Muir-Torre syndrome (MTS) is an autosomal genodermatosis that is diagnosed by the presence of at least one sebaceous gland tumor and at least one visceral malignancy. The most frequent visceral malignancies reported in literature are low-grade colon-rectal and genitourinary cancers, with prolonged survival. The authors report the case of a 52-year-old female, with a positive familial history for MTS, who developed a cutaneous sebaceous carcinoma, a synchronous colon-rectal adenocarcinoma, and a metachronous endocervical adenocarcinoma associated with a neuroendocrine small cell carcinoma of the cervix (SCNC), with lymph node metastasis. The rare occurrence in literature of the cervical SCNC and the rarest occurrence of a neuroendocrine carcinoma in the context of a MTS deviate from the usual and low-grade types of cancers normally described with MTS. It should be always appropriate to assess any symptoms that might reveal an underlying malignancy, although not within the spectrum of neoplasms most associated with this rare syndrome.

Adenocarcinoma of the cervix associated with a neuroendocrine small cell carcinoma of the cervix in the spectrum of Muir-Torre syndrome / Donati, P; Paolino, G; Donati, M; Panetta, C. - In: EUROPEAN JOURNAL OF GYNAECOLOGICAL ONCOLOGY. - ISSN 0392-2936. - 36:2(2015), p. 213-5.

Adenocarcinoma of the cervix associated with a neuroendocrine small cell carcinoma of the cervix in the spectrum of Muir-Torre syndrome

Paolino, G;
2015

Abstract

Muir-Torre syndrome (MTS) is an autosomal genodermatosis that is diagnosed by the presence of at least one sebaceous gland tumor and at least one visceral malignancy. The most frequent visceral malignancies reported in literature are low-grade colon-rectal and genitourinary cancers, with prolonged survival. The authors report the case of a 52-year-old female, with a positive familial history for MTS, who developed a cutaneous sebaceous carcinoma, a synchronous colon-rectal adenocarcinoma, and a metachronous endocervical adenocarcinoma associated with a neuroendocrine small cell carcinoma of the cervix (SCNC), with lymph node metastasis. The rare occurrence in literature of the cervical SCNC and the rarest occurrence of a neuroendocrine carcinoma in the context of a MTS deviate from the usual and low-grade types of cancers normally described with MTS. It should be always appropriate to assess any symptoms that might reveal an underlying malignancy, although not within the spectrum of neoplasms most associated with this rare syndrome.
2015
Adenocarcinoma; Carcinoma, Neuroendocrine; Carcinoma, Small Cell; Colorectal Neoplasms; Female; Humans; Middle Aged; Muir-Torre Syndrome; Neoplasms, Multiple Primary; Neoplasms, Second Primary; Uterine Cervical Neoplasms
01 Pubblicazione su rivista::01a Articolo in rivista
Adenocarcinoma of the cervix associated with a neuroendocrine small cell carcinoma of the cervix in the spectrum of Muir-Torre syndrome / Donati, P; Paolino, G; Donati, M; Panetta, C. - In: EUROPEAN JOURNAL OF GYNAECOLOGICAL ONCOLOGY. - ISSN 0392-2936. - 36:2(2015), p. 213-5.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1074086
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