Neuroendocrine tumor (NET) of the vagina in the light of WHO 2020 2-tiered grading system: clinicopathological report of the first described case

Inzani, Frediano; Santoro, Angela; Angelico, Giuseppe; Mastrosimini, Maria Gaia; Masciullo, Valeria; Salutari, Vanda; Zannoni, Gian Franco

doi:10.1007/s00428-021-03078-6

Cited by 2 publications

(1 citation statement)

References 17 publications

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“…Other gynecological organs like cervix, endometrium vagina or vulva are less frequently affected by NEN. In those localizations most NENs is diagnosed as NECs (6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma

Opalińska¹,

Sowa‐Staszczak

Olearska

et al. 2021

Front. Endocrinol.

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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.

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“…Other gynecological organs like cervix, endometrium vagina or vulva are less frequently affected by NEN. In those localizations most NENs is diagnosed as NECs (6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma

Opalińska¹,

Sowa‐Staszczak

Olearska

et al. 2021

Front. Endocrinol.

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Neuroendocrine carcinoma of vagina with prolonged survival

et al. 2022

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We report the case of a woman in her 70s with a stage IVA small cell neuroendocrine carcinoma of the vagina. The patient started chemotherapy with cisplatin and etoposide followed by concurrent chemoradiotherapy and adjuvant chemotherapy. Pelvic MRI after completion of treatment did not show residual disease. Three years and 8 months after definitive treatment, the patient remains on regular follow-up without evidence of disease.

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Neuroendocrine tumor (NET) of the vagina in the light of WHO 2020 2-tiered grading system: clinicopathological report of the first described case

Cited by 2 publications

References 17 publications

Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma

Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma

Neuroendocrine carcinoma of vagina with prolonged survival

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