Kaposi sarcoma (KS) is a systemic multifocal vascular tumor thought to be derived from the endothelial cell lining. It was initially described in 1872 by Moritz Kaposi. This tumor was brought to the attention of the medical community in a case series in which purple-colored nodular skin lesions were observed in five elderly men, with widespread cutaneous and visceral involvement reported in one patient at autopsy 1.
During the 19th century, KS was considered a rare disease because only sporadic cases were found in most parts of the world, occurring in elderly men of Mediterranean origin or of Jewish descent. However, an increased rate was suggested at the turn of the century 2,3. The epidemiology of KS has stimulated interest since 1982, when it became a sign of the AIDS 4.
KS has four variants including classic KS, endemic KS, immunosuppression-associated or transplantation-associated KS, and epidemic or AIDS-associated KS 5.
The clinical picture of KS includes mucocutaneous involvement in which KS lesions in the skin are classified clinically into six major overlapping types: patch, plaque, nodular, lymphadenopathic, infiltrative, and florid ‘exophytic’. Other rarely observed variants such as telangiectatic KS, lymphangioma-like or cavernous KS, ecchymotic KS, and keloidal KS have been described 6–8. The clinical picture of KS also includes extracutaneous KS, in which visceral KS is most evident in the gut 9 and lymph nodes 10. The liver and heart are also common extracutaneous involvement sites 11. The pancreas, brain, spleen, testes, adrenals, tonsils, kidneys, seminal vesicles, urinary bladder, thyroid, and other organs may also be involved 12.
The possible pathogenic factors implicated in KS include genetic factors, immunosuppression, infectious agents such as Epstein–Barr virus, cytomegalovirus, and Human Herpesvirus-8 (HHV-8), and environmental factors such as blood-sucking insects, volcanic soils, exposure to iron, and nitrites 13.
Four groups predisposed to KS include: older men of Mediterranean and Jewish lineage, with specific geographic foci in Europe; Africans from areas including Uganda, the Congo Republic, Congo (Brazzaville), Burundi, and Zambia; persons who are iatrogenically immunosuppressed; and men who are homosexual 14.
The seroprevalence of HHV-8 appears to mirror the risk of developing KS. In Africa, in areas highly endemic for KS, the seroprevalence of KS herpes virus in the general population has been reported to be 32–100%, which might account for the high general risk for KS in these areas 15.
The seropositivity of HHV-8 is lower in southern and northern Africa (10–40%), including Egypt, where KS is rarer 16. When KS becomes an epidemic, with a more aggressive form, it poses a difficult public health problem and remains a significant cause of morbidity and mortality in HIV-infected patients 17. Arabs experience an incidence of classic KS similar to that of other people living around the Mediterranean region 18. In Egypt, the most common clinical variant of KS is the classic variant, which was originally described by Moritz Kaposi. It accounts for 5–27% of the total skin malignancies 19.
In Egypt, KS is the most frequent post-transplant neoplasia, and a fifth of the patients have visceral involvement. Use of cyclosporine therapy and certain HLA-A and DR phenotypes may predispose the patient to this type of malignancy. Reduction of immunosuppressive drugs and use of radiotherapy or chemotherapy in selected patients with visceral involvement may lead to regression of KS, albeit at the risk of graft loss caused by rejection. The challenge in the future will be to prevent and treat KS while preserving renal function 20.
The aim of this work was to study some epidemiological features of KS in Gharbia Governorate, Egypt, from 1999 to 2005, as well as show the burden of KS in relation to other skin malignancies and to other cancers as a whole.
Patients and methods
This was a retrospective study in which data on patients clinically and histopathologically diagnosed with KS between 1999 and 2005 from the Gharbia Population-based Cancer Registry and the Outpatient Dermatology Clinic, Department of Dermatology, Tanta University, were collected, reviewed, computerized, and submitted for statistical analysis. The total nonmelanoma skin cancer count, total skin cancer count, and total cancer count (cutaneous and noncutaneous) were obtained from the registry unit at TCC to show the burden of KS in relation to other skin malignancies and to other cancers as a whole.
Gharbia Governorate is located in the Mid-Delta region of Egypt with a surface area of 1943 km2 (0.2% of the country’s total area). The population size is 3406 million (5.7% of the total population of Egypt according to the 1996 census) and population density is 1752/km2. It is considered an urban–rural Governorate according to the Central Agency for Public Mobilization and Statistics (CAPMAS).
All statistical calculations were performed using the computer program SPSS (Statistical Package for the Social Science; SPSS Inc., Chicago, IL, USA) version 16 for Microsoft Windows. Descriptive statistics were presented in the form of mean and standard deviation (SD) for quantitative data and number of patients as percentage per million (p/m) for qualitative data. Qualitative data of the groups were compared using the χ2-test . P values less than 0.05 were considered statistically significant.
On collecting data on patients clinically and pathologically diagnosed with KS from 1999 to 2005 from the registry unit at the Tanta Cancer Center and the Outpatient Dermatology Clinic (Tanta University), it was found that there were 22 patients with KS, but only 15 were from Gharbia and the other seven were from other governorates [Kafr El-Sheikh (three patients), Elbehera (two patients), Menoufiya (one patient), and Kaleoubiya (one patient)]. These seven patients were seeking medical services in Gharbia and were excluded from the present study. This study was conducted only on the 15 patients from Gharbia; the incidence rate of KS in Gharbia was 1.6 p/m.
There was significant male predominance as 86.7% of the KS patients were men (13 patients) and only 13.3% (two patients) were women, with a male : female ratio of 6.5 : 1. Statistically significant difference was found between the incidence rates of each sex: 2.7 p/m for men and 0.5 p/m for women (Fig. 1).
With respect to age, it was found that 11 of the 15 (73.3%) patients were above the age of 50 and seven (46.6%) were above the age of 60. The incidence rate was highest in the age group of 60–70 years (6.4 p/m) and was least in the age group of 30 to <40 (0.3 p/m); this difference was statistically significant (χ2=6.802, P<0.05). The mean age of patients with KS in Gharbia was 55.6 years (Fig. 2).
Analysis of these data according to time was also performed, and it was found that the highest percentage of patients [26.7% (four patients)] was in the year 1999, with an incidence rate of 3.1 p/m, whereas the least percentage [6.7% (one patient)] was in the year 2003, with an incidence rate of 0.7 p/m. In 2002, there were no reported cases. This difference was statistically significant (χ2=5.619, P<0.05) (Table 1).
The cases were distributed according to districts. It was found that the highest percentage of patients with KS [33.3% (five patients)] was in Tanta, the capital city of Gharbia, whereas the least percentage was found in Samannoud, Elsanta, and Kafr El-Zyat [6.7% (one patient)]; there were no reported cases in Zefta and Kotour. In addition, the incidence rate of KS in p/m in the age group of 30–70 years was also calculated. The highest incidence rate was in Basyoun (5.3 p/m) and the least incidence rate was in Elsanta (about 1 p/m), and this difference was statistically significant (χ2=8.519, P<0.05) (Table 2).
According to residence, KS showed a relatively higher incidence of 53% (eight patients) in rural areas, whereas in urban areas the incidence was 47% (seven patients), with a rural/urban (R/U) ratio of 1.1 : 1. The 7-year incidence rate was 0.85 p/m in rural areas, whereas it was 0.75 p/m in urban areas. This difference in the R/U distribution of KS patients was statistically nonsignificant (χ2=1.307, P>0.05) (Fig. 3).
This study found that KS represented about 2% of the total nonmelanoma skin cancer count (706 patients) and about 1.9% of the total skin cancer count (785 patients) (Table 3 and Figs 4 and 5). The percentage of KS to total cancer count differed each year from 1999 to 2005. KS represented about 0.06% of the total cancer count, both cutaneous and noncutaneous (24 684 patients) (Figs 6 and 7).
In Gharbia, clinically, KS affects mainly the lower limb [53.3% (eight patients)]. In three (20%) of the 15 patients, both upper and lower limbs were affected, whereas upper limb affection was present in one patient (6.7%). In the remaining three patients (20%), the skin was affected but the specific site in the skin was unknown (not described) (Table 4 and Fig. 8).
The incidence rates of classic KS in different population-based registries vary markedly 21,22. High rates have been reported from Israel (20.7 p/m among men and 7.5 p/m among women between 1960 and 1998) 23 and Greece (5.8 p/m among men and 3.7 p/m among women between 1979 and 1983 24, and 2.11 patients per 10 000 dermatological patients during a 5-year study period) 25. The highest rates have been reported from Sardinia (24.3 p/m among men and 7.7 p/m among women between 1977 and 1991 26 and 29.2 p/m among men and 19.6 p/m among women between 1998 and 2002) 27 and Sicily (30.1 p/m among men and 5.4 p/m among women between 1976 and 1984) 28. In Peru, an overall incidence of 2.54 patients per 10 000 attended was found between 1946 and 2004 29.
In the present study, over a period of 7 years from January 1999 to December 2005, only 15 patients were diagnosed with KS in Gharbia, with an incidence rate of 1.6 p/m (2.7 p/m among men and 0.5 p/m among women). This incidence rate in Gharbia reflects the fact that KS is still rare in Egypt, even after the AIDS epidemic. In contrast, in other countries like England and USA, the incidence rate of KS flared after AIDS 30,31.
In a previous study 19 conducted in Egypt before the flare of the AIDS epidemic, the epidemiology of KS from 1962 to 1978 was studied from the data registered at Kasr E1 Aini Centre of Radiation Oncology, Cairo. Only 27 cases were found during this period, forming 0.007% of the total cases seen during this period. In the same study, the highest incidence was observed in Cairo Governorate, followed by Menoufia and Dakahlia, whereas Gharbia was not mentioned among the governorates having a high incidence of KS.
In the present study, it was found that KS mainly affects men, as 86.7% of the patients were men and only 13.3% were women, with a male : female ratio of 6.5 : 1. This result confirms the previous results obtained by different studies on classic KS conducted across the world in which men were shown to be mainly affected, with a male :female ratio of 10–15 : 1 32. In the previous study conducted in Egypt by El Hadad et al.19 as well, a male predominance was seen, with a male : female ratio of 5.8 : 1. However, this ratio was much lower than those reported from Italy (3 : 1) 33 and Greece (2.47 : 1) 25. In Iran as well, the male : female ratio was low (2.5 : 1) 34.
KS predominantly affects elderly men. Two-thirds of patients develop the disease after the age of 50 35. This statement coincided with the present study in which the mean age of the patients was 55.6 years and 73.3% of the patients were above the age of 50. The incidence rate of KS was highest in the age group of 60–70 years (6.4 p/m) and was least in the age group of 30 to < 40 (0.3 p/m). This result was confirmed by a previous study conducted in Egypt 19 in which the peak age of incidence was in the seventh decade and the mean age of the patients reviewed was 52 years. Also, this result matched that of another study conducted in Egypt’s Al-Minya Governorate 36, in which the mean age was about 62.4 years and 91.7% of the patients were above the age of 50.
In this study the highest percentage of patients affected with KS (26.7%) was in the year 1999, with an incidence rate of 3.1 p/m, whereas the least percentage [6.7% (one patient)] was in the year 2003, with an incidence rate of 0.7 p/m. In the year 2002, there were no reported cases. It is noteworthy that a similar curve with a gradual drop from 1999 to 2002, followed by a gradual rise, was also noticed in Peru, but in Peru the study ended in 2004 29.
In this study, the highest percentage of patients affected with KS (33.3%) was in Tanta, the capital city of Gharbia, whereas the least percentage was in Samannoud, Elsanta, and Kafr El-Zyat (6.7%); there were no reported cases in Zefta and Kotour. The incidence rate of KS was highest in Basyoun (5.3 p/m) and the least rate was in Elsanta (1 p/m). Although the number of cases was smaller in Basyoun (three cases) than in Tanta (five cases), the incidence rate was higher in Basyoun as it has a much smaller population size.
This statistically significant difference in the distribution of KS in Gharbia districts requires further studies to explain why the incidence rate of KS was high in some parts of the governorate (Basyoun and Tanta) and low in other parts, and if there is a relationship between this result and the geographical and demographic characteristics of these areas. The prevalence of HHV-8 in these regions should also be studied.
In the present study, it was noticed that KS in Gharbia showed a relatively higher frequency of 53% (eight patients) in rural areas, whereas the frequency was 47% (seven patients) in urban areas, with a R/U ratio of 1.1 : 1, which was not significant. This difference in R/U distribution of KS patients was also reported in other studies; for example, in the northern area of Sardinia (Italy), most cases were observed in rural areas, suggesting a role of contact with animals and farming cereals 26. In contrast to these studies, a study in southern Sardinia (Italy) observed a higher incidence of KS in urban than in rural districts 27.
According to previous studies conducted in Egypt, the prevalence of HHV-8 was higher in urban than in rural areas 37. Studies on HHV-8 conducted in urban areas of Egypt reported a seroprevalence of approximately 40%, which is about two-folds the prevalence of HHV-8 in rural areas 38.
In this study, it was found that KS represented about 2% of the total nonmelanoma skin cancer count (15 of 706 cases) and about 1.9% of the total skin cancer count (785 cases). A higher rate was seen by another study conducted in Al-Minya Governorate, south Egypt, in which KS represented 5.5% of all cutaneous malignancies 36.
We found that KS represented about 0.06% of the total cancer count (24 684 cases) in Gharbia, a percentage much lower than the global percentage of KS (0.6% of the global cancer count) 39. All these results confirm that KS is a rare disease in Gharbia Governorate, Egypt.
In this retrospective study, the clinical data of some patients were not sufficient. Therefore, a full picture regarding the types of KS present in Gharbia could not be formed, but it was noted that KS in Gharbia is rare (only 15 cases were reported), present mainly in elderly men, and in all of these cases the skin was affected, with no mucosal or visceral involvement. The lesions in most of the patients were present in the lower limbs (53.3%) and to a lesser extent in the upper limbs (6.7%) and both upper and lower limbs were affected in 20% of the patients. In the remaining 20% of patients, the skin was affected but the specific site in the skin was not described. The lesions in most of the patients were of nodular type. In a previous study on classic KS in Al-Minya Governorate, Egypt, lower limbs were involved in 66.7% of the patients and most of the lesions were of nodular type (75%) 36.
The patients who were investigated for HIV showed negative results, whereas some patients refused to undergo HIV investigations, which made the data pertaining to this point incomplete. Moreover, the patients were not investigated for HHV-8. However, in a previous study conducted in Tanta University (Egypt) on patients with KS from the Mid-Delta region, HHV-8 was detected by PCR in skin lesions of all patients and all patients were HIV negative 40.
According to the available data, we can say that the clinical profile of KS in Gharbia matched the classic type of KS present in the Mediterranean region, which was originally described by Moritz Kaposi 1.
We found that KS in Gharbia is a rare disease with an incidence rate of 1.6 p/m. It is of the classic type, affecting mainly elderly men with a mean age of 55.6 years. It is mainly of nodular morphology, affecting the skin of lower limbs, with no mucosal or visceral involvement, and represents about 1.9% of the total skin cancer count and 0.06% of the total cancer count in Gharbia.
It is recommended that all KS patients remain admitted until they are investigated for the presence of HIV, as it is compulsory and not an option. Moreover, they should be tested for HHV-8, as it is the main factor involved in the pathogenesis of KS. Further studies on the geographical and demographic profile of Gharbia districts should be conducted to determine why some areas in Gharbia (Basyoun) have a high incidence rate, whereas others (Zefta and Kotour) are free of KS.
Conflicts of interest
There are no conflicts of interest.
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Keywords:© 2013 Egyptian Women's Dermatologic Society
epidemiology; Gharbia/Egypt; Kaposi sarcoma