Skin manifestations of neuroendocrine neoplasms: review of the literature

Abstract

Neuroendocrine neoplasms (NENs) are a heterogeneous group of rare tumours derived from peptidergic neurons and specialized neuroendocrine cells capable of secreting various peptides or amines. These cells may be present in endocrine tissue or diffused in the tissues of the digestive or respiratory system. The article reviews the characteristic features of NENs, with particular emphasis on skin manifestations, such as necrolytic migratory erythema (NME), tongue inflammation, angular cheilitis, venous thrombosis and alopecia in glucagonoma; "flushing", "lion face", pellagra skin symptoms, "scleroderma-like features without Raynaud's phenomenon" in carcinoid tumours. The paper also presents the clinical picture of the neuroendocrine tumour of the skin -Merkel cell carcinoma. The aim of this study was to draw attention to the need for precise and comprehensive diagnosis of the patients, with particular emphasis on skin lesions as a revelator of neuroendocrine tumours. This management allows for the early implementation of appropriate treatment.

Cutaneous Neuroendocrine Metastases of Visceral Origin Responsive to Surgical Resection and Targeted Radionuclide Therapy

“…There have only been 45 cases of cutaneous NEN metastases reported in the literature (including this one) [ 2 , 5 , 7 ]. Typically, NET metastases are clinically nondescript and appear as nonulcerated subcutaneous papules or nodules [ 3 , 5 ]. Clinical suspicion for cutaneous metastasis should be high if any new, changing, or symptomatic lesions of any size are encountered on physical examination in a patient with metastatic NET.…”

“…Most NENs are characterized as well-differentiated and are classified as neuroendocrine tumors (NETs). While NETs frequently spread to the liver, lymph nodes, and bone, metastases to the skin are very uncommon and often are a late manifestation of advanced disease [ 3 , 4 ]. If cutaneous NET metastases do occur, they are typically solitary, subcutaneous papules or nodules [ 3 , 5 ].…”

“…While NETs frequently spread to the liver, lymph nodes, and bone, metastases to the skin are very uncommon and often are a late manifestation of advanced disease [ 3 , 4 ]. If cutaneous NET metastases do occur, they are typically solitary, subcutaneous papules or nodules [ 3 , 5 ]. We present a case of two eruptive painful cutaneous NET metastases occurring in a patient with prior stage IV visceral disease successfully managed with resection and targeted 177 Lu-DOTATATE combined with monthly maintenance lanreotide therapy.…”