Clinico-epidemiological study of Kaposi's sarcoma in Egypt

Clinico-epidemiological study of Kaposi's sarcoma in Egypt

ClinicalRadiology (1981) 32, 355-358 0009-9260/81/00370355502.00 © 1981 Royal College of Radiologists Clinico-epidemiological Study of Kaposi's Sar...

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ClinicalRadiology (1981) 32, 355-358

0009-9260/81/00370355502.00

© 1981 Royal College of Radiologists

Clinico-epidemiological Study of Kaposi's Sarcoma in Egypt S. EL-HADDAD*, G. F. AL-WAHIDIt, N. AL-KHOLI*, S. AL-SHAMI:~ and O. ZAKI*

*Department of Radiation Oncology, Faculty of Medicine, Cairo University, Egypt,"Departments of t Radiation Oncology and }Dermatology, Faculty of Medicine, Mansoura University, Egypt The clinico-epidemiological aspects of Kaposi's sarcoma are reviewed. A study of 27 patients seen in Egypt during the years 1962-78 is reported. The treatment modalities are discussed with special reference to radiotherapeutic management.

Kaposi's sarcoma is a rare disease, and was originally described by the Hungarian dermatologist, Kaposi (1872), as 'Idiopathisches multiples pigments sarkom der haut'. It appears on the skin as nodules that vary in size from a peppercorn to that of a pea or hazel nut. They may occur as a single nodule or as plaquelike groups. The total number of cases recorded in the world literature amounts to 4000, the majority from tropical Africa. In Egypt, the incidence of the disease is not fully known. The aim of this work was to study the clinicoepidemiological aspects of Kaposi's sarcoma in the material registered at Kasr E1 Aini Centre of Radiation Oncology, Cairo, in an attempt to study the prevalence of the disease and its epidemiological aspects.

MATERIAL AND METHODS The material consisted of 27 patients seen in the period 1962-78 inclusive. These patients presented among 35 193 malignancies seen at the out-patient clinic of Kasr E1 Aini Radiation Oncology Centre (NEMROCK). The clinico-epidemiological data and the results of treatment were analysed for all patients. The treatment modalities adopted were radiotherapy, surgery and chemotherapy. 1. Radiotherapy. The following alternative techniques were used: (a) Single dose treatment of 1000 cGy (rad) was given for solitary lesions. (b) 2400cGy in 10 fractions over two weeks for bigger lesions. (c) 2400 cGy in 20 fractions over four weeks for deep infiltrating lesions. The quality of radiation used in treatment varied according to the thickness of the lesion, from 120 kV, 4 mm A1 HVT to 250 kV, 2mm Cu HVT. Either a

direct field or two paraUel opposed portals were employed. 2. Surgery was resorted to in three cases. 3. Chemotherapy was usually used with or after irradiation in the treatment of residual or recurrent lesions as well as in cases showing dissemination. Single drug (Cyclophosphamide) as well as combination chemotherapy was used, the combination regime being the standard course of COP consisting of: Cyclophosphamide 600mg/m 2 given i.v. on the first and eighth days. Vincristine 1.4mg/m 2 given i.v. on the first and eighth days. Prednisone 40rag orally for eight days. This course was repeated every three weeks for six courses. No modification was undertaken, as the blood pictures were within normal limits. Intraarterial infusion using Nitromine and Trenimon was also used in two cases. Combination chemotherapy of a type used for solid malignant tumours was also tried: this was given as follows in five day courses: Cyclophosphamide 400mg/m 2 i.v. on days one, three and five. 5-Fluorouracil 500mg/m z i.v. on days one and five. Methotrexate 20 mg/m 2 i.v. on days two and four. Vincristine I mg/m 2 i.v. on days two and four. This course was repeated every three weeks, provided the blood picture was normal.

RESULTS The total number of patients seen at NEMROCK in the period from 1962 to 1978 inclusive was 27, forming 0.007% of the total cases seen in the same period and 5.27% of the total skin malignancies.

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Males outnumbered females in the ratio 5.8 to 1. The ages ranged from 18 to 75 years, with a peak age incidence in the seventh decade. The mean age in the cases reviewed was 52 years (Fig. 1). The highest incidence was observed in the Cairo Governorate (70%), followed b y Minofia (11.7%) and Dakahlia (7.4%). In 44% o f cases occupations involving long periods o f standing were observed, while in 26% o f cases outdoor occupations were recorded. Clinical Manifestations

The cases were classified as: (a) N o d u l a r - small nodules growing superficially with no gross infiltration. (b) L o c a l l y aggressive - nodular but with a tendency to infiltrate deeply or ulcerate to the surface. (c) G e n e r a l i s e d - lesions generalised from the start in b o t h lower limbs. The commonest pattern was the nodular type (63%), followed b y the locally aggressive t y p e (11%). In 22% o f cases the lesion was o f nodular a n d locally aggressive type. Only in 4% o f cases was the disease generalised. The disease had a peripheral distribution, with

Tunisia 0-06%

Table 1 - Response of Kaposi's sarcoma to various modalities of treatment

Modality o f treatment

No. o f cases

Response No

Recurrence

recurrence

Radiotherapy Surgery Surgery and radiotherapy Chemotherapy Single agent

12 2 1

4 (one lost to follow-up) Combination 2 therapy (one lost to follow-up)

9 1 1

3 1 3 (after temporrary response) 1

the lower limb affected in 92.4% Of cases, while bilateral distribution o f lesions was n o t e d in 44.1%. Unilateral localisation was slightly more common (48%). Below-knee lesions were noticed in 74% of cases and above-knee ones were observed in 33%. Rarely the disease affects the face (4%) and the chest (4%). The disease never developed upon the soles of the feet or the palms o f the hands.

Treatment

Twelve cases were treated by radiotherapy alone, nine o f them showing a good response* (75%), while a moderate responset was observed in three cases (25%). Surgical excision was performed in three cases: one showed local recurrence; in the second no recurrence was observed after excision of the primary and draining o f the l y m p h nodes; the third did not show any relapse after excision and irradiation. Chemotherapy was used as a single agent (Cyclophospharnide) in four cases, with good response in half of them; the third patient showed mild response, while the fourth was lost to follow-up, combination chemotherapy (COP), as used for non-Hodglcin's lymphoma, was e m p l o y e d in two cases. No response was seen in the first case and the second was lost to follow-up. Another regime used was combination chemotherapy for solid tumours mentioned above; this type o f treatment was restricted to patients resistant to the previously mentioned t r e a t m e n t modalities. It was tried in one case only, which showed an unexpected response with complete disappearance of lesions after the fifth course. Intra-arterial infusion using Nitromine and Trenimon was tried in two cases Fig. 1 - The incidence of Kaposi's sarcoma throughout Africa.

*Good response signifies no recurrence up to three years. tModerate response signifies local control for more than one year.

KAPOSIS'S

SARCOMA

and both showed an immediate subjective and objective response.

DISCUSSION Kaposi's sarcoma is a rare disease in most parts of the world. In the Mayo clinic (USA) only 10 cases were collected in 38 years, forming 0.067% of the total number of cases at this institute during this period (Reynolds et al., 1965). This disease is much more common in Eastern Europe (Dorffell, 1932) and tropical Africa than in other parts of the world; other racial groups are affected, for example, 23 cases were recorded in Sweden (Hansson, 1940). On the other hand, isolated cases were reported from South America (Austregesilo, 1912), Iraq (Pautrier and Diss, 1929); Japan (Epstein, 1941; Hasegawa, 1962); China (Kocsard, 1949); New Guinea (Biggs et al., 1964); and India (Yesudian, 1969). In Africa, the incidence of Kaposi's sarcoma is roughly 200 times higher in the Congo than in Chicago, USA (Rothman, 1962). Fig. 1 shows a map of Africa with the incidence of the disease shown in different Countries. In Negroes, the incidence of the disease is at least as common as in white Americans, but American Negroes show a lower incidence than African Negroes. This suggests an environmental cause for the disease. The peak age incidence in the present series was the seventh decade (37%) followed by the third decade (18.5%) (Fig. 3). The age ranged between 18 and 75 years. A similar age incidence was recorded by Oettle (1962). However, generalised disease has been observed in young adults by many investigators (Symmers, 1941; Reynolds et al., 1965). Regarding sex incidence in the present series, the male to female ratio was 5.8 to 1. Choisser and Ramsey (1939) recorded a ratio of 15 to 1 in their cases, which is higher than in the present series. In childhood cases, the male to female ratio was 3 to 1 (Dutz and Stout, 1960; Slavin et al., 1970). In this series, Kaposi's sarcoma represented 1.5% of malignant tumours of the skin. The disease was common in people whose occupation necessitated long periods of standing and outdoor occupations. This is in agreement with the results of McCarthy and Pack (1950). The commonest clinical form was the nodular type, in keeping with findings of Reynolds et aL (1965) in the Mayo Clinic (USA) who reported an incidence of 93%, the commonest sites affected being the lower limbs followed by the upper limbs, rarely the face and the chest. This is in agreement with all series reported (Reynolds et al., 1965; Taylor et al., 1971). Nodular disease is compatible with very long survival. Cox and Helwing (1959) have reported average survival in this type from eight to 10 years. Since the

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rate of growth of the disease is slow, it is difficult to assess the results of any treatment, but we can say that surgery alone gave poor results. On the other hand radiotherapy gave good results for localised disease. The effective dose varies from 100 cGy (rad) single dose to 2400 cGy in two and a half weeks using that quality of beam appropriate to the thickness of the lesion. The results obtained were good in 75% of cases and moderate in 25% of cases. Cohen (1962) concluded that an adequate dose consisted of 1000 cGy as a single dose or 2500 cGy in four weeks. As for chemotherapy, Cyclophosphamide proved to be the most effective single drug provided it was given after radiotherapy. Although Spencer (1966) found similar results, Kyalwazi (1968) failed to show any response after Cyclophosphamide therapy. Intraarterial infusion using Trenimon or Nitromine was resorted to when the lesion was extensive and involved the whole limb. Although it showed a good response in one case, the high incidence of morbidity restricted its wide use. While Kyalwazi (1968) showed temporary benefit after intra-arterial Mustine, Keen (1962) obtained a dramatic response. The COP regime was not found effective in the two cases treated in our series. Solid tumour multiple drug chemotherapy gave unexpectedly good results in the cases treated especially those resistant to the conventional treatment given before its administration. In conclusion, we can say that greater experience will result if such a rare tumour is treated only in one centre in any individual country. REFERENCES

Austregesilo, A_ (1912). Un cas d'angiokeratome semblade an Pied de Madura Arcgfff Schfffs. Tropical Hygiene, 16, 622-624. Bigge, B., Cooke, R. A. & McGovern, V. J. (1964). Kaposi's sarcoma report of a case from New Guinea. Australian Journal of Dermatology, 7, 131. Choisser, R. M. & Ramsey, E. M. (1939). Angioreticuloendothelioma (Kaposi's disease) of the heart. American Journal of Pathology, 15, 155-158. Cohen, L. (1962). Dose, time, volume parameters in irradiation therapy of Kaposi's sarcoma. British Journal of Radiology, 35,485- 489. Cox, F. X. & Helwing, E. B. (1959). Kaposi's sarcoma. Cancer, 12, 289-291. Dorffel, J. (1932). Histogenesis of multiple idiopathic haemorrhagic sarcoma of Kaposi. Archives of Dermatology and Syphilology, 26,608. Dutz, W. & Stout, A. P. (1960). Kaposi's sarcoma in infants and children. Cancer, 13, 684-687. Epstein, E. (1941). A case for diagnosis. Kaposi's sarcoma. Archives o f Dermatology and Syphilology, 34, 409410. Hansson, C. J. (1940). Kaposi's sarcoma: clinical and radiotherapeutic studies on 23 patients. Acta Radiologica, 21, 457-459.

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Pautrier, L. M. & Diss, A. (1929). Kaposi's idiopathic sarcoma is not a genuine sarcoma but a neurovascular dysgenesis. British Journal of Dermatology and Syphilis, 41, 93-94. Reynolds, W. A., Winkelman, R. K. & Soule, E. H. (1965). Kaposi's sarcoma. Medicine, 44, 4 1 9 - 4 2 1 . Rothman, S. (1962). Remarks on sex, age and racial distribution of Kaposi's sarcoma and on possible pathogenetic factors. Acta Union [nternationalis Contra Cancrum, 18, 322-325. Slavin, G., Cameron, H. M., Forbes, C. & Morton, M. R. (1970). Kaposi's sarcoma in East African children. A report of 51 cases. Journal of Pathology, 100, 187-188. Spencer, M. C. (1966). Cytoxan treatment of Kaposi's idiopatic haemorrhagic sarcoma. Illinois Medical Journal, 129, 252-254. Symmers, D. (1941). Kaposi's sarcoma. Archives of Patholog-y, 32, 764-767. Taylor, J. F., Templeton, A, C., Kyalwzi, S. & Lubega, A. (1971). Kaposi's sarcoma in pregnancy. Two case reports. British Journal of Surgery, 58, 577-579. Yesudian, P. (1969). A case of Kaposi's sarcoma. Indian Journal of Dermatology, 14, 121-123..