© 1991 S. Kargcr AG, Basel 0042-1138/91/0462-0235S2.75/0
Urol Ini 1991;46:235-237
Kaposi’s Sarcoma of the Penis as an Initial Urological Manifestation of AIDS A Report of Two Cases Javier C. Angulo a, JoseI. Lopezb, Miguel Unda-Urzaiza, Jose R. Larrinaga a, Carmen L. Zubiaur a, Nicolas C. Floresa Departments of “Urology and bPathology, Santo Hospital Civil de Bilbao, Spain
Key Words. Kaposi’s sarcoma • Penis • AIDS, urological manifestations Abstract. Kaposi’s sarcoma of the penis is an initial manifestation of AIDS and the practising urologist should be aware of this form of presentation. We report 2 young intravenous drug abusers with this tumor in the male geni tourinary system. The clinical course is unfavorable; therefore prompt diagnosis and management, based on the early recognition of the lesion, must be achieved.
AIDS was first described as ‘the syndrome of Kaposi’s sarcoma and opportunistic infections’ [1,2], and epidemiologically related to homosexual men [3], Since then, Kaposi’s sarcoma, a slowly progressing malignant mes enchymal proliferation of atypical spindle cells and vas cular channels, clinically presenting as red-purple plaques and nodules, has become one of the characteris tic morphologic features of HIV infection [4]. We report 2 cases of Kaposi’s sarcoma of the penis, constituting both an initial manifestation and a distinct urological lesion of AIDS.
Case Reports Case 1 A 28-year-old male intravenous drug abuser presented with a rapidly growing red-purple nodule on his glans penis. Biopsy con firmed the clinical suspicion of Kaposi’s sarcoma (fig. 1). HI V infec tion was therefore investigated and diagnosed. Three months later the patient developed a miliary form of tuberculosis which is now being treated.
Case 2 A 26-year-old male, addicted to parenteral drugs for more than 10 years, was admitted to the emergency room suffering from an overdose. He presented multiple cutaneous lesions in the penis, scrotum, right calf and leg that were suspected to be Kaposi’s sarco mas. His relatives reported a history of disorientation, slowly pro gressing dementia and fever. HIV infection was diagnosed. Blood cultures were positive for Salmonella enteriditis and Candida albi cans was isolated from the sputum. He died during a sudden hypo tensive episode a few weeks after diagnosis. Autopsy revealed a single pulmonary and multiple splenic ab scesses. Pneumocystis carinii pneumonia was also present, together with a candidiasic meningitis, central nervous system demyelinating changes and intense gliosis. The adrenal glands showed a gross and microscopic picture of hemorrhagic necrosis with cytomegalovirus (CMV) inclusions. Histology confirmed that the cutaneous plaques were Kaposi’s sarcoma.
Discussion Apart from being the most constant dermatologic finding in AIDS [5], Kaposi’s sarcoma is still a remark able head and neck manifestation: oral, pharyngeal or in the cervical nodes. This last form has been clinically mis taken for persistent generalized lymphadenopathy [6, 7],
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Introduction
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Angulo/Lopez/Unda-Urzaiz/Larrinaga/Zubiaur/Flores
Even before the AIDS epidemic, the finding of CMVDNA in the genome of Kaposi’s sarcoma cells, together with certain seroepidemiologic studies, linked CMV to the causation of this tumor [15]. Nowadays, both CMV infection and Kaposi’s sarcoma arising in the context of AIDS are common findings and the coincidence of both entities in case 2 is in no way striking. The increased frequency of the HLA-DR5 antigen reported in Kaposi’s sarcoma [3] raises the possibility that the severely immunocompromised individuals that develop this tumor, in this case HIV-infected drug ad dicts, are genetically susceptible to ‘opportunistic’ Kapo si’s sarcoma. Finally, we want to emphasize the possibility that Kaposi’s sarcoma of the penis, same as primary testicu lar lymphoma [16, 17], presents as the initial manifesta tion of underlying and previously undiagnosed AIDS. Urologists should always be aware of this form of muco cutaneous presentation in order to establish a prompt diagnosis and management of the patient.
Similarly, an unreported equivalent misdiagnosis could occur for the inguinal region. Despite a great individual variability, the median sur vival of patients with Kaposi’s sarcoma seems to be sig nificantly shortened [8]. In fact, the unfavorable clinical course of our 2 patients agrees with this poor progno sis. Kaposi’s sarcoma in the genitourinary system has been described in the adrenal glands, epididymis, pros tate, seminal vesicles, bladder and penis [9-12] and therefore can be considered a distinct urological mani festation of AIDS. In the series that Kaplan et al. [13] presented, no Kaposi’s sarcoma was reported in 60 patients suffering from AIDS. Recently, Miles et al. [14] stated that only a small number of these patients shows significant urolog ical manifestations and presented 3 cases of cutaneous genitourinary Kaposi’s sarcoma of 120 patients with AIDS or AIDS-related complex. In our 2 cases, genitou rinary Kaposi’s sarcoma of the penis and scrotum is the initial manifestation, and in case 1 the only consulting sign of previously undiagnosed AIDS.
1 Durack DT: Opportunistic infections and Kaposi’s sarcoma in homosexual man. N Engl J Med 1981;305:1465-1467. 2 Fauci AS: The syndrome of Kaposi’s sarcoma and opportunistic infections. Ann Intern Med 1982;96:777-779. 3 Friedman-Kien AE, Laubenstein LJ, Rubinstein P, et al: Dis seminated Kaposi’s sarcoma in homosexual men. Ann Intern Med 1982;96:693-700. 4 Robbins S, Cotran R, Kumar V: Pathologic Bases of Disease. Philadelphia, Saunders, 1984, p 1270. 5 Kaplan MH, Sadick N, McNutt S, et al: Dermatologic findings and manifestations of acquired immunodeficiency syndrome (AIDS). J Am Acad Dermatol 1987;16:487-506. 6 Patow CA, Steis R, Longo DL, et al: Kaposi’s sarcoma of the head and neck in the acquired immunodeficiency syndrome. Otolaryngol Head Neck Surg 1984;92:255-260. 7 Levine AM, Meyer PR, Gill PS, et al: Results of initial lymph node biopsy in homosexual men with generalized lymphadenopathy. J Clin Oncol 1986;4:165-169. 8 Vadhan-Raj S, Won G, Gnecco C, et al: Immunological vari ables as predictors of prognosis in patients with Kaposi’s sar coma and the acquired immune deficiency syndrome. Cancer Res 1986;46:417-425. 9 Amberson JB, DiCarlo EF, Metroka CE, et al: Diagnostic pa thology in the acquired immunodeficiency syndrome. Arch Pa thol Lab Med 1985;109:345-351. 10 Niedt GW, Schinella RA: Acquired immunodeficiency syn drome. Clinicopathologic study of 56 autopsies. Arch Pathol Lab Med 1985;109:727-734. 11 Reichert CM, O’Leary TJ, Levens DL, et al: Autopsy pathology in the acquired immune deficiency syndrome. Am J Pathol 1983;1 12:357-382.
Downloaded by: Stockholm University Library 130.237.165.40 - 2/21/2018 1:56:15 AM
References Fig. 1. Histological high-power view of Kaposi’s sarcoma show ing vascular channels and lumina within a spindle cell and atypical neoplastic growth. HE. X 400.
Kaposi’s Sarcoma of the Penis
17 Levine AM, Gill PS, Mayer PR, et al: Retrovirus and malignant lymphoma in homosexual men. JAMA 1985;254:1921-1925.
Received: February 20, 1990 Accepted after revision: May 23, 1990 Dr. Javier C. Angulo Servicio de Urologia Santo Hospital Civil de Bilbao Avenida Montevideo, 18 E-48013 Bilbao (Spain)
Downloaded by: Stockholm University Library 130.237.165.40 - 2/21/2018 1:56:15 AM
12 Welch K, Frinkbeiner W, Alpers CE, et al: Autopsy findings in acquired immune deficiency syndrome. JAMA 1984;252:1152— 1159. 13 Kaplan MS, Wechsler M, Benson M: Urologic manifestations of AIDS. Urology 1987;30:441-443. 14 Miles BJ, Melser M, Farah R, et al: The urological manifesta tions of the acquired immunodeficiency syndrome. J Urol 1989; 142:771-773. 15 Giraldo G, Beth E, Huang ES: Kaposi’s sarcoma and its relation ship to cytomegalovirus (CMV). III. CMV, DNA and CMV anti gens in Kaposi’s sarcoma. Int J Cancer 1980;26:23-29. 16 Tirelli U, Rezza G, Lazzarin A, et al : Malignant lymphoma related to HIV infection in Italy: A report of 46 cases. JAMA 1987;258:2064.
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Urol Ini 1991;46:235-237
Kaposi’s Sarcoma of the Penis as an Initial Urological Manifestation of AIDS A Report of Two Cases Javier C. Angulo a, JoseI. Lopezb, Miguel Unda-Urzaiza, Jose R. Larrinaga a, Carmen L. Zubiaur a, Nicolas C. Floresa Departments of “Urology and bPathology, Santo Hospital Civil de Bilbao, Spain
Key Words. Kaposi’s sarcoma • Penis • AIDS, urological manifestations Abstract. Kaposi’s sarcoma of the penis is an initial manifestation of AIDS and the practising urologist should be aware of this form of presentation. We report 2 young intravenous drug abusers with this tumor in the male geni tourinary system. The clinical course is unfavorable; therefore prompt diagnosis and management, based on the early recognition of the lesion, must be achieved.
AIDS was first described as ‘the syndrome of Kaposi’s sarcoma and opportunistic infections’ [1,2], and epidemiologically related to homosexual men [3], Since then, Kaposi’s sarcoma, a slowly progressing malignant mes enchymal proliferation of atypical spindle cells and vas cular channels, clinically presenting as red-purple plaques and nodules, has become one of the characteris tic morphologic features of HIV infection [4]. We report 2 cases of Kaposi’s sarcoma of the penis, constituting both an initial manifestation and a distinct urological lesion of AIDS.
Case Reports Case 1 A 28-year-old male intravenous drug abuser presented with a rapidly growing red-purple nodule on his glans penis. Biopsy con firmed the clinical suspicion of Kaposi’s sarcoma (fig. 1). HI V infec tion was therefore investigated and diagnosed. Three months later the patient developed a miliary form of tuberculosis which is now being treated.
Case 2 A 26-year-old male, addicted to parenteral drugs for more than 10 years, was admitted to the emergency room suffering from an overdose. He presented multiple cutaneous lesions in the penis, scrotum, right calf and leg that were suspected to be Kaposi’s sarco mas. His relatives reported a history of disorientation, slowly pro gressing dementia and fever. HIV infection was diagnosed. Blood cultures were positive for Salmonella enteriditis and Candida albi cans was isolated from the sputum. He died during a sudden hypo tensive episode a few weeks after diagnosis. Autopsy revealed a single pulmonary and multiple splenic ab scesses. Pneumocystis carinii pneumonia was also present, together with a candidiasic meningitis, central nervous system demyelinating changes and intense gliosis. The adrenal glands showed a gross and microscopic picture of hemorrhagic necrosis with cytomegalovirus (CMV) inclusions. Histology confirmed that the cutaneous plaques were Kaposi’s sarcoma.
Discussion Apart from being the most constant dermatologic finding in AIDS [5], Kaposi’s sarcoma is still a remark able head and neck manifestation: oral, pharyngeal or in the cervical nodes. This last form has been clinically mis taken for persistent generalized lymphadenopathy [6, 7],
Downloaded by: Stockholm University Library 130.237.165.40 - 2/21/2018 1:56:15 AM
Introduction
236
Angulo/Lopez/Unda-Urzaiz/Larrinaga/Zubiaur/Flores
Even before the AIDS epidemic, the finding of CMVDNA in the genome of Kaposi’s sarcoma cells, together with certain seroepidemiologic studies, linked CMV to the causation of this tumor [15]. Nowadays, both CMV infection and Kaposi’s sarcoma arising in the context of AIDS are common findings and the coincidence of both entities in case 2 is in no way striking. The increased frequency of the HLA-DR5 antigen reported in Kaposi’s sarcoma [3] raises the possibility that the severely immunocompromised individuals that develop this tumor, in this case HIV-infected drug ad dicts, are genetically susceptible to ‘opportunistic’ Kapo si’s sarcoma. Finally, we want to emphasize the possibility that Kaposi’s sarcoma of the penis, same as primary testicu lar lymphoma [16, 17], presents as the initial manifesta tion of underlying and previously undiagnosed AIDS. Urologists should always be aware of this form of muco cutaneous presentation in order to establish a prompt diagnosis and management of the patient.
Similarly, an unreported equivalent misdiagnosis could occur for the inguinal region. Despite a great individual variability, the median sur vival of patients with Kaposi’s sarcoma seems to be sig nificantly shortened [8]. In fact, the unfavorable clinical course of our 2 patients agrees with this poor progno sis. Kaposi’s sarcoma in the genitourinary system has been described in the adrenal glands, epididymis, pros tate, seminal vesicles, bladder and penis [9-12] and therefore can be considered a distinct urological mani festation of AIDS. In the series that Kaplan et al. [13] presented, no Kaposi’s sarcoma was reported in 60 patients suffering from AIDS. Recently, Miles et al. [14] stated that only a small number of these patients shows significant urolog ical manifestations and presented 3 cases of cutaneous genitourinary Kaposi’s sarcoma of 120 patients with AIDS or AIDS-related complex. In our 2 cases, genitou rinary Kaposi’s sarcoma of the penis and scrotum is the initial manifestation, and in case 1 the only consulting sign of previously undiagnosed AIDS.
1 Durack DT: Opportunistic infections and Kaposi’s sarcoma in homosexual man. N Engl J Med 1981;305:1465-1467. 2 Fauci AS: The syndrome of Kaposi’s sarcoma and opportunistic infections. Ann Intern Med 1982;96:777-779. 3 Friedman-Kien AE, Laubenstein LJ, Rubinstein P, et al: Dis seminated Kaposi’s sarcoma in homosexual men. Ann Intern Med 1982;96:693-700. 4 Robbins S, Cotran R, Kumar V: Pathologic Bases of Disease. Philadelphia, Saunders, 1984, p 1270. 5 Kaplan MH, Sadick N, McNutt S, et al: Dermatologic findings and manifestations of acquired immunodeficiency syndrome (AIDS). J Am Acad Dermatol 1987;16:487-506. 6 Patow CA, Steis R, Longo DL, et al: Kaposi’s sarcoma of the head and neck in the acquired immunodeficiency syndrome. Otolaryngol Head Neck Surg 1984;92:255-260. 7 Levine AM, Meyer PR, Gill PS, et al: Results of initial lymph node biopsy in homosexual men with generalized lymphadenopathy. J Clin Oncol 1986;4:165-169. 8 Vadhan-Raj S, Won G, Gnecco C, et al: Immunological vari ables as predictors of prognosis in patients with Kaposi’s sar coma and the acquired immune deficiency syndrome. Cancer Res 1986;46:417-425. 9 Amberson JB, DiCarlo EF, Metroka CE, et al: Diagnostic pa thology in the acquired immunodeficiency syndrome. Arch Pa thol Lab Med 1985;109:345-351. 10 Niedt GW, Schinella RA: Acquired immunodeficiency syn drome. Clinicopathologic study of 56 autopsies. Arch Pathol Lab Med 1985;109:727-734. 11 Reichert CM, O’Leary TJ, Levens DL, et al: Autopsy pathology in the acquired immune deficiency syndrome. Am J Pathol 1983;1 12:357-382.
Downloaded by: Stockholm University Library 130.237.165.40 - 2/21/2018 1:56:15 AM
References Fig. 1. Histological high-power view of Kaposi’s sarcoma show ing vascular channels and lumina within a spindle cell and atypical neoplastic growth. HE. X 400.
Kaposi’s Sarcoma of the Penis
17 Levine AM, Gill PS, Mayer PR, et al: Retrovirus and malignant lymphoma in homosexual men. JAMA 1985;254:1921-1925.
Received: February 20, 1990 Accepted after revision: May 23, 1990 Dr. Javier C. Angulo Servicio de Urologia Santo Hospital Civil de Bilbao Avenida Montevideo, 18 E-48013 Bilbao (Spain)
Downloaded by: Stockholm University Library 130.237.165.40 - 2/21/2018 1:56:15 AM
12 Welch K, Frinkbeiner W, Alpers CE, et al: Autopsy findings in acquired immune deficiency syndrome. JAMA 1984;252:1152— 1159. 13 Kaplan MS, Wechsler M, Benson M: Urologic manifestations of AIDS. Urology 1987;30:441-443. 14 Miles BJ, Melser M, Farah R, et al: The urological manifesta tions of the acquired immunodeficiency syndrome. J Urol 1989; 142:771-773. 15 Giraldo G, Beth E, Huang ES: Kaposi’s sarcoma and its relation ship to cytomegalovirus (CMV). III. CMV, DNA and CMV anti gens in Kaposi’s sarcoma. Int J Cancer 1980;26:23-29. 16 Tirelli U, Rezza G, Lazzarin A, et al : Malignant lymphoma related to HIV infection in Italy: A report of 46 cases. JAMA 1987;258:2064.
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