Primary penile malignancies represent approximately 0.2% of cancers in men in the United States, with approximately 1530 new cases diagnosed in 2006, according to the American Cancer Society. Penile carcinoma is most commonly seen in uncircumcised men and is associated with poor hygiene. There are few reports of angiosarcoma of the genitalia, and local control with surgery, followed by chemotherapy or radiotherapy for widespread disease, is the suggested mode of treatment. Differential diagnosis of angiosarcoma includes epithelioid hemangioendothelioma, Kaposi's sarcoma, and alveolar rhadomyosarcoma. We report, to our knowledge, the first known case of penile angiosarcoma in an AIDS patient, and present a brief review of the pertinent literature.
A 44-year-old man with AIDS, Kaposi's sarcoma, and Pneumocystis carinii pneumonia, presented with a complaint of bleeding penile lesions, which had been enlarging and increasing in number over several months. On physical examination, there was a friable mass encompassing the left half of the penis extending to the scrotum (Fig. 1a). The foreskin was not retractable, and minimal handling of the penis caused bleeding. Inguinal lymph nodes were non-palpable. Initial laboratory values demonstrated a hematocrit of 27%, serum creatinine level of 1.4 mg/dl, and CD4 cell count of 22 cells/μl. Computed tomography of the abdomen and pelvis showed numerous low-density lesions in the liver, bilateral enlarged inguinal lymph nodes without pelvic lymphadenopathy, and multiple bony lytic lesions. Biopsy of the tumor revealed a malignant vascular tumor consistent with angiosarcoma. The patient underwent radical penectomy and partial scrotectomy, with formation of thigh pouches and perineal urethrostomy.
Pathological examination of the specimen showed multiple areas of ulceration on the surface of the penis, including a focus of condyloma acuminatum just proximal to the glans. On microscopy, irregular vascular channels infiltrating the dermis were identified among areas of spindle cells, along with red blood cell containing vacuoles (Fig. 1b). The surgical margins were negative, and the final pathology correlated with the initial biopsy results. Extensive review of the specimen confirmed that this was indeed angiosarcoma and not Kaposi's sarcoma. During his hospitalization, the patient was evaluated by the Pulmonology and Gastroenterology services, which performed bronchoscopy and colonoscopy, revealing no obvious lesions. He was discharged on postoperative day 14, but was lost to follow-up with the medical oncology service, which recommended palliative chemotherapy. He was re-admitted 3 months postoperatively with worsening cough secondary to parenchymal Kaposi's sarcoma, failure to thrive and malnutrition, with a serum albumin level of 1.0 g/dl. Examination of the perineum demonstrated gross recurrence at the penectomy site. He died 3 weeks later as a result of metastatic angiosarcoma and disseminated Kaposi's sarcoma; no postmortem examination was performed.
Non-squamous malignancy of the penis is rare, with sarcomatous lesions representing the smallest percentage of these malignancies [1,2]. Previous reports of penile angiosarcoma include lesions presenting as a fungating mass , a Peyronie's plaque , a mass extending into the bladder , in a patient with von Recklinghausen's disease , and in a patient who received radiation for penile squamous carcinoma . Another patient with low exposure to polyvinyl chloride presented with hepatic lesions, similar to this patient, which were consistent with hepatic angiomas . The natural course of sarcomata of the penis is of local recurrence, therefore total penectomy with or without radiotherapy is suggested even for superficial disease, and unless there is palpable inguinal lymphadenopathy, inguinal node dissection is not necessary [1,3]. Metastatic disease is rare with aggressive local control, and is treated with either palliative chemotherapy or radiotherapy [1,3,9]. Our report of penile angiosarcoma represents, to our knowledge, the first known case in a patient with AIDS, and underscores the unique lesions that can be seen in immunodeficiency states. Importantly, histological diagnosis of angiosarcoma, and avoiding the assumption that the patient's lesion were Kaposi's sarcoma, led to the appropriate treatment. Genitourinary malignancy is common is HIV/AIDS, and is frequently more aggressive. Renal cell carcinoma has been shown to occur 8.5 times more commonly in HIV patients, and occurs in younger patients . Similarly, prostate cancer seems to occur in younger patients and acts more aggressively . Testicular tumors are the third most common malignancy in HIV-associated cancers, frequently presenting as non-Hodgkin's lymphoma and Burkitt's lymphoma, as well as traditional germ-cell tumors . This case highlights the fact that an atypical presentation of genitourinary pathology requires the consideration of less common malignancies in the differential, particularly in a severely immunocompromised patient.
Conflicts of interest: None.
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