Primary Kaposi's sarcoma of the penis

Abstracts

Sarcoma de Kaposi é um tumor vascular que afeta a parede dos vasos linfáticos. Possui quatro formas: clássica, endêmica, iatrogênica e associada ao HIV. É uma doença sistêmica, maligna, multifatorial e de curso variável. A apresentação inicial no pênis é rara, e mais observada em pacientes HIV positivos. Em pacientes HIV negativos, os casos que ocorrem nesta região, apresentam-se com pápulas, nódulos, placas e lesões verruciformes, assintomáticas. Para o tratamento da forma clássica, dispõem-se de excisão cirúrgica, crioterapia, eletrocirurgia, laser e radioterapia. Neste trabalho, é relatado um caso raro de um paciente com a forma clássica, em região peniana tratado com sucesso com radioterapia.

Pênis; Radioterapia; Sarcoma de Kaposi

CASE REPORT

Primary Kaposi's sarcoma of the penis

Maira Mitsue Mukai^I; Talitha Chaves^II; Lucio Caldas^III; José Fillus Neto^IV; Jesus Rodriguez Santamaría^V

^ITemporary lecturer of Dermatology, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^IIUndergraduate student, Medical School, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^IIIDermatologist, member of the Sociedade Brasileira de Dermatologia

^IVAdjunct Professor of Pathological Anatomy, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^VAdjunct Professor and Head of the Department of Dermatology, Universidade Federal do Paraná (UFPR) – Curitiba (PR), Brazil

^{Mailing Address}

ABSTRACT

Kaposi's sarcoma is a vascular tumor involving the wall of lymphatic vessels. There are four types: classic, endemic, iatrogenic and HIV-associated. It is a systemic, malignant and multifactorial disease and has a variable course. The primary presentation on the penis is uncommon and is mainly observed in HIV-positive patients. In HIV-negative individuals, asymptomatic papules, nodules, plaques and verrucous lesions are found. The treatment for the classic form involves surgery, cryotherapy, electrosurgery, laser and radiation therapy. The authors present a rare case of a patient with the classic form on the penis, who was successfully treated by radiation therapy.

Keywords: Penis; Radiotherapy; Sarcoma, Kaposi

INTRODUCTION

Kaposi's sarcoma is a vascular neoplasm of multifocal origin, described by Morris Kaposi, in 1872. Since then, four clinical forms were described: classic, endemic, iatrogenic and HIV-associated, and the latter is the most frequent. The classic form restricted to the penile region is extremely rare. The authors report a case of Kaposi's sarcoma of the glans penis, successfully treated with radiation therapy.

CASE REPORT

A 64-year-old male patient with history of a violet lesion in the urethral meatus for eight months, associated with altered urinary stream and no other symptoms. He had an appointment with an urologist and the lesion was excised and diagnosed as hemangioma of urethral meatus. After the procedure new similar lesions appeared on the penis, and one was bleeding (Figure 1). Upon palpation, right inguinal adenopathy was observed. Biopsy of one of the lesions was performed and the histological findings were compatible with Kaposi's sarcoma (Figures 2 and 3).

To complement investigation, VDRL and anti-HIV were ordered and both were negative. The patient was referred to radiation therapy and completed 40 sessions. By the end of treatment, the nodules diminished and small ulcerations remained in the balanopreputial sulcus due to therapy and healed after 30 days (Figures 4A and 4B).

DISCUSSION

Kaposi's sarcoma can be classified into four distinct forms: classic, endemic, iatrogenic and AIDS-associated.¹ The classic form is frequently observed in elderly male patients of Mediterranean origin and Ashkenazi Jews, presenting as an indolent disease, with nodular lesions on the skin, and affecting more often the lower limbs and feet and rarely presenting visceral involvement.

The primary presentation on the penis is rare and it is more common in HIV-positive patients, in whom it has an aggressive behavior. ^2,3 HIV-negative patients with this tumor on the penile region present papules, nodules, plaques and verrucous lesions of erythematous violet color, which are generally asymptomatic.⁴ Most patients have few lesions, although multiple papules may be observed. The sites more often involved are glans penis (more common), foreskin, coronal sulcus, penile body, frenulum and urethral meatus.^4,5 Increased penile volume and lymphatic edema due to massive involvement were described in some cases.^6,7

The differential clinical diagnosis includes pyogenic granuloma, moluscum contagiosum, condiloma acuminata and bowenoid papulosis.⁸ The conclusive diagnosis is made by biopsy.³ The histological pattern found is similar to that of Kaposi's sarcoma seen in other areas of the body. The exact nature of the disease remains unknown. Today the association of Kaposi's sarcoma with human herpesvirus 8 (HHV-8), which is sexually transmitted by blood or saliva, is under consideration.^9,10

The literature describes the following options to treat the classic form: local surgical excision, cryotherapy, electrosurgery, chemotherapy, laser therapy and radiation therapy, besides adjuvant treatment with intralesional or systemic injections with cytostatic agents, alpha- and beta-interferon.4 Therapy should be chosen for each patient and there is no consensus established. For small and single lesions, surgical excision may be recommended.⁸ Local recurrence is rare after complete excision of the primary lesion. When present, relapse occurs from six months to two years after the procedure. In case of larger and multiple lesions, the best option is radiation therapy, and recurrence is infrequent. In more advanced cases, with visceral or generalized involvement, chemotherapy may be a good option.

Although indicated for this type tumor, radiation therapy should be employed with periodical and continuous monitoring. Fibrosis of the body of the penis, erectile dysfunction and new lesions in areas not treated may be observed.^5,9

Kaposi's sarcoma is rare in immunocompetent individuals but must be included in the differential diagnosis of diseases in the genital region.

REFERENCES

Conflict of interest: None

Financial funding: None

How to cite this article: Mukai MM, Chaves T, Caldas L, Fillus Neto J, Santamaria JR. Sarcoma de Kaposi primário do pênis. An Bras Dermatol. 2009;84(5):524-6.

• 1. Morelli L, Pusiol T, Piscioli F, Hofler H, Weirich G, Werner M, et al Herpesvirus 8-associated penile Kaposi's sarcoma in an HIV-negative patient: first report of a solitary lesion. Am J Dermatopathol. 2003;25:28-31
• 2. Bayne D, Wise GJ. Kaposi sarcoma of penis and genitalia: a disease of our times. Urology. 1988;31:22-5
• 3. Chitale SV, Peat D, Meaden JD, Johnson HB, Burgess NA. Kaposi's sarcoma of the glans penis in an HIV negative patient. Int Urol Nephrol. 2002;34:251-3
• 4. Micali G, Nasca MR, De Pasquale R, Innocenzi D. Primary classic Kaposi's sarcoma of the penis: report of a case and review. J Eur Acad Dermatol Venereol. 2003;17:320-3
• 5. Ekmekci TR, Kendirci M, Kizilkaya O, Koslu A. Sildenafil citrate-aided radiotherapy for the treatment of Kaposi's sarcoma of the penis. J Eur Acad Dermatol Venereol. 2005;19:603-4
• 6. Gönen M, Cenker A, Kiyici H, Kalkan M. Penile Kaposi's sarcomas in a circumcised and HIV-seronegative patient. Int J Urol. 2006;13:318-20
• 7. Schwartz RA, Cohen JB, Watson RA, Gascon P, Ahkami RN, Ruszczak Z, et al Penile Kaposi's sarcoma preceded by chronic penile lymphoedema. Br J Dermatol. 2000;142:153-6
• 8. Pacifico A, Piccolo D, Fargnoli MC, Peris K. Kaposi's sarcoma of the glans penis in an immunocompetent patient. Eur J Dermatol. 2003;13:582-3
• 9. Zargari O. Exclusive penile Kaposi's sarcoma: report of an HIV-negative man successfully treated with radiotherapy. J Eur Acad Dermatol Venereol. 2006;20:318-20
• 10. Monini P, de Lellis L, Fabris M, Rigolin F, Cassai E. Kaposi's sarcoma-associated herpesvirus DN sequences in prostate tissue and human semen. N Engl J Med. 1996;334:1168-72

Publication Dates

• Publication in this collection
  07 Jan 2010
• Date of issue
  Oct 2009

History

• Accepted
  20 Oct 2008
• Received
  04 Apr 2008