Breakthrough Symptoms Remain an Unmet Need in Symptomatic Patients With Neuroendocrine Tumors and Carcinoid Syndrome.

OBJECTIVES: The aims of the study were to assess the effects of breakthrough carcinoid syndrome symptoms on well-being in neuroendocrine tumor (NET) patients insufficiently controlled on long-acting somatostatin analog (SSA) and to assess patient experience with treatment options, physician communication, and disease information sources. METHODS: This study surveyed US NET patients from 2 online communities, experiencing at least one symptom, by utilizing a 64-item questionnaire. RESULTS: One hundred patients participated: 73% female, 75% age 56 to 75 years, and 93% White. Primary tumor distribution was as follows: gastrointestinal NET (n = 55), pancreatic NET (n = 33), lung NET (n = 11), and other NET (n = 13). All patients were actively treated with one long-acting SSA and experiencing breakthrough symptoms: diarrhea, flushing, or other (13% experienced one, 30% two, 57% greater than two). More than one third of treated patients experienced carcinoid-related symptoms daily. Sixty percent of respondents reported not having short-acting "rescue" treatment available, impacting well-being though anxiety or depression (45%), trouble exercising (65%), sleeping (57%), employment (54%), and maintaining friendships (43%). CONCLUSIONS: Breakthrough symptoms remain an unmet need, even in treated patients with NETs. Though still relying on physicians, NET patients are now also using the Internet. Improved awareness of optimal SSA use may improve syndrome control.

Proposing Specific Neuronal Epithelial-to-Mesenchymal Transition Genes as an Ancillary Tool for Differential Diagnosis among Pulmonary Neuroendocrine Neoplasms.

Pulmonary neuroendocrine neoplasms (PNENs) are currently classified into four major histotypes, including typical carcinoid (TC), atypical carcinoid (AC), large cell neuroendocrine carcinoma (LCNEC), and small cell lung carcinoma (SCLC). This classification was designed to be applied to surgical specimens mostly anchored in morphological parameters, resulting in considerable overlapping among PNENs, which may result in important challenges for clinicians' decisions in the case of small biopsies. Since PNENs originate from the neuroectodermic cells, epithelial-to-mesenchymal transition (EMT) gene expression shows promise as biomarkers involved in the genotypic transformation of neuroectodermic cells, including mutation burden with the involvement of chromatin remodeling genes, apoptosis, and mitosis rate, leading to modification in final cellular phenotype. In this situation, additional markers also applicable to biopsy specimens, which correlate PNENs subtypes with systemic treatment response, are much needed, and current potential candidates are neurogenic EMT genes. This study investigated EMT genes expression and its association with PNENs histotypes in tumor tissues from 24 patients with PNENs. PCR Array System for 84 EMT-related genes selected 15 differentially expressed genes among the PNENs, allowing to discriminate TC from AC, LCNEC from AC, and SCLC from AC. Functional enrichment analysis of the EMT genes differentially expressed among PNENs subtypes showed that they are involved in cellular proliferation, extracellular matrix degradation, regulation of cell apoptosis, oncogenesis, and tumor cell invasion. Interestingly, four EMT genes (MAP1B, SNAI2, MMP2, WNT5A) are also involved in neurological diseases, in brain metastasis, and interact with platinum-based chemotherapy and tyrosine-kinase inhibitors. Collectively, these findings emerge as an important ancillary tool to improve the strategies of histologic diagnosis in PNENs and unveil the four EMT genes that can play an important role in driving chemical response in PNENs.

International trends in pulmonary neuroendocrine cancer studies:a scientometric study.

INTRODUCTION: Pulmonary neuroendocrine tumors account for approximately 20% of all primary lung tumors. Few studies summarize the current body of pulmonary neuroendocrine tumors studies worldwide. OBJECTIVE: A quantitative scientometric analysis was conducted to evaluate the development of applications and innovations and to analyze their contribution to various areas of improvement in treatment and diagnosis of pulmonary neuroendocrine tumors. METHODS: We searched for studies published in the last 20 years in the databases United States National Library of Medicine (PubMed), Scientific Electronic Library Online (SciELO), Scopus, and Web of Science, using the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'typical pulmonary carcinoid', 'atypical pulmonary carcinoid', 'pulmonary carcinoid and diagnosis', 'pulmonary carcinoid and treatment', 'pulmonary carcinoid and epidemiology' and 'pulmonary carcinoid and prognosis'. RESULTS: Our results showed the number of publications increased significantly over the study period and was strongly associated with the economic or financial situation of the publications' countries of origin. We observed a predominance of studies on histological diagnosis compared to treatment, and among the studies related to treatment, a predominance of retrospective studies relative to prospective studies was found. CONCLUSION: Based on the published literature, we concluded research on pulmonary neuroendocrine tumors still seems to be incipient, because it favors studies related to histological characterization of the disease, and therapeutic studies are still predominantly of a retrospective nature.

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.

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.

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

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.

Differential Diagnosis of Diarrhea in Patients With Neuroendocrine Tumors.

Patients with neuroendocrine tumors (NETs) and carcinoid syndrome experience diarrhea that can have a debilitating effect on quality of life. Diarrhea also may develop in response to other hormonal syndromes associated with NETs, surgical complications, medical comorbidities, medications, or food sensitivities. Limited guidance on the practical approach to the differential diagnosis of diarrhea in these patients can lead to delays in appropriate treatment. This clinical review and commentary underscore the complexity in identifying the etiology of diarrhea in patients with NETs. Based on our collective experience and expertise, we offer a practical algorithm to guide medical oncologists and other care providers to expedite effective management of diarrhea and related symptoms in patients with NETs.

Endobronchial ultrasound- guided transbronchial needle aspiration for mediastinal lymph node staging in patients with typical pulmonary carcinoids.

BACKGROUND: Pulmonary carcinoids, which are well-differentiated lung neuroendocrine carcinomas, account for only 1-2 % of primary lung malignancies. Although fluorodeoxyglucose positron-emission tomography/computed tomography performs poorly in the identification of mediastinal lymph node metastases, particularly for pulmonary carcinoids, endobronchial ultrasound-guided (EBUS) transbronchial needle aspiration (TBNA) may be a useful means of preoperative nodal assessment in patients with these conditions. However, the diagnostic performance of EBUS TBNA is unknown. This study was designed to determine the sensitivity of EBUS for mediastinal staging in patients with typical carcinoid. STUDY DESIGN AND METHODS: A retrospective review of all patients with carcinoids who underwent EBUS TBNA and/or surgical resection with lymphadenectomy at The University of Texas MD Anderson Cancer Center was performed. The sensitivity of EBUS -TBNA in diagnosis of mediastinal lymph node metastases was determined. RESULTS: Of the 212 patients with pulmonary carcinoids we identified, 137 had surgery with no preoperative EBUS TBNA, 68 had EBUS TBNA followed by surgery, and 7 had EBUS TBNA only. The sensitivity rate for EBUS TBNA in diagnosis of mediastinal lymph node metastases was 77.78 % overall (95 % CI, 57.7-91.3%) and it was 87.5 % (95 % CI, 67.6-97.3%) when we considered only patients with EBUS TBNA-accessible lymph nodes. DISCUSSION: The sensitivity of EBUS TBNA for diagnosis of mediastinal lymph node metastases of pulmonary carcinoids was slightly lower than that reported previously for non-small cell lung cancer. Preoperative EBUS TBNA identified nodal metastases not previously identified by imaging.

Well-differentiated Neuroendocrine Carcinoma of the Larynx: Confusion of Terminology and Uncertainty of Early Studies.

Well-differentiated neuroendocrine carcinoma (also known as "carcinoid") of the larynx is an exceedingly rare tumor that has an epithelial origin. These tumors are malignant and have a low, but definite, risk of metastasis. Although it can be challenging, this tumor should be differentiated from moderately differentiated neuroendocrine carcinoma (also known as "atypical carcinoid"). The clinical and pathologic features of this tumor, as well as treatment and prognosis, are reviewed in detail.

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.

Evaluation of diagnostic and prognostic significance of Ki-67 index in pulmonary carcinoid tumours.

BACKGROUND: Pulmonary carcinoid (PC) tumours are classified as either typical (TC) or atypical (AC) according to mitotic index (MI) and presence of necrosis. The aim of this study was to analyse the diagnostic and prognostic values of the Ki-67 index in PC. METHODS/PATIENTS: Between January 2001 and March 2015, we evaluated 94 consecutive patients with a confirmed diagnosis of TC (n = 75) or AC (n = 19) at our institution. Diagnostic histology was centrally reviewed by a local expert neuroendocrine pathologist, with assessment of Ki-67, MI, and necrosis. RESULTS: Median patient follow-up was 35 months. Eighty-four patients who underwent curative surgical resection were included in the survival analysis for identification of prognostic factors. Ki-67 index showed high diagnostic accuracy to predict histological subtype when assessed by receiver operator characteristic curves with an area under the curve of 0.923 (95% CI 0.852-0.995, p < 0.001). Multivariate analysis showed that MI, Ki-67 index, and the presence or absence of necrosis were independent prognostic factors for relapse-free survival. Combination of MI, Ki-67, and necrosis led to the classification of patients into four different prognostic groups (very low, low, intermediate, and high risks of relapse). CONCLUSIONS: The current study proposes the incorporation of Ki-67 index in the prognostic classification of PC tumours. Due to the limited number of patients and length of follow-up, the current model needs validation by larger cohort studies. Nevertheless, our results suggest that Ki-67 index and MI have continuous effect on prognosis. Prognostic models incorporating multiple cutoffs of Ki-67 and MI might better predict outcome and inform clinical decisions.

Tumor carcinoide bronquial en pediatría: reporte de caso / Bronchial carcinoid tumor in pediatrics: Report of a case

Los tumores primarios de pulmón son poco frecuentes en el paciente pediátrico. Dentro de ellos, el tumor carcinoide bronquial es el más común. Clínicamente, su manifestación más usual es como infección respiratoria a repetición; sin embargo, su presentación puede ser variada. Reportamos el caso de una paciente de catorce años de edad con clínica de dolor torácico y síntomas respiratorios.

Lung priman- tumors are bizarra in pediátrica. Among them, carcinoid bronchtal tumor is the most common. The claaaical mamíestation is recurrent respiratory tract infectton, but the clinical presentaron can be variable. We report the case of a 14 years-old patient with chest pain and respiratory symptoms.

[Carcinoid tumor of gastrointestinal tract: about two clinical cases]. / Tumor carcinoide: a propósito de dos casos clínicos.

Carcinoid tumors belong to the families of neuroendocrine tumors. The major sites are the gastrointestinal tract 65% and lungs 25%. The small intestine, specifically the ileum, is the most common. These tumors although rare, are more common in tumors of neuroendocrine origin gastro-entero-pancreatic. In both cases we observe the different clinical presentations that may have carcinoid tumor; in case 1 ulceration of the tumor mass causing the elimination of melena, and severe diarrhea caused by neuroendocrine secretion. Case 2 typical course, totally asymptomatic incidental finding.

Primary lung tumors in children: 24 years of experience at a referral center.

PURPOSE: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years. METHODS: A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990-2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome. RESULTS: Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91-16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology. CONCLUSION: Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.

Tumor Carcinoide de testículo / Testicular carcinoid tumor

Los tumores carcinoides de testículo son extremadamente raros, representando el 0.23% de los tumores de dicha localización. Se dividen en tres grupos: tumor carcinoide primario de testículo, teratoma de testículo con áreas de carcinoide y tumor carcinoide metastásico de otra localización. Presentamos un paciente de 52 años que consultó por dolor y tumefacción testicular. Se realizó orquiectomía derecha. LDH, alfa feto proteína y beta HCG: normales. Anatomía patológica: tumor blanco amarillento, constituido por células neoplásicas. Inmunohistoquímica: inmunofenotipo correspondiente a tumor neuroendocrino. 5HIAA urinario: normal. Centellografía con (Tc99) octreotide: normal. Se decidió control. Los tumores carcinoides primarios de testículo son infrecuentes, es fundamental descartar localizaciones primarias intestinales para confirmar el diagnóstico. Las herramientas diagnósticas más utilizadas son 5HIAA en orina y centellografía con (TC99) octreotide. El tumor carcinoide primario de testículo presenta un pronóstico favorable luego de la orquiectomía (AU)

Testicular carcinoid tumors are extremely rare. They are divided into three groups: primary testicular carcinoid tumor, testicular teratoma with areas of carcinoid and metastatic carcinoid tumor from another location. We present a 52 years old patient that was admitted with testicular pain and swelling. Right orchiectomy was performed. Serum LDH and alpha, fetoprotein and beta human chorionic gonadotropin were normal. Pathology: white yellowish tumor consisting of neoplastic cells. Immunohistochemistry: immunophenotype corresponding to neuroendocrine tumor. 5HIAA urinary and somatostatin receptor scintigraphy were normal. It was decided to control the patient. Primary testicular carcinoid tumors are uncommon. It is essential to rule out intestinal primary locations to confirm the diagnosis. The diagnostic tools used are 5 HIAA urinary and somatostatin receptor scintigraphy. The primary carcinoid tumor of the testis presents a favorable prognosis after orchiectomy (AU)

[Intestinal carcinoid tumour: Case report]. / Tumor carcinoide intestinal: reporte de un caso.

BACKGROUND: Carcinoid of the small intestine, is a well-differentiated neuroendocrine tumor that rarely presents with clinical signs. This tumour can be associated with other conditions, such as inflammatory bowel disease, presenting a wide range of symptoms. In some cases they have an aggressive and highly symptomatic behaviour; thus, clinical suspicion must be high to make an early diagnosis. CLINICAL CASE: A 60 year-old male patient with Crohn's disease and gastrointestinal symptoms attributed to this disease within the last year. He presented with intestinal obstruction initially treated with conservative management with no improvement. Exploratory laparotomy was performed finding a mesenteric tumour that caused the bowel obstruction. Bowel resection with primary anastomosis was performed. The pathology report showed an intestinal carcinoid tumour with lymph node metastases. The patient recovered well, and was discharged without complications to continue medical treatment and follow-up by the Oncology department. CONCLUSION: In almost 42% of the cases, the most common site of carcinoid tumours is the small intestine, and of these, 41% are presented as locoregional disease. Patients with Crohn's disease present a higher incidence. In these cases, the most common presentation is an acute intestinal obstruction (90%). Surgery is usually curative, and follow up is important as the symptoms of Crohn's disease can hide any recurrence.

Appendiceal Nodules in the Setting of Endometriosis Can Be Carcinoid Tumors.

INTRODUCTION: Endometriosis is occasionally found in the appendix, particularly in severe forms of deep infiltrating disease. Carcinoid tumor is the most common neoplasm of the appendix and may be overlooked or misdiagnosed when there are multiple endometriosis lesions in the pelvis. CASE DESCRIPTION: We describe two cases of carcinoid tumor diagnosed in patients who underwent surgery to treat endometriosis, in whom the diagnosis of appendiceal endometriosis was presumed. DISCUSSION: In the context of endometriosis, surgery is indicated when the appendix is affected. Despite the more likely diagnosis of appendiceal endometriosis, carcinoid tumors cannot be ruled out by imaging examinations.

Adenoma carcinoide de oído medio / Middle ear carcinoid adenoma

Las neoplasias primarias de oído medio son poco frecuentes y más aún lo es el adenoma de oído medio. Se plantea que el tumor se origina de células pluripotenciales endodérmicas indiferenciadas que están presentes en la mucosa. El adenoma carcinoide está compuesto por dos tipos de células: exocrinas y neuroendocrinas, estas últimas son capaces de liberar granulaciones y neuropéptidos que se detectan en la inmunohistoquímica. La mayoría se presentan con síntomas vagos de la esfera otológica, siendo raras las manifestaciones sistémicas de síndrome carcinoide. No existe examen físico característico ni patrón imagenológico. Se debe hacer diagnóstico diferencial con colesteatoma y otros tumores de oído medio. El diagnóstico definitivo es anatomopatológico y el tratamiento de elección es quirúrgico dado el potencial destructivo local. Se presentan en este artículo dos casos de adenoma carcinoide de oído medio.

Primary neoplasms of the middle ear are rare and even more so is the middle ear adenoma. It is stated that the tumor originates from undifferentiated endodermal stem cells that are present in the mucosa. Carcinoid adenoma is compromised of two cell types; exocrine and neuroendocrine cells, the latter are able to release neuropeptides and granulations that can be detected in immunohistochemistry. Most cases present with vague symptoms of the otologic sphere, being less common the systemic manifestations of carcinoid syndrome. There is no physical examination or characteristic imaging pattern. Differential diagnosis must be done with cholesteatoma and other middle ear tumors. The definitive diagnosis is anatomopathological and the treatment of choice is surgery given the local destructive potencial. Two cases of middle ear carcinoid adenoma are presented in this article.

[Appendiceal carcinoid tumors. Evaluation of long-term outcomes in a tertiary level]. / Tumores carcinoides apendiculares. Evaluación de los resultados a largo plazo en un hospital de tercer nive.

BACKGROUND: Appendiceal tumors are found in about 1% of appendectomies performed and 0.5% of intestinal neoplasias. Appendiceal carcinoids are the predominant histology in this group and are usually casual after appendectomy for other reasons. The prognosis is excellent and survival is 95% at 5 years after surgery. METHODS: Retrospective analysis of all patients with appendiceal carcinoid surgery in our hospital for 20 years (1990-2010) and survival at 5 years. We also discuss the need for additional treatment and testing for follow-up visits. RESULTS: 42 patients underwent surgery for appendiceal carcinoid tumor. 38 of them were operated on urgently, mostly for suspected acute appendicitis, without having reached the carcinoid tumor diagnosed preoperatively in any of them. The predominant symptomatology at admission was abdominal pain. Surgical treatment was appendectomy in 34 patients (12 laparoscopic), 7 patients required colon resections over intraoperatively by colonic involvement; only one patient required reoperation to complete right hemicolectomy. 2 patients had disseminated disease at diagnosis (liver metastases). The 5-year survival is over 95%, with no recurrence. CONCLUSIONS: The appendiceal carcinoid tumor is difficult to diagnose preoperatively. Appendectomy surgical treatment is usually sufficient, although colonic resections may be needed for dissemination. The 5-year survival is over 95%.

Antecedentes: los tumores apendiculares se encuentran en cerca de 1% de las apendicectomías y representan 0.5% de las neoplasias intestinales. El tipo de tumor más frecuente es el carcinoide apendicular, que casi siempre es un hallazgo durante la apendicectomía por otro motivo. Su pronóstico es excelente y la supervivencia es mayor de 95% a cinco años de la intervención. Objetivo: reportar una serie de casos y analizar la supervivencia media a cinco años posteriores a la identificación el tumor. Material y métodos: análisis retrospectivo (1990-2010) de pacientes con tumor carcinoide apendicular intervenidos en el servicio de Cirugía General y del Aparato Digestivo del Hospital Universitario Virgen del Rocío, Sevilla, España. Se analizaron: la supervivencia a cinco años, la necesidad de tratamiento complementario y las pruebas para seguimiento en la consulta. Resultados: se encontraron 42 pacientes intervenidos por tener un tumor carcinoide apendicular. En 38 pacientes la operación fue de urgencia, la mayoría por sospecha de apendicitis aguda, sin que en ninguno se hubiera establecido el diagnóstico de tumor carcinoide antes de la operación. El síntoma predominante al ingreso fue el dolor abdominal. El tratamiento quirúrgico fue: apendicectomía en 34 pacientes (12 por laparoscopia), en el intraoperatorio siete pacientes requirieron resecciones colónicas mayores debido a la afectación del colon; sólo uno requirió la reintervención para completar la hemicolectomía derecha. Al momento del diagnóstico dos pacientes tenían enfermedad diseminada (metástasis hepáticas). La supervivencia a cinco años fue superior a 95%, sin recidivas o tratamiento posterior de la enfermedad. Conclusiones: el tumor carcinoide apendicular difícilmente se diagnostica antes del procedimiento quirúrgico. La apendicectomía suele ser suficiente aunque en algunos pacientes las resecciones colónicas son necesarias por diseminación. La supervivencia a 5 años es superior a 95%.

Presentación de una paciente con tumor carcinoide del apéndice cecal / Presentation of a Patient with Appendiceal Carcinoid Tumor

Se presentó una paciente de 35 años de edad, puérpera de 30 días, filipina con antecedentes de salud, valorada en Consulta de Cirugía en el Hospital de Dukhan, Catar en septiembre de 2013. Con dolor abdominal localizado en fosa iliaca derecha de un día de evolución, acompañado de un vómito, sin otros síntomas asociados. Luego del examen físico, hemograma completo, química sanguínea, análisis de orina y ultrasonido abdominal se decidió ingreso y tratamiento quirúrgico por el diagnóstico de apendicitis aguda. Se realizó apendicetomía de urgencia por vía videolaparoscópica, donde se observó macroscópicamente, el apéndice engrosado y aumentado de tamaño, cubierto por epiplón con discreta colección amarillenta; se envió la muestra al Departamento de Anatomía Patológica donde por técnicas especiales se confirmó el diagnóstico de tumor carcinoide del apéndice cecal. La paciente fue egresada 72 h después con evolución satisfactoria, fue seguida por consulta externa(AU)

A 35-year-old patient, Philippine female patient with 30 days of puerperium, no previous disease, who came to the Emergency Department of Surgery at Qatar, Dukhan hospital in September 2013 complaining of abdominal pain localized in right iliac fossa with a day of evolution associated by one episode of vomiting without any other symptoms. After physical examination, complete blood count, blood chemistry, urinalysis and abdominal ultrasound the patient was admitted for surgical treatment because of the diagnosis of acute appendicitis, emergent video laparoscopic appendectomy was performed which showed an enlarged and thickened appendix surrounded by the omentum with slight yellowing discharge around. Sample was sent to pathology department. Appendiceal carcinoid tumor was confirmed. The patient was discharged after 72 hours with satisfactory evolution and followed by consultation(AU)