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 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.

Clinical analysis of 11 patients with neuroendocrine carcinoma in maxillofacial region / 中华口腔医学杂志

Objective: To investigate the clinicopathological features, treatment and prognosis of maxillofacial neuroendocrine carcinoma. Methods: A total of 11 patients with maxillofacial neuroendocrine carcinoma diagnosed in the Department of Pathology of The First Affiliated Hospital of Zhengzhou University from December 2010 to July 2022 were retrospectively enrolled, including 8 males and 3 females, aged (65.2±9.5) years (ranged from 49 to 87 years), with a disease course of 0.5 to 6.0 months. The clinicopathological data including head and neck CT, MRI and treatment methods were analyzed. Results: Submandibular gland and maxilla were involved in 3 cases, parapharynx in 2 cases, and face, tongue root and soft palate in 1 case respectively. Clinically, the initial symptom is a rapidly growing painless or tender mass, which may be accompanied by restricted mouth opening, dysphagia, and local numbness after invasion of masticatory muscles and nerves. The tumors were all invasive and low-density, with unclear boundaries from the surrounding tissues. Among the patients, 9 received surgical treatment, and 5 received adjuvant treatment after surgery (2 received chemotherapy, 3 received radiotherapy+chemotherapy). According to the 5th edition of the World Health Organization classification of head and neck tumors in 2022, there were 1 case (1/11) with poorly differentiated large cells and 10 cases (10/11) with poorly differentiated small cells. Histologically, the macrocell type is composed of large cells with rough chromatin, obvious vacuolar nucleolus, protruding nucleolus, and necrosis. The small cell type is dominated by small blue round cells with neuroendocrine characteristics, with active growth and multifocal necrosis. Immunohistochemical staining showed that cytokeratin (CK), epithelial membrane antigen (EMA) and synaptophysin (Syn) were diffusively expressed, 10 cases expressed CD56, 8 cases expressed p63, 6 cases expressed weakly punctated chromograin-A (CgA), and S-100 was not expressed. The Ki-67 index ranges from 20 to 90 percent. By the end of follow-up (0.5 to 127.0 months), 3 patients were alive, and the mean progression-free survival (21.0 months) of postoperative chemoradiotherapy patients was significantly longer than that of surgery and/or chemotherapy alone (3.3 months). Conclusions: Maxillofacial neuroendocrine carcinoma is characterized by low differentiation of small cells, high degree of malignancy and poor prognosis. Radical surgery combined with chemoradiotherapy has better local control effect.

Clinicopathological analysis of superior mediastinal lymph node metastases in medullary thyroid carcinoma / 中华肿瘤杂志

Objective: To investigate the clinicopathological characteristics of superior mediastinal lymph node metastases (sMLNM) in medullary thyroid carcinoma (MTC). Methods: This retrospective analysis enrolled the patients who were treated for sMLNM of MTC in our hospital from May 2012 to January 2021. All patients were suspected of sMLNM due to preoperative imaging. According to the pathological results, the patients were divided into two groups named sMLNM group and the negative superior-mediastinal-lymph-node group. We collected and analyzed the clinical features, pathological features, pre- and post-operative calcitonin (Ctn), and carcinoembryonic antigen (CEA) levels of the two groups. Logistic regression analysis was used to analyze risk factors, and receiver operation characteristic (ROC) curves were drawn to determine the optimal cut-off values of preoperative Ctn and preoperative CEA for predicting sMLNM. Results: Among the 94 patients, 69 cases were in the sMLNM group and 25 cases were in the non-SMLNM group. Preoperative Ctn level (P=0.003), preoperative CEA level (P=0.010), distant metastasis (P=0.022), extracapsular lymph node invasion (P=0.013), the number of central lymph node metastases (P=0.002) were related to sMLNM, but the multivariate analysis did not find any independent risk factors. The optimal threshold for predicting sMLNM by pre-operative Ctn is 1500 pg/ml and AUC is 0.759 (95% CI: 0.646, 0.872). The sensitivity, specificity, positive predictive value, and negative predictive value of diagnosis are 61.2%, 77.3%, 89.1%, 39.5%, respectively. In patients who underwent mediastinal lymph node dissection through transsternal approach, the metastatic possibility of different levels from high to low were level 2R (82.3%, 28/34), level 2L (58.8%, 20/34), level 4R (58.8%, 20/34), level 3 (23.5%, 8/34), level 4L (11.8%, 4/34). Postoperative complications occurred in 41 cases (43.6%), and there was no perioperative death in all cases. 14.8% (12/81) of the patients achieved biochemical complete response (Ctn≤12 pg/ml) one month after surgery, 5 of these patients were in sMLNM group. Conclusions: For patients who have highly suspicious sMLNM through imaging, combining with preoperative Ctn diagnosis can improve the accuracy of diagnosis, especially for patients with preoperative Ctn over 1 500 pg/ml. The superior mediastinal lymph node dissection for the primary sternotomy should include at least the superior mediastinal levels 2-4 to avoid residual lesions. The strategy of surgery needs to be cautiously performed. Although the probability of biochemical cure in sMLNM cases is low, nearly 40% of patients can still benefit from the operation at the biochemical level.

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.

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.

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

Tumor neuroendocrino de colon derecho en un paciente con colitis ulcerativa: a propósito de un caso / Right colon neuroendocrine tumor in a patient with ulcerative colitis: A case study

Resumen Los tumores neuroendocrinos del colon son relativamente raros en comparación con el carcinoma colorrectal esporádico; su incidencia es muy baja y la localización más frecuente es en el ciego, colon sigmoideo y recto. Se describe con poca frecuencia la asociación de tumor neuroendocrino con la colitis ulcerativa debido a la inflamación crónica que conduce a la diferenciación de células neuroendocrinas a partir de células multipotenciales en el epitelio displásico, que puede ser responsable del desarrollo de carcinomas neuroendocrinos colorrectales. La presentación del caso se trata de un paciente de 57 años de edad con colitis ulcerativa con cuadro de dolor abdominal, pérdida de peso y diarreas líquidas con moco. En el examen físico presentó una lesión endurecida en el marco colónico derecho con tomografía de lesión neoplásica en el colon ascendente y en su hospitalización presentó un cuadro de oclusión intestinal, por lo que se le realizó una hemicolectomía más ileostomía, cuyo resultado en la biopsia fue un tumor neuroendocrino G3 de células grandes en el colon ascendente. En este artículo se revisan de forma práctica los aspectos relacionados con la fisiopatología, diagnóstico y tratamiento de la asociación de estas dos patologías.

Abstract Neuroendocrine colon tumors are relatively unknown compared with sporadic colorectal cancer; its incidence is low, and it is generally located in the cecum, sigmoid colon, and rectum. The existing relation between neuroendocrine tumors and ulcerative colitis is not frequently described due to the chronic inflammation that leads to neuroendocrine cell differentiation from multipotential cells in the dysplastic epithelium, which can be responsible for colorectal neuroendocrine carcinomas development. The study refers to the case of a patient of 57 years old with ulcerative colitis, abdominal pain, weight loss, and liquid diarrhea with mucus. Physical examination revealed a hardened lesion in the right colonic framework with the tomography of a neoplastic lesion in the ascending colon. When the patient was hospitalized, he developed an intestinal obstruction. A hemicolectomy plus ileostomy procedure was performed resulting in a large cell G3 neuroendocrine tumor in the ascending colon revealed in the biopsy. In this article, the aspects related to the pathophysiology, diagnosis, and treatment of the association of these two pathologies are reviewed in a practical way.

Comportamiento del cáncer de tiroides en la Unidad de Cirugía Adulto del Hospital Clínico Herminda Martín de Chillán: período 2017 al 2019 / Thyroid cancer behavior in the adult surgery unit of the Herminda Martin de Chillan Clinical Hospital: period from 2017 to 2019

Resumen Objetivo: Describir las características demográficas del cáncer de tiroides en la Región de Ñuble en pacientes operados en el servicio de cirugía del Hospital Clínico Herminda Martin del 2017 al 2019. Materiales y Método: Mediante estudio descriptivo, se revisaron las historias clínicas de los pacientes operados en la Unidad de Cirugía de Adultos del hospital entre enero de 2017 y diciembre de 2019, tabulando las variables demográficas, clínicas e histopatológicas y método diagnóstico. Resultados: Se operaron 124 pacientes con afecciones tiroideas, 58 resultaron tener cáncer. Predominó el sexo femenino (50 pacientes). Se realizaron 43 tiroidectomías totales como primera cirugía y 15 disecciones cervicales. El carcinoma papilar fue el más diagnosticado (93,1%). Hubo discrepancias entre las categorías Bethesda II y IV en cuanto al diagnóstico definitivo. En mujeres el 36% de los tumores tuvo extensión extra tiroidea y el 54% tenían un diámetro mayor a 1 cm. Discusión: La prevalencia en el sexo femenino corresponde con los datos que se aportan a nivel mundial y en Chile. La discrepancia en el sistema Bethesda pudo corresponder a errores de la toma de muestra o su interpretación citológica. Los hallazgos de extensión y tamaño tumoral pueden estar en relación con el tiempo de espera para la cirugía. Conclusiones: El cáncer tiroideo es más frecuente en el sexo femenino. El carcinoma papilar fue el más diagnosticado. La asociación de tiroiditis con cáncer tiroideo fue de 98%. No se realizó ninguna cirugía conservadora.

Aim: To describe the demographic characteristics of thyroid cancer in the Ñuble Region in patients operated on by the Herminda Martín de Chillán Clinical Hospital surgery service from 2017 to 2019. Materials and Method: Through a descriptive study, the medical records of the patients operated on in the Adult Surgery Unit of the hospital between January 2017 and December 2019 were reviewed, tabulating the demographic, clinical and histopathological variables and method diagnosis. Results: 124 patients with thyroid conditions were operated on, 58 had cancer. The female sex predominated (50 patients). 43 total thyroidectomies were performed as the first surgery and 15 cervical dissections. Papillary carcinoma was the most diagnosed (93.1%). There were discrepancies between Bethesda categories II and IV regarding the definitive diagnosis. In women, 36% of the tumors had an extra-thyroid extension and 54% had a diameter greater than 1 cm. Discussion: The prevalence in the female sex corresponds to the data provided worldwide and in Chile. The discrepancy in the Bethesda system could correspond to errors in the sampling or its cytological interpretation. The findings of tumor extension and size may be related to the waiting time for surgery. Conclusions: Thyroid cancer is more frequent in females. Papillary carcinoma was the most diagnosed. The association of thyroiditis with thyroid cancer was 98%. No conservative surgery was performed.

Carcinoma neuroendócrino de células pequeñas, como hallazgo incidental / Incidental Finding of Small Cell Neuroendocrine Carcinoma

Hombre de 45 años de edad, oriundo del Gran Buenos Aires, ocupación: administrativo, ex- tabaquista de 15 p/y. El paciente se realiza un examen preocupacional que incluía una radiografía de tórax (Figura 1). Se observa como hallazgo incidental una radiopacidad en región superior de campo pulmonar derecho de bordes netos El paciente no refería tos, disnea, descenso de peso o sudoración nocturna, así como tampoco otros síntomas. Consulta al médico de cabecera quien deriva a nuestra institución para diagnóstico y tratamiento. Se le realiza TACAR de tórax, donde se observa una masa sólida que abarca lóbulo superior derecho, sin plano de clivaje con el mediastino y que desplaza tráquea y esófago hacia la izquierda. La misma presenta realce heterogéneo tras la administración de contraste. Además, presenta bullas subpleurales en lóbulos superiores y adenopatías a nivel retrocavopretraqueal e infracarinal (Figura 2). Ante los diversos diagnósticos diferenciales, de masas mediastinales en un paciente joven (linfoma, tumores carcinoides atípicos, tumores mixtos), se decidió realizar un método diagnóstico mínimamente invasivo pero que aportara material suficiente para determinar el origen

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

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.

Surgical treatment of gastric neuroendocrine neoplasms / 中华胃肠外科杂志

Neuroendocrine neoplasms (NEN) are rare neoplasms originating from all major systems, in which gastric neuroendocrine neoplasms (G-NEN) is rarely malignant neoplasm originated in stomach. In 2019, the 5th WHO classification of digestive system tumors updated the classification of G-NEN and solved several naming problems. Since the classification of G-NEN has become more specific and more scientific, the surgical treatment of G-NEN is becoming more individual and more precise. Generally, endoscopic resection is often recommended for the treatment of type I gastric neuroendocrine tumors (NET). Type II gastric NET is mostly secondary to gastrinoma originating from the duodenum or pancreas, and thus surgical treatment of primary gastrinoma deserves enough attention. The decision of operation for type III gastric NET needs comprehensive consideration of tumor size, invasive depth and lymph node metastasis. For gastric neuroendocrine carcinomas without distant metastasis, aggressive surgery should be performed, and the resection range of primary site and lymph nodes can refer to the standard of gastric adenocarcinoma. For locally advanced gastric NEC, it has not been reported whether neoadjuvant chemotherapy or neoadjuvant chemoradiotherapy could reduce tumor stage and improve radical resection rate. In addition, for functional gastric NEN with distant metastasis, radical resection or palliative surgery can be performed to control hormone secretion and may improve the survival. In general, it is an important principle to thoroughly consider biological behavior, extent of primary and metastatic sites, resectability and function of tumor before surgery of gastric neuroendocrine neoplasm, and thus multi-disciplinary treatment (MDT) is recommended.

Progress of Immune Checkpoint Inhibitors in the Treatment of Advanced Pulmonary Neuroendocrine Tumors / 中国肺癌杂志

Pulmonary neuroendocrine tumors (PNETs) are a kind of epithelial tumors originating from pulmonary neuroendocrine cells, accounting for about 20% of primary lung tumors, including typical carcinoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma. The morphologic and clinical characteristics of these four types of PNETs are relatively highly heterogeneous. Immune checkpoint inhibitors (ICIs) have been shown robust antitumor activity in a variety of solid tumors. Treatment regimens of advanced PNETs have developed greatly in the past decade, but ICIs are still in their infancy in the field of PNETs. This review focuses on the landscape of current clinical trials and research as well as the situation of ICIs-related biomarkers in PNETs.
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Growth hormone-releasing hormone-secreting pulmonary neuroendocrine tumor associated with pituitary hyperplasia and somatotropinoma

SUMMARY Acromegaly caused by ectopic growth hormone-releasing hormone (GHRH)-secreting tumor is exceedingly rare. We report a case of acromegaly secondary to GHRH secretion by an incidentally diagnosed pulmonary neuroendocrine tumor (NET) and review 47 similar cases in literature. A 22-year-old male patient presented with symptoms of pituitary apoplexy. Magnetic resonance imaging (MRI) showed apoplexy of a pituitary adenoma. Routinely prior to surgery, a chest radiography was performed which revealed a mass in the left lung. During investigation, the patient was diagnosed with metastatic GHRH-secreting pulmonary NET. In retrospect, it was noted that the patient had pituitary hyperplasia 20 months prior to the MRI which showed the presence of a pituitary adenoma. The histological findings confirmed somatotroph hyperplasia adjacent to somatotropinoma. This case suggests that GHRH secretion can be associated with pituitary hyperplasia, which may be followed by pituitary adenoma formation.

Robotic transanal ressection for rectal tumor: a description of the technique / Ressecção transanal robótica para tumor retal: uma descrição da técnica

ABSTRACT Introduction: Robotic transanal surgery (RTS) is the analog of TAMIS and represents a new focus for the advancement of transanal platforms robotic transanal surgery is sometimes referred to as robotic TAMIS. Though limited to only a few centers world-wide, experience with robotic transanal surgery has been encouraging. Most research with robotic transanal surgery has concentrated on local excision of rectal neoplasia, although more complex procedures such as transanal proctectomy are possible using the robotic approach. This article reports the surgical technique of R-TAMIS performed in the Brazilian National Cancer Institute (INCA, Rio de Janeiro). Methods: 71-year-old, female with cardiologic disease (heart failure), with a 1.5 cm rectal neoplasm at 4 cm from the anal verge in the right anterolateral position. Biopsy revealed neuroendocrine tumor. A compete colonoscopy revealed no evidence of synchronous lesions. Work-up included 3D endorectal ultrasonography and magnetic resonance imaging, which demonstrated the lesion to be uT1uN0. The patient was counseled about surgical options − local excision versus low anterior resection. Due to the cardiologic condition, the patient was elected to proceed with local excision with robotic transanal surgery. Conclusion: Robotic TAMIS is a safe and effective operative procedure for high selected cases of rectal neoplasm. It enhances surgeon ergonomics and facilitates tumor removal and suture in the transanal approach.

RESUMO Introdução: A cirurgia transanal robótica (RTS,Robotic Transanal Surgery) é análoga da TAMIS (cirurgia minimamente invasiva transanal) e representa um novo foco para o avanço das plataformas transanais. A cirurgia transanal robótica é algumas vezes chamada de TAMIS robótica. Embora limitada a apenas alguns centros em todo o mundo, a experiência com a cirurgia transanal robótica tem sido encorajadora. A maioria das pesquisas com cirurgia transanal robótica tem se concentrado na excisão local da neoplasia retal, embora procedimentos mais complexos, como a proctectomia transanal, sejam possíveis utilizando a abordagem robótica. Este artigo relata a técnica cirúrgica de R-TAMIS realizada no Instituto Nacional do Câncer (INCA, Rio de Janeiro). Métodos: paciente de 71 anos, sexo feminino, com doença cardiológica (insuficiência cardíaca), com neoplasia retal de 1,5 cm a 4 cm da borda anal em posição anterolateral direita. A biópsia revelou tumor neuroendócrino. Uma colonoscopia completa não revelou evidências de lesões sincrônicas. A investigação incluiu ultrassonografia endorretal em 3D e ressonância magnética, que demonstrou que a lesão era uT1uN0. A paciente foi aconselhada sobre as opções cirúrgicas - excisão localversus ressecção anterior baixa. Devido à condição cardiológica, optou-se por proceder à excisão local com cirurgia transanal robótica. Conclusão: A TAMIS robótica é um procedimento cirúrgico seguro e eficaz para casos altamente selecionados de neoplasia retal. Ela melhora a ergonomia do cirurgião e facilita a remoção do tumor e a sutura na abordagem transanal.

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.

Tumor carcinoide na vesícula biliar em cão / Carcinoid gallbladder tumor in a dog

Carcinoid is a neoplasia that arises from dispersed cells of the neuroendocrine system. This tumor is uncommon in animals, and its occurrence in the gallbladder is rare. A male Basset Hound dog's corpse was taken to the Univerdade Federal de Minas Gerais to be analyzed by the Veterinary Pathology sector, without a description of its previous history. Necropsy revealed the presence of pale oral, ocular and penile mucous membranes. The gallbladder had a thickened wall and a dilated lumen, which was filled with dark and lumpy bile. Its mucosa had a whitish-red nodule, with solid and friable areas. Microscopically, there was a focal neoplastic proliferation, which wasn't encapsulated and had imprecise limits, which cells were distributed in a solid pattern and separated by a delicate fibrovascular stroma. The neoplastic cells presented oval or round shaped nucleus, which had a chromatin predominantly loose, and one or two nucleoli. Their cytoplasm was moderately abundant, and in most of the cells it was eosinophilic, granular, and had well-defined limits. Using the Grimelius coloration, neoplastic cells' cytoplasmic granules stained brownish or black, confirming the neuroendocrine origin of the neoplasia. Based on the macroscopic and microscopic findings, the diagnosis of a gallbladder carcinoid was established.(AU)