Primary Hepatic Neuroendocrine Carcinoma Associated With Carcinoid Syndrome and Pellagra: A Case Report

Abdelmounem Eltayeb Abdo, Susanna Caminada, Salma Barakat Mudawi, Sami Medani Abd Elwahab, Ahmed Rafei, Christos Vosinakis, Damiano Pizzol, Lee Smith
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Abstract

Carcinoid tumours are slow-growing neuroendocrine neoplasms mainly affecting the gastrointestinal tract, the respiratory tree, ovaries and kidneys. Approximately 20% of patients with carcinoid tumour are affected by carcinoid syndrome, characterised by chronic diarrhoea and/or flushing in the presence of systemically elevated levels of specific markers as serotonin or 5-hydroxyindolacetic acid. Skin manifestations include flushing of the face, neck and anterior surface of the chest but after repeated and prolonged relapses, the skin lesions become fixed, acquiring a bluish-red tint with telangiectasias. Patients may also develop pellagra skin symptoms including erythema, xerosis, scaling, hyperkeratosis and pigmentation, caused by a deficiency of tryptophan, due to its high consumption for serotonin synthesis.

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原发性肝神经内分泌癌合并类癌综合征及糙皮病1例报告
类癌肿瘤是生长缓慢的神经内分泌肿瘤,主要累及胃肠道、呼吸道、卵巢和肾脏。大约20%的类癌患者患有类癌综合征,其特征是慢性腹泻和/或在血清素或5-羟基吲哚乙酸等特定标志物全身性水平升高时出现潮红。皮肤表现包括面部、颈部和胸部前表面潮红,但反复和长时间复发后,皮肤病变变得固定,获得带毛细血管扩张的蓝红色。患者还可能出现糙皮病皮肤症状,包括红斑、干燥、脱屑、角化过度和色素沉着,这是由于合成血清素需要大量消耗色氨酸而缺乏色氨酸引起的。
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