Neuroendocrine breast carcinoma

ABSTRACT Neuroendocrine breast cancer (NEBC) is a rare and heterogeneous entity. It most commonly presents a luminal phenotype and a worse prognosis. When diagnosed in an advanced stage, metastasis from another neuroendocrine tumor should be excluded. This case features a premenopausal woman with an oligometastatic breast large cell neuroendocrine carcinoma, estrogen receptor (ER) positive, and human epidermal growth factor receptor 2 (HER2) negative. Since the patient was very symptomatic at the presentation of the disease, chemotherapy was started. Complete radiological response of the metastatic disease was achieved, and the patient was then submitted to radical breast surgery and bilateral oophorectomy. She subsequently underwent radiation therapy. Since then and to date, she has been under endocrine therapy (ET) and a CDK4/6 inhibitor (CDK4/6i), with no evidence of malignant disease. Evidence to guide the choice of treatment for these tumors is currently scarce. In cases with oligometastatic disease, radical treatment should be considered. Given that this entity is rare, its reporting should be encouraged.

Combined larynx large cell neuroendocrine and squamous cell carcinoma: a case report

ABSTRACT Laryngeal cancer ranks third among the most common head and neck neoplasms. The most common histological subtype is squamous cell carcinoma, and neuroendocrine tumors are rare. An even rarer entity is a composite tumor with both these histologies. This case reports a metastatic combined carcinoma of squamous cells and large neuroendocrine cells, presenting favorable response to treatment with a total laryngectomy followed by adjuvant therapy including chemo-, radio-, and immunotherapy.

Progress of diagnosis and treatment for gastrointestinal neuroendocrine neoplasms / 肿瘤研究与临床

Gastrointestinal neuroendocrine neoplasm (GI-NEN) is an important part of digestive system tumor. With the progress of diagnosis and the further understanding of GI-NEN, the diagnostic rate is increasing. However, GI-NEN is a rare tumor, thus clinicians are not familiar with the diagnosis and treatment. Misdiagnosis and missed diagnosis of GI-NEN often occur, and the treatment regimen is not standardized. In recent years, some progresses of the diagnosis and treatment have been made in GI-NEN, especially in the aspects of pathology, imaging diagnosis, radionuclide therapy, targeted therapy and immunotherapy, which have improved the diagnosis rate and efficacy for GI-NEN. This paper reviews recent literatures of GI-NEN, summarizes its epidemiology, pathological diagnosis, image diagnosis, surgery, targeted therapy, radionuclide therapy, chemotherapy, immunotherapy, and separates GI-NEN into metastatic and non-metastatic groups for detailed review to provide a reference for guiding clinical diagnosis and treatment as well as exploring the new treatment regimens of GI-NEN.

68Ga-DOTATATE PET/CT versus 111In-octreotide scintigraphy in patients with neuroendocrine tumors: a prospective study / PET/CT com 68Ga-DOTATATE versus cintilografia com 111In-octreotide em pacientes com tumores neuroendócrinos: estudo prospectivo

Abstract Objective: To compare 68Ga-DOTA-DPhe1,Tyr3-octreotate (68Ga-DOTATATE) positron-emission tomography/computed tomography (PET/CT) findings with those of conventional 111In-octreotide scintigraphy in patients with neuroendocrine tumors (NETs). Materials and Methods: This was a single-center prospective study including 41 patients (25 males; mean age, 55.4 years) with biopsy-proven NETs who underwent whole-body 111In-octreotide scintigraphy and whole-body 68Ga-DOTATATE PET/CT. The patients had been referred for tumor staging (34.1%), tumor restaging (61.0%), or response evaluation (4.9%). Images were compared in a patient-by-patient analysis to identify additional lesions, and we attempted to determine the impact that discordant findings had on treatment planning. Results: Compared with 111In-octreotide scintigraphy, 68Ga-DOTATATE PET/CT revealed more lesions, the additional lesions typically being in the liver or bowel. Changes in management owing to the additional information provided by 68Ga-DOTATATE PET/CT occurred in five patients (12.2%), including intermodal changes in three (7.3%) and intramodal changes in two (4.9%). In addition, 68Ga-DOTATATE PET/CT yielded incidental findings unrelated to the primary NET in three patients (7.3%): Hürthle cell carcinoma of the thyroid, bowel non-Hodgkin lymphoma, and a suspicious breast lesion. Conclusion: We conclude that 68Ga-DOTATATE PET/CT is superior to conventional 111In-octreotide scintigraphy for the management of NETs because of its ability to determine the extent of the disease more accurately, which, in some cases, translates to changes in the treatment plan.

RESUMO Objetivo: Comparar os achados da PET/CT com 68Ga-DOTATATE em relação aos da cintilografia com 111In-octreotide em pacientes com tumores neuroendócrinos (TNEs). Materiais e Métodos: Estudo prospectivo unicêntrico incluindo 41 pacientes (25 homens; média de idade: 55,4 anos) com TNEs comprovados por biópsia submetidos a cintilografia de corpo inteiro com 111In-octreotide e PET/CT com 68Ga-DOTATATE. Os pacientes incluídos foram encaminhados para estadiamento do tumor (34,1%), reestadiamento (61,0%) ou avaliação da resposta (4,9%). As imagens foram comparadas para identificar lesões adicionais e o impacto dos achados discordantes no planejamento terapêutico. Resultados: Na comparação com a cintilografia com 111In-octreotide, a PET/CT com 68Ga-DOTATATE revelou mais lesões, mais frequentemente localizadas no fígado e intestino. Mudanças no tratamento devidas às informações adicionais reveladas pela PET/ CT ocorreram em 5/41 pacientes (12,2%), incluindo mudanças intermodalidade em três casos (7,3%) e intramodalidade em dois casos (4,9%). A PET/CT também identificou achados incidentais não relacionados ao TNE em 3/41 pacientes (7,3%), incluindo um carcinoma de células de Hürthle da tireoide, um linfoma não Hodgkin de intestino e uma lesão mamária suspeita. Conclusão: A PET/CT com 68Ga-DOTATATE é superior à cintilografia convencional com 111In-octreotide para o manejo de pacientes com TNEs, em virtude da sua capacidade de detectar a extensão da doença com mais precisão, o que se traduz, em alguns casos, em alterações terapêuticas.

Crisis carcinoide como una causa poco frecuente de choque distributivo / Carcinoid crisis as a rare cause of distributive shock

Los tumores neuroendocrinos son neoplasias que suelen tener un comportamiento clínico maligno, son provenientes de células entero cromafines y/o células productoras de gastrina. Según su origen anatómico, se clasifican en tumores del intestino anterior (comprometen estómago, duodeno, pulmones y páncreas), intestino medio (parte distal del duodeno) e intestino posterior (colon transverso hasta el recto). El síndrome carcinoide, presente en la mitad de los casos al momento del diagnóstico, se caracteriza por desencadenar episodios de diarrea, taquicardia, hipotensión, rubor (por el desarrollo de telangiectasias), y según la gravedad, valvulopatías cardiacas. Por otro lado, la crisis carcinoide, una complicación infrecuente, está relacionada con episodios de choque, que cuando ocurren son consecuencia de la liberación en la circulación sistémica de aminas vasoactivas, posterior a un evento desencadenante. A continuación, se presenta el caso de un paciente masculino con antecedente de un tumor neuroendocrino de intestino delgado, quien luego de ser llevado a embolización de metástasis hepáticas, presentó una crisis carcinoide, y finalmente un choque distributivo refractario a tratamientos convencionales, incluyendo octreotide y vasopresores, que culminó con la muerte del paciente. Se realiza la discusión del caso clínico y la presentación de la literatura disponible, donde se describe la epidemiología, patogénesis, diagnóstico, clínica y tratamiento de esta entidad

Neuroendocrine tumors are neoplasms that usually have a malignant clinical behavior. They arise from enterochromaffin-like and/or gastrin-producing cells. According to their anatomical location they can be classified as foregut tumors (af- fecting stomach, duodenum, lungs and pancreas), midgut tumors (affecting distal portion of the duodenum) and hindgut tumors (affecting transverse colon to rectum). Carcinoid syndrome, occurring in half of the cases at the time of diagnosis, is characterized by episodes of diarrhea, tachycardia, hypotension, flushing (due to telangiectasia), and heart valve disease depending on their severity. On the other hand, the carcinoid crisis, a rare complication that is related to episodes of shock, occur as a consequence of the release of vasoactive amines into the systemic circulation after a triggering event. Here we describe a case of a male patient with a history of neuroendocrine neoplasm, who after embolization of hepatic metastatic lesions presented a carcinoid crisis, ending with a vasodilatory shock, refractory to conventional treatment including octreotide and vasopressors, which resulted in the death of the patient. A discussion of the clinical report and a review of the available literature are presented, including the epidemiology, pathogenesis, diagnosis, clinical manifestations and treatment of this entity

Clinical diagnosis and treatment progress of duodenal neuroendocrine neoplasms / 肿瘤研究与临床

Duodenal neuroendocrine neoplasms (D-NEN) is a kind of heterogeneous tumor originated from neuroendocrine cells. With the continuous progress of diagnostic technology, more and more examination methods and treatment methods are applied. This article reviews the characteristics, classification, imaging applications, biochemical markers' monitoring and treatment of D-NEN.

Clinical analysis of neuroendocrine carcinoma of the breast / 中华普通外科杂志

Objective:To investigate the clinicopathological features, treatment and prognosis of neuroendocrine carcinoma of the breast.Methods:Clinical data of 26 patients with neuroendocrine carcinoma of the breast admitted to the Northern Jiangsu People's Hospital from July 2013 to Mar 2021 were analyzed.Results:All 26 cases were female, the average aged of (62.81±11.95) years, the first clinical manifestations were painless breast masses, the average size being of (23.34±9.47) mm. At the time of diagnosis, regional lymph node metastasis was found in 4 cases, 1 case developed distant metastasis. Most patients' were on stage Ⅱ by TNM staging, molecular typing was Luminal A, and invasive mammary carcinoma with neuroendocrine differentiation was most common, with positive rates of ER and PR of 96%, the positive rate of CgA and Syn were 69% and 100%, and there was not positive expression of HER2. All patients received surgical treatment, 25 patients underwent postoperative adjuvant therapy. Twenty-five patients were followed up for a median follow-up time of 39.50 months. During the follow-up, 3 cases developed distant metastasis, 1 case died, the mean survival time was (40.81±26.90) months, there was ao satistically significant difference compared with invasive mammary carcinoma ( t=1.291, P=0.209). The mean disease free interval is (39.96±27.58) months. The overall survival and disease free survival at 1, 2 and 5 years are 100%, 100% and 87%, respectively. Conclusions:Neuroendocrine carcinoma of the breast occurs more frequently in elderly women, often with large tumor size, low rate of regional lymph node and distant metastasis, moderate histological grade, early clinical stage, and the molecular typing is mostly Luminal A.The overall prognosis is fair.

Neoplasias neuroendocrinas del intestino: serie de casos / Intestinal neuroendocrine tumors: case series

RESUMEN: Los tumores neuroendocrinos (TNE) intestinales representan el mayor porcentaje de este tipo de lesiones a nivel del aparato digestivo. El tratamiento de elección es la extirpación de la lesión primaria y sus linfonodos regionales. El objetivo de este estudio es reportar el resultado de pacientes portadores de TNE intestinales, tratados quirúrgicamente, en términos de morbilidad postoperatoria (MPO) y mortalidad. Serie de casos de pacientes con TNE intestinales intervenidos de forma consecutiva en Clínica RedSalud Mayor Temuco, entre 2006 y 2020. Las variables resultado fueron MPO y mortalidad. Otras variables de interés fueron localización y diámetro del tumor, tipo de cirugía y estancia hospitalaria. Se utilizó estadística descriptiva. Se trató a 11 pacientes (54,5 % mujeres), con una mediana de edad de 56 años. El 54,5 % de los tumores se localizó en yeyuno-íleon. La mediana del diámetro tumoral, tiempo quirúrgico y estancia hospitalaria fueron 2 cm, 75 min y 4 días, respectivamente. El tipo de resección más frecuente fue hemicolectomía derecha (63,6 %). La MPO fue 9,1 % (seroma en un paciente). No hubo reintervenciones ni mortalidad operatoria. Con una mediana de seguimiento de 18 meses, no se verificaron recurrencias. Los resultados reportados en relación a MPO y mortalidad, son adecuados en relación con la evidencia publicada.

SUMMARY: Intestinal neuroendocrine tumors (INETs) represent the highest percentage of this type of lesion in the digestive system. The treatment of choice is removal of the primary lesion and its regional lymph nodes. The aim of this study is to report the results of patients with INETs treated surgically, in terms of postoperative morbidity (POM) and mortality. Series of cases of patients with intestinal INETs operated consecutively at Clínica RedSalud Mayor Temuco, between 2006 and 2020. Result variables were POM and mortality. Other variables of interest were location and diameter of the tumor, type of surgery, and hospital stay. Descriptive statistics were used. Eleven patients (54.5 %) were treated, with a median age of 56 years. 54.5 % of the tumors were located in the jejunum-ileum. The median tumor diameter, surgical time, and hospital stay were 2 cm, 75 min, and 4 days, respectively. The most frequent type of resection was right hemicolectomy (63.6 %). The MPO was 9.1 % (seroma in one patient). There were no reoperations or operative mortality. With a median follow-up of 18 months, there were no recurrences. Reported results in relation to POM and mortality are adequate in relation to the published evidence.

Tumor neuroendocrino en recto simulando una neoplasia benigna: reporte de caso y revisión de a literatura / Neuroendocrine tumor in the rectum simulating a benign neoplasm: case report and literature review

Los tumores neuroendocrinos se definen como un grupo heterogéneo de neoplasias de origen epitelial, provenientes de células enterocromafines diseminadas por todo el organismo, y representan alrededor del 1 al 4 % de todas las neoplasias. Su mayor distribución se encuentra en el tracto gastrointestinal, donde se localiza el 75 % de los tumores neuroendocrinos, siendo los ubicados en el recto, el 27 % de todos los que afectan el tracto gastrointestinal. A propósito de esta revisión de tema, presentamos el caso de un paciente de 71 años de edad, que consultó por sangrado rectal rojo rutilante, sin otra sintomatología asociada, y se le diagnosticó un tumor neuroendocrino grado 1, que se comportaba como una lesión benigna del recto

Neuroendocrine tumors are defined as a heterogeneous group of neoplasms of epithelial origin from enterochromaffin cells disseminated throughout the body, and represent about 1% to 4% of all neoplasms. Its largest distribution is found in the gastrointestinal tract, where 75% of neuroendocrine tumors are located, being 27% of those in the rectum. We present the case of a 71-year-old patient who consulted for bright red blood per rectum, with no other associated symptoms, and was diagnosed with a grade 1 neuroendocrine tumor, which behaved as a benign lesion of the rectum

Comparative study of 68Ga-DOTATATE and 18F-FDG PET/CT imaging in the detection of bone metastasis in neuroendocrine neoplasm / 中华核医学与分子影像杂志

Objective:To compare the value of 68Ga-1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid- D-Phe1-Tyr3-Thr8-octreotide (DOTATATE) and 18F-fluorodeoxyglucose (FDG) PET/CT imaging in the detection of bone metastasis in neuroendocrine neoplasm (NEN). Methods:From January 2014 to July 2019, 29 NEN patients (19 males, 10 females, age: 35-76 years) with bone metastasis who underwent 68Ga-DOTATATE and 18F-FDG PET/CT imaging within one month in Peking University Cancer Hospital & Institute were retrospectively enrolled. Patients were divided into Ki-67≤20% and Ki-67>20% groups according to the tumor proliferation activity, and osteolysis, osteogenesis and no change groups according to the CT findings of bone metastases. The differences of the number and radioactive uptake (maximum standardized uptake value (SUV max) ratio of bone lesion to normal bone (SUV T/B)) of detected bone metastases between 68Ga-DOTATATE and 18F-FDG PET/CT imaging were analyzed. χ2 and Mann-Whitney U tests were used to analyze the data. Results:The sensitivity of 68Ga-DOTATATE and 18F-FDG PET/CT imaging were 75.9%(22/29) and 82.8% (24/29) respectively, and there was no significant difference between the two modalities ( χ2=0.42, P>0.05). The numbers of cases with bone lesions detected by 68Ga-DOTATATE PET/CT imaging in pelvis, spine, ribs, proximal limbs, sternoclavicular scapula and skull were all higher than those of 18F-FDG PET/CT imaging (23, 22, 20, 14, 14, 10 vs 12, 19, 13, 11, 10, 6, respectively). The 68Ga-DOTATATE PET/CT imaging was significantly superior to 18F-FDG imaging in detecting bone metastases (9(3, 36) and 3(0, 18)) and SUV T/B(11.10(3.35, 22.30) and 1.60(1.05, 2.70); U values: 281.000, 77.000, both P<0.001). 68Ga-DOTATATE PET/CT imaging found more bone lesions in well differentiated NEN (Ki-67≤20%) group (11(2, 38) and 2(0, 13)) and osteogenic bone metastasis group (31(3, 100) and 3(0, 31); U values: 105.500, 69.500, both P<0.05). SUV T/B of 68Ga-DOTATATE PET/CT imaging was significantly higher than 18F-FDG PET/CT imaging in all subgroups ( U values: 3.000-22.000, all P<0.05). Conclusion:The 68Ga-DOTATATE PET/CT imaging is superior to 18F-FDG PET/CT imaging in the detection of bone metastasis in NEN.

Sincronismo colorrectal: adenocarcinoma de colon ascendente y carcinoma neuroendocrino de células pequeñas de recto / Colorectal synchronism: ascending colon adenocarcinoma and small-cell neuroendocrine carcinoma of the rectum

RESUMEN El cáncer colorrectal (CCR) es el tercer cáncer más frecuente en el mundo y la segunda causa de muerte de origen neoplásico. El sincronismo en CCR es de aproximadamente 3-6%. El tracto gastrointestinal es el lugar más frecuente de asiento de neoplasias neuroendocrinas (NNE), y de estos los carcinomas neuroendocrinos (CNE) son poco frecuentes. El tratamiento es un desafío, debido a la agresividad de la neoplasia y la falta de protocolos bien establecidos. Todo esto conlleva a la necesidad de un enfoque multidisciplinario, sin embargo, en la mayoría de casos el pronóstico resulta no ser el mejor. Presentamos el caso de un varón de 83 años que acude a emergencia con historia de 3 meses de diarrea, dolor perianal, pérdida ponderal y hematoquezia. El estudio endoscópico evidenció dos neoplasias y el estudio histológico confirmó la presencia de CNE de células pequeñas de recto y adenocarcinoma de colon ascendente, recibió quimioterapia sistémica sin embargo evolucionó desfavorablemente falleciendo a las 3 semanas.

ABSTRACT Colorectal cancer (CRC) is the third most frequent cancer in the world and the second cause of death of neoplastic origin. Synchronism in CCR is approximately 3-6%. The gastrointestinal tract is the most frequent place where neuroendocrine neoplasms (NNE) settle and a special type of these neuroendocrine carcinomas (CNE) are rare. Treatment is challenging, due to the aggressiveness of the malignancy and the lack of well-established protocols. Therefore a multidisciplinary approach is needed, however, in most cases the result is not the best. We present the case of an 83-year-old man who has an emergency with a 3-month history of diarrhea, perianal pain, weight loss, and hematochezia. The endoscopic study shows evidence of two malignancies and the histological study confirms the presence of CNE in small cells at the rectum and adenocarcinoma in the ascending colon. He underwent systematic chemotherapy, however, he evolved unfavorably, dying after 3 weeks.

Hepatic neuroendocrine neoplasm: imaging patterns / Neoplasia neuroendócrina hepática: padrões de imagem

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors with distinct morphological and biological manifestations, the liver being the main organ affected by its metastases. However, primary hepatic involvement is quite rare. Hepatic NENs can have a variety of radiological presentation forms and can therefore mimic other lesions, making their diagnosis challenging. Nonetheless, certain imaging aspects allow NENs to be included among the main differential diagnoses of hepatic lesions and can guide the search for an extrahepatic primary site when the probable diagnosis is metastases.

As neoplasias neuroendócrinas (NNEs) são um grupo heterogêneo de tumores com manifestações morfológicas e biológicas distintas, sendo o fígado o principal órgão acometido por suas metástases. Por outro lado, o acometimento primário deste órgão é muito raro. Podem-se observar as mais diversas formas de apresentação radiológica das NNEs hepáticas primária e secundária e, em razão dessa variedade e por mimetizar outras lesões, o seu diagnóstico pode se revelar um desafio. No entanto, alguns desses aspectos permitem incluir as NNEs entre os principais diagnósticos diferenciais de lesões hepáticas e, nos casos de metástases, orientar na busca de um eventual sítio primário extra-hepático.

The role of conventional echoendoscopy (eus) in therapeutic decisions in patients with neuroendocrine gastrointestinal tumors / O papel da ecoendoscopia convencional nas decisões terapêuticas em pacientes com tumores neuroendócrinos gastrointestinais

ABSTRACT Background: Gastrointestinal neuroendocrine tumors are rare, usually presented as subepithelial or polypoid tumors. Accurate diagnosis and indication of the type of resection are still challenging. Aim: To determine the effectiveness of echoendoscopy in determining the depth of the lesions (T) identified by endoscopy in order to evaluate surgical and/or endoscopic indication, and to evaluate the results of endoscopic removal in the medium term. Methods: Twenty-seven patients were included, all of whom underwent echoendoscopy for TN tumor staging and the evaluation of possible endoscopic resection. The parameters were: lesion size, origin layer, depth of involvement and identified perilesional adenopathies. The inclusion criteria for endoscopic resection were: 1) high surgical risk; 2) those with NET <2 cm; 3) absence of impairment of the muscle itself; and 4) absence of perilesional adenopathies in echoendoscopy and in others without distant metastases. Exclusion criteria were TNE> 2 cm; those with infiltration of the muscle itself; with perilesional adenopathies and distant metastases. The techniques used were: resection with polypectomy loop; mucosectomy with saline injection; and mucosectomy after ligation with an elastic band. The anatomopathological study of the specimens included evaluation of the margins and immunohistochemistry (chromogranin, synaptophysin and Ki 67) to characterize the tumor. Follow-up was done at 1, 6 and 12 months. Results: Resections with polypectomy loop were performed in 15 patients; mucosectomy in five; mucosectomy and ligation with elastic band in three and the remaining four were referred for surgery. The anatomopathological specimens and immunohistochemical analyzes showed positive chromogranin and synaptophysin, while Ki 67 was less than 5% among all cases. The medium-term follow-up revealed three recurrences. The average size of tumors in the stomach was 7.6 mm and in the duodenum 7.2 mm. Well-demarcated, hypoechoic, homogeneous lesions occurred in 75%; mucous layer in 80%; and the deep and submucosal mucosa in 70%. Conclusions: Echoendoscopy proved to be a good method for the study of subepithelial lesions, being able to identify the layer affected by the neoplasm, degree of invasion, echogenicity, heterogeneity, size of the lesion and perilesional lymph node involvement and better indicate the treatment option.

RESUMO Racional: Tumores neuroendócrinos gastrointestinais são raros geralmente apresentados como tumores subepiteliais ou polipoides. O diagnóstico preciso e a indicação do tipo de ressecção ainda são desafiadores. Objetivo: Determinar a eficácia da ecoendoscopia em determinar a profundidade das lesões (T) identificadas pela endoscopia com objetivo de avaliar indicação cirúrgica e/ou endoscópica, e avaliar os resultados da remoção endoscópica em seguimento em médio prazo. Métodos: Foram incluídos 27 pacientes todos submetidos à ecoendoscopia para estadiamento tumoral TN e à avaliação de possível ressecção endoscópica. Os parâmetros estudados foram: tamanho da lesão, camada de origem, profundidade do acometimento e adenopatias perilesionais identificadas. Os critérios de inclusão para ressecção endoscópica foram: 1) risco cirúrgico elevado; 2) aqueles com TNE <2 cm; 3) ausência de comprometimento da muscular própria; e 4) ausência de adenopatias perilesionais na ecoendoscopia e em outros sem metástases à distância. Os critérios de exclusão foram TNE >2 cm; os com infiltração da muscular própria; com adenopatias perilesionais e metástases à distância. As técnicas utilizadas foram: ressecção com alça de polipectomia; mucosectomia com injeção de solução salina; e mucosectomia após a ligadura com banda elástica. O estudo anatomopatológico dos espécimes incluiu avaliação das margens e imunoistoquímica (cromogranina, sinaptofisina e Ki 67) para caracterizar o tumor. O seguimento foi feito com 1, 6 e 12 meses. Resultados: Ressecções com alça de polipectomia foram realizadas em 15 pacientes; mucosectomia em cinco; mucosectomia e ligadura com banda elástica em três e os quatro restantes foram encaminhados para cirurgia. O anatomopatológico dos espécimes e as análises imunoistoquímicas mostraram cromogranina e sinaptofisina positivas, enquanto que o Ki 67 foi menor que 5% dentre todos os casos. O seguimento em médio prazo revelou três recidivas. A média de tamanho dos tumores no estômago foi de 7,6 mm e no duodeno 7,2 mm. As lesões bem demarcadas, hipoecóicas, homogêneas ocorreram em 75%; da camada mucosa em 80%; e da mucosa profunda e submucosa em 70%. Conclusões: A ecoendoscopia mostrou ser bom método para o estudo de lesões subepiteliais podendo identificar a camada acometida pela neoplasia, grau de invasão, ecogeneicidade, heterogeneidade, tamanho da lesão e acometimento linfonodal perilesional e melhor indicar a opção de tratamento.

Clinicopathological features, diagnosis and treatment of primary and metastatic hepatic neuroendocrine neoplasm originating from digestive tract / 中华消化杂志

Objective@#To explore the clinicopathological characteristics of primary hepatic neuroendocrine neoplasm (HNEN) and metastatic HNEN from digestive tract, to screen the risk factors of hepatic metastasis of gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) and to analyze the differences between primary and metastatic HNEN in clinical features, diagnosis, treatment and prognosis.@*Methods@#From January 2010 to June 2017, the clinical data of 182 patients with HNEN admitted at The First Affiliated Hospital of Zhengzhou University were retrospectively analyzed, including 39 cases of primary HNEN, 129 cases of metastatic HNEN and 14 cases of HNEN with unknown primary lesions. Chi-square test and t test were performed to analyze the pathologic characteristics among groups. Logistic regression method was used to analyze the risk factors of hepatic metastasis. Kaplan-Meier method and log-rank test were used for survival analysis. Cox model was used for the prognostic multivariate survival analysis.@*Results@#Metastatic HNEN from digestive tract was more common in male (70.5%, 91/129). The case number of serological tumor biomarkers neuronspecific enolase and alpha-fetoprotein positive in primary HNEN were two cases and one case, respectively, and the positive rates in metastatic HNEN were 37.2% (32/86) and 6.4% (7/110). Most primary HNEN was single lesion (61.5%, 24/39), while multiple lesions were more common in metastatic HNEN (78.3%, 90/115). Primary HNEN mainly occurred in the right lobe of the liver (44.7%, 17/38), while metastatic HNEN located simultaneously in the left and right lobes of the liver (68.4%, 78/114). There were significant differences between primary HNEN and metastatic HNEN in tumor number, pathological grading, location of tumors and maximum diameter of tumors (χ2=21.264, 11.696, 19.461 and 4.547, all P<0.05). The median survival time of patients with primary HNEN and metastatic HNEN were 17.0 months and 10.0 months, and there was a significant difference in survival curves between the two groups (χ2=7.235, P=0.007). The type of hepatic tumors (primary or metastatic)(P=0.002), pathological grading of hepatic tumors (P=0.044), lymph node metastasis (P=0.024), the growth pattern of tumors (P<0.01) and treatment methods (P=0.018) were the independent factors for the prognosis of patients.@*Conclusions@#There are significant differences between primary HNEN and metastatic HNEN in tumor number, size and location. The type of hepatic tumors, pathological grading, lymph node metastasis, growth pattern of tumors and treatment methods are the independent factors for the prognosis of patients. Early topical treatment and combination treatment can help to prolong survival time of HNEN patients.

Gastric mixed adenoneuroendocrine carcinoma: a clinicopathological analysis of 36 cases / 中华病理学杂志

Objective@#To investigate the clinicopathological characteristics of gastric mixed adenoneuroendocrine carcinoma (MANEC).@*Methods@#The clinical and pathologic data of 36 cases of gastric MANEC collected from January 2011 to December 2018 in the Department of Pathology, Fujian Provincial Hospital were retrospectively analyzed. Light microscopy and EnVision immunohistochemical (IHC) staining were used in the study. The results were compared with 40 cases of gastric neuroendocrine carcinoma collected within the same period.@*Results@#The 36 cases were collected, there were 29 males and 7 females. The patients′ age ranged from 43 to 87 years (mean 66 years). The tumor diameter ranged from 1.0 to 9.0 cm (mean 5.0 cm). Clinical staging showed that four cases were at T1+T2 stages and 32 cases were at T3+T4 stages. The main clinical manifestations were upper abdominal pain, abdominal distension and dysphagia. Complete follow-up data were obtained in 20 (eleven living and nine dead) patients. Pathologic analysis showed that all tumors were composed of neuroendocrine carcinoma and adenocarcinoma and each of the components was more than 30%. IHC staining showed that CK was expressed in adenocarcinoma; whereas the neuroendocrine carcinoma mainly expressed CD56 and Syn. There was no significant difference in age, sex, T stage and prognosis between gastric MANEC and gastric neuroendocrine carcinoma(P>0.05). Gastric neuroendocrine carcinoma predominantly occurred in the esophagogastric junction and the gastric body but only rarely in the gastric antrum; gastric MANEC mainly occurred in the esophagogastric junction and the gastric antrum but rarely in the gastric body. Gastric MANEC was more prone to lymph node metastasis than gastric neuroendocrine cancer(P<0.05).@*Conclusions@#Most of the gastric MANEC patients are middle-aged and elderly males, and the tumors predominantly occur in the esophagogastric junction and the gastric antrum. Most of the patients are found to have higher stages, and most of them have lymph node metastases and poor prognosis.

Clinicopathological features, diagnosis and treatment of primary and metastatic hepatic neuroendocrine neoplasm originating from digestive tract / 中华消化杂志

Objective To explore the clinicopathological characteristics of primary hepatic neuroendocrine neoplasm (HNEN) and metastatic HNEN from digestive tract ,to screen the risk factors of hepatic metastasis of gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) and to analyze the differences between primary and metastatic HNEN in clinical features , diagnosis, treatment and prognosis.Methods From January 2010 to June 2017, the clinical data of 182 patients with HNEN admitted at The First Affiliated Hospital of Zhengzhou University were retrospectively analyzed , including 39 cases of primary HNEN, 129 cases of metastatic HNEN and 14 cases of HNEN with unknown primary lesions .Chi-square test and t test were performed to analyze the pathologic characteristics among groups .Logistic regression method was used to analyze the risk factors of hepatic metastasis .Kaplan-Meier method and log-rank test were used for survival analysis . Cox model was used for the prognostic multivariate survival analysis .Results Metastatic HNEN from digestive tract was more common in male ( 70.5%, 91/129 ).The case number of serological tumor biomarkers neuronspecific enolase and alpha-fetoprotein positive in primary HNEN were two cases and one case , respectively, and the positive rates in metastatic HNEN were 37.2%( 32/86) and 6.4%(7/110).Most primary HNEN was single lesion (61.5%, 24/39), while multiple lesions were more common in metastatic HNEN (78.3%, 90/115).Primary HNEN mainly occurred in the right lobe of the liver (44.7%, 17/38), while metastatic HNEN located simultaneously in the left and right lobes of the liver (68.4%, 78/114).There were significant differences between primary HNEN and metastatic HNEN in tumor number , pathological grading, location of tumors and maximum diameter of tumors ( χ2 ＝21.264, 11.696, 19.461 and 4.547, all P?0.05).The median survival time of patients with primary HNEN and metastatic HNEN were 17.0 months and 10.0 months, and there was a significant difference in survival curves between the two groups (χ2 ＝7.235, P＝0.007).The type of hepatic tumors (primary or metastatic) ( P＝0.002), pathological grading of hepatic tumors (P＝0.044), lymph node metastasis ( P ＝0.024), the growth pattern of tumors ( P ?0.01) and treatment methods (P＝0.018) were the independent factors for the prognosis of patients .Conclusions There are significant differences between primary HNEN and metastatic HNEN in tumor number , size and location. The type of hepatic tumors, pathological grading , lymph node metastasis, growth pattern of tumors and treatment methods are the independent factors for the prognosis of patients .Early topical treatment and combination treatment can help to prolong survival time of HNEN patients .

Stage and histology of cervical cancer in women under 25 years old / 부인종양

OBJECTIVE: To evaluate the histological and stage characteristics of cervical cancer in women under 25 years old, and to compare them with older women. METHODS: Cross-sectional study of cases from the Hospital Cancer Registry of São Paulo State/Brazil from 2000 to 2015. Variables were age, International Federation of Gynecology and Obstetrics stage and histological type. Prevalence ratio (PR) and its 95% confidence interval (CI) were calculated. RESULTS: Out of 18,423 cervical cancer cases 204 (1.1%) were in women under 25 years old. The most frequent stage was stage I in women under 25 (36.2%) and between 25 and 34 (43.4%), and stage III in older women (31.8%). No statistically significant difference was observed in stages by age group. Squamous carcinomas were the most frequent in 73.5% of women under 25 and 78.5% of older women. In women under 25 the following histological types were more frequent: neuroendocrine carcinomas (PR=6.10, 95% CI=2.03–18.35), malignant germ cell tumors (PR=54.98, 95% CI=26.53–113.95), mesenchymal tumors (sarcomas) (PR=5.67, 95% CI=2.58–12.45) and hematopoietic/lymphoid tumors (PR=0.72, 95% CI=2.90–36.69). CONCLUSION: In women under 25 years old cervical cancer was an uncommon diagnosis and in about one third occurred at early stage. Squamous carcinoma was the most frequent histological type regardless age, but rare histological types were more frequent in young women.

Clinical and pathological characteristics of 107 esophageal neuroendocrine carcinoma / 中华消化杂志

Objective To investigate the clinical and pathological features of patients with esophageal neuroendocrine carcinoma (ENEC).Methods From January 2011 to November 2018,107 patients with pathologically confirmed ENEC were enrolled at the First Affiliated Hospital of Zhengzhou University.The clinical manifestation,tumor location,tumor size,clinical pathological classification and immunohistochemical markers were analyzed.Statistical description was used for measurement data analysis,and chi-square test was performed for classification data analysis.Results Among 107 patients with ENEC,feeling obstruction during eating was the most common initial symptom,accounting for 63.6％ (68/107);followed by chest and back pain,accounting for 13.1％ (14/107).About 60.7％ (65/107) patients were diagnosed by biopsy under endoscopy and 39.3％ (42/107) patients were confirmed by pathological diagnosis after surgery.The proportion of tumor located in the upper thoracic esophagus and middle and lower thoracic segments was 13.1％ (14/107),45.8％ (49/107) and 41.1％ (44/107),respectively.The length of tumor was 0.7 cm to 9.0 cm,and the median was 2.5 cm.Among them,57.0％ (61/107) were less than 2.5 cm and 43.0％ (46/107) were over 2.5 cm.Among 107 patients,50 (46.7％) patients were ulcerative type,32 (29.9％) patients were medullary type,16 (15.0％) patients were mushroom type and nine (8.4％) patients were protrude type.Among 107 patients,96 (89.7％) patients were pure neuroendocrine carcinoma (P-NEC;including 95 small cell types,one large cell type);11 (10.3％) patients were mixed neuroendocrine carcinoma (M-NEC;including nine small cell carcinoma mixed with squamous cell carcinoma,two small cell carcinoma mixed with adenocarcinoma).The positive rates of synaptophysin,CD56 and chromogranin A were 99.0％ (104/105),98.0％ (100/102) and 31.5％ (17/54),respectively.Ki-67 proliferation index of 47.7％ tumors (51/107) was between 90％ and 100％.P-NEC with the maximum diameter over 2.5 cm accounting for 42.1％ (45/107),and M-NEC accounting for 0.9％ (1/107).The maximum diameter of P-NEC group was larger than that of M-NEC group,and the difference was statistically significant (x2 =4.311,P =0.038).Conclusions ENEC is a kind of highly aggressive malignant tumor with nonspecific manifestations.The diagnosis mainly depends on histopathology and immunohistochemistry.

Primary endometrial large cell neuroendocrine carcinoma with melanocytic differentiation

High-grade endometrial carcinomas are aggressive neoplasms of difficult histological classification. Neuroendocrine differentiation in endometrial carcinomas is rare. This is the report of an endometrial large cell neuroendocrine carcinoma with foci of melanocytic differentiation in a 75-year-old woman with abnormal post-menopausal uterine bleeding for 2 years. Two initial biopsies were inconclusive. Histopathological examination of the uterus revealed large cell neuroendocrine carcinoma associated with endometrioid carcinoma and foci of melanocytic differentiation, pT3a (FIGO IIIA). There were metastases in the rectum serosa and lungs. After 8 months of diagnosis and surgical treatment, the patient is on chemotherapy and radiotherapy. We highlight the morphological characteristics and criteria that allow the definitive anatomopathological diagnosis, including immunohistochemical markers used to identify the cell types present in this unprecedented association.

Carcinoma neuroendocrino de esófago: reporte de caso / Neoendocrine carcinoma of the esophagus: report of a case

El carcinoma neuroendocrino de esófago representa menos del 2% de casos de cáncer de esófago. Esta forma de presentación es extremadamente inusual, ya que este tipo de carcinoma se suele presentar con mayor frecuencia en zonas broncopulmonares. Los síntomas suelen asociarse a la obstrucción esofágica e incluyen odinofagia, disfagia y pérdida de peso, siendo infrecuente la clínica de síndrome carcinoide. Para el diagnóstico se requiere de uno o más de los siguientes marcadores de inmunohistoquímica positivos: cromogranina A, sinaptofisina o CD56 positivos, así como la presencia de los anticuerpos EMA y PANCK positivos. Se reporta el caso de un paciente varón con diagnóstico de tumor neuroendocrino de esófago.

Neuroendocrine carcinoma of esophagus represents less than 2% of cases of cancer of the esophagus. This presentation is extremely unusual, as this type of carcinoma typically presents with greater frequency in bronchopulmonary regions. The symptoms are usually associated with esophageal obstruction and include odynophagia, dysphagia and weight loss and are not usually associated with Carcinoid syndrome. The diagnosis requires one or more of the following positive immunohistochemical markers: Chromogranin A, synaptophysin or CD56 positive, as well as the presence of EMA and PANCK positive. We report the case of a male patient with diagnosis of neuroendocrine tumor of esophagus.