Data from small cell neuroendocrine carcinoma of the cervix: FIGO 2018 staging is more accurate than FIGO 2009

Objective To compare the prognostic value of International Federation of Gynecology and Obstetrics (FIGO) 2009 and 2018 staging systems in surgical patients with small cell neuroendocrine carcinoma of the cervix (SCNEC). Methods We re-staged 64 surgical IB–IIA (FIGO 2009) SCNEC patients according to the FIGO 2018 system and refined stage IIIC of FIGO 2018 based on tumor local invasion. The prognostic factors were analyzed, and the advantages of FIGO 2018 were compared with 2009. Results The 5-year overall survival rate (OS) was 78.5% for stage I and 22.2% for stage II (FIGO 2009). In FIGO 2018, there was no difference between stage I and II, and the 5-year OS was 74.1%, 60.2%, and 0% for stage I/II, IIIC1, and IIIC2. After combining stage IIIC with the local invasion stage (T1 was limited to the cervix and vagina; T2 involved the parametrium; T3 involved the pelvic or abdominal cavity), the 5-year OS for stage IIICT1, IIICT2, and IIICT3 was 83.3%, 30.0%, and 0%, respectively. Conclusions For stage II SCNEC patients, FIGO 2009 underestimated the prognosis, while FIGO 2018 was more accurate. For stage IIIC, FIGO 2018 might be more individualized and accurate after combining stage IIIC with tumor local invasion.

Histological Origin and Clinicopathological Analysis of Primary Ovarian Neuroendocrine Neoplasms

Purpose To investigate the histological origin and clinical and pathological features of primary ovarian neuroendocrine neoplasms. Methods We retrospectively analyzed nine cases of ovarian neuroendocrine neoplasms diagnosed at our hospital from January 2009 to January 2021. Results The mean age of the nine patients was 44.9 ± 15.2 years (range, 28–68 years). Six cases manifested ovarian carcinoid cancer, and the pathological types were insular and trabecular carcinoid; microscopic observation showed that the carcinoid components were limited and that stromal reaction was slight. Four cases showed teratomas, with the carcinoid components not displaying adjacent mucinous glands; and the other cases exhibited carcinoid cancer as the only tumor component, without adjacent or migratory epithelial components. The six patients were followed up for 76.6 ± 41.2 (6–123) months after resection, without disease. Two cases manifested adenocarcinoma admixed with neuroendocrine carcinoma, and the intermigration of neuroendocrine carcinoma and adenocarcinoma components could be observed; and one case was an isolated small cell neuroendocrine carcinoma with no epithelial proximity or migration observed. Adenocarcinoma admixed with neuroendocrine carcinoma and small-cell neuroendocrine carcinoma exhibited an obviously promoted interstitial reaction and damaging infiltration: these three patients underwent radical surgery supplemented by postoperative radiotherapy and chemotherapy, and follow-up lasted 34.6 ± 24.2 (7–52) months; two patients died and one showed recurrence. Conclusions Ovarian neuroendocrine neoplasms may reflect multiple tissue origins, carcinoid and simple neuroendocrine neoplasms with no adjacent, transitional epithelium, and may originate from original/transformed neuroendocrine cells or stem cells of the ovarian stroma. In addition, the adenocarcinoma admixed with neuroendocrine carcinoma may originate from dedifferentiated epithelium. The prognosis with carcinoid cancer is favorable, while the prognosis for neuroendocrine carcinoma is poor.

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

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.

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

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.

Neuroendocrine Carcinoma of Oral Cavity: A Case Report of an Unusual Presentation

Neuroendocrine carcinoma (NEC) are tumors affecting lungs in the first line. Extra-pulmonary locations are rare, and involvement of the oral cavity is uncommon. The therapeutic strategy of this clinically aggressive entity is not codified. Nevertheless, multimodal treatment combining surgery and radio-chemotherapy is associated with the best results in terms of local control and overall survival. We report a case of a 67-year-old patient, diagnosed with a NEC, the computed tomography (CT) of the facial mass objectified a jugal mass of 2x2.4 cm with two lymph nodes. The patient benefited from an excisional biopsy of the mass, and whose histological examination and immunohistochemical testing returned in favor of a small cell neuroendocrine carcinoma.

Primary small cell neuroendocrine carcinoma of oropharynx: A potential diagnostic pitfall with Human papillomavirus (HPV)-related squamous cell carcinoma

Introduction/Objective Small cell neuroendocrine carcinoma (SCNEC) primary to the head and neck region, especially from oropharynx, is very rare. Published data have shown that Human papillomavirus (HPV) positivity may be associated with a better overall survival in these tumors. Here we report a case of p16 positive SCNEC arising from the tonsil. Methods/Case Report A 51-year-old man with former smoking history was evaluated for sore throat and difficulty swallowing. Nasopharyngolaryngoscopy showed a left tonsillar mass and imaging revealed left jugular chain lymphadenopathy with no other evidence of a primary tumor elsewhere, including the lung. Biopsy of the mass was performed which revealed nested proliferation of uniform, hyperchromatic tumor cells with scant cytoplasm, indistinct nucleoli, areas of nuclear molding, and brisk mitotic activity. No keratinization was present, but a vague peripheral palisade was seen in some of the nests. The major differential diagnostic considerations included HPV-related nonkeratinizing squamous cell carcinoma, basaloid squamous cell carcinoma, and SCNEC. Immunohistochemistry revealed tumor cells were positive for AE1/AE3 with partial dot-like perinuclear pattern, cytokeratin 8/18, synaptophysin, and strong p16; negative for P40, P63, cytokeratin 5/6, cytokeratin 7, chromogranin, and CD56. Because SCNEC at any site may be positive for p16, testing for HPV RNA by in-situ hybridization was performed and showed negative for HPV subtypes 6, 11, 16, and 18. Results (if a Case Study enter NA) NA Conclusion This case highlights the differential diagnosis of a “basaloid” appearing carcinoma in the oropharynx. It also reinforces the fact that although p16 is considered a reliable surrogate marker for HPV infection in oropharyngeal squamous cell carcinoma, the same does not hold true for SCNEC.