Nodular image in the appendix observed on ultrasound: endometriosis or neuroendocrine neoplasia?

Objective: To evaluate the association between clinical and imaging with surgical and pathological findings in patients with suspected neuroendocrine tumor of appendix and/or appendix endometriosis. Methods: Retrospective descriptive study conducted at the Teaching and Research Institute of Hospital Israelita Albert Einstein, in which medical records and databases of patients with suspected neuroendocrine tumor of appendix and/or endometriosis of appendix were analyzed by imaging. Results: Twenty-eight patients were included, all of which had some type of appendix alteration on the ultrasound examination. The pathological outcome of the appendix found 25 (89.3%) lesions compatible with endometriosis and three (10.7%) neuroendocrine tumors. The clinical findings of imaging and surgery were compared with the result of pathological anatomy by means of relative frequency. Conclusion: It was possible to observe a higher prevalence of appendix endometriosis when the patient presented more intense pain symptoms. The image observed on ultrasound obtained a high positive predictive value for appendicular endometriosis.

Which is the appropriate surgical procedure for appendiceal adenocarcinoma: appendectomy, partial colectomy or right hemicolectomy?

OBJECTIVE: The purpose of this study was to explore the appropriate surgical procedure and clinical decision for appendiceal adenocarcinoma. METHODS: A total of 1,984 appendiceal adenocarcinoma patients from 2004 to 2015 were retrospectively identified from the Surveillance, Epidemiology, and End Results (SEER) database. All patients were divided into three groups based on the extent of surgical resection: appendectomy (N = 335), partial colectomy (N = 390) and right hemicolectomy (N = 1,259). The clinicopathological features and survival outcomes of three groups were compared, and independent prognostic factors were assessed. RESULTS: The 5-year OS rates of patients who underwent appendectomy, partial colectomy and right hemicolectomy were 58.3%, 65.5% and 69.1%, respectively (right hemicolectomy vs appendectomy, P < 0.001; right hemicolectomy vs partial colectomy, P = 0.285; partial colectomy vs appendectomy, P = 0.045). The 5-year CSS rates of patients who underwent appendectomy, partial colectomy and right hemicolectomy were 73.2%, 77.0% and 78.7%, respectively (right hemicolectomy vs appendectomy, P = 0.046; right hemicolectomy vs partial colectomy, P = 0.545; partial colectomy vs appendectomy, P = 0.246). The subgroup analysis based on the pathological TNM stage indicated that there was no survival difference amongst three surgical procedures for stage I patients (5-year CSS rate: 90.8%, 93.9% and 98.1%, respectively). The prognosis of patients who underwent an appendectomy was poorer than that of those who underwent partial colectomy (5-year OS rate: 53.5% vs 67.1%, P = 0.005; 5-year CSS rate: 65.2% vs 78.7%, P = 0.003) or right hemicolectomy (5-year OS rate: 74.2% vs 53.23%, P < 0.001; 5-year CSS rate: 65.2% vs 82.5%, P < 0.001) for stage II disease. Right hemicolectomy did not show a survival advantage over partial colectomy for stage II (5-year CSS, P = 0.255) and stage III (5-year CSS, P = 0.846) appendiceal adenocarcinoma. CONCLUSIONS: Right hemicolectomy may not always be necessary for appendiceal adenocarcinoma patients. An appendectomy could be sufficient for therapeutic effect of stage I patients, but limited for stage II patients. Right hemicolectomy was not superior to partial colectomy for advanced stage patients, suggesting omission of standard hemicolectomy might be feasible. However, adequate lymphadenectomy should be strongly recommended.

¿Plastrón o tumor apendicular? / Plastron appendicitis or appendiceal tumor?

Un plastrón apendicular es una masa apendicular palpable que contiene el apéndice inflamado, vísceras adyacentes y el omento mayor, asociado a o no a la presencia de pus. El manejo del plastrón apendicular abscedado es controvertido. Cuando hay signos de sepsis asociados, la indicación es una intervención quirúrgica de urgencia, de lo contrario puede optarse por un tratamiento conservador. La utilización de drenaje percutáneo tiene una alta tasa de efectividad, aunque la tasa de neoplasias no detectadas presenta un porcentaje no despreciable, especialmente en los pacientes mayores de 40 años. El objetivo de esta presentación es comunicar una paciente de 65 años con diagnóstico presuntivo de plastrón apendicular abscedado quien luego del fracaso de los drenajes percutáneos fue sometida a una laparotomía exploratoria y hemicolectomía derecha. El diagnóstico histopatológico fue de adenocarcinoma mucinoso del apéndice. (AU)

A palpable inflammatory appendiceal mass may contain the inflamed appendix, adjacent viscera, and the greater omentum, associated or not with the presence of pus. The management of an inflammatory appendiceal mass is controversial. When there are associated signs of sepsis, the indication is emergency surgery, otherwise, you can opt for a conservative treatment. The use of percutaneous drainage has a high rate of effectiveness, although the rate of undetected neoplasia is not negligible, especially in patients older than 40 years. We present the case of a 65-year-old female patient with a presumptive diagnosis of an appendiceal mass with abscess, who underwent exploratory laparotomy and right hemicolectomy after percutaneous drainage failure. The histopathological diagnosis was mucinous adenocarcinoma of the appendix. (AU)

Adenomiosis apendicular: reporte de dos casos / Appendicular adenomiosis: report of two cases

Resumen Los adenomiomas son tumoraciones benignas constituidas por un agregado nodular de músculo liso, glándulas endometriales y estroma endometrial. La presencia de adenomiomas fuera del útero es un hallazgo infrecuente. Presentamos dos casos de adenomiomas extrapélvicos localizados en el apéndice. El estudio histológico resultó esencial para el diagnóstico.

Abstract Adenomyomas are a benign tumor compound of smooth muscle nodular aggregate, endometrial glands and endometrial stroma. Adenomyomas presenting outside uterus are a rare finding. Here we report two extrapelvic adenomyomas of the appendix. Histological examination was essential for diagnosis.

Mucinous adenocarcinoma of the appendix with inguinal node metastases

Mucinous adenocarcinoma of the appendix is a rare neoplasm with a low propensity for lymph node metastasis. The present case refers to an appendicular mucinous adenocarcinoma with inguinal lymph node metastasis. A 71-year-old woman underwent an appendectomy due to a clinical presentation of acute appendicitis. However, the histological examination of the surgical specimen revealed a mucinous adenocarcinoma of the appendix. After staging, the patient underwent a right hemicolectomy and was proposed for adjuvant chemotherapy. At the 3rd year of follow-up, inguinal lymphadenopathy was diagnosed, which biopsy confirmed inguinal node metastases from primary colorectal cancer, with areas of extracellular mucin. Restaging revealed liver and peritoneal metastasis, and the patient was proposed for palliative chemotherapy. Appendicular neoplasms, due to their rarity, represent a diagnostic and therapeutic challenge. This clinical case depicts an unusual metastasis pathway for an unusual neoplasm.

A rare cecal subepithelial tumor in a Crohn´s Disease patient

Appendiceal tumors comprise a variety of histologic types, including appendiceal mucinous neoplasms, which can be grouped as premalignant lesions, tumors of uncertain malignant potential, and malignant lesions. The appendiceal mucinous neoplasms are characterized by mucinous epithelial proliferation with extracellular mucin and pushing tumor margins, commonly an incidental finding during operative exploration. We report the case of a low-grade appendiceal mucinous neoplasm presenting as a subepithelial lesion in Crohn´s Disease patient. The diagnosis was not straightforward, and only surgical resection allowed an accurate diagnosis. Although Inflammatory Bowel Disease is a risk factor for the development of colorectal neoplasms, the absolute risk for appendiceal tumors is uncertain. The frequency of progression to malignancy remains to be determined.

[Appendiceal collision tumor: Mucinous epithelial neoplasm and neuroendocrine tumor]. / Tumor de colisión apendicular: Neoplasia epitelial mucinosa y tumor neuroendocrino.

Introduction: An appendiceal collision tumor is defined as the presence of histologically distinct types of tumors in the appendix without transition between them. Appendiceal tumors are rare, with epithelial neoplasms and neuroendocrine tumors being the most common. Objective: Report the case of woman with acute appendicitis and diagnosis of an appendiceal collision tumor. Case presentation: A 35 years old woman, who consulted for a 24 hours of continuous epigastric pain with migration to the right iliac fossa; the routine blood test showed leukocytosis with neutrophilia and an ultrasound scan abdomen demonstratedsigns in favor of acute appendicitis; a diagnosis of acute appendicitis and surgical treatment. The anatomopathological study and immunostaining revealed well-differentiated neuroendocrine neoplasia and low-grade mucinous epithelial neoplasia. A follow-up with postsurgical tumor markers was performed with normal results, in addition to computed tomography and postoperative colonoscopy without alterations. Discussion: Appendiceal collision tumor is infrequently. Generally the diagnosis is made with anatomopathological study and supposes great challenges for postoperative control because there are no guidelines to follow up. Conclusion: More research should be done in order to have recommendations for this disease. Conclusion: More research should be done in order to have recommendations for this disease.

Introducción: Se define a un tumor de colisión apendicular, como la presencia de dos tipos de tumores histológicamente distintos, dentro del apéndice sin transición entre ellos. Los tumores apendiculares son poco frecuentes, siendo las neoplasias epiteliales y los tumores neuroendocrinos los más comunes. Objetivo: Presentar el caso de una paciente con diagnóstico de tumor de colisión apendicular que se presentó como apendicitis aguda. Caso clínico: Mujer de 35 años de edad sin antecedentes patológicos que consultó por dolor epigástrico de 24 horas de evolución, con migración a fosa ilíaca derecha, de tipo continuo. El examen físico reveló un abdomen blando con defensa en fosa ilíaca derecha; el análisis de sangre mostró leucocitosis con neutrofilia y una ecografía en favor de apendicitis aguda. Se realizó apendicectomía mediante incisión de Mc Burney. El estudio anatomopatológico e inmunomarcación informó neoplasia neuroendocrina bien diferenciada y neoplasia epitelial mucinosa de bajo grado. Se realizó seguimiento con marcadores tumorales postquirúrgicos con resultados normales, además de tomografía computada y colonoscopia postquirúrgicos normales. Dsicusión: El tumor de colisión apendicular es muy poco frecuente. Generalmente el diagnóstico se realiza con el estudio anatomopatológico y plantea grandes desafíos para el control, ya que no existe consenso de cómo realizar el seguimiento postquirúrgico . Conclusión: Se debe investigar más al respecto para contar con recomendaciones de vigilancia para dicha enfermedad

Primary Functioning Neuroendocrine Tumor of the Appendix with Hypoglycemia Syndrome: A Case Report and Review of Neuroendocrine Tumors.

INTRODUCTION: Primary neuroendocrine tumors (NETs) of the appendix are uncommon, but when present are usually not hyperfunctioning. This case represents an extraordinarily rare primary hyperfunctioning NET of the appendix with a clinical presentation of symptomatic hypoglycemia in an otherwise healthy man. CASE PRESENTATION: A previously healthy 34-year-old man was found to be symptomatically hypoglycemic in early 2018. After the workup, the apparent explanation was a tumor in his midappendix, for which he was referred to our surgical oncology service for resection. The patient's clinical course is described in detail, including imaging, as well as surgical and pathologic descriptions of the appendiceal NET. DISCUSSION: A literature review demonstrates no other case series or reports of a primary hyperfunctioning NET of the appendix presenting with hypoglycemic crisis. The symptoms of tumor-induced hypoglycemia more typically arise with islet cell tumors, such as pancreatic NETs or insulinomas. We believe this case represents a nonislet cell tumor-induced hypoglycemia. This patient's elevated serum proinsulin level preoperatively implies secretion of proinsulin by the tumor. However, tumor-induced hypoglycemia caused by proinsulin has been described previously only in pancreatic tumors. This unique case adds knowledge to the possible glycemic endocrine effects of nonpancreatic NETs, specifically those that arise primarily in the appendix.

ΔNp73 status in peritoneal and ovarian dissemination of appendicular adenocarcinoids (goblet cells).

INTRODUCTION: Goblet cell carcinoma (GCC) is an appendicular neoplasia representing less than 5% of all appendicular tumors, found in 0.3-0.9% of the appendectomies, 35-58% of all appendicular neoplasms, and less than 14% of malign appendix tumors. The most frequent clinical presentation is abdominal pain associated with a picture of acute appendicitis. MATERIALS AND METHODS: We present 3 clinical cases of appendix GCC, 2 subjected to cytoreductory surgery plus intraperitoneal hyperthermic chemotherapy and a third, who is currently receiving neoadjuvant treatment with a good response to chemotherapy and who will be offered the same treatment as the first two patients. Given the unpredictable behavior of these tumors, the use of molecular markers could help us to predict their behavior and prognosis. In this context, the TP73 gene would make an interesting putative marker. ∆Np73 has been described as overexpressed in a great variety of tumor types including colon cancer and this up-regulation is associated with a poor prognosis. To evidence its role in this malignancy, we evaluate here the status of ∆Np73 in the primary tumor and normal counterpart tissues, in the metastatic implants and in healthy areas of the peritoneum from the appendicular GCC patients. In addition, we checked the expression levels of this p73 variant in the tumor and normal tissue of 26 patients with colon cancer. RESULTS: Remarkably, 2 patients showed significant ∆Np73 down-regulation in both the primary tumor and the implants. Case 1 presented a fourfold decrease of levels in the primary tumor and 20-fold decrease in the implants. Case 2 showed a seven- and fourfold down-regulation in the primary tumor and implants, respectively. However, Case 3 showed an up-regulation of 53- and threefold in the primary tumor and implants, respectively. CONCLUSION: Goblet cell carcinoma of the appendix is very rate. It tends to seed throughout the peritoneum, making aggressive surgical cytoreduction and chemotherapy viable treatment options. Investigation into the molecular basis of these tumors may improve the diagnosis, prognosis and therapeutic decisions regarding these patients. ∆Np73 seems a good candidate for further analysis in longer series.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei and appendix tumours in elderly patients: Is it justified?

BACKGROUND: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) for pseudomyxoma peritonei and appendix tumours are widespread in the world. It is unclear what should be the attitude in elderly patients. METHODS: This retrospective multicenter study collected the database from ten Spanish centers from Spanish Group of Peritoneal Cancer Surgery. The study period was between November 2002 and March 2014. Seventeen patients with age greater than or equal to 75 years with peritoneal carcinomatosis from pseudomyxoma peritonei and appendix tumours met the selection criteria for the study. Outcomes in terms of morbidity and mortality such as disease-free and overall survival were analyzed. RESULTS: Median PCI was 16 (range 6-39). Ten postoperative adverse events were detected in nine patients (44.4%). 28% were grade I-II and 17% were grade III-IV. Disease-free survival at 1 and 3 years was 67 and 44%, respectively. Overall survival at 1 and 3 years was 100 and 88%, respectively. Only cytoreduction was related to worst disease free survival after univariate (p = 0.007) and multivariate (OR 11.639, 95% CI 1.24-109.74, p = 0.03) analyses. Cytoreduction was related to the worst overall survival after univariate analysis (p = 0.046). CONCLUSION: Cytoreductive surgery and HIPEC for pseudomyxoma peritonei and appendix tumours in elderly patients it is a procedure with feasible postoperative morbi-mortality and survival outcomes. TRIAL REGISTRATION: researchregistry1587 (retrospectively registered).

Análisis de casos de adenocarcinoma apendicular y su manejo en una serie de 10 años en el Hospital Doctor Sótero del Río / Analysis of cases of appendicular adenocarcinoma and its management in a series of 10 years in Hospital Doctor Sótero del Río

Introducción: Los tumores del apéndice cecal son una patología infrecuente. De ellos, los adenocarcinomas apendiculares no superan el 0,1% de las apendicectomías. Objetivos: Describir el manejo y la sobrevida de pacientes con diagnóstico de adenocarcinoma apendicular en el Complejo Asistencial Dr. Sótero del Río. Materiales y métodos: Análisis retrospectivo descriptivo de pacientes operados de apendicectomía y diagnóstico patológico de adenocarcinoma apendicular entre enero de 2003 y diciembre de 2013. Se evaluaron características demográficas, síntomas, manejo y sobrevida. Resultados: Se analizaron un total de 14.582 apendicectomías. Se obtuvieron 84 biopsias positivas para tumores apendiculares primarios (0,58%). De este grupo, 9 biopsias correspondían a un adenocarcinoma apendicular (0,06%). Siete de los 9 pacientes poseían registro completo. La mediana de edad fue de 53 años. Seis pacientes cursaron con un cuadro de abdomen agudo. Se realizó una hemicolectomía derecha en 6 pacientes posterior al diagnóstico de adenocarcinoma. De ellos, 4 recibieron quimioterapia adyuvante. La sobrevida a 3 años fue del 58%. Conclusiones: El adenocarcinoma apendicular es un hallazgo poco frecuente durante una apendicectomía y se asocia a una sobrevida similar a la reportada internacionalmente.

Introduction: Appendiceal tumors are a rare pathology, not exceeding 0.1% of appendectomies. Objectives: Describe the management and survival of patients diagnosed with appendiceal adenocarcinoma at the Complejo Asistencial Doctor Sótero del Río. Materials and methods: Descriptive retrospective analysis of patients underwent appendectomy, with histopathologic diagnosis of appendiceal adenocarcinoma, in the period from January 2003 to December 2013. Demographic characteristics, symptoms, management and survival were evaluated. Results: Data from 14,582 appendectomies was analyzed; 84 positive biopsies for primary appendiceal tumors (0.58%) were obtained. Of this group, 9 biopsies corresponded to an appendiceal adenocarcinoma (0.06%). Seven of the 9 patients had complete record. The median age was 53 years. Six patients presented with an acute abdomen. Right hemicolectomy underwent 6 patients. Of these, 4 received adjuvant chemotherapy. The 3-year survival was 58%. Conclusions: Appendiceal adenocarcinoma is a rare finding during an appendectomy and is associated with a low overall survival.

Giant appendiceal mucinous cystadenoma treated by laparoscopy: a case report and review of the literature / Cistadenoma mucinoso apendicular gigante tratado por laparoscopia: relato de caso e revisão de literatura

ABSTRACT Appendiceal mucinous cystadenoma is a rare entity, which causes appendicular mucocele. It is more frequent in women over 50 years old. In half of the cases it is asymptomatic. Tomography of the abdomen is the gold standard in its preoperative diagnosis. The treatment is surgical, with good prognosis, the complete resection evolves without appendicular rupture and extravasation. We report a case of a 64-year-old man with appendiceal mucinous cystadenoma. A laparoscopic right hemicolectomy was performed. This therapy that can be safely used to treat appendiceal mucocele, as long as it is cautious.

RESUMO O cistadenoma mucinoso apendicular é entidade rara que causa mucocele apendicular, sendo mais frequente em mulheres acima dos 50 anos. Em metade dos casos, o cistadenoma mucinoso apendicular é assintomático. A tomografia do abdome é o padrão-ouro para um diagnóstico pré-operatório. O tratamento é cirúrgico e tem bom prognóstico; a ressecção completa evolui sem ruptura apendicular e sem extravasamento. Relatamos um caso de paciente homem de 64 anos com cistadenoma mucinoso apendicular. Foi realizada hemicolectomia laparoscópica direita. Esse é um procedimento que pode ser usado com segurança no tratamento de mucocele apendicular, desde que seja executado com cautela.

Management and outcome of children with neuroendocrine tumors of the appendix in Spain: Is there room for improvement?

PURPOSE: Neuroendocrine tumors (NETs) are, after lymphomas, the most frequent gastrointestinal tumors in children, mainly located in the appendix. Best management remains unclear, given the absence of pediatric guidelines. We present the first Spanish series of pediatric patients with NETs. PATIENTS AND METHODS: Retrospective study of all pediatric patients (<18 years) with NET treated in four oncology reference institutions in Spain between 1994 and 2015. RESULTS: Seventeen patients were included. All patients presented with acute abdomen. TNM stage was T1a (82%) and T1b (12%). Extension study was heterogenous, with only 4 patients undergoing an OctreoScan. Four patients met criteria for second surgery (affected surgical margins or mesoappendix invasion), but it was only performed in two. Despite the diverse management, none of the patients relapsed during follow-up. CONCLUSIONS: The disparity in diagnostic tests, second surgery criteria and follow-up shown in this study highlights the need for specific pediatric guidelines.

Appendiceal carcinoid tumor in children: implications for less radical surgery?

The discovery of an appendiceal carcinoid tumor found incidentally or during the course of diagnostic or therapeutic procedures is a burden to both the patient and clinician. The role of the correct surgical operation is paramount for lesions suspected to be malignant. In the pediatric population, appendiceal carcinoids continue to challenge the clinician in choosing the optimal treatment when lesions are larger than 2 cm or involve the appendical base. While the criteria used to define these distinct lesions are available in most cases, the management and treatment are still debated and controversial when considering more radical surgical intervention. The purpose of this article is to give an overview regarding the history, diagnosis, histopathology, management, and controversies associated with appendiceal carcinoid in the pediatric population.

Carcinoid tumor of cecal appendix: one-year incidence at the Santa Marcelina Hospital / Tumor Carcinoide de apêndice cecal: incidência em um ano no Hospital Santa Marcelina

Introduction: Carcinoid tumors are neuroendocrine malignancies that originate in the neuroectodermal cells of the Amine, Peptide Uptake and Decarboxylation system dispersed in the gastrointestinal mucosa and representing about 80-88% of tumors of cecal appendix. These are tumors usually diagnosed at appendectomies, and it is estimated that from each 100 appendectomies yearly performed, at least one case is a neuroendocrine tumor. Objectives: To report the experience of an University Teaching Hospital in health and reference at the east side of São Paulo and great São Paulo in cases of these rare appendicular tumors, with emphasis on the importance of these descriptions, as probably are rare those surgeons in particular who will acquire extensive wisdom in these cases. Method: Retrospective analysis of 237 patients who underwent appendectomy from September 2010 to September 2012 in the Hospital Santa Marcelina-SP. We evaluated data on age, gender, initial clinical presentation and surgical findings of patients undergoing appendectomy with subsequent anatomic and immunopathological diagnosis of carcinoid tumor of cecal appendix. Results: The presence of a carcinoid tumor of the appendix was observed in 5 patients, which corresponds to 2.1% of all appendectomies performed. Regarding gender, 4 patients (80%) were female and the average age was 34.2 years, with a range from 17 to 68 years. In all patients the initial hypothesis for surgery indication was acute appendicitis, with an intraoperative finding of necroperforated phase acute appendicitis in 3 patients (60%). Conclusion: The therapeutical conduct after the diagnosis of carcinoid tumors of the appendix must be based on the data provided by pathological and immunohistochemical studies, besides the judicious judgment of the attending physician. (AU)

Introdução: Os tumores carcinoides são neoplasias malignas neuroendócrinas que se originam em células neuroectodérmicas do sistema APUD (Amine, Peptide Uptake and Decarboxylation), dispersas na mucosa gastrointestinal e que representam cerca de 80-88% das neoplasias do apêndice cecal. São tumores diagnosticados geralmente durante apendicectomias e estima-se que de cada 100 apendicectomias realizadas por ano, ao menos um caso será TNE. Objetivos: Objetiva-se nesse artigo relatar experiência de Hospital Universitário e de Ensino (HUE) em saúde e referência na zona leste de São Paulo e grande São Paulo em casos desses raros tumores apendiculares, com ênfase na importância dessas descrições, já que provavelmente raros cirurgiões em particular irão adquirir uma extensa sapiência nesses casos. Método: Análise retrospectiva de 237 pacientes submetidos à apendicectomia no período de setembro de 2010 a setembro de 2012 no Hospital Santa Marcelina-SP. Foram avaliados os dados referentes a idade, sexo, quadro clínico inicial, achados operatórios dos pacientes submetidos à apendicectomia com posterior diagnóstico anatomopatológico e imunopatológico de tumor carcinoide de apêndice. Resultados: Verificou-se a presença de tumor carcinoide de apêndice em 5 pacientes, o que corresponde a 2,1% das apendicectomias realizadas. Com relação ao gênero, 4 pacientes (80%) eram mulheres e a média de idade foi de 34,2 anos, com variação de 17 a 68 anos. Em todos os pacientes a hipótese inicial para indicação de cirurgia fora de apendicite aguda, com achado intra-operatório de apendicite aguda em fase necroperfurada em 3 pacientes (60%). Conclusão: A conduta após o diagnóstico de tumores carcinoides de apêndice cecal deve ser alicerçada nos dados fornecidos por exames anatomopatológicos e imunoistoquímicos, além do julgamento criterioso do médico assistente. (AU)

[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%.

Progression of peritoneal adenomucinosis to the scrotum: a rare occurrence treated with cytoreductive surgery and hyperthermic chemoperfusion of the scrotum in two patients.

INTRODUCTION: Disseminated Peritoneal Adenomucinosis (DPAM) is an infrequent presentation of appendiceal cancer. Infrequently, umbilical or inguinal hernias could be the first clinical manifestation of this condition; DPAM extension to the scrotum may be anatomically viable. Treatment with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is the standard of treatment for DPAM. We hypothesize that these same treatment principles, consisting of CRS with hyperthermic chemoperfusion of the scrotum (HCS), could be applied to the scrotal dissemination of DPAM. METHODS: We reviewed our Institution's prospective cancer database and identified two cases of DPAM with extension to the scrotum. Their medical records were examined, and close follow-up was performed. Tumor histopathology and cytoreduction scores were evaluated. Tumor progression was monitored on follow-up by physical examination, tumor markers (CEA, CA 125, CA 19.9) and abdomino-pelvic CT scan. RESULTS: Two patients who previously had CRS/ HIPEC for DPAM were successfully treated with HSC. Both patients are alive and free of disease at 88 and 57 months following initial CRS/HIPEC, and 50 and 32 months following CRS/HCS, respectively. CONCLUSION: Increased awareness by surgeons to the coexistence of inguinal hernia with peritoneal neoplasm and the need for a surgical repair is raised. CRS/HCS may be employed to treat patients with DPAM extension to the scrotum. Successful outcome is dependent on complete cytoreduction of metastatic tumor.

INTRODUCCIÓN: La Adenomucinosis Peritoneal Diseminada (DPAM, por el término en inglés) es una presentación no frecuente del cáncer de apéndice. Infrecuentemente, las hernias umbilicales o inguinales pueden ser la primera manifestación clínica de esta condición; la extensión al escroto puede ser anatómicamente viable. La cirugía citoreductiva (CRS, por el término en inglés) con quimioterapia hipertérmica intraperitoneal (HIPEC, por el término en inglés) es el tratamiento estándar para DPAM. Nuestra hipótesis es que los mismos principios terapéuticos, consistentes en CRS con quimioterapia hipertérmica del escroto (HCS), pueden ser aplicados para DPAM con extensión al escroto. MÉTODOS: Revisamos una base de datos prospectiva en nuestra Institución donde se identificaron dos casos de DPAM con extensión al escroto. Se examinaron sus historias clínicas, y se realizaron controles cercanos. La histopatología tumoral y la citoreducción fueron evaluados. La progresión tumoral fue monitorizada en los controles mediante examen físico, marcadores tumorales (CEA, CA 125, CA 19.9) y TAC abdomino-pélvico. RESULTADOS: Dos pacientes a quienes se les practicó previamente CRS/HIPEC por DPAM fueron exitosamente tratados con CRS/HSC. Ambos pacientes se encuentran vivos y sin evidencia de enfermedad 88 y 57 meses después de la CRS/HIPEC inicial y a 50 y 32 meses post CRS/HCS, respectivamente. CONCLUSIÓN: La precaución de los cirujanos sobre la coexistencia de hernias inguinales con neoplasias peritoneales y la necesidad de reparo quirúrgico debe ser incrementada. La CRS/HCS puede ser empleada en el tratamiento a pacientes con DPAM con extensión al escroto. Los resultados clínicos son dependientes de una citoreducción completa del tumor metastásico.

GNAS is frequently mutated in both low-grade and high-grade disseminated appendiceal mucinous neoplasms but does not affect survival.

We analyzed a series of 55 disseminated appendiceal mucinous neoplasms treated at our institution for GNAS and KRAS mutations in an attempt to correlate mutation status with clinicopathological findings and patient survival. GNAS mutations (p.R201H, c.602G>A and p.R201C, and c.602C>T) were identified in 17 (31%) of 55 of disseminated mucinous neoplasms and were found in 8 (35%) of 23 low-grade mucinous neoplasms, 7 (37%) of 19 high-grade mucinous adenocarcinomas lacking a signet ring cell component, and 2 (15%) of 13 high-grade mucinous adenocarcinomas with a signet ring cell component. All 7 mucinous adenocarcinomas composed of pure (>95%) signet ring cells harbored wild-type GNAS. There was no significant association between GNAS mutations and sex and age (both with P > .05) or between GNAS mutations and individual adverse histologic features including cytologic grade, destructive invasion, tumor cellularity, angiolymphatic invasion, perineural invasion, and signet ring cells (all with P > .05). KRAS mutations were identified in 22 (40%) of 55 disseminated mucinous neoplasms. GNAS-mutated disseminated appendiceal mucinous neoplasms more frequently harbored concurrent KRAS mutations compared with GNAS wild-type tumors (65% versus 29%, P = .018). GNAS mutations were not significantly associated with overall survival (both with P > .05). Only overall tumor grade was an independent predictor of overall survival in the multivariate analysis (P = .01). Our results indicate that GNAS mutations are frequently identified in both low-grade and high-grade disseminated appendiceal mucinous neoplasms indicating that GNAS mutation status cannot be used to distinguish between low-grade from high-grade appendiceal mucinous neoplasms.