Chinese Medical Association guideline for clinical diagnosis and treatment of lung cancer (2023 edition) / 中华肿瘤杂志

To standardize the prevention and clinical management of lung cancer, improve patients' survival outcomes, and offer professional insight for clinicians, the Oncology Society of Chinese Medical Association has summoned experts from departments of pulmonary medicine, oncology, thoracic surgery, radiotherapy, imaging, and pathology to formulate the Oncology Society of Chinese Medical Association guideline for clinical diagnosis and treatment of lung cancer in China (2023 edition) through consensus meetings. Updates in this edition include 1) cancer screening: deletion of high-risk traits of lung cancer based on epidemiological investigations in the Caucasian population, while preserving features confirmed by research on the Chinese population. Advice on screening institutions is also added to raise awareness of the merits and demerits of lung cancer screening through detailed illustrations. 2) Principles of histopathologic evaluation: characteristics of four types of neuroendocrine tumors (typical carcinoid, atypical carcinoid, large cell carcinoma, and small cell carcinoma) are reviewed. 3) Surgical intervention: more options of resection are available for certain peripheral lesions based on several clinical studies (CALGB140503, JCOG0802, JCOG1211). 4) neoadjuvant/adjuvant therapy: marked improvement in the prognosis of non-small cell lung cancer (NSCLC) patients receiving neoadjuvant immunotherapy are reviewed; more options for consolidation immunotherapy after radiochemotherapy have also emerged. 5) Targeted and immune therapy: tyrosine kinase inhibitors of sensitive driver mutations such as EGFR and ALK as well as rare targets such as MET exon 14 skipping, RET fusion, ROS1 fusion, and NTRK fusion have been approved, offering more treatment options for clinicians and patients. Furthermore, multiple immune checkpoint inhibitors have been granted for the treatment of NSCLC and SCLC, resulting in prolonged survival of late-stage lung cancer patients. This guideline is established based on the current availability of domestically approved medications, recommendations of international guidelines, and present clinical practice in China as well as integration of the latest medical evidence of pathology, genetic testing, immune molecular biomarker detection, and treatment methods of lung cancer in recent years, to provide recommendations for professionals in clinical oncology, radiology, laboratory, and rehabilitation.

An unusual finding of tumorlet carcinoid and endobronchial hamartoma in the lobar bronchus / Hallazgo inusual de tumorlet carcinoide y hamartoma endobronquial en el bronquio lobar

SUMMARY: Neuroendocrine lung tumours are a group of different tumours that have similar morphological, immunohistochemical and molecular characteristics, and represents 1-2 % of all malignant lung tumours. Tumorlet carcinoids represent the nodular proliferation of hyperplastic neuroendocrine cells that is smaller than 5 mm in size. In this study, we reported the unusual finding of tumour carcinoid and endobronchial hamartoma in the same bronchus. A 49-year-old male patient with symptoms of prolonged severe cough and fever, and was treated for pneumonia. Since he did not adequately respond to antibiotic therapy lung CT scan was performed which showed middle lobe bronchus obstruction. Bronchoscopy revealed a lobulated whitish tumour which was biopsied and histopathological diagnosis was hamartoma. Tumour could not be completely removed during bronchoscopy, it was decided to surgically remove it. On serial section, during gross examination in the same bronchus, an oval yellowish area with a diameter of 3 mm was found along the bronchial wall. According to gross and histomorphological characteristics and immunophenotype of tumour cells, the diagnosis of tumour carcinoid was set. Diagnosis of carcinoids of the tumorlet type is usually an accidental finding and it can be seen on CT in the form of subcentimeter, single or multiple, nodular changes. Considering that the clinical picture is nonspecific, they should always be kept in mind as a possible differential diagnosis.

RESUMEN: Los tumores neuroendocrinos de pulmón son un grupo de tumores de diferentes características morfológicas, inmunohistoquímicas y moleculares similares, y representan el 1- 2 % de todos los tumores malignos de pulmón. Los carcinoides tumorales representan la proliferación nodular de células neuroendocrinas hiperplásicas de tamaño inferior a 5 mm. En este estudio reportamos el hallazgo inusual de tumor carcinoide y hamartoma endobronquial en el mismo bronquio. Un paciente varón de 49 años con síntomas de tos severa prolongada y fiebre fue tratado por neumonía. Al no responder adecuadamente a la terapia con antibióticos, se realizó una tomografía computarizada de pulmón que mostró obstrucción del bronquio del lóbulo medio. La broncoscopia reveló una tumoración blanquecina lobulada de la cual se tomó biopsia y el diagnóstico histopatológico fue hamartoma. No fue posible extirpar el tumor por completo durante la broncoscopia y se decidió extirparlo quirúrgicamente. En la sección seriada, durante el examen macroscópico en el mismo bronquio, se encontró un área amarillenta ovalada de 3 mm de diámetro a lo largo de la pared bronquial. De acuerdo a las características macroscópicas e histomorfológicas y de inmunofenotipo de las células tumorales, se estableció el diagnóstico de tumor carcinoide. El diagnóstico de carcinoides de tipo tumorlet suele ser un hallazgo accidental y se observan en la TC en forma de cambios nodulares subcentimétricos, únicos o múltiples. En consideración de que el cuadro clínico es inespecífico, siempre debe tenerse en cuenta como posible diagnóstico diferencial.

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

Cutaneous metastasis on the nasal tip: first clinical sign of pulmonary carcinoid tumor

Abstract Skin metastases are rare and may occur in the context of a known metastatic disease or be the first clinical sign of an underlying primary tumor. In the case of carcinoid neoplasms, determining whether the cutaneous tumor is primary or secondary and identifying the tumor origin in metastatic cases is not always an easy task. This is the report of a case of cutaneous metastasis presenting as the first clinical manifestation of a previously unknown pulmonary carcinoid tumor, including the discussion of histopathological and immunohistochemical findings that allowed an adequate diagnosis of the tumor etiology and reinforces the importance for dermatologists and dermatopathologists to be familiar with these findings.

Cyclic ACTH-secreting thymic carcinoid: a case report and review of the literature

SUMMARY Cyclic Cushing's syndrome (CS) due to thymic carcinoid is a rare disorder. We report a case of cyclic CS due to ectopic adrenocorticotropic hormone (ACTH)-secreting atypical thymic carcinoid tumor and reviewed similar cases published in the literature. Our patient had hypercortisolemia lasting approximately one month, followed by normal cortisol secretion, with relapse one year later. Histopathology revealed an atypical ACTH-positive thymic carcinoid. Ectopic CS can be derived from atypical thymic carcinoids, which can be aggressive tumors with early relapse, suggesting that this type of tumor probably needs aggressive treatment.

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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Observation of Short-term and Long-term Efficacy of Bronchoscopic Interventional Therapy in the Treatment of Typical Carcinoid / 中国肺癌杂志

BACKGROUND@#The curative potential of various bronchoscopic treatments such as electric snare, carbon dioxide freezing, argon plasma coagulation (APC), Neudymium-dopted Yttrium Aluminium Garnet (Nd:YAG) laser and photodynamic therapy (PDT) for the treatment of intraluminal tumor has been administered previously, but this regimen is not common in the treatment of typical carcinoid. The aim of this study is to investigate the curative effects both in short-term and long-term of interventional bronchoscopy in the treatment of typical carcinoid.@*METHODS@#We retrospectively reviewed the clinical data of typical carcinoid patients who were treated with interventional bronchoscopy for tumor suppression and they were hospitalized in the Emergency General Hospital from December 2010 to December 2020, and Wilcoxon rank sum test and chi-square test were used for analysis.@*RESULTS@#A total of 32 patients were included, including 18 cases of preoperative bronchial artery embolization (embolization rate 56%, 95%CI: 31%-79%). The grade score of dyspnea decreased from before treatment to after treatment, and the difference was statistically significant [(1.44±1.03) score vs (0.25±0.58) score, P=0.003]; The degree of bronchial stenosis decreased from pre-treatment to post-treatment, and the difference was statistically significant [(87.50%±13.90%) vs (17.50%±6.83%), P<0.001]; There was significant difference in bronchial diameter before and after treatment [(0.14±0.18) cm vs (0.84±0.29) cm, P<0.001].@*CONCLUSIONS@#Bronchoscopic interventional therapy has significant short-term and long-term effects in the treatment of typical carcinoid.

Chinese expert consensus on lung and thymus neuroendocrine neoplasms / 中华肿瘤杂志

Lung and thymus neuroendocrine neoplasms (NENs) are rare tumors. According to the fifth edition of the World Health Organization classification of thoracic tumors published in 2021, lung and thymus NENs include typical carcinoids, atypical carcinoids, large cell neuroendocrine carcinomas, and small cell carcinomas. Although the incidence of lung and thymus NENs has gradually increased in recent years, there is a lack of randomized controlled clinical study results to guide clinical practice. The treatment of early-stage lung and thymus NENs is complete surgical resection, and the treatment methods for unresectable advanced diseases include different medical treatments, peptide receptor radionuclide therapy, and local therapy. To improve the standardization of diagnosis and treatment of lung and thymus NENs in China, the Expert Committee of Neuroendocrine Neoplasms, Chinese Society of Clinical Oncology developed the expert consensus after multidisciplinary expert discussions based on existing clinical study evidences and guidelines from different neuroendocrine tumor societies. The contents of the consensus cover the epidemiology, diagnosis, pathological classification, staging, treatment and follow-up of lung and thymus NENs (except small cell lung cancer).

Tumor carcinoide atípico solitario de timo: una entidad inusual que no debemos olvidar / Solitary atipic carcinoid tumor of the thymus: an unusual entity that we should not forget

Resumen: Los tumores neuroendocrinos primarios (NET) del mediastino son muy raros. Presentamos el caso de un tumor carcinoide atípico primario de timo. Un hombre de 52 años que fue a un examen médico porque se quejaba de tos no productiva sin hemoptisis. Se solicitó una radiografía de tórax donde se evidenció un mediastino notablemente ensanchado, con una tomografía de tórax que mostro una masa tumoral de 90 x 50 mm en el mediastino anterosuperior que comprime el arco aórtico y la arteria pulmonar sin un plano de clivaje adecuado. Debido a que el tumor era infiltrativo, se proporcionó escisión quirúrgica completa, quimioterapia y radioterapia al mediastino. El objetivo principal de nuestro estudio fue evaluar los hallazgos de CT y MRI con relación a la literatura mundial.

Abstract: Primary neuroendocrine tumors (NET) of the mediastinum are very rare. We present the case of a primary atypical carcinoid tumor of the thymus. A 52-year-old man who went to a medical examination because he complained of a non-productive cough without hemoptysis. A chest radiograph was requested where a markedly widened mediastinum was evidenced, with a thoracic tomography showing a tumor mass of 90 x 50 mm in the anterosuperior mediastinum that compresses the aortic arch and pulmonary artery without an adequate cleavage plane. Because the tumor was infiltrative, complete surgical excision, chemotherapy and radiotherapy were provided to the mediastinum. The main objective of our study was to evaluate the findings of CT and MRI in relation to universal literature.

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)

Tumor neuroendocrino primario testicular: reporte de un caso y revisión de la literatura / Primary neuroendocrine tumor of the testis. A case report and literature review

Los tumores neuroendocrinos primarios del testículo son una entidad muy infrecuente, dando cuenta del 1% de las neoplasias testicu-lares. Clínicamente se presentan indolentes, por una masa testicular. En tumores localizados, rara vez presentan síndrome carcinoide. La orquiectomía radical es el tratamiento de elección; el pronóstico es excelente en etapas precoces. Las terapias adyuvantes no han mostrado utilidad.Se presenta un caso de un hombre de 53 años con una masa testicular palpable de larga evolución. Se realiza orquiectomía radical. La biopsia muestra un tumor neuroendocrino bien diferenciado. Sin evidencia de metástasis en etapificación. Se define en comité onco-lógico, realizar seguimiento.

Rectal carcinoid tumor: diagnosis and management / Tumor carcinoide de reto: diagnóstico e orientação

ABSTRACT The authors present a case of rectal carcinoid tumor in an asymptomatic patient who sought a coloproctology service with the purpose of colorectal cancer prevention. During colonoscopy, a polyp lesion was found in the rectum, and it was resected with a polypectomy loop. Anatomopathological examination revealed a rectal carcinoid tumor with compromised margins. The lesion site was resected again and pathological exam no longer showed neoplasia. A systematic review of the issue was performed, discussing diagnosis aspects, difficulties in the choice of therapeutic approaches, and prognosis. The conclusion is that the rarity of the disease brings difficulties in the choice of treatment; although it has a good prognosis in most cases, its malignant potential cannot be underestimated.

RESUMO Apresentamos um caso clínico de um tumor carcinóide de reto em um paciente assintomático que procurou um serviço de colo-proctologia com o objetivo de fazer prevenção ao câncer colorretal. Durante a colonoscopia observou-se uma lesão polipóide no reto, a qual foi ressecada com alça de polipectomia. O exame anatomopatológico evidenciou tumor carcinóide de reto com comprometimento de margem. O local foi novamente ressecado e o resultado patológico não mais evidenciou neoplasia. Neste artigo é feita uma revisão bibliográfica do assunto abordando aspectos diagnósticos, dificuldade na escolha da conduta terapêutica e prognóstica. O trabalho conclui que a raridade do caso implica em dificuldades na escolha da conduta e que apesar de ter bom prognóstico, em uma parcela dos casos o seu potencial maligno não deve ser desprezado.

Distribución Topográfica y Manejo de Tumores Neuroendocrinos del Tubo Digestivo / Topographic Distribution and Management of Neuroendocrine Tumors of the Digestive Tube

Introducción: Los tumores neuroendocrinos (TNE) son un grupo de neoplasias que se originan a partir de células enterocromafínicas, especialmente ubicadas en el tubo digestivo. El objetivo del presente estudio es escribir la distribución topográfica, el manejo multidisciplinario y diagnóstico patológico según la OMS de los tumores neuroendocrinos del tubo digestivo. Métodos: El presente es un estudio descriptivo, retrospectivo de los casos con diagnóstico anatomopatológico confirmado de tumor neuroendocrino localizados en el tubo digestivo entre enero del 2011 a diciembre del 2018 en el Instituto Oncológico Nacional del Ecuador- SOLCA de Guayaquil. Se describe topografía y tipo de tratamiento establecido con frecuencias y porcentajes. Resultados: Ingresaron al estudio 21 casos. La localización más frecuente fue el apéndice cecal n = 8 (38 %), en estómago n=4 (19 %), intestino delgado n=3 (14 %). El diagnóstico patológico en estadio G1 (65 %); G2 (24%) y G3 (12%). La primera línea de tratamiento fue la cirugía con intención curativa n=19 (90.5 %) y tratamiento endoscópico n=2 (9.5 %). Los pacientes sometidos a cirugía, n=16/19; (84 %) obtuvieron niveles de resección 0 (R0) y permanecieron en observación clínica, los demás sujetos en el estudio recibieron tratamiento adyuvante con somatostatina sola n=1 (4.8 %) o combinación de somatostatina y radioterapia n=2 (9.5 %). Conclusión: El diagnóstico de tumor neuroendocrino de tubo digestivo es una etiología oncológica poco frecuente. El tratamiento quirúrgico en esta serie de casos está enfocada en una acción curativa de tipo quirúrgico.

Introduction: Neuroendocrine tumors (NETs) are a group of neoplasms that originate from enterochromaffin cells, especially located in the digestive tract. The objective of the present study is to write the topographic distribution, the multidisciplinary management and pathological diagnosis according to the WHO of the neuroendocrine tumors of the digestive tract. Methods: This is a descriptive, retrospective study of the cases with a confirmed anatomopathological diagnosis of neuroendocrine tumor located in the digestive tract between January 2011 to December 2018 at the National Oncology Institute of Ecuador- SOLCA of Guayaquil. The topography and type of treatment established with frequencies and percentages are described. Results: 21 cases were entered into the study. The most frequent location was the cecal appendix n = 8 (38%), stomach n = 4 (19%), small intestine n = 3 (14%). The pathological diagnosis in stage G1 (65%); G2 (24%) and G3 (12%). The first line of treatment was surgery with curative intent n = 19 (90.5%) and endoscopic treatment n = 2 (9.5%). Patients undergoing surgery, n = 16/19; (84%) obtained resection levels 0 (R0) and remained under clinical observation, the other subjects in the study received adjuvant treatment with somatostatin alone n = 1 (4.8%) or combination of somatostatin and radiotherapy n = 2 (9.5%). Conclusion: The diagnosis of neuroendocrine tumor of the digestive tract is a rare oncological etiology. The surgical treatment in this series of cases is focused on a surgical action of a surgical type.

Bronchial carcinoid tumors: second primary neoplasms and outcomes of surgical treatment / Tumores carcinoides brônquicos: segunda neoplasia primária e desfechos do tratamento cirúrgico

ABSTRACT Objective: To analyze determinants of prognosis in patients with bronchial carcinoid tumors treated surgically and the potential concomitance of such tumors with second primary neoplasms. Methods: This was a retrospective analysis of 51 bronchial carcinoid tumors treated surgically between 2007 and 2016. Disease-free survival (DFS) was calculated by the Kaplan-Meier method, and determinants of prognosis were evaluated. Primary neoplasms that were concomitant with the bronchial carcinoid tumors were identified by reviewing patient charts. Results: The median age was 51.2 years, 58.8% of the patients were female, and 52.9% were asymptomatic. The most common histology was typical carcinoid (in 80.4%). Five-year DFS was 89.8%. Ki-67 expression was determined in 27 patients, and five-year DFS was better among the patients in whom Ki-67 expression was ≤ 5% than among those in whom it was > 5% (100% vs. 47.6%; p = 0.01). Concomitant primary neoplasms were observed in 14 (27.4%) of the 51 cases. Among the concomitant primary neoplasms that were malignant, the most common was lung adenocarcinoma, which was observed in 3 cases. Concomitant primary neoplasms were more common in patients who were asymptomatic and in those with small tumors. Conclusions: Surgical resection is the mainstay treatment of bronchopulmonary carcinoid tumors and confers a good prognosis. Bronchial carcinoid tumors are likely to be accompanied by second primary neoplasms.

RESUMO Objetivo: Analisar os determinantes do prognóstico em pacientes com tumores carcinoides brônquicos tratados cirurgicamente e possível segunda neoplasia primária concomitante. Métodos: Trata-se de uma análise retrospectiva de 51 tumores carcinoides brônquicos tratados cirurgicamente entre 2007 e 2016. A sobrevida livre de doença (SLD) foi calculada pelo método de Kaplan-Meier, e os determinantes do prognóstico foram avaliados. As neoplasias primárias concomitantes aos tumores carcinoides brônquicos foram identificadas por meio da análise dos prontuários dos pacientes. Resultados: A mediana de idade foi de 51,2 anos, 58,8% dos pacientes eram do sexo feminino e 52,9% eram assintomáticos. A classificação histológica mais comum foi carcinoide típico (em 80,4%). A SLD em cinco anos foi de 89,8%. A expressão de Ki-67 foi determinada em 27 pacientes, e a SLD em cinco anos foi melhor nos pacientes nos quais a expressão de Ki-67 foi ≤ 5% do que naqueles nos quais a expressão de Ki-67 foi > 5% (100% vs. 47,6%; p = 0,01). Neoplasias primárias concomitantes foram observadas em 14 (27,4%) dos 51 casos. Entre as neoplasias primárias malignas concomitantes, a mais comum foi o adenocarcinoma pulmonar, observado em 3 casos. Neoplasias primárias concomitantes foram mais comuns em pacientes assintomáticos e naqueles com tumores pequenos. Conclusões: A resseção cirúrgica é o principal tratamento de tumores carcinoides broncopulmonares e propicia um bom prognóstico. É provável que tumores carcinoides brônquicos se relacionem com segunda neoplasia primária.

Long-term postresection prognosis of primary neuroendocrine tumors of the liver

PURPOSE: Primary hepatic neuroendocrine tumor (PHNET) is a very rare neoplasm, requiring strict exclusion of metastasis from possible extrahepatic primary sites for its diagnosis. METHODS: We reviewed our clinical experience of 13 patients with primary hepatic NET who underwent liver resection from January 1997 to December 2015. RESULTS: The mean age of the 13 patients (8 males and 5 females) was 51.1 ± 12.8 years; the most common clinical manifestation was vague, nonspecific abdominal pain (n = 9). Of them, 11 patients underwent preoperative liver biopsy, 7 of which correctly diagnosed as neuroendocrine tumor (NET). Ten patients underwent R0 resection, and 3 underwent R1 resection. Diagnosis of PHNET was confirmed both immunohistochemically and by absence of extrahepatic primary sites. All tumors were single lesions, with a mean size of 9.6 ± 7.6 cm and a median size of 4.3 cm; all showed positive staining for synaptophysin and chromogranin. During a mean follow-up period of 95.1 ± 86.6 months, 7 patients died from tumor recurrence, whereas the other 6 remain alive to date, making the 5-year tumor recurrence rate 56.0% and the 5-year patient survival rate 61.5%. When confined to R0 resection, 5-year recurrence and survival rates were 42.9% and 70.0%, respectively. Univariate analysis showed that Ki-67 proliferative index was the only risk factor for tumor recurrence. CONCLUSION: PHNET is a very rare tumor with no specific clinical features, and its final diagnosis depends primarily on pathology, immunohistochemistry, and exclusion of metastasis from other sites. Aggressive surgical treatment is highly recommended for PHNET because of acceptably favorable postresection outcomes.

Atypical Thymic Carcinoid in a Patient with Zollinger-Ellison Syndrome / 대한흉부외과학회지

Atypical thymic carcinoid is an extremely rare tumor with a poor prognosis. In addition to its known association with multiple endocrine neoplasia type 1, its hallmark characteristics include local invasion and early distant metastasis. In this report, we share our experience treating atypical thymic carcinoid in a patient with Zollinger-Ellison syndrome.

A Case of Asymptomatic Multiple Endocrine Neoplasia Type I with Thymic Carcinoid

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant hereditary disorder caused by germline mutation of the MEN1 gene. It is characterized by tumors of the anterior pituitary gland, parathyroid glands, and endocrine pancreas. Thymic carcinoid tumor is uncommon and associated with a high mortality, but its natural history has not been investigated yet. We report a case of asymptomatic MEN 1 with a thymic carcinoid tumor. A 37-year-old man underwent a routine medical checkup and upper gastrointestinal endoscopy revealed a duodenal neuroendocrine tumor (NET). Further studies showed the coexistence of pancreatic tumor, parathyroid hyperplasia, pituitary adenoma, and thymoma. The patient underwent duodenal endoscopic mucosal resection, distal pancreatectomy, subtotal parathyroidectomy, and thymectomy. The pathological test revealed a duodenal NET, pancreatic NET, parathyroid hyperplasia, and thymic carcinoid tumor. He was treated for MEN 1. We report this asymptomatic case of MEN 1 with a literature review.

Tumor carcinoide atípico de timo / Atypic Thymic Carcinoid Tumor

Los tumores de timo son formaciones poco frecuentes que constituyen el 50% de las masas ocupantes del mediastino anterior, con una incidencia de 0.5/millón personas/año. Componen un grupo heterogéneo de lesiones, con una amplia gradación de aspectos morfológicos siendo el timoma y el carcinoma de timo los más frecuentes. Sin embargo, hay otros subtipos más raros como los timolipomas, timomas quísticos, linfomas, tumores de células germinales y los carcinoides típicos y atípicos. Estos últimos representan el 2% de los tumores de timo1, 2. Los tumores neuroendocrinos en el timo, fueron descriptos por primera vez por Rosai e Higa3 . El número total de casos reportados internacionalmente hasta la fecha es de aproximadamente 400 y el carcinoide atípico de esta glándula constituye un grupo muy poco frecuente entre éstos, con una incidencia anual cercana al 0.18/1.000.000 de personas. El número total de casos de carcinoides atípicos de timo publicados internacionalmente hasta la fecha es algo mayor de 1004, 5 . Con la finalidad de contribuir a la bibliografía, se presenta una observación de carcinoide atípico de timo