TAEHO Tanzania

Skin cancers are a major risk associated with albinism and are thought to be a major cause of death in Tanzania albinos. The challenges associated with the care of these patients are numerous and need to be addressed. The aim of this research report was to outline the needs and treatment of skin cancers among albinos through our Organization and to highlight challenges associated with the care of these patients and proffer solutions for improved outcome.

Albinism is a genetically inherited disorder characterized by hypopigmentation of the skin, hair and eyes due to a reduced or lack of cutaneous melanin pigment production. Generally, there are two principal types of albinism, oculocutaneous, affecting the eyes, skin and hair, and ocular affecting the eyes only. The mode of inheritance of albinism is thought to vary, depending on the type. The oculocutaneous type is considered autosomal recessive, and the ocular variant sex-linked [1-4]. Oculocutaneous albinism exists in four forms. One form involves the tyrosinase gene (OCA1), whereas the other form (OCA2) has recently been associated with alterations of the P gene on chromosome 15. The other two forms include OCA3 due to TYRP1 mutations and OCA4 due to SLC45A2/MATP . OCA 2 is about twice as common as OCA1 in African and African-American populations.

Albinism has a worldwide distribution and tends to affect people of all ethnic backgrounds; its frequency worldwide is estimated to be approximately 1 in 20,000 in most populations and in Africa, incidences ranging from 1 in 2,700 to 1 in 10,000 people have been reported in various studies with the highest incidence of 1 in 1,000 people in Zimbabwe. In Tanzania the frequency of albinism has been estimated to be approximately 1 in 2,500. Melanin is a photo protective pigment, protecting the skin from the harmful effects of ultraviolet radiation. Its deficiency in people with albinism predisposes them to the harmful effects of ultraviolet radiation exposure, resulting in issues such as photo-phobia, decreased visual acuity, extreme sun sensitivity, and skin cancers. High levels of exposure to ultraviolet radiation increase the risk of all three major forms of skin cancer and are responsible for the anatomical site distribution. No use of protection for the skin increased the risk of skin cancer in these patients.

The head and the neck is the site most commonly affected and squamous cell carcinoma has been reported to be the commonest skin malignancy seen in albinos. In Africa the incidence of squamous cell carcinoma in the general population ranges from 7.8 to 16% of all diagnosed skin malignancies. In the African albino, the risk of developing these malignancies in comparison to the general population has been reported to be as high as up to 1000 fold.

The management of skin cancers among albinos in resource-limited countries like Tanzania poses major therapeutic challenges which need to be addressed. Late presentation with advanced lesion coupled with lack of therapeutic facilities such as radiotherapy services are among the hallmarks of the disease in developing countries. The outcome of treatment of skin cancers among albinos in most developing countries has been poor because the majority of these patients present late to the hospital with advanced stage. This is partly due to paucity of local data regarding this condition and lack of community awareness on the importance of early reporting to hospital for early diagnosis and treatment.