Skin involvement in neuroendocrine neoplasia

Neuroendocrine neoplasia (NEN), priory known as neuroendocrine tumours represent the modern approach of traditionally named “carcinoid” tumours. We shortly introduce benign and malign dermatological aspects that are linked to NEN field. This is a multidisciplinary perspective. The paper is a mini-re...

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Main Authors: Ana VALEA, Florica SANDRU, Simona Elena ALBU, Mihai Cristian DUMITRASCU, Dana TERZEA, Ana Maria Alexandra STANESCU, Mara CARSOTE
Format: Article
Language:English
Published: Amaltea Medical Publishing House 2020-03-01
Series:Romanian Journal of Medical Practice
Subjects:
Online Access:https://rjmp.com.ro/articles/2020.1/RJMP_2020_1_Art-07.pdf
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author Ana VALEA
Florica SANDRU
Simona Elena ALBU
Mihai Cristian DUMITRASCU
Dana TERZEA
Ana Maria Alexandra STANESCU
Mara CARSOTE
author_facet Ana VALEA
Florica SANDRU
Simona Elena ALBU
Mihai Cristian DUMITRASCU
Dana TERZEA
Ana Maria Alexandra STANESCU
Mara CARSOTE
author_sort Ana VALEA
collection DOAJ
description Neuroendocrine neoplasia (NEN), priory known as neuroendocrine tumours represent the modern approach of traditionally named “carcinoid” tumours. We shortly introduce benign and malign dermatological aspects that are linked to NEN field. This is a multidisciplinary perspective. The paper is a mini-review. 30 articles are included as references. NEN represents 2% of all cancers, being considered orphan disease. Skin and soft tissue are exceptional sites of NEN, the majority are actually secondary spreading of NEN of other locations. Little data have been published related to the topic. Some cases reports showed invasion of dermis and subcutis, probably more frequent in older adults. The entity has been named low-grade neuroendocrine carcinoma of the skin, also named primary cutaneous carcinoid tumour. As seen in NEN diagnosis, the immunohistochemistry is essential like chromogranin A, synaptophysin, neuron specific enolase which are classical neuroendocrine markers and non-neuroendocrine parameters are useful for differential diagnosis like estrogens, androgens and progesterone receptors, GATA3, carcinoembryonic antigen. Merkel cell carcinoma represents the high grade NEN. It has a poor prognosis, with a 30% risk of local metastasis. The risk factors are either viral infection like Merkel cell polyomavirus, either long time exposure to ultraviolet light. The prognostic is similar between the two factors. Surgical removal is the first line therapy and adjuvant treatment is radiotherapy and chemotherapy. Skin NEN represents an exceptional finding of aggressive profile. Their prompt recognition may help the prognosis.
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spelling doaj.art-08025de06997415fa7a09cb85b32aa3f2022-12-21T20:05:52ZengAmaltea Medical Publishing HouseRomanian Journal of Medical Practice1842-82582069-61082020-03-01151333610.37897/RJMP.2020.1.7Skin involvement in neuroendocrine neoplasiaAna VALEA0Florica SANDRU1Simona Elena ALBU2Mihai Cristian DUMITRASCU3Dana TERZEA4Ana Maria Alexandra STANESCU5Mara CARSOTE6Clinical County Hospital, Cluj-Napoca, Romania; “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania; Elias Emergency University Hospital, Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania; Emergency University Hospital, Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania; Emergency University Hospital, Bucharest, Romania“C.I. Parhon” National Institute of Endocrinology, Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania; “C.I. Parhon” National Institute of Endocrinology, Bucharest, RomaniaNeuroendocrine neoplasia (NEN), priory known as neuroendocrine tumours represent the modern approach of traditionally named “carcinoid” tumours. We shortly introduce benign and malign dermatological aspects that are linked to NEN field. This is a multidisciplinary perspective. The paper is a mini-review. 30 articles are included as references. NEN represents 2% of all cancers, being considered orphan disease. Skin and soft tissue are exceptional sites of NEN, the majority are actually secondary spreading of NEN of other locations. Little data have been published related to the topic. Some cases reports showed invasion of dermis and subcutis, probably more frequent in older adults. The entity has been named low-grade neuroendocrine carcinoma of the skin, also named primary cutaneous carcinoid tumour. As seen in NEN diagnosis, the immunohistochemistry is essential like chromogranin A, synaptophysin, neuron specific enolase which are classical neuroendocrine markers and non-neuroendocrine parameters are useful for differential diagnosis like estrogens, androgens and progesterone receptors, GATA3, carcinoembryonic antigen. Merkel cell carcinoma represents the high grade NEN. It has a poor prognosis, with a 30% risk of local metastasis. The risk factors are either viral infection like Merkel cell polyomavirus, either long time exposure to ultraviolet light. The prognostic is similar between the two factors. Surgical removal is the first line therapy and adjuvant treatment is radiotherapy and chemotherapy. Skin NEN represents an exceptional finding of aggressive profile. Their prompt recognition may help the prognosis.https://rjmp.com.ro/articles/2020.1/RJMP_2020_1_Art-07.pdfskinneuroendocrine neoplasiamelanoma
spellingShingle Ana VALEA
Florica SANDRU
Simona Elena ALBU
Mihai Cristian DUMITRASCU
Dana TERZEA
Ana Maria Alexandra STANESCU
Mara CARSOTE
Skin involvement in neuroendocrine neoplasia
Romanian Journal of Medical Practice
skin
neuroendocrine neoplasia
melanoma
title Skin involvement in neuroendocrine neoplasia
title_full Skin involvement in neuroendocrine neoplasia
title_fullStr Skin involvement in neuroendocrine neoplasia
title_full_unstemmed Skin involvement in neuroendocrine neoplasia
title_short Skin involvement in neuroendocrine neoplasia
title_sort skin involvement in neuroendocrine neoplasia
topic skin
neuroendocrine neoplasia
melanoma
url https://rjmp.com.ro/articles/2020.1/RJMP_2020_1_Art-07.pdf
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