AIDS PRESENTING AS PRIMARY TESTICULAR LYMPHOMA NOEL A. ARMENAKAS, M.D. MARIA M. SCHEVCHUK, M.D. MICHAEL BRODHERSON, M.D. JOHN A. FRACCHIA, M.D.
From the Departments of Urology and Pathology, Lenox Hill Hospital, New York, New York ABSTRACT-A case of AIDS presenting as a primary testicular lymphoma is reported. Despite the lack of evident systemic disease, such a presentation in a young patient should alert the physician to the possible presence of an underlying human immunodeficiency virus infection.
Acquired immunodeficiency syndrome (AIDS) is an abnormal immune state which alters the onset and evolution of certain opportunistic infections and neoplasms. For example, primary Kaposi sarcoma, previously rare in men under sixty years of age, is now considered a classic finding in all age groups with AIDS. Similarly, malignant lymphomas, of several histologic types, have become a designated manifestation of this syndrome. 1 We report the first case of primary testicular lymphoma as the initial presentation of AIDS.
of 5.1 x 10 with 58% polymorphonuclear leukocytes, 37 % lymphocytes, and 5 % monocytes, a lactate dehydrogenase of 750 U/L, and undetectable amounts of alpha-fetoprotein and betahuman chorionic gonadotropin. A left radical orchiectomy was performed, and pathologic examination disclosed a high-
Case Report A thirty-six-year-old white homosexual male was hospitalized because of painful left testicular enlargement and fever. He was well until two weeks prior to admission at which time he was prescribed a course of oral tetracycline and scrotal elevation, for a presumptive diagnosis of left epididymo-orchitis. The patient’s symptoms persisted, and an ultrasound examination confirmed an enlarged left testicle with a small centrally located hyperechoic mass (Fig. 1). Physical examination revealed a well-developed, well-nourished male in no acute distress. Rectal temperature was 38.5 OC. Pertinent findings included a markedly tender, indurated, enlarged left testicle and epididymis. There was no palpable lymphadenopathy. Significant laboratory data included a white blood cell count
FIGURE 1. Left testicular sonogram shows moderate enlargement with slight parenchymal inhomogeneity and distinct centrally-located hyperechoic lesion.
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FIGURE 2. (A) Longitudinal section of left testis showing involvement of entire parenchyma (original magnification 9 x 5 x 4 cm). (B) High magnijication of testicular lymphoma shows high-grade plasmacytoid features (original magnification x 400).
grade large-cell plasmacytoid malignant lymphoma (Fig. 2). Immunoperoxidase staining showed a monoclonal IgM positive, B-cell tumor. Because of the unusually young age of the patient for testicular lymphoma, a serum human immunodeficiency virus (HIV) test was drawn and found to be positive. Western Blot analysis confirmed the existence of the HIV infection detected by enzyme-linked immunosorbent assay. Metastatic evaluation included computerized tomograms of the chest, abdomen, and pelvis, which were normal. A bone scan detected increased uptake in the proximal left femur and left acetabulum; the nature of this was unclear, but lymphoma was never proved, on biopsy, in this location. The patient was treated with combination chemotherapy, using methotrexate, doxorubitin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP-B). His clinical course rapidly deteriorated with a 50-pound weight loss in less than two months. He succumbed to overwhelming opportunistic infections within three months of his orchiectomy. Autopsy revealed small foci of metastatic lymphoma in the liver, para-aortic lymph nodes, and brain. Opportunistic infections were extensive and included cytomegalovirus in the lungs, duodenum, adrenals, and seminal
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vesicles, as well pneumonitis.
as pneumocystis
carinii
Comment The incidence of malignant lymphomas of Bcell origin is markedly increased in the AIDS population, and their presence in an HIV-positive individual is diagnostic of AIDS. In AIDS patients, at the time of initial diagnosis, wide dissemination is expected with extranodal sites of disease described in 65 to 98 percent of patients.2 Mohler, Jarow, and Marshall3 described a case of AIDS initially presenting as disseminated lymphoma, including testicular involvement. We believe our case is the second reported in the literature of AIDS presenting as testicular lymphoma. However, we believe it is the first to present as primary testicular lymphoma, without overt systemic disease. Primary lymphoma of the testis is not an uncommon lesion, but it is present almost exclusively in patients older than fifty years. Therefore, the presence of a primary B-cell testicular lymphoma in a patient younger than fifty, may imply a significantly altered immune state and is highly suggestive of AIDS. This patient presented with primary testicular lymphoma, without clinical systemic
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100 East 77th Street New York, New York 10021-1883 (DR. ARMENAKAS)
manifestations of AIDS or lymphoma. He was found to be HIV positive only on testing, and many of the AIDS-associated opportunistic infections rapidly developed, to which he succumbed. With the increasing AIDS epidemic (365,000 diagnosed cases projected by 1992 in the United States), urologists will need to familiarize themselves with the genitourinary manifestations of the disease.4 Any patient younger than fifty years of age with a B-cell lymphoma of the testis warrants HIV testing. In this way, proper precautions and early diagnosis and initiation of treatment can be instituted.
1. Fauci SF, et al: Acquired immunodeficiency syndrome: epidemiologic, clinical, immunologic and therapeutic considerations (NIH Conference), Ann Intern Med 166: 92 (1984). 2. Levine A: Lymphoma in acquired immunodeficiency syndrome, Semin Oncol 17: 104 (1990). 3. Mohler JL, Jarow JP, and Marshall FF: Unusual urological presentations of acquired immune deficiency syndrome: large cell lymphoma, J Urol 138: 627 (1987). 4. AIDS and Human Immunodeficiency Virus Infection in the United States (Printed and distributed by Massachusetts Medical Society), Morbidity and Mortality Weekly Report, 38(S-4), (1989).
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From the Departments of Urology and Pathology, Lenox Hill Hospital, New York, New York ABSTRACT-A case of AIDS presenting as a primary testicular lymphoma is reported. Despite the lack of evident systemic disease, such a presentation in a young patient should alert the physician to the possible presence of an underlying human immunodeficiency virus infection.
Acquired immunodeficiency syndrome (AIDS) is an abnormal immune state which alters the onset and evolution of certain opportunistic infections and neoplasms. For example, primary Kaposi sarcoma, previously rare in men under sixty years of age, is now considered a classic finding in all age groups with AIDS. Similarly, malignant lymphomas, of several histologic types, have become a designated manifestation of this syndrome. 1 We report the first case of primary testicular lymphoma as the initial presentation of AIDS.
of 5.1 x 10 with 58% polymorphonuclear leukocytes, 37 % lymphocytes, and 5 % monocytes, a lactate dehydrogenase of 750 U/L, and undetectable amounts of alpha-fetoprotein and betahuman chorionic gonadotropin. A left radical orchiectomy was performed, and pathologic examination disclosed a high-
Case Report A thirty-six-year-old white homosexual male was hospitalized because of painful left testicular enlargement and fever. He was well until two weeks prior to admission at which time he was prescribed a course of oral tetracycline and scrotal elevation, for a presumptive diagnosis of left epididymo-orchitis. The patient’s symptoms persisted, and an ultrasound examination confirmed an enlarged left testicle with a small centrally located hyperechoic mass (Fig. 1). Physical examination revealed a well-developed, well-nourished male in no acute distress. Rectal temperature was 38.5 OC. Pertinent findings included a markedly tender, indurated, enlarged left testicle and epididymis. There was no palpable lymphadenopathy. Significant laboratory data included a white blood cell count
FIGURE 1. Left testicular sonogram shows moderate enlargement with slight parenchymal inhomogeneity and distinct centrally-located hyperechoic lesion.
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FIGURE 2. (A) Longitudinal section of left testis showing involvement of entire parenchyma (original magnification 9 x 5 x 4 cm). (B) High magnijication of testicular lymphoma shows high-grade plasmacytoid features (original magnification x 400).
grade large-cell plasmacytoid malignant lymphoma (Fig. 2). Immunoperoxidase staining showed a monoclonal IgM positive, B-cell tumor. Because of the unusually young age of the patient for testicular lymphoma, a serum human immunodeficiency virus (HIV) test was drawn and found to be positive. Western Blot analysis confirmed the existence of the HIV infection detected by enzyme-linked immunosorbent assay. Metastatic evaluation included computerized tomograms of the chest, abdomen, and pelvis, which were normal. A bone scan detected increased uptake in the proximal left femur and left acetabulum; the nature of this was unclear, but lymphoma was never proved, on biopsy, in this location. The patient was treated with combination chemotherapy, using methotrexate, doxorubitin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP-B). His clinical course rapidly deteriorated with a 50-pound weight loss in less than two months. He succumbed to overwhelming opportunistic infections within three months of his orchiectomy. Autopsy revealed small foci of metastatic lymphoma in the liver, para-aortic lymph nodes, and brain. Opportunistic infections were extensive and included cytomegalovirus in the lungs, duodenum, adrenals, and seminal
UROLOGY
/ AUGUST
1992
i
VOLUME
40, NUMBER 2
vesicles, as well pneumonitis.
as pneumocystis
carinii
Comment The incidence of malignant lymphomas of Bcell origin is markedly increased in the AIDS population, and their presence in an HIV-positive individual is diagnostic of AIDS. In AIDS patients, at the time of initial diagnosis, wide dissemination is expected with extranodal sites of disease described in 65 to 98 percent of patients.2 Mohler, Jarow, and Marshall3 described a case of AIDS initially presenting as disseminated lymphoma, including testicular involvement. We believe our case is the second reported in the literature of AIDS presenting as testicular lymphoma. However, we believe it is the first to present as primary testicular lymphoma, without overt systemic disease. Primary lymphoma of the testis is not an uncommon lesion, but it is present almost exclusively in patients older than fifty years. Therefore, the presence of a primary B-cell testicular lymphoma in a patient younger than fifty, may imply a significantly altered immune state and is highly suggestive of AIDS. This patient presented with primary testicular lymphoma, without clinical systemic
163
100 East 77th Street New York, New York 10021-1883 (DR. ARMENAKAS)
manifestations of AIDS or lymphoma. He was found to be HIV positive only on testing, and many of the AIDS-associated opportunistic infections rapidly developed, to which he succumbed. With the increasing AIDS epidemic (365,000 diagnosed cases projected by 1992 in the United States), urologists will need to familiarize themselves with the genitourinary manifestations of the disease.4 Any patient younger than fifty years of age with a B-cell lymphoma of the testis warrants HIV testing. In this way, proper precautions and early diagnosis and initiation of treatment can be instituted.
1. Fauci SF, et al: Acquired immunodeficiency syndrome: epidemiologic, clinical, immunologic and therapeutic considerations (NIH Conference), Ann Intern Med 166: 92 (1984). 2. Levine A: Lymphoma in acquired immunodeficiency syndrome, Semin Oncol 17: 104 (1990). 3. Mohler JL, Jarow JP, and Marshall FF: Unusual urological presentations of acquired immune deficiency syndrome: large cell lymphoma, J Urol 138: 627 (1987). 4. AIDS and Human Immunodeficiency Virus Infection in the United States (Printed and distributed by Massachusetts Medical Society), Morbidity and Mortality Weekly Report, 38(S-4), (1989).
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