Oral and Maxillofacial Neuroendocrine Carcinoma: A Systematic Review

Author(s):  
Lauren Frenzel Schuch ◽  
Tuany Rafaeli Schmidt ◽  
Gisele de Oliveira Zigmundo ◽  
Laura Borges Kirschnick ◽  
Felipe Martins Silveira ◽  
...  
2020 ◽  
pp. 1-14
Author(s):  
Corrado Tinterri ◽  
Alberto Bottini ◽  
Alessandro De Luca ◽  
Andrea Sagona ◽  
Corrado Tinterri ◽  
...  

Introduction: Neuroendocrine neoplasm of the breast (bNENs) are considered a rare disease, even if in WHO data they represent about 2-5 % of all breast cancer. The last WHO classification includes: welldifferentiated neuroendocrine tumor (bNET), neuroendocrine carcinoma (NEC) and invasive carcinoma with neuroendocrine differentiation. The current knowledge on clinical management of bNENs is poor and patients are usually treated according to non-endocrine tumor components guidelines. Materials and Methods: We presented our experience of six cases of bNENs. Moreover, we conducted a systematic review of published data on diagnosis, treatment and outcome of this kind of tumors. Results: bNENS usually presented as palpable breast masses, classically appearing as irregular hypoechoic lesions at US examination and as hyperdense masses at mammography. Usually pre-operative tumor biopsy is not able to recognize the neuroendocrine components and the final diagnosis is performed only on definitive histopathological assessment. The most frequent subtype seems to be neuroendocrine carcinoma and synaptophysin is positive in most specimens. Treatment strategies, including surgical treatment, radiotherapy and medical treatment are nowadays based on current non-endocrine breast cancer guidelines, independently from neuroendocrine components, even if some studies have proposed the use of somatostatin analogues for bNET and cisplatin-etoposide for NEC. Prognosis of all bNENs, especially of poorly differentiated neoplasia, seems worse compared to non-neuroendocrine breast cancer and stage and morphology seem the best predictor of tumor outcome. Conclusions: We provide an algorithm for clinical management of bNETs, basing on available data. More studies are necessary for confirming the best treatment strategy for these patients, in order to improve clinical outcome


2019 ◽  
Vol 103 (4) ◽  
pp. 383-390 ◽  
Author(s):  
Xiang Tu ◽  
Tiancong Chang ◽  
Ling Nie ◽  
Shi Qiu ◽  
He Xu ◽  
...  

BMC Cancer ◽  
2018 ◽  
Vol 18 (1) ◽  
Author(s):  
Clemens B. Tempfer ◽  
Iris Tischoff ◽  
Askin Dogan ◽  
Ziad Hilal ◽  
Beate Schultheis ◽  
...  

2010 ◽  
Vol 1 (2) ◽  
pp. 99-102 ◽  
Author(s):  
Duncan F Hanby ◽  
Andrew McWhorter ◽  
Eric Wallace ◽  
Eugene A Woltering ◽  
Anthony Harton ◽  
...  

Abstract Objective To describe three patients with nonsinonasal neuroendocrine carcinoma (NSNEC) of the head and neck and present a systematic review of literature. Study design Retrospective chart review Methods Three patients with pathologically proven NSNEC of the head-neck who presented to our institution were identified. Relevant demographic, clinic-pathological, and radiological data was recorded. A web-based search was conducted to identify relevant scientific literature on “neuroendocrine carcinoma (NEC) of the head and neck” and a systematic review of literature is presented. Results Two female and one male patient aged 44 to 66 years presented to our service with NEC of the supraglottis (2/3), and of the thymus (1/3). Diagnosis was confirmed with immunohistochemical staining such as Ki-67, synaptophysin, chromogranin, and also with octreotide scanning. The first case, a T4bN0M0 large cell NEC of the thymus with tracheal invasion received conservative airway management and emergent radiotherapy. The second case, moderately differentiated NEC of the supraglottis was managed with a laser supraglottic laryngectomy followed by adjuvant treatment. The third case, a laryngeal NEC was treated with chemoradiotherapy. Conclusions NSNEC of the head and neck are extremely rare tumors with variability in clinical presentation that present challenges in diagnosis and treatment planning. Consideration of the histological subtype and staining characteristics of the individual tumor, accurate diagnosis, and classification of the tumor is vital in order to tailor therapeutic intervention.


2021 ◽  
Vol 11 ◽  
Author(s):  
Hoda Saghaeiannejad Esfahani ◽  
Cory M. Vela ◽  
Aman Chauhan

IntroductionLarge cell neuroendocrine carcinoma (LCNEC) is a rare and highly aggressive high-grade neuroendocrine neoplasm, which can arise from anywhere in the body. Due to its rarity there is a lacuna in our understanding of LCNEC’s molecular biology. In 2016, Rekhtman and colleagues presented one of the largest molecular sequencing series of pulmonary LCNEC. They differentiated genomic profiles of LCNEC into two major subsets: small cell lung cancer (SCLC)-like, characterized by TP53 + RB1 co-mutation/loss, and non-small cell lung cancer (NSCLC)-like, characterized by the lack of co-altered TP53 + RB1. This finding is of significance because at present LCNEC patients are often treated like SCLC. However, the universal genomic SCLC biomarker of TP53 and RB1 co-mutation was only found in 40% of their cohort. Since then various other scientists have looked into molecular profiling of LCNEC with markedly discordant results. The objective of this study was to conduct a systematic review of publicly available next generation sequencing (NGS) data to evaluate the prevalence of TP53 + RB1 co-mutation in LCNEC.MethodWe conducted a literature search using PubMed. Seven studies including 302 patients with pulmonary LCNEC and four studies including 20 patients with extra-pulmonary LCNEC underwent final analysis.ResultsThe prevalence of TP53 + RB1 co-mutation was 36% (109/302) among pulmonary LCNEC patients and 35% (7/20) among the extra-thoracic LCNEC cohort. This finding is in stark contrast to >90% TP53 + RB1 co-mutation in SCLC.ConclusionIt is now well established that LCNEC is molecularly distinct from SCLC. LCNEC seems to have two molecularly defined sub-cohort based on TP53 + RB1 co-mutation status. Future studies should look into prognostic and predictive implication of TP53 + RB1 co-mutation status in LCNEC. Prospective studies should be designed to characterize molecular subtypes and direct treatment accordingly. We are currently conducting a prospective pilot clinical trial wherein LCNEC patients are treated based on TP53 + RB1 co-mutation status. The study is currently enrolling. “Next Generation Sequencing-Based Stratification of Front Line Treatment of Neuroendocrine Carcinoma (PRECISION-NEC).Systematic ReviewClinicalTrials.gov, identifier NCT04452292.


Cancers ◽  
2021 ◽  
Vol 13 (23) ◽  
pp. 5948
Author(s):  
Hao Chen ◽  
Masashi Ishihara ◽  
Nobuyuki Horita ◽  
Hiroki Kazahari ◽  
Ryusuke Ochiai ◽  
...  

Background: Pulmonary large cell neuroendocrine carcinoma (LCNEC) is a rare subset of lung carcinoma with poor overall survival. Methods: A systematic review following a meta-analysis of studies was performed to identify the effect of different selections of chemotherapy in LCNEC. Articles providing overall survival data for adjuvant chemotherapy or palliative chemotherapy for LCNEC were eligible. The odds ratio (OR) of mortality at one or two years after chemotherapy was evaluated. Results: A total of 16 reports were finally included in the quantitative synthesis, involving a total of 5916 LCNEC patients. Adjuvant chemotherapy was administered to 1303 patients, and palliative chemotherapy was administered to 313 patients using either a small cell lung cancer (SCLC) or a non-small cell lung cancer (NSCLC) regimen. The OR for adjuvant chemotherapy was 0.73 (95% confidence interval (CI): 0.59 to 0.89, p = 0.002). The SCLC regimen showed an OR of 0.52 (95% CI: 0.11 to 2.38, p = 0.40) after one year, and 0.32 (95% CI: 0.11 to 0.89, p = 0.03) after two years, compared with the NSCLC regimen. Conclusions: Adjuvant chemotherapy for pulmonary large cell neuroendocrine carcinoma improved the outcome after surgery. The SCLC regimen showed better survival than the NSCLC regimen as palliative chemotherapy.


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