Primary malignant melanomas of the lung and adrenal

Primary Melanoma of the Adrenal Gland, a Continuous Dilemma: Report of a Case

“…It is difficult to prove that a visceral malignant melanoma is a primary lesion because malignant melanomas in more common locations may metastasize widely. 4 Metastatic involvement of the regional lymph nodes, small bowel, and bone have been noted in two cases at the time the adrenal lesion was detected, 5 and there are other cases in the literature with hepatic metastasis, 4,7 but our case is the only one to demonstrate splenic metastasis.…”

“…Primary adrenal melanoma is a highly aggressive tumor that affects middle-aged adults and manifests itself as a painful flank mass, although distant lymph node metastasis can be seen on the initial examination. 6 The size of the lesions ranges from 8 to 17 cm in maximal diameter [1][2][3][4][5][6] and the tumors are com- posed of soft gray-brown to brown-black tissue with areas of hemorrhage and necrosis. 5,7 A histologic study reveals polygonal or spindle cells displaying an oval-toround nucleus with a prominent nucleolus and containing brown intracytoplasmic pigment.…”

“…7,8 Malignant melanoma originating outside the skin, squamous mucous membranes, eyes, and leptomeninges is rare. 4 The existence of primary melanoma of the adrenal gland is explained by the neuroectodermal origin of the medulla; chromaffin cells and melanocytes have a common embryogensis. 7,9 Many authors have expressed great reluctance to accept malignant melanomas as primary in the adrenal gland because the frequency of adrenal involvement by metastasis from cutaneous or ocular melanomas can be as high as 50%.…”

“…11 For this reason we tried to establish the criteria to differentiate primary from metastatic melanoma. In 1984, Carstens et al 4 established four strict criteria for diagnosing adrenal melanoma as primary: (1) the presence of a primary malignant melanoma in only one of the adrenals; (2) no prior or current malignant melanoma elsewhere; (3) no previous removal of pigmented skin, or mucosal or eye lesions; (4) exclusion by autopsy of the presence of a hidden primary lesion. Our patient satisfied the first three of these criteria, but this could not be confirmed by autopsy because the patient is still alive, asymptomatic and free of disease after 1 year.…”

“…Primary malignant melanoma of the adrenal gland is extremely rare, with only 18 cases [1][2][3][4][5][6] reported in the English literature. It is always difficult to differentiate a primary melanoma from either a metastatic melanoma of the adrenal gland or from a pigmented pheochromocytoma.…”