La Revue de médecine interne
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Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. ⋯ Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases.
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Acanthosis nigricans is a dermatosis characterized by the presence of a hyperpigmented, velvety cutaneous thickening in the flexural areas, especially axillary and inguinal fossas, and lateral faces of the neck. AN is usually a benign condition but can sometimes reveal an internal malignancy corresponds to a cutaneous paraneoplasic syndrome. Literature shows a predominant association with gastric adenocarcinoma. Here, we report a rare association between AN and cholangiocarcinoma. ⋯ AN should be considered as cutaneous sign either of malignancy or endocrinopathy and therefore requires further investigations. The existence of extensive lesions, pruritus, tripe palms syndrome, florid cutaneous papillomatosis or mucous lesions, associated to an AN is a sign of malignancy should be investigated urgently the early diagnosis of which can lead to a better prognosis.