|Year : 2015 | Volume
| Issue : 2 | Page : 68-70
Malignant melanoma in a Nigerian oculocutaneous albino
Oluwafemi Olasupo Awe1, Emmanuel Ehizome Esezobor1, Quincy O Aigbonorga2, Clifford I Owobu3
1 Department of Surgery, Plastic Surgery Unit, Irrua Specialist Teaching Hospital, Irrua; Department of Surgery, Ambrose Alli University, Ekpoma, Edo, Nigeria
2 Department of Surgery, Irrua Specialist Teaching Hospital, Irrua, Nigeria
3 Department of Histopathology and Morbid Anatomy, Irrua Specialist Teaching Hospital, Irrua, Nigeria
|Date of Web Publication||10-Mar-2016|
Oluwafemi Olasupo Awe
Department of Surgery, Plastic Surgery Unit, Irrua Specialist Teaching Hospital, Irrua, Edo, Department of Surgery, Faculty of Clinical Sciences, Ambrose Alli University, Ekpoma, Edo
Source of Support: None, Conflict of Interest: None
Albinism is a rare autosomal recessive disorder occurring as a result of decrease or absence of tyrosinase enzyme resulting in a reduction in melanin synthesis. This is characterized by depigmentation, nystagmus, and photophobia and decrease visual acuity. Malignant melanoma though on the increase worldwide, it is very rare in the albino who have deficient melanocytes that express melanin. We present a case of malignant melanoma in a 26-year-old male undergraduate albino.
Keywords: Albino, malignant melanoma, Nigerian, oculocutaneous
|How to cite this article:|
Awe OO, Esezobor EE, Aigbonorga QO, Owobu CI. Malignant melanoma in a Nigerian oculocutaneous albino. Nigerian J Plast Surg 2015;11:68-70
|How to cite this URL:|
Awe OO, Esezobor EE, Aigbonorga QO, Owobu CI. Malignant melanoma in a Nigerian oculocutaneous albino. Nigerian J Plast Surg [serial online] 2015 [cited 2019 Jun 17];11:68-70. Available from: http://www.njps.org/text.asp?2015/11/2/68/178453
| Introduction|| |
Oculocutaneous albinism is a rare recessive autosomal disorder occurring as a result of lack of tyrosinase enzyme. The incidence in Caucasians is 1:39,000. It is characterized by pink-white skin, white hair, nystagmus, and photophobia and decrease visual acuity. Patients with albinism are predisposed to skin cancers because of lack of protection from sunlight (ultraviolet radiation). However, the incidence of malignant melanoma is rare in albinos. In the general population, malignant melanomas consist of 1% of all malignant tumors and 3% of malignant tumors of the skin.
| Case Report|| |
The patient is a 26-year-old albino, undergraduate presenting with 14 months history of right-sided neck swelling and 4 months neck pain. There was no difficulty in swallowing. There was no similar swelling on any part of his body. No cough, weight loss, drenching night sweats nor low-grade fever. No history of trauma. He does not smoke cigarette nor consumes alcohol in any form. No family history of similar swelling.
Examination revealed a young fair-skinned man with yellow hair and nystagmus, who was afebrile, not pale, anicteric, cyanosed, and well hydrated. All the vital signs were within normal ranges. He had multiple brown macules measuring 1–3 cm scattered across the face, neck, and upper limbs.
There was a fleshy mass on the posterior angle of the right lateral neck, irregular in shape, measuring about 14 cm in its widest diameter, with associated ulcer at the summit of the mass of 4 cm in diameter with a central area of necrosis and contact bleeding [Figure 1]. The edge was elevated and the base is firm. Mass was neither attached to the underlying structures nor tender. Carotid pulses were palpable. There was no cervical lymph node enlargement.
A preliminary diagnosis of squamous cell carcinoma was made to rule out basal cell carcinoma. The patient was scheduled for a 2-stage excision biopsy and split thickness skin graft. Routine laboratory studies and biochemical tests were normal. The first stage of the excision was done with near total excision of the mass [Figure 2]. The histopathology result of the excised mass showed a tumor composed of slightly pleomorphic nevus-like cells demonstrating mild irregular nuclei with no prominent eosinophilic nucleolus. Atypical mitotic figures were present. There was no melanin in the cytoplasm of the neoplastic cells and no intraepidermal component. Malignant melanomas are quite rare in albinos. Morphology alone will not be convincing since the hallmark of the MM-large prominent eosinophilic nucleolus is absent in this case. Ahistochemical stain such as Manson–Fontana reaction or immunohistochemical staining reaction to HMB 45 or S-100 will be more convincing. These findings were in keeping with amelanotic malignant melanoma [Figure 3].
The second stage of the surgery was done after about 2 weeks and the wound grafted with split thickness skin graft [Figure 4]. He is currently on up at the plastic surgery outpatient clinic. He has been advised to have adjuvant radiotherapy.
| Discussion|| |
Albinism is an autosomal recessive disorder of hypopigmentation of the skin, hair, and eyes due to lack of tyrosinase enzyme., There are two main types, i.e.,oculocutaneous albinism and ocular albinism. Oculocutaneous albinism is a genetically heterogeneous group of disorders in which there is generalized decrease or absence of melanin pigment in the eyes, hair, and the skin. There are, at least, ten forms of this condition identified, each presumably resulting from a different biochemical mechanism blocking the synthesis of melanin. Consequently, albinos lack the protective effect of melanin against ultraviolet radiation damage. The Nigerian albino is particularly predisposed to skin cancers because of the proximity to the equator., The skin disorders associated with albinism include accelerated photo-aging and increased the incidence of solar keratosis, squamous cell carcinoma, and basal cell carcinoma. Though rarely, there is also malignant melanoma.,,
The malignant melanoma occurrence is very rare in oculocutaneous albinism though the low incidence is unexplained. Only about thirty cases of malignant melanoma associated with oculocutaneous albinism have been reported in the English literature., This had also been documented once in a Nigerian albino., Most of the documented cases are small lesions but the index patient presented with an extensive lesion due to late presentation.
Most of the cancers in the albinos are located on the head and neck, just as in our index patient. These cancers are mainly squamous cell carcinoma, less commonly basal cell carcinoma, and rarely malignant melanoma. Our patient's lesion was malignant melanoma. There is also problem of late presentation in this patient due to financial constraint and ignorance, which had also been highlighted by Asuquo et al. Surgical removal of this lesion remains the mainstay of treatment as indicated in our patient. There may be a need for adjuvant radiotherapy, chemotherapy, and immunotherapy as additional treatment options, especially in late presentation. There is a need for high index of suspicion in these patients, especially when presenting with fleshy ulcerated skin cancers.
| Conclusion|| |
We conclude that malignant melanoma though rare in albino can sometimes present as observed in this case. All patients with albinism should be treated like individuals with Fitzpatrick typeI skin. This means they must practice strict sun avoidance and year round broad-spectrum sunscreen use, wear protective clothing, and receive skin cancer screening every 6 months. Education of the patients, the public and nondermatologist regarding prevention, and early detection and excision of these lesions will lead to a possible cure.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]