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BACKGROUND
A case of a very large congenital melanocytic naevus (VLCMN) is reported. Both this and a review of the current literature raise the question of the adequacy of standard surgical intervention for melanoma arising in VLCMN. Such naevi extend into deeper tissue layers with involvement of neurovascular and deep striated muscle bundles not usually addressed by standard surgical excision. VLCMN overlying the scalp or spinal cord also commonly involve the leptomeninges. In contrast, conventional melanoma usually starts in the epidermis (level I) and invades the papillary dermis (level II) to the junction of the papillary and reticular dermis (level III) and reticular dermis (level IV) before entering the subcutaneous fat (level V). Given the differences in the depth of anatomical involvement of the disease, melanoma derived from VLCMN should be viewed as a special variant of melanoma for management and follow-up. We present a case of VLCMN treated by conventional therapies, which had rapid malignant transformation and spread, resulting in death from metastases within months. The incidence of VLCMN has been reported as 1 per 500000 live births. 1
CASE PRESENTATION
A 19-year-old man with a VLCM ("garment naevus) ( fig 1 ) involving approximately 20% of the skin surface area presented with serosanguinous drainage from a left lateral chest nodule within his naevus, which he had first noted 8 months previously. He was otherwise well.
Upon physical examination, a VLCMN was seen, which extended from the posterior hairline down to his waistline with multiple areas of hyperkeratosis and hypertrichosis. On the left upper chest near the axilla there was pedunculated, bleeding nodule, 2.5 cm in diameter. A diagnostic excisional biopsy was performed on this lesion, which was found to be an ulcerated melanoma, 10 mm thick, Clark level IV ( figs 2 and 3 ). There was also a small palpable left axillary mass that was later determined by fine needle aspiration to be metastatic melanoma.
INVESTIGATIONS
Further investigations included whole-body postitron emission tomography (PET)/CT and brain MRI. The brain MRI was negative for intracranial metastasis. PET/CT was remarkable for increased uptake in the right hilar and sacral regions. Both of these lesions were considered for biopsy via fine needle aspiration. The sacral lesion, which was the only lesion deemed reachable by...