Carcinoma neuroendocrino de la piel: Carcinoma de células de Merkel. Revisión de la Literatura, a propósito de un caso / Neuroendocrine skin carcinoma: Merkel cell carcinoma. Review of literature, about a case

El carcinoma de células de Merkel, también llamado carcinoma neuroendocrino de la piel, es un tipo de cáncer de piel muy poco frecuente que generalmente aparece como un nódulo de color carne o rojo azulado, más frecuentemente en región facial, cabeza y cuello. El carcinoma de células de Merkel se desarrolla principalmente en personas mayores ya que la exposición al sol a largo plazo o un sistema inmunitario débil pueden aumentar el riesgo de desarrollarlo. Las células de Merkel se encuentran en la base de la capa más externa de la piel (epidermis) y están conectadas a las terminaciones nerviosas que son responsables del sentido del tacto. Tiende a crecer rápido y diseminarse a otras partes del cuerpo. Por tanto, las opciones de tratamiento para el carcinoma de células de Merkel dependen de si el cáncer se ha diseminado más allá de la piel

Merkel cell carcinoma, also called neuroendocrine skin of the skin, is a very rare type of skin cancer that generally appears as a bluish meat or red color nodule, more frequently in the facial, head and neck region. Merkel cell carcinoma develops mainly in older people since long -term exposure or a weak immune system can increase the risk of developing it. Merkel cells are at the base of the outermost layer of the skin (epidermis) and are connected to nerve endings that are responsible for the sense of touch. It tends to grow quickly and spread to other parts of the body. Therefore, the treatment options for Merkel cell carcinoma depend on whether cancer has spread beyond the skin

Analysis of clinicopathological characteristics, therapeutic strategy and prognosis of 501 patients with gastric neuroendocrine neoplasms attending a single center / 中华胃肠外科杂志

Objective: To explore the clinicopathological features, treatment strategy and to analysis of prognosis-related risk factors of gastric neuroendocrine neoplasms(G-NEN). Methods: In this study, a retrospective observational study method was used to collect the clinicopathological data of patients diagnosed with G-NEN by pathological examination in the First Medical Center of PLA General Hospital from January 2000 to December 2021. The basic information of the patients, tumor pathological characteristics, and treatment methods were entered, and the treatment information and survival data after discharge were followed up and recorded. The Kaplan-Meier method was used to construct survival curves, and the log-rank test to analyze the differences in survival between groups. Cox Regression model analysis of risk factors affecting the prognosis of G-NEN patients. Results: Among the 501 cases confirmed as G-NEN, 355 were male and 146 were female, and their median age was 59 years. The cohort comprised 130 patients (25.9%) of neuroendocrine tumor (NET) G1, 54 (10.8%) of NET G2, 225 (42.9%) of neuroendocrine carcinoma (NEC), and 102 cases (20.4%) of mixed neuroendocrine-non-neuroendocrine(MiNEN). Patients NET G1 and NET G2 were mainly treated by endoscopic submucosal dissection (ESD) and endoscopic mucosal resection (EMR). The main treatment for patients with NEC/MiNEN was the same as that for gastric malignancies, namely radical gastrectomy+lymph node dissection supplemented with postoperative chemotherapy. There were significant differences in sex, age, maximum tumor diameter, tumor morphology, tumor numbers, tumor location, depth of invasion, lymph node metastasis, distant metastasis, TNM staging and expression of immunohistological markers Syn and CgA among NET, NEC, and MiNEN patients (all P<0.05). Further for NET subgroup analysis, there were significant differences between NET G1 and NET G2 in the maximum tumor diameter, tumor shape and depth of invasion(all P<0.05). 490 patients (490/501, 97.8%) were followed up with a median of 31.2 months. 163 patients had a death during follow-up (NET G1 2, NET G2 1, NEC 114, MiNEN 46). For NET G1, NET G2, NEC and MiNEN patients,the 1-year overall survival rates were 100%, 100%, 80.1% and 86.2%, respectively; the 3-year survival rates were 98.9%, 100%, 43.5% and 55.1%, respectively. The differences were statistically significant (P<0.001). Univariate analysis showed that gender, age, smoking history, alcohol history, tumor pathological grade, tumor morphology, tumor location, tumor size, lymph node metastasis, distant metastasis, and TNM stage were associated with the prognosis of G-NEN patients (all P<0.05). Multivariate analysis showed that age ≥60 years, pathological grade of NEC and MiNEN, distant metastasis, and TNM stage III-IV were independent factors influencing the survival of G-NEN patients (all P<0.05). 63 cases were stage IV at initial diagnosis. 32 of these were treated with surgery and 31 with palliative chemotherapy. Stage IV subgroup analysis showed that the 1-year survival rates were 68.1% and 46.2% in the surgical treatment and palliative chemotherapy groups, respectively, and the 3-year survival rates were 20.9% and 10.3%, respectively; the differences were statistically significant (P=0.016). Conclusions: G-NEN is a heterogeneous group of tumors. Different pathological grades of G-NEN have different clinicopathological features and prognosis. Factors such as age ≥ 60 years old, pathological grade of NEC/MiNEN, distant metastasis, stage III, IV mostly indicate poor prognosis of patients. Therefore, we should improve the ability of early diagnosis and treatment, and pay more attention to patients with advanced age and NEC/MiNEN. Although this study concluded that surgery improves the prognosis of advanced patients more than palliative chemotherapy, the value of surgical treatment for patients with stage IV G-NEN remains controversial.

Asociación de tumorlet pulmonar con segundas neoplasias / Pulmonary tumorlet associated with second neoplasia

Los tumorlets son tumores neuroendocrinos pulmonares menores a 0.5 cm, de evolución benigna y habitualmente asintomáticos. Su diagnóstico es importante para realizar la diferenciación con otras afecciones neuroendocrinas y enfermedad metastásica de otro origen, que requerirán una intervención terapéutica. Se presenta un caso de dicha entidad asociada a otros tumores.

Tumorlets are pulmonary neuroendocrine tumors smaller than 0.5 cm. They are benign and usually asymptomatic. Their diagnosis is important so as to differentiate them from other neuroendocrine pathologies that require therapeutic intervention. We report a case of such entity and a discussion on the subject that can contribute to highlight the importance of diagnosing this entity.

Small cell neuroendocrine carcinoma of the nasopharynx: a rare case report

Small cell neuroendocrine carcinoma rarely appears primarily in the head and neck and exhibits aggressive behavior with a poor prognosis. The pathologist has a significant role in the diagnosis, and a consensual treatment still does not exist. The authors report the case of a middle-aged male patient who presented repeated episodes of massive epistaxis. The diagnostic work-up disclosed the diagnosis of small cell neuroendocrine carcinoma of the nasopharynx. The patient was treated with chemotherapy followed by radiotherapy. Imaging examinations performed after the end of treatment showed apparent complete remission of the disease. The patient was kept under active surveillance with no signs of local relapse or distant metastasis after 4 years of follow-up.

Estudio de carcinoma medular de tiroides a partir de un caso índice / Study of Medullary Thyroid Carcinoma from a proband

El carcinoma de tiroides es un tumor infrecuente; constituye menos del 1% de las neoplasias malignas en la población general y el 0,5%-3% en la edad pediátrica. Existen cuatro tipos: papilar (80%-90% de los casos), folicular (5%-10%), medular (5%) y anaplásico (2%-3%). En el tipo medular, el 80% son esporádicos, y un 20% se asocia a un síndrome hereditario que se divide, fundamentalmente, en tres grupos: neoplasia endócrina múltiple 1, neoplasia endócrina múltiple 2 y carcinoma medular de tiroides familiar. Las formas hereditarias se producen por una mutación en el protooncogén RET, localizado en el brazo largo del cromosoma 10. Se presenta un caso de carcinoma medular de tiroides detectado a raíz de un estudio genético familiar con el propósito de resaltar la importancia del diagnóstico precoz y la intervención de equipos multidisciplinares expertos en esta patología para su manejo y seguimiento.

Thyroid cancer is an uncommon type of cancer, accounting less than 1% of all cancers in adults, and 0.5-3% of all cancers in children. There are four different types: papillary carcinoma (80-90% of cases), follicular (5-10%), medullary (5%) and anaplastic cell (2-3%). Eighty per cent of cases of medullary thyroid cancer are sporadic, but 20% are associated with an inherited syndrome that is divided into three groups: multiple endocrine neoplasia type 1, multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma. The inherited forms are caused by a disruption in the RET oncogene, which is located in the long arm of chromosome 10. A hereditary case of medullary thyroid carcinoma is presented. It was detected because of a familial genetic study. The purpose of the paper is emphasize the importance of the early diagnosis and the intervention of multidisciplinary teams of experts.

Mixed adenoneuroendocrine carcinoma of the gastric stump following Billroth II gastrectomy: case report and review of the literature / Adenocarcinoma neuroendócrino misto do coto gástrico após gastrectomia à Billroth II: relato de caso e revisão de literatura

CONTEXT: Gastric stump cancer after gastric resection is a well-known disease. It may be a newly developed cancer after resection due to benign disease, or recurrent or residual disease after oncological surgery. The predominant histological type is usually adenocarcinoma. This study aimed to report on a rare occurrence of a mixed adenoneuroendocrine carcinoma (MANEC) on the gastric stump. CASE REPORT: The case of an 83-year-old female who presented a locally aggressive gastric stump MANEC, 35 years after Billroth II gastrectomy to treat a peptic ulcer, is reported. The patient underwent resection and adjuvant therapy. She has been followed up for one year without signs of recurrence. CONCLUSION: MANEC is a rare type of gastrointestinal neoplasm. The classification, histopathology, clinical features, treatment issues and prognosis are discussed along with a brief review of the literature.

CONTEXTO: O câncer de coto gástrico após gastrectomia é uma condição extensamente documentada. Pode se tratar de doença desenvolvida após a ressecção por doença benigna, ou ainda doença recorrente ou residual após cirurgia oncológica. Geralmente, o tipo histológico predominante é o adenocarcinoma. Este estudo tem como propósito relatar a rara ocorrência de um adenocarcinoma neuroendócrino misto (MANEC) no coto gástrico. RELATO DE CASO: É relatado o caso de uma mulher de 83 anos que apresentou um MANEC localmente agressivo 35 anos após uma gastrectomia à Billroth II devido a úlcera péptica. Foi submetida a ressecção e terapia adjuvante e foi seguida por 12 meses sem sinais de recorrência. CONCLUSÃO: Os MANECs constituem raro tipo de neoplasia gastrointestinal. Sua classificação, histopatologia, aspectos clínicos, tratamento e prognóstico são discutidos junto com uma breve revisão de literatura.

Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment

Introduction: Malignant sinonasal tumors comprise less than 1% of all neoplasms. A wide variety of tumors occurring primarily in this site can present with an undifferentiated or poorly differentiated morphology. Among them are esthesioneuroblastomas, sinonasal undifferentiated carcinomas, and neuroendocrine carcinomas. Objectives: We will discuss diagnostic strategies, recent advances in immunohistochemistry and molecular diagnosis, and treatment strategies. Data Synthesis: These lesions are diagnostically challenging, and up to 30% of sinonasal malignancies referred to the University of Texas MD Anderson Cancer Center are given a different diagnosis on review of pathology. Correct classification is vital, as these tumors are significantly different in biological behavior and response to treatment. The past decade has witnessed advances in diagnosis and therapeutic modalities leading to improvements in survival. However, the optimal treatment for esthesioneuroblastoma, sinonasal undifferentiated carcinoma, and neuroendocrine carcinoma remain debated. We discuss advances in immunohistochemistry and molecular diagnosis, diagnostic strategies, and treatment selection. Conclusions There are significant differences in prognosis and treatment for esthesioneuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. Recent advances have the potential to improve oncologic outcomes but further investigation in needed...

Tumores endócrinos del páncreas / Endocrine tumors of the pancreas

Multiple endocrine neoplasia (MEN) is a rare familial disorder that affects multiple endocrine organs. it is inherited in an autosomal dominant pattern with variable penetrance. There are two distinct types of MEN. Dominantly inherited neoplasia are believed to occur from the recessive loss of tumor suppressor gene function. The first mutational event affects the germ cell, is hereditary, and predisposes to neoplasia. Because tumors occur in multiple organs in MEN, the second mutational evento probably occurs in common precursor cells, such as the amine precursor uptake and decarboxylation cells (APUD). The gene locus for tupe 1 MEN has been mapped to cromosome 11q. Type 1 organs affected: parathyroids, pancreas and pituitary. The gene locus for type 2 MEN is thought to be located for chromosome 10, (other organs). Fifty to 60 % of patients with type 1 MEN have pancreaticislet cell tumors. Insulinomas are tumors that originate in the beta cells of the islets of Langerhans, which compose the APUD system, the meaning of which is: A=Amino, P=Precursor, U=Uptake, D=Decarboxylation. The cells fo the APUD system have common cytochemical characteristics with the ability to secret polypeptides and amines. Symptoms are related to the peptide secreted by the tumor, and some tumors can produce multiple peptides. Gastrinomas comprise about 60 % of type 1 MEN-associated islet cell tumors. Gastric acid hypersecretion results from excess gstrin secretion and causes multiple gastric and duodenal ulcers (Zollinger-Ellison syndrome). The author describe an experience with this pathology with special reference to diagnostic methods, treatment, follow-up, laboratory studies and localization of the tumor.

Câncer de pulmão: histologia, estádio, tratamento e sobrevida / Lung cancer: histology, staging, treatment and survival

OBJETIVO: Analisar os principais tipos histológicos, estádio, tratamento e sobrevida dos portadores de câncer de pulmão. MÉTODOS: Estudo retrospectivo a partir da análise dos prontuários de pacientes acompanhados no Hospital das Clínicas da Faculdade de Medicina de Botucatu, num período de seis anos. RESULTADOS: De janeiro de 2000 a janeiro de 2006, foram acompanhados 240 doentes com câncer de pulmão, com predominância do sexo masculino (64 por cento). O tipo histológico mais freqüente foi o carcinoma escamoso (37,5 por cento), seguido pelo adenocarcinoma (30 por cento), carcinoma neuroendócrino (19,6 por cento) e carcinoma de grandes células (6,6 por cento). Apenas 131 pacientes (54,6 por cento) foram tratados. Destes, 52 pacientes (39,7 por cento) foram submetidos à quimioterapia exclusiva, 32 (24,4 por cento) realizaram quimioterapia associada à radioterapia e 47 (35,9 por cento) foram submetidos à cirurgia associada ou não à quimioterapia exclusiva e/ou radioterapia. Somente 27 pacientes (20,6 por cento) foram submetidos à cirurgia exclusiva.Em relação ao estadiamento, 34,4 por cento apresentavam, no momento do diagnóstico, estádio IV, 20,6 por cento estádio IIIB, 16,8 por cento estádio IIIA e os outros 28,2 por cento pertenciam aos estádios I e II. A sobrevida em cinco anos foi de 65 por cento para o estádio I e 25 por cento para os estádios remanescentes. CONCLUSÕES: O tipo histológico predominante foi o carcinoma escamoso e o de menor freqüência foi o carcinoma de grandes células. A maioria se encontrava em estádio avançado ao diagnóstico, estando nos estádios iniciais menos de 30 por cento dos casos. Isto justifica a baixa sobrevida e a pequena quantidade de pacientes submetidos ao tratamento cirúrgico exclusivo, em comparação à maioria que foi submetida à quimioterapia exclusiva.

OBJECTIVE: To analyze principal histological types of lung cancer, as well as the staging, treatment and survival of lung cancer patients. METHODS: This was a retrospective study based on the analysis of medical charts of patients treated at the Botucatu School of Medicine Hospital das Clínicas over a six-year period. RESULTS: From January of 2000 to January of 2006, 240 patients with lung cancer, most (64 percent) of whom were male, were treated. The most common histological type was squamous cell carcinoma (37.5 percent), followed by adenocarcinoma (30 percent), neuroendocrine carcinoma (19.6 percent) and large cell carcinoma (6.6 percent). Only 131 patients (54.6 percent) were treated. Of those, 52 patients (39.7 percent) received only chemotherapy, 32 (24.4 percent) were treated with chemotherapy combined with radiotherapy, and 47 (35.9 percent) were submitted to surgery alone or surgery accompanied by chemotherapy, with or without radiotherapy. Only 27 patients (20.6 percent) were submitted to surgery alone. Concerning staging, 34.4 percent presented stage IV at the time of diagnosis, 20.6 percent presented stage IIIB, 16.8 percent presented stage IIIA, and the remaining 28.2 percent were classified as stage I or II. Five-year survival was 65 percent for those in stage I and 25 percent for those in the remaining stages. CONCLUSIONS: Of the various histological types, the most common was squamous cell carcinoma and the least common was large cell carcinoma. Most cases presented advanced stages at the moment of diagnosis, and less than 30 percent of the cases presented early stages. This accounts for the low survival rate and the small number of patients submitted to surgical treatment alone, the majority being submitted to chemotherapy alone.

Tumor carcinóide neuroendócrino da mama: estudo clínico-patológico de seis casos / Neuroendocrine carcinoid tumors of breast: clinicopathologic study of six cases

O tumor carcinóide primário da glândula mamária é uma neoplasia muito rara, de existência controversa. Sua histogênese ainda é discutida, sendo a origem mais reconhecida as células basais primitivas ductais. No estudo retrospectivo de 243 carcinomas infiltrativos e "in situ" de tipo ductal e lobular, seguindo estritos critérios de seleçäo, foram achados seis tumores carcinóides. O material correspondia a mulheres com idade média de 43 anos, que apresentaram-se com massas palpáveis notadamente nos quadrantes externos da glândula mamária direita. Näo foram comprovadas metástases axilares nem síndrome carcinóide. A mamografia mostrou opacidades densas de contornos regulares. A punçäo citológica com agulha fina sugeriu células neoplásicas de tipo lobular em três casos; hiperplasia lobular atípica em um caso e presença de mucina näo específica no resto. No estádio II da doença foi realizada mastectomia de tipo Madden em todos os casos. Macroscopicamente os tumores tinham uma média de 29,8 mm de diâmetro, aspecto sólido em quatro casos e cístico-gelatinoso em dois, e margens bem definidas. O padräo histopatológico mais freqüente foi o basalóide e presença de mucina extracelular compondo 28 porcento do tumor em três casos. Näo foram comprovadas mitoses. Os diagnósticos diferenciais incluíram: o carcinoma lobular, o carcinoma mucinoso e as metástases mamárias de carcinóides de outras topografias. Na evoluçäo pós-operatória näo foi comprovada recorrência local, disseminaçäo a distância ou óbito numa média de 3,8 anos, sendo que em nenhuma das pacientes realizou-se rádio, químio ou hormonioterapia. Baseados nesses dados propöe-se a quadrantectomia com esvaziamento axilar como primeira forma de tratamento, reservando a mastectomia para um segundo momento.