Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma

BackgroundNeuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and le...

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Autores principales:	Marta Opalińska, Anna Sowa-Staszczak, Helena Olearska, Magdalena Ulatowska-Bialas, Aleksandra Gilis-Januszewska, Alicja Hubalewska-Dydejczyk
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	NET teratoma ovary tumor neuroendocrine tumors ovarian carcinoid Diseases of the endocrine glands. Clinical endocrinology RC648-665
Acceso en línea:	https://doaj.org/article/91943ef9e7324292973a1ad795f44e8a
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spelling	oai:doaj.org-article:91943ef9e7324292973a1ad795f44e8a2021-12-01T14:56:51ZClinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma1664-239210.3389/fendo.2021.770266https://doaj.org/article/91943ef9e7324292973a1ad795f44e8a2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fendo.2021.770266/fullhttps://doaj.org/toc/1664-2392BackgroundNeuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and less than 5% of all neuroendocrine tumors. They may arise alone (as monodermal, specialized teratoma – ovarian carcinoid) or as a part of other ovarian lesion: cystic mature or immature teratomas. Due to the rarity and limited amount of such cases reported in the literature, there is no consensus on diagnostic and therapeutic procedures in this group of patients.Materials and MethodsThe group of 10 patients at the age of 19 to 77 years (mean 42.8 ± 17.9), diagnosed with unilateral NET within ovarian teratoma were analyzed. The histopathological type of tumor, progression free survival after surgical treatment and presence of hormonally active syndrome were assessed.Results70% (n=7) of patients was diagnosed with mature cystic teratomas containing NET component and 30% (n=3) with monodermal teratoma (strumal carcinoid). All cases of monodermal teratomas were found in women at premenopausal age. Determined Ki67 ranged from 2% to 9%. Ninety percent of lesions (n=9) stained positive for synaptophysin and chromogranin, while markers: CK20, CK7, TTF-1 and CDX2 were negative in all cases, which ruled out their metastatic nature. None of the patients presented with carcinoid syndrome. All followed-up patients remain progression-free, which confirms surgical intervention being a crucial and sufficient method of treatment.ConclusionsThe prognosis and clinical behavior of NETs associated with ovarian teratomas are good with long progression-free survival.Marta OpalińskaAnna Sowa-StaszczakHelena OlearskaMagdalena Ulatowska-BialasAleksandra Gilis-JanuszewskaAlicja Hubalewska-DydejczykFrontiers Media S.A.articleNETteratomaovary tumorneuroendocrine tumorsovarian carcinoidDiseases of the endocrine glands. Clinical endocrinologyRC648-665ENFrontiers in Endocrinology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	NET teratoma ovary tumor neuroendocrine tumors ovarian carcinoid Diseases of the endocrine glands. Clinical endocrinology RC648-665
spellingShingle	NET teratoma ovary tumor neuroendocrine tumors ovarian carcinoid Diseases of the endocrine glands. Clinical endocrinology RC648-665 Marta Opalińska Anna Sowa-Staszczak Helena Olearska Magdalena Ulatowska-Bialas Aleksandra Gilis-Januszewska Alicja Hubalewska-Dydejczyk Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma
description	BackgroundNeuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and less than 5% of all neuroendocrine tumors. They may arise alone (as monodermal, specialized teratoma – ovarian carcinoid) or as a part of other ovarian lesion: cystic mature or immature teratomas. Due to the rarity and limited amount of such cases reported in the literature, there is no consensus on diagnostic and therapeutic procedures in this group of patients.Materials and MethodsThe group of 10 patients at the age of 19 to 77 years (mean 42.8 ± 17.9), diagnosed with unilateral NET within ovarian teratoma were analyzed. The histopathological type of tumor, progression free survival after surgical treatment and presence of hormonally active syndrome were assessed.Results70% (n=7) of patients was diagnosed with mature cystic teratomas containing NET component and 30% (n=3) with monodermal teratoma (strumal carcinoid). All cases of monodermal teratomas were found in women at premenopausal age. Determined Ki67 ranged from 2% to 9%. Ninety percent of lesions (n=9) stained positive for synaptophysin and chromogranin, while markers: CK20, CK7, TTF-1 and CDX2 were negative in all cases, which ruled out their metastatic nature. None of the patients presented with carcinoid syndrome. All followed-up patients remain progression-free, which confirms surgical intervention being a crucial and sufficient method of treatment.ConclusionsThe prognosis and clinical behavior of NETs associated with ovarian teratomas are good with long progression-free survival.
format	article
author	Marta Opalińska Anna Sowa-Staszczak Helena Olearska Magdalena Ulatowska-Bialas Aleksandra Gilis-Januszewska Alicja Hubalewska-Dydejczyk
author_facet	Marta Opalińska Anna Sowa-Staszczak Helena Olearska Magdalena Ulatowska-Bialas Aleksandra Gilis-Januszewska Alicja Hubalewska-Dydejczyk
author_sort	Marta Opalińska
title	Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma
title_short	Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma
title_full	Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma
title_fullStr	Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma
title_full_unstemmed	Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma
title_sort	clinical approach to neuroendocrine neoplasm associated with ovarian teratoma
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/91943ef9e7324292973a1ad795f44e8a
work_keys_str_mv	AT martaopalinska clinicalapproachtoneuroendocrineneoplasmassociatedwithovarianteratoma AT annasowastaszczak clinicalapproachtoneuroendocrineneoplasmassociatedwithovarianteratoma AT helenaolearska clinicalapproachtoneuroendocrineneoplasmassociatedwithovarianteratoma AT magdalenaulatowskabialas clinicalapproachtoneuroendocrineneoplasmassociatedwithovarianteratoma AT aleksandragilisjanuszewska clinicalapproachtoneuroendocrineneoplasmassociatedwithovarianteratoma AT alicjahubalewskadydejczyk clinicalapproachtoneuroendocrineneoplasmassociatedwithovarianteratoma
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Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma

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