Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso / Surgical treatment of advanced facial Merkel cell carcinoma. A case report

Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

Carcinoma neuroendocrino de canal anal: presentación de un caso y revisión bibliográfica

Introducción: Los carcinomas neuroendocrinos (NEC) de canal anal son neoplasias extremadamente raras, representando del 1 a 1,6% de la totalidad de los tumores neuroendocrinos (NET). Suelen ser poco diferenciados, muy agresivos y con alta tendencia a metastatizar. Caso clínico: Mujer de 52 años diagnosticada de fisura anal. Durante la esfinterotomía lateral interna (ELI) se evidencia un pólipo milimétrico aparentemente hiperplásico. Biopsia: NEC de alto grado. En el estudio de extensión se observa engrosamiento de la mucosa del canal anal que invade el esfínter interno, sin enfermedad a distancia. Se realiza amputación abdominoperineal laparoscópica donde se objetiva infiltración del tabique rectovaginal, por lo que se realiza resección y vaginoplastia. AP: NEC con estadio PT4B N2A, por lo que se indica quimioterapia adyuvante. Discusión: La presentación clínica de los NEC de canal anal es inespecífica, diferenciándose de otros tumores colorrectales en que hasta el 67% de los pacientes presentan metástasis al diagnóstico, siendo la supervivencia media de 11 meses. Si diagnosticamos un NEC localizado de forma incidental, es fundamental la celeridad en su tratamiento, dada su agresividad.

Introduction: Neuroendocrine carcinomas (NEC) of the anal canal are extremely rare neoplasms, representing 1 to 1.6% of all neuroendocrine tumors (NET). They are usually poorly differentiated, very aggressive and with a high tendency to metastasize. Clinical case: A 52-year-old woman diagnosed with anal fissure. During the LIS, an apparently hyperplastic millimetric polyp is evidenced. Biopsy: high-grade NEC. The imaging study shows thickening of the mucosa of the anal canal that invades the internal sphincter, without metastases. We performed a laparoscopic abdominoperineal amputation, and noticed an infiltration of the rectovaginal septum, so resection and vaginoplasty was performed. Pathology: NEC with stage PT4B N2A, for which adjuvant chemotherapy is indicated. Discussion: The clinical presentation of NEC of the anal canal is nonspecific, differing from other colorectal tumors in that up to 67% of patients have metastases at diagnosis, with a median survival of 11 months. When an incidentally localized NEC is diagnosed, prompt treatment is essential, given its aggressiveness.

Small cell neuroendocrine carcinoma with primary orbital involvement – The unseen, and a review of literature

Neuroendocrine neoplasms are derived from the epithelial lineages mainly of respiratory tract, with predominant neuroendocrine differentiation. There are only a handful of documented cases of paranasal small cell neuroendocrine carcinomas (SNEC) with primary orbital involvement. Here, the authors describe a 33-year-old male patient with rapidly progressive swelling of the right lower lid with proptosis since 4 weeks. On contrast-MRI orbit, an ill-defined multilobulated mass measuring 3.6 × 3.1 cm with intense homogenous enhancement was seen in the right retrobulbar space involving the right ethmoid sinus. On incisional biopsy, a poorly differentiated mass containing numerous small round blue cells and scanty intervening stroma with prominent necrosis and apoptosis was seen. Immunohistochemistry was strongly positive for synaptophysin. He was diagnosed as a case of SNEC and received chemotherapy, with good response till date of 9 months of follow up. The authors present a literature review and describe challenges in management of a primary orbital SNEC.

Tumores neuroendócrinos pancreáticos: revisão histopatológica / Pancreatic neuroendocrine tumors: histopathological review

Introdução: Os tumores neuroendócrinos pancreáticos são considerados raros. Eles são classificados em funcionantes e não funcionantes. Objetivo: Definir e classificar tumores neuroendócrinos pancreáticos de acordo com sua avaliação histopatológica e imunoistoquímica, associado aos critérios diagnósticos. Método: Trata-se de revisão narrativa sobre publicações encontradas no PubMed, SciELO e Google Acadêmico. Resultados: Esses tumores podem ser bem ou pouco diferenciados e apresentam características microscópicas distintas. As células bem diferenciadas têm formato pequeno, núcleos uniformes redondos ou ovais, citoplasma finamente granular indicando forte capacidade secretória e mantêm a estrutura organoide. A presença de necrose tumoral, atividade mitótica aumentada e índice de Ki-67 elevado indicam alta probabilidade de neoplasia neuroendócrina. Cromogranina A e sinaptofisina favorecem o diagnóstico do bem diferenciado. Já a marcação positiva do BCL 10 em conjunto com a ausência de expressão da cromogranina A e da sinaptofisina mostram a precária diferenciação tumoral. A presença de marcação positiva para as expressões hormonais não define o tumor como funcionante. Conclusão: Houve aumento do diagnóstico de tumores neuroendócrinos pancreáticos com o uso de técnicas de imagem e a conscientização sobre a doença. A análise histopatológica com imunoistoquímica, especialmente quando há sintomas consumptivos, podem indicar o tipo do carcinoma e induzir ao mais adequado tratamento.

Introduction: Pancreatic neuroendocrine tumors are considered rare. They are classified into functioning and non-functioning. Objective: To define and classify pancreatic neuroendocrine tumors according to their histopathological and immunohistochemical evaluation, associated with diagnostic criteria. Method: This is a narrative review of publications found in PubMed, SciELO and Google Scholar. \Results: These tumors can be well or poorly differentiated and have distinct microscopic characteristics. Well-differentiated cells are small in shape, have uniform round or oval nuclei, finely granular cytoplasm indicating strong secretory capacity, and maintain the organoid structure. Presence of tumor necrosis, increased mitotic activity and high Ki-67 index indicate a high probability of neuroendocrine neoplasia. Chromogranin A and synaptophysin favor the diagnosis of well-differentiated. The positive staining of BCL 10 together with the absence of expression of chromogranin A and synaptophysin show poor tumor differentiation. The presence of positive staining for hormone expressions does not define the tumor as functioning. Conclusion: There was an increase in the diagnosis of pancreatic neuroendocrine tumors with the use of imaging techniques and awareness of the disease. Histopathological analysis with immunohistochemistry, especially when there are consuming symptoms, can indicate the type of carcinoma and lead to the most appropriate treatment.

Clinicopathological Features of Primary Small Cell Neuroendocrine Carcinoma of the Bladder / 中国医学科学院学报

Objective To investigate the clinicopathological features,immunohistochemical features,diagnosis,and relationship with sporadic prostate cancer in primary small cell neuroendocrine carcinoma of the bladder. Methods We retrospectively analyzed the clinical characteristics of 12 patients with primary small cell neuroendocrine carcinoma of the bladder diagnosed at Beijing Chao-Yang Hospital affiliated to Capital Medical University from January 2013 to September 2022.The histological features of primary small cell neuroendocrine carcinoma of the bladder were re-evaluated by two pathologists according to the 2022 revision of the World Health Organization Classification of Tumors of the Urinary System and Male Genital Organs.Electronic medical records were retrieved,and telephone follow-up was conducted from the time of histopathological diagnosis to the death or the end of the last follow-up until January 31,2023. Results The 12 patients include 7 patients in pT3 stage and 1 patient in pT4 stage.Eight patients were complicated with other types of tumors,such as high-grade urothelial carcinoma of the bladder and squamous cell carcinoma.Five patients had sporadic prostate cancer.Immunohistochemical staining showed that 12 (100.0%),10 (83.3%),and 8 (66.7%) patients were tested positive for CD56,Syn,and CgA,respectively.The Ki67 proliferation index ranged from 80% to 90%.Five patients with urothelial carcinoma were tested positive for CK20,GATA3,and CK7.P504S was positive in all the 5 patients with prostate cancer,while P63 and 34βE12 were negative.The follow-up of the 12 patients lasted for 3-60 months.Eight of these patients died during follow-up,with the median survival of 15.5 months.Four patients survived. Conclusions Primary small cell neuroendocrine carcinoma of the bladder is a rare urological tumor with high aggressiveness and poor prognosis.In male patients with bladder prostatectomy,all prostate tissue should be sampled.If prostate cancer is detected,the prostate-specific antigen level should be monitored.

Treatment and Outcome of Merkel Cell Carcinoma in A Single Center / 肿瘤防治研究

Objective To investigate the clinical features, treatment, and outcome characteristics of patients with Merkel cell carcinoma. Methods The clinical manifestations, laboratory tests, diagnosis and treatment, and follow-up data of six patients with Merkel cell carcinoma were retrospectively analyzed. Results Among the six patients with Merkel cell carcinoma, four were males and two were females, with a median age of 66 years old (57-76 years old). All six patients presented with skin swelling, and the clinical stages were as follows: stageⅠ in three patients, stage Ⅲ in one patient, and stage IV in two patients. Two patients were treated with surgery alone, three patients with surgery combined with radiotherapy and/or chemotherapy, and one patient with immunotherapy combined with chemotherapy. Until the follow-up time, four patients had no disease progression, one patient died because of disease progression, and one patient remained under treatment. Conclusion Limited-stage Merkel cell carcinoma is primarily treated with surgery and radiotherapy, meanwhile, metastatic Merkel cell carcinoma needs systemic therapy, and first-line immune checkpoint inhibitors targeting PD-1/ PD-L1 pathway can achieve better therapeutic results.

Poorly differentiated neuroendocrine carcinoma of the tongue base: a case report / 临床耳鼻咽喉头颈外科杂志

Neuroendocrine carcinoma(NEC) is a malignant tumor derived from neuroendocrine cells, with distinct clinical, morphological and immunohistochemical characteristics. Neuroendocrine carcinoma of the head and neck is very rare in clinic. Larynx is the most common affected site, and the root of the tongue is extremely rare. The clinical manifestations are mainly eating pain, cauliflower like mass in the mouth, and ulcerative lesions that have not healed for a long time. Maxillofacial MRI and contrastenhanced CT are the most commonly used examination tools for such diseases, which can detect the spaceoccupying lesions of tumors. Neuroendocrine granules found in the cytoplasm under pathological light microscope can be diagnosed as neuroendocrine carcinoma. However, for most cases, it is difficult to make a diagnosis only under light microscope, and it is often necessary to make a diagnosis by means of immunohistochemistry and other technical means. This paper reports a case of neuroendocrine carcinoma of the root of the tongue, introduces its characteristics, diagnosis and treatment, and reviews the relevant literature of this case.

Primary adrenal small cell neuroendocrine carcinoma complicated with renal venous carcinoma thrombus: a case report / 中华泌尿外科杂志

Primary adrenal small cell neuroendocrine carcinoma is clinically rare. This article reported a patient, who was diagnosed as primary adrenal small cell neuroendocrine carcinoma complicated with renal vein cancer thrombus, and underwent laparoscopic left adrenal + left kidney + left renal vein tumor embolectomy.The carcinoma relapsed after 19 months of follow-up after surgery. The patient and his family refused further treatment.

High-grade mixed neuroendocrine non-neuroendocrine neoplasm of the gastroesophageal junction: A rare case report and review of literature

Mixed neuroendocrine non-neuroendocrine neoplasm (MiNeN) is a recently described entity of the esophagus in the latest (fifth) edition of WHO Classification of Digestive System Tumors. It is often a difficult pathological diagnosis, especially in small preoperative biopsies. We herein report a case of high-grade MiNeN of gastroesophageal junction diagnosed as a squamous cell carcinoma in preoperative biopsy and subsequently as a high-grade MiNeN in esophagogastrectomy specimen comprising areas of mucoepidermoid carcinoma and large-cell neuroendocrine carcinoma (NEC). This report accentuates the importance of deeper multisite preoperative biopsies as the management is completely different in a MiNeN from esophageal squamous cell carcinoma.

A journey of neuroendocrine carcinoma of the brain from the primary to metastatic tumor

The neuroendocrine tumors most commonly originate in the lung, liver, and gastrointestinal tract. They can be graded into a three-tiered system depending on their proliferation index. These tumors frequently metastasize to the regional lymph nodes. Neuroendocrine tumors of the brain are very rare. Most of them are metastatic and a handful of cases are primary. Hereby, we present a case of brain neuroendocrine carcinoma, which was solitary with no evidence of the primary tumor in the body at the time of neurosurgery. However, treating physicians vigilantly kept him on regular follow-up and investigated further. The patient was diagnosed to have a lesion in the lung 3 months after surgery. Thus, it was considered primary neuroendocrine carcinoma of the brain; however, regular follow-up and further positron emission tomography scan showed a small mass in the lung and adrenal gland. Based on this, a final diagnosis of metastatic neuroendocrine carcinoma of the brain was made.

Large cell neuroendocrine carcinoma of cervix: A case report

Neuroendocrine carcinoma of the cervix is a rare aggressive tumor with a poor prognosis. Neuroendocrine carcinomas account for 1–1.5% of cervical cancer and around 12.5% were of large cell types. We report the case of a 55-year-old woman who came with complaints of pain abdomen and was reported as large cell neuroendocrine carcinoma of the cervix on biopsy. Immunohistochemistry was done which confirmed the diagnosis.

O prognóstico dos diferentes subtipos de carcinomas de esôfago neuroendócrinos: um estudo de base populacional / The prognosis of the different esophageal neuroendocrine carcinoma subtypes: a population-based study

ABSTRACT Background Neuroendocrine neoplasms are extremely rare and account for 0.4% to 2% of all malignant esophageal neoplasms. The burden of the neuroendocrine histological type on the patients' prognosis and survival is poorly debated. This study aimed to compare the survival rates of primary neuroendocrine neoplasms compared with adenocarcinoma and squamous cell carcinoma of the esophagus. Methods This is a retrospective cohort from the Surveillance, Epidemiology, and End Results Program database. Overall survival and cancer-specific survival were evaluated with Kaplan-Meier curves and logrank tests. Proportional Cox regression models were used to evaluate variables related to overall survival. Results After eligibility criteria, 66,528 patients were selected. The mean follow-up was 22.6 months (SD 35.6). Adenocarcinoma was predominant (62%), followed by squamous cell carcinoma (36%). Large cell carcinoma, small cell carcinoma, and mixed adenoneuroendocrine carcinoma each account for less than 1% each. On the long-term overall survival analysis, esophageal adenocarcinoma showed a better prognosis than all the other histologic types (P-value for logrank test <0.001). With adenocarcinoma as a reference, HR was 1.32 for large cell carcinoma (95%CI 1.2 to 1.45) and 1.37 for small cell carcinoma (95%CI 1.23 to 1.53). The HR was 1.22 for squamous cell carcinoma (95%CI: 1.2 to 1.24); and 1.3 for adenoneuroendocrine carcinoma (95%CI 1.01 to 1.66). For multivariate Cox regression analysis, besides age and stage, the neuroendocrine subtypes large cell carcinoma and small cell carcinoma were considered independent prognostic variables. Conclusion In the esophagus, large cell carcinoma and small cell carcinoma show poorer long-term survival rates than squamous cell carcinoma and adenocarcinoma.

RESUMO Contexto As neoplasias neuroendócrinas são extremamente raras e representam 0,4% a 2% de todas as neoplasias malignas do esôfago. A determinação prognóstica e avaliação de sobrevida para o tipo histológico neuroendócrino é pouco debatida. Este estudo teve como objetivo comparar as taxas de sobrevida de neoplasias neuroendócrinas primárias comparadas com adenocarcinoma e carcinoma espinocelular de esôfago. Métodos Este é um estudo coorte retrospectivo do banco de dados do Surveillance, Epidemiology, and End Results Program. A sobrevida global e a sobrevida específica do câncer foram avaliadas com curvas de Kaplan-Meier e testes de logrank. Modelos de regressão de Cox proporcional foram utilizados para avaliar as variáveis relacionadas à sobrevida global. Resultados Após critérios de elegibilidade, foram selecionados 66,528 pacientes. O seguimento médio foi de 22,6 meses (DP 35,6). O adenocarcinoma foi predominante (62%), seguido pelo carcinoma espinocelular (36%). Carcinoma de grandes células, carcinoma de pequenas células e carcinoma adenoneuroendócrino misto representam menos de 1% cada. Na análise de sobrevida global, o adenocarcinoma de esôfago apresentou um prognóstico melhor do que todos os outros tipos histológicos (P valor para teste de logrank < 0,001). Com adenocarcinoma como referência, HR foi de 1,32 para carcinoma de grandes células (IC95% 1,2 a 1,45) e 1,37 para carcinoma de pequenas células (IC95% 1,23 a 1,53). O HR foi de 1,22 para carcinoma espinocelular (IC95%: 1,2 a 1,24); e 1,3 para carcinoma adenoneuroendócrino (IC95% 1,01 a 1,66). Para a análise multivariada da regressão de Cox, além da idade e do estadiamento, os subtipos neuroendócrinos carcinoma de grandes células e carcinoma de pequenas células foram considerados variáveis prognósticas independentes. Conclusão No esôfago, o carcinoma de grandes células e o carcinoma de pequenas células apresentam menores taxas de sobrevida a longo prazo do que o carcinoma espinocelular e o adenocarcinoma.

Anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma: a case report / 中华神经科杂志

Paraneoplastic neurological syndromes (PNS) are heterogeneous disorders caused by autoimmune responses of cancer, which can affect any part of the nervous system. Anti-amphiphysin antibody is one of the high-risk PNS antibodies, which is usually associated with small cell lung cancer and breast cancer. However, extrapulmonary neuroendocrine carcinoma is rare in patients with anti-amphiphysin antibody. A case of anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma is reported. The tumor was detected by fluorine 18 fluorodeoxyglucose positron emission tomography and pathologically confirmed by gastroscopic biopsy. The patient′s neurological symptoms were partially improved after treatment of intravenous immunoglobulin and glucocorticoids. However, the disease prognosis is closely related to the accompanying tumor.

The Advance in Research into Immunotherapy for Neuroendocrine Carcinoma of Digestive System / 罕见病研究

Gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) is a rare heterogeneous tumor. The heterogeneity in pathology, differentiation, grade and clinical stages results in different prognosis and treatment strategy. Patients with recurrent or metastatic NEN have limited treatment options and poor prognosis.Programmed cell death 1(PD-1) blockade has played indispensable roles in management of cancers recently.Immunotherapy is being explored in NENs in the world with emerging clinical trials. The results of the trials provide evidence and guidance in the application of NEC.The article analizes and summarizes of the findings of major investigations in this field.

Carcinoma neuroendocrino moderadamente diferenciado (carcinoide atípico) de cavidad nasal: Reporte de un caso y revisión del tema / Moderately differentiated neuroendocrine carcinoma of the nasal cavity, a case report and literature review

Resumen Los carcinomas neuroendocrinos son tumores poco frecuentes en nariz y senos paranasales. Se consideran tumores primitivos y son los menos diferenciados del sistema neuroendocrino difuso. Se discute el caso de una paciente femenina de 49 años quien acude al servicio de otorrinolaringología en el Hospital Central Militar refiriendo masa en cavidad nasal izquierda de crecimiento progresivo en un periodo de 9 meses, así como epistaxis y obstrucción nasal izquierda. Mediante resonancia magnética y tomografía computada se observó tumoración de la cavidad nasal izquierda en su totalidad hasta nasofaringe, con captación heterogénea del medio de contraste. Se decide resección de tumoración mediante abordaje endoscópico (sinusotomía maxilar tipo III, etmoidectomía anterior y posterior izquierda, Draf esfenoidal tipo II izquierdo, Draf IIa del seno frontal izquierdo, así como septectomía posterior). El estudio histopatológico reportó un carcinoma neuroendocrino moderadamente diferenciado, por lo que se trató de forma conjunta con servicio de oncología médica. Este tipo de tumores son un reto diagnostico por la complejidad para diferenciarlos en un estudio histopatológico. La diferenciación efectiva de los mismos puede tener un impacto clínico, por lo que los avances en la intervención terapéutica podrían prolongar la supervivencia del paciente, mejorar la calidad de vida e incluso la cura.

Abstract Neuroendocrine carcinomas are rare tumors of the nose and sinuses. They are considered primitive tumors and are the least differentiated from the diffuse neuroendocrine system. We report a 49-year-old female patient who attends the otolaryngology service at the Central Military Hospital referring mass in the left nasal cavity with a progressive growth over a period of 9 months, including epistaxis, as well as left nasal obstruction. Magnetic Resonance Imaging and Computed Tomography imaging studies showed a tumor occupying the entirety of the left nasal cavity to the nasopharynx, with heterogeneous uptake of the contrast medium. Tumor resection is decided by endoscopic approach (maxillary sinusotomy type III, left anterior and posterior ethmoidectomy, left sphenoid Draf type II, left frontal Draf IIa as well as posterior septectomy). The pathology service reported a moderately differentiated neuroendocrine carcinoma. The treatment was decided joint session with the medical oncology service. These types of tumors are a diagnostic challenge because of the complexity to differentiate them in a histopathological study. Their effective differentiation can have a clinical impact, that's why the advances in therapeutic intervention could prolong patient survival, improve quality of life and even find a cure.

Características clínicas y sociodemográficas de una población adulta con tumores neuroendocrinos

Introducción: Los tumores neuroendocrinos representan un grupo de neoplasias de baja incidencia que derivan de células neuroendocrinas distribuidas en todo el cuerpo en especial sistema respiratorio y gastrointestinal. Objetivo: Determinar las características clínicas y sociodemográficas de una población adulta con padecimiento de tumores neuroendocrinos. Materiales y métodos: Estudio descriptivo transversal, se evaluaron 91 historias clínicas con diagnóstico de tumores neuroendocrinos confirmados por patología entre los años 2013 a 2020. Análisis realizado en Microsoft Excel 2013 y EpiInfo 7.2. Resultados: La media de edad fue 60 años, con predominio en hombres (57%). Los principales antecedentes fueron el tabaquismo (35%), hipertensión arterial (22%) y EPOC (9%). Los principales síntomas fueron el dolor abdominal (43%), pérdida de peso (31%) y tos (26%). Según el origen, fueron más frecuentes los de intestino anterior (75%), predominando los de tracto respiratorio (39,5%). En el 21,9%, el origen fue desconocido. Teniendo en cuenta la clasificación 2019 de la OMS, predominaron los carcinomas neuroendocrinos (56%), de los cuales el más frecuente fue el carcinoma de células pequeñas. Entre los bien diferenciados (44%), fueron más frecuentes los de bajo grado (58%) seguido grado intermedio (24%) y bajo grado (17%). Las metástasis se registraron en 37% de los casos con afectación principalmente hepática (49%), ganglios (21%) y sistema nervioso central (9%). La muerte se presentó en el 24% de los casos. Conclusiones: Los resultados del presente estudio concuerdan con lo reportado a nivel mundial, resaltando el predominio de los tumores de origen pulmonar, como también clínica semejante según los órganos afectados.

Introduction: Neuroendocrine tumors represent a group of low-incidence neoplasms derived from neuroendocrine cells distributed throughout the body, especially the respiratory and gastrointestinal systems. Objective: To determine the clinical and sociodemographic characteristics of an adult population with neuroendocrine tumors. Materials and methods: In a descriptive cross-sectional study, 91 medical records with a diagnosis of neuroendocrine tumors confirmed by pathology were evaluated between the years 2013 and 2020. Analysis carried out in Microsoft Excel 2013 and EpiInfo 7.2. Results: The mean age was 60 years, with a predominance in men (57%). The main antecedents were smoking (35%), arterial hypertension (22%), and COPD (9%). The main symptoms were abdominal pain (43%), weight loss (31%), and cough (26%). According to the origin, those of the foregut were more frequent (75%), predominantly those of the respiratory tract (39.5%). In 21.9%, the origin was unknown. Taking into account 2019 WHO classification, neuroendocrine carcinomas predominated (56%), of which the most frequent was small cell carcinoma. Among the well-differentiated (44%), low-grade (58%) followed by intermediate grade (24%) and low-grade (17%). Metastases were registered in 37% of the cases with mainly liver involvement (49%), lymph nodes (21%), and central nervous system (9%). Death occurred in 24% of cases. Conclusions: The results of the present study coincide with those reported worldwide, highlighting the predominance of tumors of pulmonary origin, as well as similar clinical symptoms according to the affected organs

Efficacy and Safety of PD-1 Inhibitor Combined with Anlotinib on Advanced Neuroendocrine Carcinoma / 肿瘤防治研究

Objective To analyze the efficacy and safety of PD-1 inhibitor combined with anlotinib on advanced neuroendocrine carcinoma. Methods We collected the data of patients with advanced neuroendocrine carcinoma who had failed the first-line standard chemotherapy and treated with PD-1 inhibitor combined with anlotinib from the First Affiliated Hospital of Zhengzhou University. Results A total of 45 patients, including 24 males and 21 females, were included. The median age was 57 years old. The primary tumor sites were lung (23 cases, 51.1%), esophagus (8 cases, 17.8%), pancreas (7 cases, 15.6%) and rectum (7 cases, 15.6%). Eighteen cases (40%) had failed the first- and second-line treatments, and 27 cases (60%) had failed the third-line and above treatments. All patients received 2-15 cycles of treatment, 3 cases died due to disease progression, overall objective response rate was 11.1%, disease control rate was 53.5%, median progression-free survival was 5.8 months, and 10-month progression-free survival rate was 25.5%. Adverse events were mainly grade 1-2 myelosuppression and digestive tract reactions. Conclusion PD-1 combined with anlotinib show better efficacy and good tolerance on advanced neuroendocrine carcinoma. It can be used as a choice after the failure of standard first-line treatment of advanced neuroendocrine carcinoma.

Pregnancy-preserving and maternal-fetal management in a patient with rare large cell neuroendocrine carcinoma of the uterine cervix / 南方医科大学学报

OBJECTIVE@#To explore the strategy of pregnancy-preserving and maternal- fetal management in patients with primary gynecologic neuroendocrine tumors (gNETs) during pregnancy.@*METHODS@#We performed whole genome sequencing (WGS) for analyzing maternal and fetal somatic and germline single nucleotide variations (SNVs) and small insertions and deletions (InDels) for a 29-year-old pregnant woman diagnosed with stage IB2 large cell neuroendocrine carcinoma (LCNEC) and adenocarcinoma in the cervix. A systematic literature review was performed to explore the strategies for treatment of such rare histological type while maintaining pregnancy.@*RESULTS@#Global case analysis of cervical NETs during pregnancy suggested that negative lymph node metastasis and an early FIGO stage were potentially associated with a good prognosis of the patients. In the case presented herein, a pregnancy-preserving strategy was adopted and favorable maternal-fetal outcomes were achieved after neoadjuvant chemotherapy, radical surgery and postoperative systemic chemotherapy. At 35@*CONCLUSIONS@#Although gNETs in pregnancy are rare and highly risky, pregnancy-preserving managements of gNETs can still be considered and favorable maternalfetal outcomes are possible with proper assessment of the clinical indications and implementation of multimodal treatments. Precise treatment and follow-up strategies based on the results of WGS for risk-reducing intervention of cancer recurrence or occurrence can potentially benefit the patient and the neonate.

Correlation Analysis of Serum LDH Concentration before and after Operation and Prognosis of Large Cell Neuroendocrine Lung Cancer Patients / 中国肺癌杂志

BACKGROUND@#Studies have shown that elevated serum lactate dehydrogenase (LDH) concentration can lead to poor prognosis in patients with small cell lung cancer and lung adenocarcinoma, but its relationship with the prognosis of patients with lung large-cell neuroendocrine carcinoma (L-LCNEC) is not clear. This study aims to explore the influence of L-LCNEC preoperative serum LDH concentration and postoperative LDH concentration change trend on the disease-free survival (DFS) of patients after surgery, so as to judge the clinical prognosis of L-LCNEC provides new ideas.@*METHODS@#Collected the clinical data. The receiver operating characteristic (ROC) curve was used to determine the optimal cut-off value, while the Kaplan-Meier and Cox proportional hazard model were used to analyze data.@*RESULTS@#DFS was shortened in patients with high serum LDH concentration before operation and increased LDH concentration after operation (P<0.001, P<0.001). The preoperative LDH concentration and postoperative LDH concentration change trend were independent prognostic factors for patients (P<0.001, P=0.037).@*CONCLUSIONS@#Preoperative LDH concentration and its postoperative concentration change trend in patients with L-LCNEC are independent prognostic factors for DFS of patients.