(368) Stuttering Priapism: A Rare Manifestation of Underlying Malignancy

Abstract

Abstract Introduction First described by Peacock in 1938, malignant priapism (MP) is defined as persistent erection in the absence of sexual stimulation or other triggering factors. The proposed mechanism is due to neoplastic invasion of cavernous sinuses and venous system resulting in poor outflow and painful penile engorgement. Though rare, MP due to penile tumors has been described in cases of hematologic malignancies, primary penile neoplasms, local invasion from urologic primary tumors including prostate and bladder cancer, and penile metastasis from other primary sources. Objective Here we describe a case of metastatic epithelioid sarcoma that presented with recurrent priapism. Methods An otherwise healthy 43 year old male presented with two weeks of intermittent priapism. Initially thought to be traumatic in nature, the patient was managed conservatively with penile irrigation and phenylephrine. Though the patient achieved some improvement in his pain, full detumescence was never achieved. Approximately one month later the patient elected for placement of an inflatable penile prosthesis (IPP) due to persistent incomplete detumescence. At the time of surgery, extensive corporal induration was noted bilaterally. Upon entry into the corpora, copious purulent material was expressed from the tissues and the decision was made to abort the case. Prior to closure, several representative biopsies were collected. Results Blood gas performed at initial presentation was consistent with low flow priapism. Doppler studies showed intact arterial blood flow to the penis bilaterally and pelvic MRI was negative for penile injury though asymmetric enhancement of the corpora was noted incidentally. Surgical pathology revealed an unclassified malignancy and increased proliferation concerning for metastasis. A PET scan was performed which was consistent with widely metastatic disease with mets to the liver, lung, bone, muscles, adrenal glands, brain, and penis. Percutaneous biopsy of an abdominal mass confirmed the diagnosis of epithelioid sarcoma. Despite palliative chemotherapy and radiation the patient succumbed to his cancer just two months after diagnosis. Conclusions Epithelioid sarcoma is a rare soft tissue neoplasm primarily affecting young adult males and representing less than 1% of soft tissue sarcomas. Even rarer is the incidence of metastatic penile tumors, with fewer than 500 cases reported in the literature since 1850. A literature review by Lin YH et al based on reports from 2006 to 2011 suggested that the true incidence of penile metastasis may be higher given that 12% of penile metastasis may be asymptomatic and discovered only at autopsy. To our knowledge, this is the first case of widely metastatic epithelioid sarcoma presenting with priapism secondary to penile metastasis. Disclosure No