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.

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.

Medición rutinaria de calcitonina sérica en nódulos tiroideos / Routine serum calcitonin measurement in thyroid nodules

Existe mucha controversia sobre los beneficios de la medición de la calcitonina sérica (CT) durante la evaluación inicial de pacientes con nódulos tiroideos. El objetivo del estudio fue evaluar la identificación temprana del carcinoma medular de tiroides (CMT) a través de la medición rutinaria de CT sérica en una cohorte de Buenos Aires, Argentina. Se estudiaron consecutivamente a los pacientes con enfermedad nodular de la tiroides (n=1017). La CT se midió por quimioluminiscencia (valor normal: hasta 18 pg/ml en hombres y 12 pg/ml en mujeres). En dos pacientes, la hipercalcitoninemia se confirmó en mediciones repetidas. La aspiración con aguja fina con medición de CT en el líquido obtenido identificó la presencia del CMT. El estudio genético fue positivo en uno (mutación exón 14, Val804Met, CMT familiar). El otro presentó un polimorfismo (heterocigoto exón 13 L769L - heterocigoto exón 15 S904S). En ambos casos, la CT se normalizó 3 meses después de la cirugía y se mantuvo en valores normales después de 6 años de seguimiento. La medición rutinaria de la CT en nódulos tiroideos fue útil para detectar dos casos de CMT, uno de ellos esporádico y el otro familiar en la cohorte seguida. La prevalencia de CMT fue de 0.2%.

There is much controversy about the benefits of the use of serum calcitonin (CT) in the initial evaluation of patients with thyroid nodules. The objective of the study was to early identify medullary thyroid carcinoma (MTC) through the routine measurement of CT in thyroid nodular pathology in a large cohort of patients from Buenos Aires, Argentina. Consecutive patients with nodular thyroid disease (n=1017) were studied. CT was measured by chemiluminescence, normal value: up to 18 pg/ml in men and 12 pg/ml in women. In two patients, hypercalcitoninemia was confirmed in repeated measurements. Fine needle aspiration with CT measurement in the needle wash fluid identified MTC in nodules with citology abnormalities. The genetic study was positive in one patient (mutation exon 14, Val804Met, MTC familiar). The other presented a polymorphism (exon 13 L769L heterozygous - exon 15 S904S heterozygous). In both cases, CT was normalized 3 months after surgery and remained normal after 6 years of follow-up. The routine measurement of CT in thyroid nodular pathology was useful to detect two cases of MTC, one of them sporadic and the other familiar in this cohort. The prevalence of MTC was 0.2%.

The 4Ds of ectopic ACTH syndrome: diagnostic dilemmas of a difficult disease

SUMMARY Cushing's syndrome (CS) is an uncommon condition that leads to high morbidity and mortality. The majority of endogenous CS is caused by excessive ACTH secretion, mainly due to a pituitary tumor - the so-called Cushing's disease (CD) - followed by ectopic ACTH syndrome (EAS), an extra-pituitary tumor that produces ACTH; adrenal causes of CS are even rarer. Several methods are used to differentiate the two main etiologies: specific laboratory tests and imaging procedures, and bilateral inferior petrosal sinus sampling (BIPSS) for ACTH determination; however, identification of the source of ACTH overproduction is often a challenge. We report the case of a 28-year-old woman with clinical and laboratory findings consistent with ACTH-dependent CS. All tests were mostly definite, but several confounding factors provoked an extended delay in identifying the origin of ACTH secretion, prompting a worsening of her clinical condition, with difficulty controlling hyperglycemia, hypokalemia, and hypertension. During this period, clinical treatment was decisive, and measurement of morning salivary cortisol was a differential for monitoring cortisol levels. This report shows that clinical reasoning, experience and use of recent methods of nuclear medicine were decisive for the elucidation of the case.

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.

A pioneering RET genetic screening study in the State of Ceará, Brazil, evaluating patients with medullary thyroid cancer and at-risk relatives: experience with 247 individuals

ABSTRACT Objective: Initial diagnosis of medullary thyroid carcinoma (MTC) is frequently associated with advanced stages and a poor prognosis. Thus, the need for earlier diagnoses and detection in relatives at risk for the disease has led to increased use of RET genetic screening. Subjects and methods: We performed RET screening in 247 subjects who were referred to the Brazilian Research Consortium for Multiple Endocrine Neoplasia (BRASMEN) Center in the State of Ceará. Direct genetic sequencing was used to analyze exons 8, 10, 11, and 13-16 in MTC index cases and specific exons in at risk relatives. Afterward, clinical follow-up was offered to all the patients with MTC and their affected relatives. Results: RET screening was performed in 60 MTC index patients and 187 at-risk family members. At the initial clinical assessment of the index patients, 54 (90%) were diagnosed with apparently sporadic disease and 6 (10%) diagnosed with hereditary disease. After RET screening, we found that 31 (52%) index patients had sporadic disease, and 29 (48%) had hereditary disease. Regarding at-risk relatives, 73/187 were mutation carriers. Mutations in RET codon 804 and the rare p.M918V mutation were the most prevalent. Conclusions: Performing RET screening in Ceará allowed us to identify a different mutation profile in this region compared with other areas. RET screening also enabled the diagnosis of a significant number of hereditary MTC patients who were initially classified as sporadic disease patients and benefited their relatives, who were unaware of the risks and the consequences of bearing a RET mutation.

Caso Clínico: Tumor Neuroendocrino Gástrico / Case Report: Gastric Carcinoid

INTRODUCCIÓN: Los tumores neuroendocrinos gástricos son tumores de muy baja prevalencia, constituyendo el aproximadamente 1.8 % de los tumores gástricos. Suelen ser solitarios, con un tamaño entre 4 y 8 cm, con más frecuencia en el sexo masculino con edad promedio de presentación entre 60 - 70 años. Su diagnóstico es cada vez mayor, gracias al uso generalizado de la endoscopia digestiva alta, siendo ésta la prueba de oro. En cuanto a su tratamiento, la resección quirúrgica de los tumores carcinoides gástricos es de elección cuando no se puede optar por resección endoscópica. CASO CLÍNICO: Paciente de 45 años con cuadro clínico de epigastralgia, náusea y diarrea, de dos meses de evolución. Se realiza endoscopía digestiva alta con reporte de úlcera de antro Forrest III. Biopsia: neruroendocrino grado II. Tomografia abdomen: sin datos de actividad en otro sitio fuera de estómago. EVOLUCIÓN: Se realizó gastrectomía subtotal, con reporte negativo en el estudio transoperatorio de líquido de lavado peritoneal. El reporte de patología confirmó el diagnóstico de tumor neuroendócrino GII 3.5 cm unifocal; en estadio III B según las guías clínicas, con vigilancia por un período de un año sin actividad tumoral. CONCLUSIÓN: Al ser el tumor neuroendocrino gástrico un tipo de cáncer poco frecuente, la endoscopía digestiva alta fue fundamental en el diagnóstico de este caso que le llevó al paciente a buscar evaluación médica. Sin embargo, para disminuir el riesgo de su incidencia se recomienda una alimentación rica en frutas, y verduras frescas acompañado de una actividad física adecuada.

BACKGROUND: Gastric neuroendocrine are very low tumors prevalence, constituting approximately 1.8 % of gastric tumors. They are usually solitary, with a size between 4 and 8 cm, most often in the male with average age of presentation between 60 - 70 years. It is diagnosis is increasing, thanks to the widespread use of upper digestive endoscopy, this being the gold standar. Regarding it is treatment; surgical resection of gastric neruroendocrine tumors is one of the choices when endoscopic resection is not possible. CASE REPORT: 45-year-old patient refers epigastralgia, nauseas and diarrhea, since two months ago. Upper digestive endoscopy is performed with report of Forrest III antrum ulcer. Biopsy: grade II neuroendocrine tumor. Abdominal tomography: no activity data in another place outside the stomach. EVOLUTION: Subtotal gastrectomy was performed, with a negative report in the transoperative study of peritoneal lavage fluid. The pathology report confirmed the diagnosis of unifocal neuroendocrine GII 3.5 cm tumor; in stage III B according to clinical guidelines, with surveillance for a period of one year without tumor activity. CONCLUSION: The gastric neuroendocrine is a rare type of cancer; upper gastrointestinal endoscopy was a fundamental in the diagnosis of this case that led the patient to seek medical evaluation. However, to reduce it is incidence is recommended to eat a diet rich in fruits, and fresh vegetables accompanied by adequate physical activity.

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.

Carcinoma neuroendocrino primario de vagina. Revisión de literatura a propósito de un caso / Primary neuroendocrine carcinoma of the vagina. Literature review on the subject of a case

RESUMEN El carcinoma primario de vagina representa 1 a 2% de los cánceres ginecológicos, siendo la diferenciación histológica neuroendocrina de células pequeñas extremadamente infrecuente, sólo se han reportado 28 casos en la literatura, describiéndose en orden de frecuencia en cérvix, endometrio, ovario, vagina y vulva. Se observa con más frecuencia en mujeres postmenopáusicas. Se presenta el caso de paciente femenino, de 39 años de edad, quien acude a la consulta por presentar secreción genital serohemática, fétida, de 1 mes de evolución, evidenciándose al examen físico lesión exofítica, friable, renitente, de aproximadamente 4 cm de diámetro, en tercio superior y cara posterolateral izquierda de vagina, por lo que se realiza biopsia excisional de dicha lesión, siendo el diagnóstico anatomopatológico carcinoma neuroendocrino de células pequeñas, grado histológico: 3. A propósito de este caso, se realiza una revisión del tema, haciendo hincapié en la importancia de la exploración ginecológica exhaustiva que incluya tomas de citologías y colposcopias periódicas, tomando en cuenta la vagina y así, realizar diagnóstico precoz en vista que el riesgo de carcinoma en dicha zona es infrecuente, mejorando el pronóstico y sobrevida de las pacientes.

ABSTRACT The primary carcinoma of the vagina represents 1 to 2 % of gynecologic cancers, being the histological differentiation neuroendocrine of small cells extremely infrequent, only 28 cases have been reported in the literature, describing in order of frequency: in the cervix, endometrium, ovary, vagina and vulva. It is most commonly seen in postmenopausal women. We present the case of a female patient, 39 years old, who comes to medical consult due to serohematic, fetid, genital discharge of 1 month of evolution, evidencing at physical examination an exophytic lesion, friable, renitent, with a diameter of approximately 4 cm, in the upper third and posterolateral left side of the vagina, for which an excisional biopsy of said lesion is performed, being the anatomopathological diagnosis neuroendocrine carcinoma of small cells, histological grade: 3. A bibliography reviewed was made, emphasizing the importance of exhaustive gynecological exploration with periodic cytology and colposcopy examinations, including the vagina for early diagnosis in view of carcinoma risk in this area is uncommon, improving the prognosis and survival of patients.

Caso Clínico: Hepatocarcinoma Combinado con Carcinoma Nueroendocrino Primario de Higado. Hospital de Especialidades José Carrasco Arteaga / Case Report: Management of Septic Abdomen Using VAC System

INTRODUCCIÓN: El hepatocarcinoma asociado con carcinoma neuroendocrino primario de hígado es una neoplasia rara; existiendo así el de tipo combinado y por colisión. CASO CLÍNICO: Paciente masculino de 52 años de edad, se detectó una masa a nivel de ambos lóbulos hepáticos. Se realizó una segmentectomia más resección ganglionar con resultado de patología positivo para hepatocarcinoma multinodular grado II combinado con carcinoma neuroendocrino. Estudios de extensión negativos para malignidad. EVOLUCIÓN: No se evidenció recurrencia de la lesión en los controles por imagen y los marcadores de antígeno carbohidrato 125, antígeno carbohidrato 19-9 yalfa feto proteína permanecieron negativos a los 6 meses luego de la cirugía. CONCLUSIÓN: El hepatocarcinoma combinado con carcinoma neuroendocrino primario de hígado, es una neoplasia poco frecuente que requieremás estudios que corroboren el tratamiento y pronóstico. (au)

INTRODUCTION: Hepatocarcinoma associated with primary neuroendocrine carcinoma of the liver is a rare neoplasm; There is the type of combined and collision. CASE REPORT: A 52 year old male patient who was diagnosed with a mass at both hepatic lobes. A segmentectomy plus nodal resection was performed, with results for multinodular grade II hepatocarcinoma combined with neuroendocrine carcinoma. Extension studies were negative for malignancy. EVOLUTION: There was no recurrence of lesion in the image controls; carbohydrate antigen 125, carbohydrate antigen 19-9 and Alpha-fetoprotein remained negative after 4 months of surgery. CONCLUSION: Hepatocarcinoma combined with primary neuroendocrine carcinoma of the liver is a rare neoplasm that requires more studies to strengthen treatment and prognosis.(au)

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.

Carcinoma neuroendocrino de timo con enfermedad de pompe del adulto / Thymic neuroendocrine carcinoma with Pompe's disease of the adult

La enfermedad de Pompe (glucogenosis tipo II) es una enfermedad de depósito lisosomal, autosómica recesiva causada por una deficiencia de ácido alfa-glucosidasa. Los tumores neuroendocrinos tímicos son neoplasias primarias con diferenciación neuroendocrina que generalmente se presentan como una masa en el mediastino anterior. Ambas enfermedades se consideran raras en sí mismas. En nuestro conocimiento, la enfermedad de Pompe y un tumor neuroendocrino del timo en el mismo paciente no ha sido antes comunicada. No pudimos encontrar la plausibilidad biológica entre ambas enfermedades. Se necesitan más estudios para confirmar el hallazgo y para aumentar aún más nuestra comprensión de esta asociación. Los datos clínicos de los estudios epidemiológicos, los informes de casos, las series de casos y los pequeños ensayos clínicos abiertos o controlados pueden definir tanto la plausibilidad clínica como la causalidad entre las dos enfermedades.

Pompe disease (glycogenosis type II) is an inherited autosomal recessive lysosomal storage disease caused by a deficiency of acid alpha-glucosidase. Thymic neuroendocrine tumors, are primary thymic neoplasms with neuroendocrine differentiation that generally present as a mass within the anterior mediastinum. Both diseases are considered rare. To our knowledge the co-existence of Pompe disease and thymic neuroendocrine tumor in the same patient has not been previously reported. We could not find biological plausibility between both diseases. Further studies are needed to confirm the finding and to further increase our understanding of this association. Clinical data from epidemiological studies, case reports, case series and small formal open or controlled clinical trials may define both clinical plausibility and causality between the two conditions.

Tumor pancreático no funcionante en paciente de 18 años / Non-functioning pancreatic tumor in a patient of 18 years old

Los tumores pancreáticos neuroendocrinos son raros y representan aproximadamente de menos del 3 por ciento de los tumores pancreáticos. Cerca del 85 por ciento de ellos son no funcionantes y son de localización intrapancreática y característicamente grandes, por lo que se diagnostican generalmente en estadios avanzados. Tienen mayor incidencia en el sexo masculino y en mayores de 50 años. Se presentan clínicamente con síntomas debidos al tumor per se, que pueden incluir dolor abdominal, pérdida de peso o hictericia. El objetivo de este trabajo es presentar el caso de una paciente de 18 años que aquejó de aumento de volumen del hemiabdomen superior sin asociarse a otros síntomas, al concluir el estudio se diagnosticó carcinoma neuroendocrino de cuerpo de páncreas, entidad poco frecuente en esta edad y sexo, por lo cual requirió pancreatectomía distal con esplenectomía(AU)

Neuroendocrine pancreatic tumors are rare and account for 3 percent of pancreatic tumors approximately. Almost 85 percent of them are non-functioning, generally big, located in the intrapancreatic region and diagnosed at advanced staging. Its highest incidence is found in males over 50 years of age. Its symptoms may include abdominal pain, loss of weight and jaundice. The objective of this article was to present an 18 years-old man affected by increase volume in upper hemiabdomen with no relation with other symptoms. On the conclusion of the studies, he was diagnosed with neuroendocrine carcinoma of the pancreatic body, an unusual entity at this age and sex, and he required to undergo distal pancreatectomy with splenectomy(AU)

Tumor neuroendocrino en cérvix uterino: reporte de caso / Neuroendocrine tumor of the uterine cervix: case report

Los tumores neuroendocrinos de cuello uterino son extremadamente raros. Las mujeres con diagnóstico de carcinoma neuroendocrino de células pequeñas del cuello uterino tienen mayor frecuencia de metástasis en los ganglios linfáticos, invasión linfovascular, recurrencia y peor pronóstico en comparación con aquellos con otros tipos de neoplasias cervicales. Se presenta el caso de una mujer de 58 años, con un tiempo de enfermedad de seis años antes del ingreso, caracterizado por sangrado vaginal irregular posmenopáusica, además de sintomatología relacionada a anemia crónica. En el examen ginecológico, se evidenció tumoración de 4 cm que ocupaba tercio superior de vagina y protruía por el cérvix. Fue diagnosticado como mioma abortivo y enviada a estudio anatomopatológico. El resultado fue carcinoma neuroendocrino de células pequeñas grado III en el 90% y carcinoma epidermoide en el 10%. La paciente fue sometida a histerectomía radical más salpingo-ooferectomía bilateral y linfadenectomía pélvica bilateral y para-aortica. El estudio anatomopatológico de la pieza quirúrgica encontró endometrio y miometrio comprometido por neoplasia maligna. Parametrios, anexos y ganglios linfáticos se encontraron libres de neoplasia. A la microscopía el resultado fue carcinoma neuroendocrino grado III (carcinoma de células pequeñas, infiltrante), con extensa embolia linfovascular. El estudio de inmunohistoquímica arrojó sinaptofisina positivo en las áreas con diferenciación neuroendocrina.

Neuroendocrine tumors of the cervix are extremely rare. Women diagnosed with small cell neuroendocrine carcinoma of the cervix have a higher frequency of metastases in the lymph nodes, lymphovascular invasion, recurrence and worse prognosis compared to those with other types of cervical neoplasia. We report the case of a 58-year-old female, with a history of six years of postmenopausal irregular vaginal bleeding, in addition to symptoms related to chronic anemia. Gynecological examination showed a tumor of 4 cm that occupied the upper third of the vagina and protruded through the cervix initially diagnosed as an abortifacient myoma, and sent to histopathology study. 90% of the tumor was small cell neuroendocrine carcinoma grade III, and the remaining 10% was squamous cell carcinoma. The patient underwent into a radical hysterectomy plus bilateral salpingo-oophorectomy, and bilateral pelvic and para-aortic lymphadenectomy. Histopathologic examination of the surgical specimen found endometrium and myometrium compromised by malignancy. Parametrium, annexes and lymph nodes were free of neoplasia. At microscopy, the result was a grade III neuroendocrine carcinoma (small cell carcinoma, infiltrating), with extensive lymphovascular emboli. The immune-histochemical study showed synaptophysin positive in areas with neuroendocrine differentiation.

Large Cell Poorly Differentiated Neuroendocrine Carcinoma of the Maxilla: An extremely Rare Tumor.

Neuroendocrine carcinomas are epithelial neoplasm with predominant neuroendocrine differentiation. Mostly they are found in gastrointestinal and respiratory system. In the nasal and paranasal sinus regions, squamous cell carcinoma is the most common tumor, followed by adenocarcinoma, malignant lymphoma, sinonasal undifferentiated carcinoma, and olfactory neuroblastoma. Large cell neuroendocrine carcinoma of head and neck region is extremely rare. Until now, very few cases of neuroendocrine tumors of paranasal sinuses have been documented. Compared to other organ systems, the neuroendocrine carcinoma involving sinuses are much more aggressive and with poor prognosis. The rarity of this carcinoma has restricted the understanding of its etiology and clinical outcome. We herein reporting a case of large cell poorly differentiated neuroendocrine tumor of maxilla with orbital extension with the purpose of analysis of the available information of this rare malignancy.

Primary Neuroendocrine Tumor of the Breast: Imaging Features

Focal neuroendocrine differentiation can be found in diverse histological types of breast tumors. However, the term, neuroendocrine breast tumor, indicates the diffuse expression of neuroendocrine markers in more than 50% of the tumor cell population. The imaging features of neuroendocrine breast tumor have not been accurately described due to extreme rarity of this tumor type. We present a case of a pathologically confirmed, primary neuroendocrine breast tumor in a 42-year-old woman, with imaging findings difficult to be differentiated from that of invasive ductal carcinoma.

A Case of Colonic Collision Tumor (Adenocarcinoma and Neuroendocrine Carcinoma) / 대한소화기학회지

Collision tumors of the colon are rare. A 54-year-old man was referred to our hospital for the evaluation of hematochezia. Colonoscopy demonstrated the presence of about 3 cm sized mass in the rectosigmoid junction. After surgical resection, the colonic lesion was histologically composed of two discrete lesions: adenocarcinoma in the superficial layer and poorly differentiated neuroendocrine carcinoma in the deeper layer. We report this case of colonic collision tumor (adenocarcinoma and neuroendocrine carcinoma) with a review of the literature.

Composite epithelial kidney tumor comprising of adeno and neuroendocrine carcinomatous components: An unusual variant of renal cell carcinoma.

Renal cell carcinomas accounts for an approximately 2% of human malignancies with atleast ten different histological subtypes recognized by the World Health Organization (WHO) 2004 classification. Composite carcinomas with dual divergent epithelial differentiation in kidneys are extremely uncommon. We report an unusual case of a 37-year-old female who presented with symptoms related to renal tumor for the last three years. Computed tomography scan revealed a large heterogenously contrast enhancing left kidney mass comprising of two distinct histological components of low grade adenocarcinomatous and carcinoid-like low grade neuroendocrine carcinomas with presence of hilar lymph nodal metastases of both the components. The component of adenocarcinoma was immunoreactive to E-cadherin, cytokeratins 7 and 19 with negativity for cluster of differentiation 10, cytokeratin 20, CD117, and vimentin; while the neuroendocrine component was immunoreactive for vimentin, chromogranin and synaptophysin with negativity for CD10, CD117, and cytokeratins 7, 19 and 20. MIB-1 labeling index in the both the components was 2-3%. The present case is the first of its kind to be reported in the kidney and emphasizes the diversity potential of kidney tumors.