British Journal of Urology (1975). 41, 315-318
Kaposi’s Sarcoma of the Penis WILLIAM HOUSTON, ALAN PONTIN, TRUDI KUHN
and
NOBE MAMBO
Department of Urology, Mpilo Hospital, Bulawayo
Kaposi’s sarcoma is not commonly seen in non-African countries, but with the increase in travel, “rare” diseases may be seen far from their normal habitat. Kaposi’s sarcoma of the penis is extremely rare; only 12 cases have previously been recorded (Hayes, 1971). Of these, only 9 involved the glans penis, but a 10th such case was reported by Summers (1972). An 1lth case is now added, which is also of interest in that a correct preoperative diagnosis was made. Case Report A 40-year-old Bantu patient was admitted from a rural clinic which he had attended complaining of swelling of his penis which had been present a few weeks. The doctor who referred him considered that the lesion might be a carcinoma. The patient was well-nourished and not in pain. He gave no history of haematuria or other symptoms of urinary tract disease. Physical examination showed slight bleeding, and a moderate purulent discharge from beneath a swollen prepuce. The latter could be retracted sufficiently to reveal a red raised nodule, 2 mm high and 10 mm in diameter, on the right side of the glans penis. There was no significant inguinal lymphadenopathy, and general examination was negative, except for the right foot. This was mildly oedematous, with nodules typical of Kaposi’s sarcoma (Fig. 1). Because the lesion of the glans penis did not resemble the florid, papilliferous carcinoma of the penis commonly seen in Central Africa, and of the other evidence of Kaposi’s sarcoma, a provisional diagnosis of Kaposi’s sarcoma of the glans penis and right foot was made. Radiography of the right ankle and foot showed “an area of translucency just above the distal end of the tibia, with a depression on the lateral aspect. There is considerable bone absorption in the fifth metatarsal, and rather less in the third and fourth. There is also absorption of bone in the proximal phalanges of most toes. The appearances are compatible with Kaposi’s sarcoma.” Biopsy of the skin nodules and excision biopsy of the lesion of the glans penis were carried out. Histological examination of the specimens from the foot showed a fibroblastic angiomatous tumour in the deeper dermis with considerable cellular pleomorphism and mitotic activity (Figs. 2 and 3). Histological examination of the penile tumours showed that the lesion was fibroblastic, being composed of whorls of spindle cells in an angiomatous stroma, in which there were areas of red cell extravasation and haemosiderin deposition (Fig. 4). Higher power examination showed sarcomatous features with nuclear pleomorphism (Fig. 5). 1 year later the patient showed no evidence of recurrence of the penile lesion, but has since been lost to follow-up.
Discussion Involvement of every organ except the brain has been described in patients with Kaposi’s sarcoma (Davey, 1968). But even in Bantu patients in whom the incidence of this tumour is about 10 times that in Caucasians (Oettle, 1962), involvement of structures other than the skin, in the earlier stages, is uncommon, and involvement of the penis, even when the disease has become generalised, is extremely rare. Hayes et al. (1971) reported a case involving the glans penis, and found 8 similar cases in the literature and 4 other cases affecting the shaft of the penis. They quote Philippson (1902), who described a case in which the initial lesion on the glans penis had not recurred 8 years after local excision although generalised cutaneous lesions had appeared at 4 years. Summers et al. (1972) found 9 cases involving the glans penis in the literature, and reported another in a 60-year-old Italian. In Africa, Kaposi’s sarcoma follows a geographic distribution similar to that of Burkitt’s lymphoma, which is a disease of the reticulo-endothelial system (Oettle, 1962). It accounts for 315
316
BRITISH JOURNAL OF UROLOGY
Fig. 1. Right foot showing nodules typical of Kaposi’s sarcoma.
10% of malignancies in natives of the Congo and a large proportion in the Bantu of Southern Africa (Rothman, 1962; Davies, 1962), but is unusual in American Negroes (Herring, 1963). It was originally considered to be confined to the Jewish and Italian races, but Bluefarb (1957) documented its occurrence in all major ethnic groups. All the evidence suggests that Kaposi’s sarcoma is linked to a generalised disorder of the reticulo-endothelial system (Dayan et ul., 1967). This would suggest that treatment of local lesions should be conservative. The tumour is known to be radio-sensitive, and responds well to local perfusion with Methotrexate. A 10-year survival without treatment, and showing n o change, is recorded (McCarthy and Pack, 1950). The preferred treatment of a penile lesion would therefore seem to be conservative, by local excision, possibly supplemented by a small dose of irradiation. Summers et ul. (1972) suggest Fig. 2. Skin from dorsum of foot. A fibroblastic angiomatous tumour in the deeper dermis. H & E.
x 80.
(See right)
Fip. 3. Skin from dorsum of foot. High power of tumour in Figure 1. There is considerable cellular pleomorphism and mitotic activity. H & E. x 200. (See right) Fig. 4. Penile tumour. The lesion is fibroblastic, being composed of whorls of spindle cells in an angiomatous stroma in which there are areas of red cell extravasation and haernosiderin deposition. H & E. x 32. (See right) Fig. 5. Penile tumour. High power of Figure 3, showing sarcomatous features with nuclear pleornorphism. H & E. ~200. (See right)
KAPOSI'S SARCOMA OF THE PENIS
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1,800 R. Although Hayes el al. (1971) favour radical measures, and advise amputation on the basis of rapid recurrence after 6,000 rads in their case, our experience supports the adoption of a very conservative approach. It should be emphasised that local application of 5-fluorouracil is without effect in our experience. Summary
The 1 1th case of Kaposi’s sarcoma of the glans penis is reported. Aetiology and treatment are discussed and evidence adduced to support a conservative surgical approach by local excision, supplemented if necessary by low dosage irradiation and possibly methotrexate infusion. I should like to thank Dr Andrew Watson for the radiological report, and Dr Alan Mills for the histological reports and microphotographs.
References BLUEFARLI, S . M. (1957). Kaposi’s Sarcoma: Multiple Idiopathic Huemorrhagic Sarcoma, Springfield, 111. : Thomas, p. 140. W. W. (1968). Companion to Surgery in Africa, Edinburgh: Livingstone, p. 17. DAVEY, J. N. (1962). Kaposi’s sarcoma: re-evaluation based on the disease in Africans. Acta Unio Internationalis DAVIES, contra Cancrum, 18, 372-375. DAYAN, A. D. and LEWIS,P. D. (1967). Origin of Kaposi’s sarcoma from the reticulo-endothelial system. Nature, 213, 889-893. HAYES, C. W., CLARK,R. M. and POLITANO, V. A. (1971). Kaposi’s sarcoma of the penis. Journal of Urology, 105, 525-527. HERRING, B. D. (1963). Kaposi’s sarcoma in the negro. Journal of the American Medical Association, 185, 540-542. W. D. and PACK,G. T. (1950). Malignant blood vessel tumors: report of 56 cases of angiosarcoma and MCCARTHY, Kaposi’s sarcoma. Surgery, Gynecology and Obstetrics, 91, 465-482. OETTLE,A. G. (1962). Geographical and racial differences in the frequency of Kaposi’s sarcoma as evidence of environmental or genetic causes. Acta Unio Internationalis contra Cancrum, 18, 330-363. PHILIPPSON, L. (1902). Ueber das Sarcoma Idiopathicum cutis Kaposi: ein Beitrag zur Sarcomlehre. Virchows Archiv fur pathologische Anatornie, 167, 58. ROTHMAN, S . (1962). Medical research in Africa. Archives of Dermatology, 85, 31 1-324. SUMMERS, J. L., WILKERSON, J. R. and WEGRYN, J. F. (1972). Conservative therapy for Kaposi’s sarcoma of the external genitalia. Journal of Urology, 108, 287-289.
The Authors William Houston, MD, MCh, FRCS, Consultant in Urology. Alan Pontin, FRCS, Senior House Officer (now Registrar in Urology, Groote Schuur Hospital, Cape Town). Trudi Kuhn, MB, Surgical Registrar. Nobe Mambo, MB, Junior House Officer (now Registrar in Pathology, Harari Hospital, Salisbury, Rhodesia).
Kaposi’s Sarcoma of the Penis WILLIAM HOUSTON, ALAN PONTIN, TRUDI KUHN
and
NOBE MAMBO
Department of Urology, Mpilo Hospital, Bulawayo
Kaposi’s sarcoma is not commonly seen in non-African countries, but with the increase in travel, “rare” diseases may be seen far from their normal habitat. Kaposi’s sarcoma of the penis is extremely rare; only 12 cases have previously been recorded (Hayes, 1971). Of these, only 9 involved the glans penis, but a 10th such case was reported by Summers (1972). An 1lth case is now added, which is also of interest in that a correct preoperative diagnosis was made. Case Report A 40-year-old Bantu patient was admitted from a rural clinic which he had attended complaining of swelling of his penis which had been present a few weeks. The doctor who referred him considered that the lesion might be a carcinoma. The patient was well-nourished and not in pain. He gave no history of haematuria or other symptoms of urinary tract disease. Physical examination showed slight bleeding, and a moderate purulent discharge from beneath a swollen prepuce. The latter could be retracted sufficiently to reveal a red raised nodule, 2 mm high and 10 mm in diameter, on the right side of the glans penis. There was no significant inguinal lymphadenopathy, and general examination was negative, except for the right foot. This was mildly oedematous, with nodules typical of Kaposi’s sarcoma (Fig. 1). Because the lesion of the glans penis did not resemble the florid, papilliferous carcinoma of the penis commonly seen in Central Africa, and of the other evidence of Kaposi’s sarcoma, a provisional diagnosis of Kaposi’s sarcoma of the glans penis and right foot was made. Radiography of the right ankle and foot showed “an area of translucency just above the distal end of the tibia, with a depression on the lateral aspect. There is considerable bone absorption in the fifth metatarsal, and rather less in the third and fourth. There is also absorption of bone in the proximal phalanges of most toes. The appearances are compatible with Kaposi’s sarcoma.” Biopsy of the skin nodules and excision biopsy of the lesion of the glans penis were carried out. Histological examination of the specimens from the foot showed a fibroblastic angiomatous tumour in the deeper dermis with considerable cellular pleomorphism and mitotic activity (Figs. 2 and 3). Histological examination of the penile tumours showed that the lesion was fibroblastic, being composed of whorls of spindle cells in an angiomatous stroma, in which there were areas of red cell extravasation and haemosiderin deposition (Fig. 4). Higher power examination showed sarcomatous features with nuclear pleomorphism (Fig. 5). 1 year later the patient showed no evidence of recurrence of the penile lesion, but has since been lost to follow-up.
Discussion Involvement of every organ except the brain has been described in patients with Kaposi’s sarcoma (Davey, 1968). But even in Bantu patients in whom the incidence of this tumour is about 10 times that in Caucasians (Oettle, 1962), involvement of structures other than the skin, in the earlier stages, is uncommon, and involvement of the penis, even when the disease has become generalised, is extremely rare. Hayes et al. (1971) reported a case involving the glans penis, and found 8 similar cases in the literature and 4 other cases affecting the shaft of the penis. They quote Philippson (1902), who described a case in which the initial lesion on the glans penis had not recurred 8 years after local excision although generalised cutaneous lesions had appeared at 4 years. Summers et al. (1972) found 9 cases involving the glans penis in the literature, and reported another in a 60-year-old Italian. In Africa, Kaposi’s sarcoma follows a geographic distribution similar to that of Burkitt’s lymphoma, which is a disease of the reticulo-endothelial system (Oettle, 1962). It accounts for 315
316
BRITISH JOURNAL OF UROLOGY
Fig. 1. Right foot showing nodules typical of Kaposi’s sarcoma.
10% of malignancies in natives of the Congo and a large proportion in the Bantu of Southern Africa (Rothman, 1962; Davies, 1962), but is unusual in American Negroes (Herring, 1963). It was originally considered to be confined to the Jewish and Italian races, but Bluefarb (1957) documented its occurrence in all major ethnic groups. All the evidence suggests that Kaposi’s sarcoma is linked to a generalised disorder of the reticulo-endothelial system (Dayan et ul., 1967). This would suggest that treatment of local lesions should be conservative. The tumour is known to be radio-sensitive, and responds well to local perfusion with Methotrexate. A 10-year survival without treatment, and showing n o change, is recorded (McCarthy and Pack, 1950). The preferred treatment of a penile lesion would therefore seem to be conservative, by local excision, possibly supplemented by a small dose of irradiation. Summers et ul. (1972) suggest Fig. 2. Skin from dorsum of foot. A fibroblastic angiomatous tumour in the deeper dermis. H & E.
x 80.
(See right)
Fip. 3. Skin from dorsum of foot. High power of tumour in Figure 1. There is considerable cellular pleomorphism and mitotic activity. H & E. x 200. (See right) Fig. 4. Penile tumour. The lesion is fibroblastic, being composed of whorls of spindle cells in an angiomatous stroma in which there are areas of red cell extravasation and haernosiderin deposition. H & E. x 32. (See right) Fig. 5. Penile tumour. High power of Figure 3, showing sarcomatous features with nuclear pleornorphism. H & E. ~200. (See right)
KAPOSI'S SARCOMA OF THE PENIS
317
318
BRITISH JOURNAL OF UROLOGY
1,800 R. Although Hayes el al. (1971) favour radical measures, and advise amputation on the basis of rapid recurrence after 6,000 rads in their case, our experience supports the adoption of a very conservative approach. It should be emphasised that local application of 5-fluorouracil is without effect in our experience. Summary
The 1 1th case of Kaposi’s sarcoma of the glans penis is reported. Aetiology and treatment are discussed and evidence adduced to support a conservative surgical approach by local excision, supplemented if necessary by low dosage irradiation and possibly methotrexate infusion. I should like to thank Dr Andrew Watson for the radiological report, and Dr Alan Mills for the histological reports and microphotographs.
References BLUEFARLI, S . M. (1957). Kaposi’s Sarcoma: Multiple Idiopathic Huemorrhagic Sarcoma, Springfield, 111. : Thomas, p. 140. W. W. (1968). Companion to Surgery in Africa, Edinburgh: Livingstone, p. 17. DAVEY, J. N. (1962). Kaposi’s sarcoma: re-evaluation based on the disease in Africans. Acta Unio Internationalis DAVIES, contra Cancrum, 18, 372-375. DAYAN, A. D. and LEWIS,P. D. (1967). Origin of Kaposi’s sarcoma from the reticulo-endothelial system. Nature, 213, 889-893. HAYES, C. W., CLARK,R. M. and POLITANO, V. A. (1971). Kaposi’s sarcoma of the penis. Journal of Urology, 105, 525-527. HERRING, B. D. (1963). Kaposi’s sarcoma in the negro. Journal of the American Medical Association, 185, 540-542. W. D. and PACK,G. T. (1950). Malignant blood vessel tumors: report of 56 cases of angiosarcoma and MCCARTHY, Kaposi’s sarcoma. Surgery, Gynecology and Obstetrics, 91, 465-482. OETTLE,A. G. (1962). Geographical and racial differences in the frequency of Kaposi’s sarcoma as evidence of environmental or genetic causes. Acta Unio Internationalis contra Cancrum, 18, 330-363. PHILIPPSON, L. (1902). Ueber das Sarcoma Idiopathicum cutis Kaposi: ein Beitrag zur Sarcomlehre. Virchows Archiv fur pathologische Anatornie, 167, 58. ROTHMAN, S . (1962). Medical research in Africa. Archives of Dermatology, 85, 31 1-324. SUMMERS, J. L., WILKERSON, J. R. and WEGRYN, J. F. (1972). Conservative therapy for Kaposi’s sarcoma of the external genitalia. Journal of Urology, 108, 287-289.
The Authors William Houston, MD, MCh, FRCS, Consultant in Urology. Alan Pontin, FRCS, Senior House Officer (now Registrar in Urology, Groote Schuur Hospital, Cape Town). Trudi Kuhn, MB, Surgical Registrar. Nobe Mambo, MB, Junior House Officer (now Registrar in Pathology, Harari Hospital, Salisbury, Rhodesia).
