Archivio Italiano di Urologia e Andrologia 2017; 89, 2164 CASE REPORT Kaposi’s sarcoma: An unusual penile lesion in a HIV negative patient Aldo Franco De Rose, Matteo Justich, Guglielmo Mantica, Nicolò Testino, Carlo Terrone Department of Urology, Ospedale Policlinico San Martino - Istituto di Ricerca e Cura a Carattere Scientifico per l’Oncologia, University of Genova, Genoa, Italy. Kaposi's sarcoma (KS) of the penis is a very rare lesion and it is usually observed in HIV-infected patients. We introduce a case of KS of the penis in a 75 years old HIV negative patient with a peripheral T-cell lymphoma. He came to our attention with a painful ulcerated red lesion on the glans that stretched from the urethral meatus to the coronal skin. This lesion was found to be a KS bal- anopreputial in the classical variant. Penile KS must be includ- ed in the differential diagnosis of genital diseases especially when the clinical features of the lesion are aspecific and diag- nosis can be made histologically by performing a biopsy. KEY WORDS: Kaposi sarcoma; Penile lesion; HIV negative; HHV-8; T-cell lymphoma. Submitted 17 January 2017; Accepted 1 March 2017 Summary No conflict of interest declared. The abdominal CT scan showed no inguinal lymph nodes or other suspected lesions. Due to an extended lesion and to the absence of a clear diagnose it was decided to perform a glansectomy. The histopathology examination described a classical balanopreputial KS. Six months later the patient was negative for local recur- rence but a secondary gastrointestinal lesion was detect- ed during a whole body CT scan and a gastric biopsy was performed. The patient is currently under therapy with interferon. DISCUSSION KS, described for the first time by Moritz Kaposi in 1872, is a rare neoplasm that origins from the endovascular cells in a multifocal way (1). The behavior of the disease varies from a singular indo- lent lesion localized in the skin, to a fleeting extensive respiratory and gastrointestinal visceral involvement (2). Four clinical variants have been described: classic, African endemic, iatrogenic or transplanted associated and HIV-related (3). Before the 1980s classic KS was rare and it increased because of the AIDS epidemic. Nowadays the introduc- tion of highly active anti-retroviral therapy (HAART) resulted in a reduction of the KS incidence (4, 5). Primary classical KS of the penis is rare in HIV negative patients (6) and in the literature has been reported only in DOI: 10.4081/aiua.2017.2.164 INTRODUCTION Kaposi's sarcoma (KS) is a malignant neoplasm that typi- cally arises in the skin of the extremities but any area of the face and trunk could be involved. Furthermore it often occurs in the lymph nodes and in the visceral organs (1-3). Penile occurrence is very uncommon so the diagnose and the treatment could be a hard challenge to the urologist. We introduce a very rare case of penile KS in a patient with peripheral T-cell lymphoma without HIV infection. CASE REPORT A 75 year-old man came to our attention with a paraphi- mosis arisen 2 days earlier. He reported a 5 month pain- less ulcerated red lesion on the glans extending from the urethral meatus to the coronal skin. (Figure 1). He did not complain urethral discharge nor there were palpable inguinal lymph nodes. He was diabetic, hyper- tensive and in 2014 it was diagnosed a T-cell lymphoma. So he was treated with COMP 6 cycles, then ben- damustin 4 cycles and finally romidepsin 6 cycles with the remission of the disease. A subtotal circumcision and a glans biopsy were per- formed. The histological examination could only provide evidence of immunohistochemical staining for HHV-8 both in the stromal cells and in the endothelial ones. There were no localizations of T-cell lymphoma. Urinary exam and blood laboratory tests were normal. HIV antibody test by ELISA was negative. Figure 1. Ulcerated red lesion on the glans extending from the urethral meatus to the coronal skin. De Rose_Stesura Seveso 20/06/17 10:05 Pagina 164 165Archivio Italiano di Urologia e Andrologia 2017; 89, 2 A rare case of penile Kaposi’s sarcoma 19 cases (7). It usually affects patients between the fifth and eighth decade of life living on the Mediterranean coastal areas where the HHV-8 infection is widespread (6). The pathogenesis of the disease is unclear but HHV-8 infection seems to be an essential cofactor for the devel- opment of the KS irrespective of the variant, clinical presentation or prognosis (1, 8). Beside a KS our patient presented an immunological T cell disorder and an HHV-8 infection. Probably the T-cell immunity plays a major role in the HHV-8 control and in the development of KS both in viral immunosuppressed patients both in iatrogenic ones. However no studies have shown a clear correlation between the presence of an immunological T disorder and a KS (9). The typical clinical feature of a penile KS is a purple papule or macule located on the glans, foreskin, frenulum, ure- thral meatus or coronal sulcus (6, 10). It rarely affects the shaft or the scrotum. Nodules, plaques, multiple papules, wart-like or pedunculated lesions are less common (5-6). The differential clinical diagnosis includes pyogenic granuloma, moluscum contagiosum, condiloma acumi- nate, glomus tumor, bowenoid papulosis, nevi, mela - noma and lymphoma (7). The conclusive diagnosis is made by biopsy. The histological pattern is not so differ- ent from KS seen in other areas of the body showing groups of spindle cells, extravascular erythrocytes, and macrophages filled with hemosiderin (1). The main tar- get of therapies is to decrease the symptoms, reduce the size, the number of the lesions and to delay the disease progression (11). Many therapeutic approaches have been described: Surgical excision, cryosurgery, radiation therapy, thermo-photoablative, laser therapy, local and systemic chemoterapy, α- and β-interferon (6). For small and single lesions surgical excision is recom- mended, while for multiple skin lesions or large-size is recommended radiation therapy. Systemic chemotherapy has been employed in systematic forms (10). In the case of radical surgical excision, local recurrence is rare (6). However it is important not to neglect the patient’s follow-up because a penile unique lesion could be the manifestation of a disseminated Kaposi's sarcoma. As reported by a Greek Author, 82% of patients with classic KS refer a gastric asymptomatic lesion. They rec- ommend therefore the execution of a gastroscopy in every patient with a new diagnose of KS (11-12). CONCLUSIONS In conclusion the spectrum of penile lesions is broad and the clinical assessment is often insufficient to reach a diagnosis and an adequate choice of treatment. Penile KS must be included in the differential diagnosis of the gen- ital diseases in particular when there are not specific-fea- ture penile lesions so a biopsy is advisable for reaching a histological diagnosis. REFERENCES 1. Seleit I, Attia A, Maree A, et al. Isolated Kaposi sarcoma in two negative patients. J Dermatologic Case Rep. 2011; 2:24-26. 2. Schwartz Ra, Micali G, Nasca MR, et al. Kaposi Sarcoma: a con- tinuing conundrum. J Am Acad Dermatol. 2008; 59:179-206 3. Tschachler E. Kaposi sarcoma. In: Wolff K, Goldsmith LA, Katz SI, et al, editors, Fitzpatrick’s dermatology in general medicine, 7th ed., New York: McGraw-Hill. 2008; p. 1183-7. 4. Pinto-Almeida T, Torres T, Rosmaninho A, et al. Penile kaposi sarcoma: a case of complete resolution with highly active antiretro- viral therapy alone. Dermatology Online Journal. 2011; 17:12. 5. Ho Kim K, Il Choi J, Ho Ryu K, et al. Primary Classic Kaposi's Sarcoma of the Penis in an HIV-Negative Patient Korean J Urol. 2010; 51:803-806. 6. Micali G, Nasca MR, De Pasquale R, et al. Primary classic Kaposi’s sarcoma of the penis: report of a case and review. J Eur Acad Derm Venereol. 2003; 17:320-323. 7. Attwa E, Gharib K, Albalat W, et al.Classical Kaposi sarcoma: case reports with unusualpresentation on the penis and scrotum Int. J Dermatol. 2016; 55:533-538. 8. Fatahzadeh M. Kaposi sarcoma: review and medical management. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012; 113:2-16. 9. Sabbah S. Jagne YJ, Zuo J, et al. T-cell immunity to Kaposi sarco- ma-associated herpesvirus: recognition of primary effusion lym- phoma by LANA-specific CD4+ T cells. Blood 2012; 119:2083-92. 10. 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. 11. Kolios G, Kaloterakis A, Filiotou A, et al. Gastroscopic findings in Mediterranean Kaposi’s sarcoma (non-AIDS). Gastrointest Endosc 1995; 42:336-339. 12. Balachandra B, Tunitsky E, Dawood S, et al. Classic Kaposi’s sarcoma presenting first with gastrointestinal tract involvement in a HIV-negative Inuit male - a case report and review of the literature. Pathol Res Pract. 2006; 202:623-6. Correspondence Aldo Franco De Rose, MD aldofrancoderose@gmail.com Matteo Justich, MD teojus@gmail.com Guglielmo Mantica, MD (Corresponding Author) guglielmo.mantica@gmail.com Nicolò Testino, MD nicolo.testino@live.it Carlo Terrone, MD carlo.terrone@med.unipo.it Department of Urology, Ospedale Policlinico San Martino - Istituto di Ricerca e Cura a Carattere Scientifico per l’oncologia, University of Genova Largo Rosanna Benzi 10 - 16132 Genova, Italy De Rose_Stesura Seveso 20/06/17 10:05 Pagina 165