1/3. Malignant melanoma in a patient with oculocutaneous albinism.BACKGROUND: Sun-induced malignancies (basal cell and squamous cell carcinomas) are common in oculocutaneous albinism, however, the incidence of malignant melanoma is a topic of controversy. OBJECTIVE: We have reviewed the literature and report a case of a woman with oculocutaneous albinism with an amelanotic melanoma of the anterior chest wall. RESULTS: There are only 26 previously reported cases (both case reports and African albino population studies) in 25 patients in the literature. A 27th case with immunohistochemical and ultrastructural evaluation is presented. CONCLUSIONS: It appears that melanoma, a malignancy for which sun exposure and light colouration are felt to be major risk factors, has a low incidence among a population that is both hypopigmented and often exposed to high levels of ultraviolet light. This low incidence is poorly understood and frequently disputed.- - - - - - - - - - ranking = 1keywords = albinism, oculocutaneous (Clic here for more details about this article) |
2/3. Cutaneous malignancy in albinism.albinism is a disorder of hypopigmentation affecting the skin, appendages, and eyes. Ultraviolet light-induced cutaneous tumors are common in patients with albinism due to reduced or absent protection from melanin. Squamous cell carcinoma (SCC) is the number one skin tumor seen in patients who are albinos. Although nonmelanomatous skin cancers are more common in patients with albinism, dysplastic nevus and melanoma present a greater diagnostic challenge in this group because of their hypopigmented appearance. We report 2 cases of cutaneous malignancies in patients who had oculocutaneous albinism (OCA). The first case involves a 45-year-old man with OCA type 2 (OCA2) who developed a large SCC of the neck. The second case involves a 24-year-old man with Hermansky-Pudiak syndrome (HPS) who developed amelanotic melanoma. In both cases, hypopigmentation of the lesions contributed to a delay in diagnosis. We review the clinical, diagnostic, and therapeutic concerns for patients with albinism who have cutaneous malignancies.- - - - - - - - - - ranking = 1.1688104503432keywords = albinism, oculocutaneous (Clic here for more details about this article) |
3/3. Choroidal amelanotic melanoma in a patient with oculocutaneous albinism.CASE REPORT: We describe the clinical presentation of a choroidal amelanotic melanoma in a 46-year-old woman with oculocutaneous albinism. Clinical aspects, investigations, and management are presented, and findings from computed tomography and magnetic resonance imaging are described. Microscopic findings and histopathological features, demonstrating a spindle B-cell melanoma of the choroid, are also reviewed. COMMENTS: magnetic resonance imaging may be helpful in diagnosing amelanotic melanoma in patients with oculocutaneous albinism.- - - - - - - - - - ranking = 1keywords = albinism, oculocutaneous (Clic here for more details about this article) |