruction . angioleiomyomas are common deep dermal , or subcutaneous benign , well - circumscribed smooth muscle tumors that originate from the walls of blood vessels in particular veins . although the neoplasm can affect any age , it is commonly seen in adults between the age of 30 and 60 years of age . there is mild overall male predominance , however , larger studies show that there is female predominance when angioleiomyomas are seen involving lower extremities and male predominance in the head and neck region and upper extremities . typically , the lesion presents as a solitary , small ( less than 2  cm ) , slow growing , nodule affecting the extremities , and is often painful or tender on compression . only rare cases have been reported in literature that arise in urethral location . to our knowledge , less than five cases grossly these lesions are sharply circumscribed white rubbery nodules that are mostly 12  cm in size . histologically , these are mostly round encapsulated lesions that are composed of many blood vessels of variably thickened walls that are surrounded by interlacing fascicles and bundles of uniform spindled cell . there may be areas of myxoid change , thrombosis , lipomatous metaplasia , or dystrophic calcifications . occasional cases may exhibit epithelioid changes of the cells and few cells with enlarged hyperchromatic nuclei , however , no increase in mitotic activity is seen in these cases . such atypia without mitotic activity is considered secondary to degenerative or reactive changes and does not indicate malignancy . true angiomyolipmas belong to the family of perivascular epithelioid cell tumor ( pecomas ) , however , angioleiomyomas are not related to pecomas and do not show the characteristic hmb-45 positive staining of pecomas . three histologic variants have been recognized ; the most common one being  capillary or solid  type representing approximately 67% of cases . identification of these histologic variants is important for pathologists to be able to identify them and recognize them as variants of the same benign entity , however , has no clinical significance . immunohistochemistry shows cells to be positive for smooth muscle actin , calponin , and h - caldesmin . cytogenetic studies show chromosomal imbalances , with most consistent finding being recurrent losses in chromosome 22q11 . surgical excision is the recommended treatment for any urethral lesion mainly to identify the lesion histologically , and to distinguish reactive from neoplastic , and benign from malignant tumors . examples include smooth muscle tumors to differentiate benign leiomyoma from leiomyosarcoma based on the number of mitotic figures and benign versus malignant urothelial neoplasms based on architectural and cytologic features . recurrence of angioleiomyoma after simple excision is extremely rare and malignant transformation has not been convincingly documented or reported in literature . in conclusion , we herein , report a case of a primary angioleiomyoma arising from a rarely encountered location , urethra , in a male patient who presented with only gross hematuria . angioleiomyomas are benign smooth muscle neoplasms of vessel walls that are commonly seen in subcutaneous and deep dermal tissue of extremities . only rare cases are reported to arise from other sites with only less than 5 cases reported in literature involving urethra .
highlightsa rare presentation of angioleiomyoma arising in the urethra , with only less than 5 cases reported in literature in such a location.angioleiomyoma is a smooth muscle tumor commonly seen in the blood vessels of deep dermal and subcutaneous tissue.recommended treatment of a urethral lesion is surgical excision in order to be able to identify this lesion and distinguish between reactive , benign , and malignant conditions .