Recurrent lymphoma presenting as a penile ulcer in a patient with AIDS
Published Web Locationhttps://doi.org/10.5070/D32k49p3gp
Recurrent lymphoma presenting as a penile ulcer in a patient with AIDS
Internal Medicine, Peace Harbor Hospital, Associates of PeaceHealth, 380 9th street, Florence, OR 97439
Fadi I Jabr MD
Dermatology Online Journal 11 (2): 29
Penile lymphoma is a rare neoplasm. We report a 45-year-old man with history of AIDS and previous retroperitoneal lymphoma who presented with painless penile ulceration and multiple inguinal lymphadenopathy. Fine needle biopsy showed diffuse, large B-cell lymphoma. The patient was treated with systemic chemotherapy with remission and healing of the ulcer. We review the literature and discuss this uncommon presentation of lymphoma.
Penile malignant tumors are uncommon . The most common type is squamous carcinoma . Penile lymphoma, however, is rare and not reported in association with HIV infection. Chemotherapy is a good option of treatment even in primary penile lymphoma because it preserves the erectile function and avoids disfigurement [3, 4]. We discuss the case of recurrent penile lymphoma presenting as penile ulcer with inguinal lymphadenopathy in an AIDS patient. Chemotherapy resulted in good response and healing of the ulceration.
|Figure 1||Figure 2|
|Fig. 1. Penile ulceration and swelling with an enlarged right inguinal lymph node.|
|Fig. 2. Healing of the ulcer with scarring after chemotherapy.|
A 45-year-old man was evaluated for a penile lesion. He had a history of hepatitis C, intravenous drug use, and AIDS for 4 years, and was diagnosed with localized, retroperitoneal, diffuse large-B-cell lymphoma 1½ years ago. He received chemotherapy comprising of cyclophosphamide, vincristine, oncovin, and prednisone (CHOP); he developed complete remission accompanied by a drop of serum marker lactic dehydrogenase (LDH) from 1194 u/l to 164 u/l. He was lost to followup until he presented again with a 2-month history of fever, lower abdominal pain, increasing swelling, multiple lesions of the groin and scrotum, and painless ulcers on the penile shaft. Patient was not taking highly active antiretroviral therapy (HAART) for 3 months prior to presentation. His physical examination revealed mild abdominal tenderness, scrotal and penile shaft swelling, 2 x 2 cm well-demarcated penile ulcer with black necrotic base, and multiple inguinal lymphadenopathy (Fig. 1). No urethral discharge or hepatosplenomegaly were noted. Blood tests revealed CD4 lymphocytes count 25 cells / mm3, HIV PCR 175,000 copies / ml, LDH 408 u / l, AST 43 U / L, ALT 24 U / L, albumin 1.7 g / dl, white cell count 8450 / mm3 (neutrophils 90 %), hemoglobin 11 g/dl, platelet count 310,000 / mm3, blood urea nitrogen 12 mg / dl, serum creatinine 1.2 mg / dl, and normal serum electrolytes. Computed tomograph of the abdomen and pelvis showed new appearance of extensive bilateral inguinal, anterior lower abdominal wall, and pelvic lymphadenopathy and complete resolution of the previous retroperitoneal lymphadenopathy. Liver and spleen were not involved. A fine needle aspirate of one of the inguinal masses and biopsy of the penile ulcer revealed atypical lymphocytes (B-cells) consistent with diffuse large B-cell lymphoma. Staining was negative for acid-fast bacilli and cultures for bacteria and fungi had no yield. Bone marrow biopsy revealed no infiltration by lymphoma.
Initially, HAART was given but this was complicated by lactic acidosis so it was stopped. After lactate level normalized, six cycles of CHOP were given with healing of the penile ulcer and resolution of the inguinal lymphadenopathy (Fig. 2). He was restarted and maintained on HAART and remained disease free after 6-months followup.
Soft tissue tumors of the penis occur in 1-2 per 100,000 cases per year in the United States . Among malignant tumors, squamous carcinoma is the most common. In a review of 50 patients with penile cancer, the most common type of cancer was squamous carcinoma in 46 patients, followed by verrucous carcinoma in 3 patients and melanoma in one patient . Penile lymphoma is rare and only 17 cases are reported in the literature [3 - 11]. Some of these were secondary localizations. Both B-cell [7, 8] and T-cell subtypes [3, 5, 9] are reported.
HIV-infected patients may have a higher prevalence of penile cancer than the general population. In a study of HIV patients with unusual malignant tumors, 2 out of 43 patients have penile cancer . However, none of the reported cases of penile lymphoma are associated with HIV infection or AIDS.
The clinical presentations of penile lymphoma includes indurated plaques, nodules, and diffuse penile swelling, and ulceration with or without induration [3, 4, 6]. Localization on the penis can be either primary or secondary to systemic lymphoma and differentiation is important in terms of treatment and prognosis . The diagnosis of penile lymphoma presenting as an ulceration can be difficult. The differential diagnosis includes carcinoma, drug reaction, vasculitis, trauma, and sexually transmitted diseases. Genital self-mutilation is also described as a cause of penile ulcer . Interestingly, some patients with penile lymphoma underwent extensive psychiatric evaluation and subsequent delay in management before the diagnosis was finally made . Workup should include serological studies for sexually transmitted diseases, and biopsy. In some cases, rebiopsy is required to make the diagnosis . If lymphoma is confirmed then computed tomography and bone marrow biopsy may be needed to exclude systemic involvement.
Treatment for penile lymphoma varies depending if it is primary or secondary. For secondary presentation, the treatment is systemic chemotherapy. In primary type, the treatment guidelines are difficult to establish because of the very limited number of cases; recommendations have included chemotherapy, local radiotherapy, surgery, or combined modalities of both surgery and chemotherapy or radiotherapy and chemotherapy [3-11]. In many cases, surgery and radiotherapy result in significant morbidity with disfigurement or loss of erectile function. Yet, good outcome with complete remission and preservation of penile function with chemotherapy alone has been documented [3, 4, 10]. Reported 2-year disease-free survival after chemotherapy has been as high as 83 percent for primary diffuse large cell lymphoma . The initial chemotherapy regimen is CHOP; it is reported that healing of the ulcerated lesion occurs after the completion of the second cycle . In many reports, as in our case, the healed lesion did not require further cosmetic surgery or skin grafting after chemotherapy [3, 4]. Recurrence may occur, and in some reports a second line of chemotherapy (hyperCVAD) is given with good response and remission for 2 years of follow-up . In primary penile lymphoma, good prognostic factors include young age and presence of CD30+ lymphocyte marker . An unfavorable prognostic sign is a high LDH level .
In brief, we report a case of recurrent penile lymphoma presenting as a penile ulcer in AIDS patient The patient received chemotherapy with good response. Finally, though penile lymphoma is rare, we should consider it in the differential diagnosis of penile ulceration.
References1. Schellhammer P, Jordan GH, Schlossberg SM. Tumors of the penis. In: Campbell's Urology, 6th ed. Edited by Walsh PC, Retik AB, Stamey TA, Vaughn ED. Philadelphia: W. B. Saunders Co., 1992; vol. 2, chapt. 31, pp. 1264-1291
2. Heyns CF, van Vollenhoven P, Steenkamp JW, Allen FJ. Cancer of the penis--a review of 50 patients. S Afr J Surg. 1997; 35: 120-4.
3. Tomb RR, Stephan F, Klein-Tomb L, Chahine G, Grosshans E. Recurrent primary CD30+ lymphoma of the penis. Br J Dermatol. 2003; 149: 903-5
4. Fairfax CA, Hammer CJ, Dana BW, Hanifin JM, Barry JM. Primary penile lymphoma presenting as a penile ulcer. J Urol 1995; 153: 1051-2
5. Lin DW, Thorning DR, Krieger JN. Primary penile lymphoma: diagnostic difficulties and management options. Urology 1999; 54: 366-71
6. Cribier B, Lipsker D, Grosshans E et al. Genital ulceration revealing a primary cutaneous anaplastic lymphoma. Genitourin Med 1997; 73: 325
7. Arena F, di Stefano C, Peracchia G, Barbieri A, Cortellini P. Primary lymphoma of the penis: diagnosis and treatment. Eur Urol. 2001; 39: 232-5.
8. Lo HC, Yu DS, Lee CT, Chen A, Chang SY, Sun GH. Primary B cell lymphoma of the penis: successful treatment with organ preservation. Arch Androl. 2003; 49: 467-70.
9. Wang HT, Lo YS, Huang JK. Primary lymphoma of the penis. J Chin Med Assoc. 2003; 66: 379-81.
10. Marks D, Crosthwaite A, Varigos G, Ellis D, Morstyn G. Therapy of primary diffuse large cell lymphoma of the penis with preservation of function. J Urol 1988; 139: 1057.
11. El-Sharkawi A, Murphy J. Primary penile lymphoma. The case for combined modality therapy. Clin Oncol 1996; 8: 334-5.
12. Santos J, Palacios R, Ruiz J, Gonzalez M, Marquez M. Unusual malignant tumors in patients with HIV infection. Int J STD AIDS. 2002; 13: 674-6.
13. Greilsheimer H, and Groves JE. Male genital self-mutilation. Arch Gen Psychiat. 1979; 36: 441
© 2005 Dermatology Online Journal