Gallbladder Neuroendocrine Tumors (NETs): Is there a need for a specific approach?

Biliary Tree ◽

Small Cell ◽

Specific Approach ◽

Results Of Treatment ◽

Rare Tumours ◽

Current Guidelines

Neuroendocrine tumors (NETS) of the Gallbladder or the biliary tree are rare. Most of the current guidelines and protocols are derived from the experience of managing Lung small cell neuroendocrine carcinoma (SCNEC) or gastrointestinal (GI) NETS. But the overall outcome of Gallbladder NETS (GB NETS) seems worse than similarly staged lung NETS and adenocarcinoma of the gallbladder. This may be due to its rarity and lack of literature for a focused approach towards its treatment. Hence the need for a specifically designed approach might help improve the results of treatment for these rare tumours. We share our experience of two patients with GB NETS and their 5-year outcome.

Emerging Therapeutic Concepts and Latest Diagnostic Advancements Regarding Neuroendocrine Tumors of the Gynecologic Tract

Medicina ◽

10.3390/medicina57121338 ◽

2021 ◽

Vol 57 (12) ◽

pp. 1338

Author(s):

Tiberiu-Augustin Georgescu ◽

Roxana Elena Bohiltea ◽

Octavian Munteanu ◽

Florentina Furtunescu ◽

Antonia-Carmen Lisievici ◽

...

Keyword(s):

Neuroendocrine Tumors ◽

Classification System ◽

Who Classification ◽

Small Cell ◽

International Agency ◽

Low Grade ◽

High Grade ◽

Female Genital

Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on histologic subtype and site of origin. Following the expert consensus proposal of the International Agency for Research on Cancer (IARC), the approach in the latest World Health Organization (WHO) Classification System of the Female Genital Tumours is to use the same terminology for NENs at all body sites. The main concept of this novel classification framework is to align it to all other body sites and make a clear distinction between well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The previous WHO Classification System of the Female Genital Tumours featured more or less the same principle, but used the terms ‘low-grade neuroendocrine tumor’ and ‘high-grade neuroendocrine carcinoma’. Regardless of the terminology used, each of these two main categories include two distinct morphological subtypes: NETs are represented by typical and atypical carcinoid and NEC are represented by small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). High-grade NECs, especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade NETs usually occur in the ovary. NENs of the vulva, vagina and fallopian tube are exceptionally rare, with scattered case reports in the scientific literature.

A case of a Small Cell Neuroendocrine Carcinoma of the Nasal Septum

Korean Journal of Otolaryngology - Head and Neck Surgery ◽

10.3342/kjorl-hns.2009.52.6.529 ◽

2009 ◽

Vol 52 (6) ◽

pp. 529 ◽

Cited By ~ 3

Author(s):

Young-Soo Kim ◽

Chang Hoon Bae ◽

Si-Youn Song ◽

Yong-Dae Kim

Keyword(s):

Small Cell Neuroendocrine Carcinoma

Nasal Septum ◽

Small Cell ◽

Small cell neuroendocrine carcinoma of the prostate after androgen deprivation therapy for low-risk prostatic adenocarcinoma

10.26226/morressier.5b4709866f4cb30010951884 ◽

2018 ◽

Author(s):

Sofia Mavropoulou ◽

Zoi Tatsiou

Keyword(s):

Androgen Deprivation Therapy ◽

Small Cell ◽

Androgen Deprivation ◽

Prostatic Adenocarcinoma ◽

Low Risk ◽

Carcinoma Of The Prostate

Evaluation of RET Tyrosine Kinase as a Novel Driver of Prostatic Small Cell Neuroendocrine Carcinoma

10.21236/ada621075 ◽

2015 ◽

Author(s):

Justin M. Drake ◽

Owen Witte

Keyword(s):

Tyrosine Kinase ◽

Small Cell Neuroendocrine Carcinoma

Small Cell ◽

A rare case of primary small cell neuroendocrine carcinoma of the Bartholin gland

International Journal of Gynecology & Obstetrics ◽

10.1002/ijgo.13782 ◽

2021 ◽

Author(s):

Ling Han ◽

Ai Zheng ◽

YaLi Chen

Keyword(s):

Small Cell Neuroendocrine Carcinoma

Rare Case ◽

Small Cell ◽

Mixed large and small cell neuroendocrine carcinoma and endometrioid carcinoma of the endometrium with high microsatellite instability: A case report and literature review

SAGE Open Medical Case Reports ◽

10.1177/2050313x21999200 ◽

2021 ◽

Vol 9 ◽

pp. 2050313X2199920

Author(s):

Kotaro Inoue ◽

Kentaro Kai ◽

Shimpei Sato ◽

Haruto Nishida ◽

Koji Hirakawa ◽

...

Keyword(s):

Microsatellite Instability ◽

Immune Checkpoint Blockade ◽

Japanese Woman ◽

Endometrial Biopsy ◽

Small Cell ◽

Endometrioid Carcinoma ◽

Mutation Burden ◽

Solid Part

A 65-year-old, gravida 3, para 2 Japanese woman was referred to our hospital for symptomatic thickening of the endometrial lining. Endocervical and endometrial cytology revealed an adenocarcinoma. The endometrial biopsy specimen was mixed, with a glandular part diagnosed as endometrioid carcinoma and a solid part diagnosed as high-grade mixed large and small cell neuroendocrine carcinoma (L/SCNEC). She underwent extra-fascial hysterectomy with bilateral salpingo-oophorectomy, complete pelvic and para-aortic lymphadenectomy, and omentectomy (FIGO IIIB, pT3b pN0 M0). She currently has no deleterious germline mutation, but high tumor mutation burden and high microsatellite instability (MSI) were identified. She underwent six cycles of platinum-based frontline chemotherapy and achieved complete remission. Immune checkpoint blockade therapy is a promising second-line therapy for MSI-high solid tumors. However, the MSI or mismatch repair (MMR) status of endometrial L/SCNEC remains unclear in the literature. Universal screening for MSI/MMR status is needed, particularly for a rare and aggressive disease.