Apendicitis aguda por intususcepción secundaria a endometriosis. Reporte de caso / Acute appendicitis due to intussusception secondary to endometriosis. Case report

Introducción. La intususcepción del apéndice corresponde a su invaginación en el ciego. Existen varias causas, pero la endometriosis ha sido informada pocas veces. Aunque el diagnóstico se debe sospechar clínicamente, por lo general su causa solo se determina en el intraoperatorio, donde se deben tener en cuenta causas oncológicas que requieran una resección amplia. Caso clínico. Mujer de 21 años que consultó por dolor abdominal agudo generalizado. Se practicó una tomografía computarizada de abdomen, observando una intususcepción del apéndice en el ciego, estriación de la grasa pericecal y adenomegalias. Se realizó laparoscopia diagnóstica encontrando intususcepción casi completa del apéndice cecal, de aspecto neoplásico. Se convirtió a laparotomía para proceder a hemicolectomía derecha, con vaciamiento ganglionar y anastomosis del íleon al colon transverso. Discusión. La sospecha clínica de intususcepción debe corroborarse mediante ecografía, tomografía o estudios baritados. El tratamiento siempre es quirúrgico, como en el caso de nuestra paciente, quien evolucionó de forma adecuada y continuó asintomática después de un año de seguimiento. Conclusión. El diagnóstico temprano de la intususcepción permite realizar tratamientos quirúrgicos menos agresivos y disminuye el riesgo de filtración de la anastomosis. Se debe tener en cuenta el diagnóstico de endometriosis como posible causa. Se debe realizar el manejo complementario por parte de ginecología.

Introduction. The intussusception of the appendix corresponds to its invagination in the cecum. There are several causes, endometriosis being rarely reported. Although the diagnosis must be suspected clinically, its cause is generally only determined intraoperatively, where oncological causes that require extensive resection must be taken into account. Clinical case. A 21-year-old woman who consulted due to acute generalized abdominal pain, an abdominal tomography was performed, finding an intussusception of the appendix in the cecum, striation of pericecal fat, and lymph nodes. A diagnostic laparoscopy was performed, finding almost complete intussusception of the appendix, with a neoplastic appearance. She was converted to laparotomy to perform a right hemicolectomy, with lymph node dissection and ileal to transverse anastomosis. Discussion. Clinical suspicion of intussusception should be confirmed by ultrasound, abdominal tomography, or barium studies. Treatment is always surgical, as in the case of our patient, who evolved adequately and remained asymptomatic after one year of follow-up. Conclusion. Early diagnosis of intussusception allows for less aggressive surgical treatment and decreases the risk of anastomosis leakage. The diagnosis of endometriosis should be taken into account as a possible cause. Complementary management by gynecologists should be performed.

Analysis of appendiceal neoplasms in 1,423 appendectomy specimens: a 10-year retrospective cohort study from a single institution

SUMMARY OBJECTIVE: This study aimed to reveal the incidence, clinicopathological, and oncological outcomes of appendiceal neoplasms. METHODS: This is a retrospective cohort study from a single institution. Patients with a pathological diagnosis of malignancy who underwent appendectomy between January 2011 and 2021 were included in the study, and groups were formed according to pathological type. Clinical, pathological, and oncological results were compared in these groups. RESULTS: The incidence of neoplasia was 2.38% (n=34) in a cohort of 1,423 appendectomy cases. Of the cases, 56% (n=19) were female. The median age in the entire cohort was 55.5 (range: 13-106) years. In the cohort, the rate of neuroendocrine tumor mucinous cystadenoma adenocarcinoma, and low-grade appendiceal mucinous neoplasm, according to the American Joint Committee on Cancer classification of appendiceal neoplasms, was 32.3% (n=11), 26.4% (n=9), 26.4% (n=9), and 14.7% (n=5), respectively. Neuroendocrine tumor patients (median age: 35 years) were younger than the other groups (p=0.021). Secondary complementary surgery was performed in 66.7% (n=6) of adenocarcinoma patients and 27.3% (n=3) of neuroendocrine tumor patients. Right hemicolectomy was performed in all neuroendocrine tumor patients requiring secondary surgery, while right hemicolectomy was performed in three adenocarcinoma patients and cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in three adenocarcinoma patients. After a median follow-up of 44.4 months (95% confidence interval: 18.6-70.1), the mean survival rate was 55% in appendiceal adenocarcinoma patients compared to 100% in neuroendocrine tumor patients. CONCLUSION: Appendiceal neoplasms are rare but remain an important cause of mortality. Appendiceal adenocarcinomas are associated with poorer oncological outcomes compared to other neoplasms.

The diagnostic value of KRAS gene mutation in appendiceal adenocarcinoma / 中国医师杂志

Objective:To analyze the mutation characteristics of Kirsten rat sarcoma virus oncogene homology (KRAS) gene in patients with appendiceal adenocarcinoma and its relationship with the activity of Ras Raf Mitogen activated protein kinase/extracellular regulated protein kinase (MAPK/ERK) signaling pathway.Methods:A total of 41 patients with appendiceal adenocarcinoma who were treated in the Lishui Central Hospital from January 2014 to January 2020 were selected as the observation group, and 50 patients with Appendicitis who were operated at the same time were randomly selected as the control group. Clinical and follow-up data were collected, and the mutation of the KRAS gene in the patient′s tissue was measured using the snapshot method. The expression of key proteins in the MAPK/ERK signaling pathway in cancer tissue was measured using Western blotting (WB) assay. We compared the clinical characteristics and prognosis of patients with KRAS mutation and non KRAS mutation appendiceal adenocarcinoma.Results:The KRAS gene mutation rate in the observation group was higher than that in the control group (41.5% vs 10.0%), and the expression levels of p-ARAF/ARAF, p-MEK1/MEK1, and p-ERK1/ERK1 proteins were also higher than those in the control group. The differences between the groups were statistically significant (all P<0.05). The protein expression levels of p-ARAF/ARAF, p-MEK1/MEK1, p-ERK1/ERK1 in KRAS mutation patients in the observation group were significantly higher than those in non KRAS mutation patients. The proportion of stage IV, positive rates of carcinoembryonic antigen (CEA), carbohydrate antigen (CA)199 and CA125 in KRAS mutation patients were higher than those in non KRAS mutation patients, and the survival time and progression free survival time were shorter than those in non KRAS mutation patients, with statistical significance (all P<0.05). Conclusions:The mutation rate of KRAS in appendix adenocarcinoma is high, and the activation of MAPK/ERK signaling pathway caused by KRAS mutation may play a role in the pathogenesis of appendix adenocarcinoma, which has the value of in-depth research.

Mucocele of the appendix: what to expect / Mucocele do apêndice: o que esperar

Abstract Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be neoplastic or non-neoplastic. The typical imaging appearance of a mucocele of the appendix is that of a cystic structure with a tubular morphology. This structure is defined by having a blind-ending and being contiguous with the cecum. Radiologists should be familiar with key anatomical landmarks and with the various imaging features of mucoceles of the appendix, in order to provide a meaningful differential diagnosis of a lesion in the right lower abdominal quadrant. In addition, a neoplastic mucocele can rupture, resulting in pseudomyxoma peritonei, which will change the prognosis dramatically. Therefore, prompt diagnostic imaging is crucial.

Resumo Mucoceles do apêndice são raras e podem ter uma apresentação clínica e imagiológica bastante variável, por vezes mimetizando patologia anexial. As causas subjacentes podem ser neoplásicas ou não neoplásicas. O aspecto de imagem típico de mucoceles do apêndice é o de uma estrutura de natureza cística com morfologia tubular. Esta estrutura deverá terminar "em fundo cego" e ser contígua com o ceco. Os radiologistas devem estar familiarizados com os pontos anatômicos de referência e com as diferentes características imagiológicas de mucoceles do apêndice, de modo a fornecer um adequado diagnóstico diferencial de uma lesão localizada no quadrante abdominal inferior direito. Para além disso, uma mucocele neoplásica pode sofrer ruptura, resultando em pseudomixoma peritoneal, o que altera drasticamente o prognóstico. Assim, o diagnóstico por imagem em tempo útil é crucial.

Clinicopathologic features and prognosis of appendendicular goblet cell adenocarcinoma / 中华普通外科杂志

Objective:To investigate the clinicopathological characteristics, and prognosis of appendiceal goblet cell adenocarcinoma(GCA).Methods:The clinical and pathological data of 21 GCA cases were retrospectively analyzed, and their pathological morphology, immunohistochemical phenotype, genetic alteration and clinical treatment and prognosis were studied.Results:Most of the 21 patients showed appendicitis, with appendectomy alone or extensive resection of the tumor, followed by chemotherapy. There were 12 low-grade and 9 moderate and high-grade patients by pathological examination, and tumor cells express CEA, CK20, SATB-2, Syn(19/21), CgA(18/21), CD56(18/21), Ki67(+,10%-50%),and one patient had mutations in the BRAF gene.Depth of tumor infiltration ( r=0.716, P<0.001), and TNM stage ( r=0.816, P<0.001) were all positively correlated with the grade of GCA. After 19 to 98 months of follow-up, one patient relapsed and one patient died. Conclusions:Appendiceal GCA is a kind of tumor with bidirectional differentiation characteristics. Its morphology and biological behavior lineage are relatively broad, especially the high level is more aggressive, hence more active treatment should be adopted.

Clinicopathological analysis and surgical strategy of primary appendiceal neoplasms / 中华胃肠外科杂志

Objective: To investigate the clinicopathological features, surgical methods and prognosis of primary appendiceal neoplasms. Methods: A descriptive case series study was performed. Clinical data of patients diagnosed with primary appendiceal neoplasms in Peking University First Hospital from 2006 to 2017 were retrospectively analyzed. Patients who underwent surgery and were confirmed as appendiceal neoplasms by postoperative pathology were included. Cases of cecal tumor invading the appendix and other organ tumors implanting in the appendix and cases of recurrent appendix tumors were excluded Pathological classification was based on the 4th edition of the WHO classification of digestive tract tumors (2010 edition), and the efficacy of operation methods of low grade appendiceal mucinous neoplasm (LAMN) were analyzed. Results: A total of 115 patients were enrolled, including 52 males and 63 females with a median age of 59 (51, 71) years. Clinical symptoms usually manifested as dormant pain in the right lower quadrant, migrating right lower abdominal pain, fever and bloating. Twenty-four cases were accidentally discovered during surgery, and 21 cases were found by physical examination. The preoperative diagnosis rate of CT and ultrasound was 40.2% (43/107) and 25.5% (24/94) respectively. The postoperative pathological types contained 83 cases of LAMN, 12 cases of mucinous adenocarcinoma, 9 cases of appendiceal neuroendocrine neoplasms (aNEN), 2 cases of mucinous adenocarcinoma with signet ring cells, 3 cases of serrated adenoma, 2 cases of goblet cell carcinoid, 2 cases of lymphoma, 1 case of leiomyoma and 1 case of schwannomas. All the patients underwent surgical resection, including 41 cases of appendectomy, 21 cases of partial cecectomy, 48 cases of right hemicolectomy, and 5 cases of combined organ resection due to appendiceal tumor infiltration or dissemination. Eighteen cases were diagnosed with pseudomyxoma peritonei (PMP) during operation. A total of 98 patients were enrolled for follow-up. The median follow-up time was 58 (5-172) months. The 5-year disease-free survival (DFS) rate was 84.5% and 5-year overall survival (OS) was 88.2%. Multivariate analysis revealed that high-grade malignancy tumors (HR=25.881, 95% CI: 2.827-236.935, P=0.004) and PMP formation (HR=42.166, 95% CI: 3.470-512.439,P=0.003) were independent risk factors for prognosis. Patients undergoing right hemicolectomy presented longer operation time, more blood loss and higher morbidity of complication as compared to those undergoing appendectomy and partial cecectomy (all P<0.05), while no significant differences in 5-year DFS (P=0.627) and 5-year OS (P=0.718) were found. Conclusions: Primary appendiceal neoplasms usually have no typical features, accompanied with low preoperative diagnosis rate. The common pathological types are LAMN, mucinous adenocarcinoma and aNEN. Appendectomy or partial cecectomy for LAMN may achieve satisfactory prognosis. High-grade malignancy tumors and PMP formation are independent risk factors for prognosis.

Neoplasia Mucinosa de apéndice. Abordaje laparoscópico. Reporte de caso / Neoplastic appendiceal mucinous lesion. Laparoscopic approach. Case report

El mucocele apendicular es la dilatación quística del apéndice con acumulación de material mucinoso. Su forma de presentación más frecuente es dolor en fosa ilíaca derecha, sin embargo, la mitad de los casos son asintomáticos. En el presente trabajo se presenta caso de un paciente con diagnóstico incidental de mucocele apendicular, al cual se le realizó apendicectomía laparoscópica como tratamiento definitivo. Caso clínico: Paciente masculino de 44 años sin antecedentes patológicos conocidos, quien se realiza ultrasonido abdominal que informa tumor quístico en fosa ilíaca derecha. Se decide resolución quirúrgica laparoscópica. Informe histológico: mucocele apendicular sin evidencia de malignidad y recuperación satisfactoria del paciente. Conclusión: La neoplasia mucinosa del apéndice es una patología poco frecuente, puede presentarse con una variedad de manifestaciones clínicas hasta un hallazgo incidental. El tratamiento es fundamentalmente quirúrgico y el abordaje laparoscópico es de elección en pacientes sin evidencia de ruptura y diseminación de la enfermedad(AU)

The appendicular mucocele is a cystic dilation of the appendix with accumulation of mucinous material. The most frequent form of presentation is pain in the right iliac fossa; however, half of the cases are asymptomatic patients. In the present work we present a case of a patient with an incidentally diagnosis of appendicular mucocele who underwent laparoscopic appendectomy as definitive treatment. Clinical case: A 44-year-old male patient with no known pathological history, who underwent an abdominal ultrasound that reported a cystic tumor in the right iliac fossa. Laparoscopic surgical resolution was decided, the histological examination revealed appendicular mucocele without evidence of malignancy and satisfactory recovery of the patient. Conclusion: Mucinous neoplasia of the appendix is an uncommon pathology, patients are often asymptomatic or have nonspecific symptoms, and the diagnosis is made incidentally in the course of other examination. Treatment is primarily surgical and the laparoscopic approach is the choice in patients without evidence of rupture and dissemination of the disease(AU)

Primary malignant neoplasms of the cecal appendix: unusual findings in acute appendicitis

Introduction: To evaluate the prevalence of appendix neoplasia correlating with patient profile, histological types and frequency. Methods: Data collection was performed in the Pathology Department of a General Hospital, with the objective of identifying patients diagnosed with malignant cecal appendix tumors by histopathologic study of specimens from acute appendicitis. Results: The prevalence of malignant primary epithelial neoplasia of the appendix was 1%. Fifty percent of the cases were neuroendocrine tumors, 35% were mucinous, and 15% were adenocarcinomas. The mean age at diagnosis was 41.3 (SD, 20.4) years (range 16-81), with a women/men ratio of 3:1. Discussion: Appendiceal neoplasms are rare and should be suspected manly in women over 40 years of age with suggestive symptoms of acute appendicitis. The size, location, extent, margins and presence of mucin are essential findings for the treatment of these patients. (AU)

Cistoadenoma mucinoso del apéndice: un caso con presentación inusual / Appendiceal mucinous cystoadenom: inusual presentation case

RESUMEN Fundamento: los tumores mucinosos representan alrededor del 8 % de las neoplasias apendiculares y originan dilatación quística del apéndice debido a la acumulación de material gelatinoso. El cistoadenoma mucinoso del apéndice es una rara enfermedad, que cursa de manera asintomática y se diagnostica de manera incidental mediante estudios imagenológicos o intra operatorio y representa de un 0,2 % a 0,6 % de las apendicectomías. Objetivo: presentar el caso inusual de una joven operada por apendicitis aguda que tenía además un cistoadenoma mucinoso del apéndice. Caso clínico: paciente nuligesta, de 19 años de edad con antecedentes de pérdida de peso en un período de seis meses y cuadros recurrentes de dolor en epigastrio que aliviaban con analgésicos, acudió por dolor en el cuadrante inferior derecho del abdomen de 48 horas de evolución, acompañado de náuseas y pérdida del apetito. Al examen físico se constató taquicardia y dolor abdominal a la palpación en la fosa iliaca derecha, con maniobra de Blumberg positiva, el tacto vaginal y rectal resultaron dolorosos. En el acto quirúrgico se constató apéndice cecal engrosado en el tercio proximal, turgente, con una tumoración dura en tercio distal y se realizó apendicectomía sin complicaciones. La evolución fue favorable y fue dada de alta. Conclusiones: a pesar de lo raro de esta afección, el cirujano debe conocer que en un cuadro apendicular puede estar presente este tipo de tumor aún en pacientes jóvenes a fin de tomar la conducta quirúrgica adecuada y evitar complicaciones al enfermo.

ABSTRACT Background: mucinous tumors represent about 8% of appendix neoplasms and cause cystic dilatation of the appendix due to the accumulation of gelatinous material. Mucinous cystadenoma of the appendix is a rare disease, which occurs asymptomatically and is diagnosed incidentally through imaging studies or intra-operatively and accounts for 0.2% to 0.6% of appendectomies. Objective: to present the unusual case of a 19-year-old girl operated on for acute appendicitis who also had a mucinous cystadenoma of the appendix. Clinical case: patient without previous pregnancy, with a history of weight loss in a period of 6 months and recurrent pain in the epigastrium that relieved with analgesics, came for pain in the lower right quadrant of the abdomen of 48 hours of evolution, accompanied by nausea and loss of appetite. Physical examination revealed tachycardia and abdominal pain on palpation in the right iliac fossa, with a positive Blumberg maneuver; vaginal and rectal examinations were painful. In the surgical act, a cecal appendix was found thickened in the proximal third, turgid, with a hard mass in the distal third and an appendectomy was performed without complications. The evolution was favorable and the patient was discharged. Conclusions: despite the rareness of this condition, the surgeon must know that this type of tumor may be present in an appendicular frame even in young patients in order to take the appropriate surgical behavior and avoid complications to the patient.

Pathological prognostic factors of pseudomyxoma peritonei / 中华病理学杂志

Objective@#To analyze the pathological features of pseudomyxoma peritonei (PMP) in correlation with the survival status and independent prognostic factors.@*Methods@#One-hundred and fifty-five PMP specimens were collected at Beijing Shijitan Hospital, Capital Medical University, from 2012 to 2018. Conventional histopathological evaluation was performed to document the primary tumor site, histopathological type, lymph nodes metastasis, tumor emboli in the blood and lymph vessels, nerve invasion and cellular density. The immunohistochemical parameters including Ki-67, p53, MMR-related protein, MUC2 and MUC5AC were analyzed. Clinical follow-up data were reviewed to correlate with pathological prognostic factors using Kaplan-Meier estimator and Cox proportional hazards regression model for univariate and multivariate analysis.@*Results@#Among 155 PMP patients, there were 77 males and 78 females. There were 98 cases (63.2%) of low-grade peritoneal mucinous carcinomatosis, 49 cases (31.6%) of high-grade peritoneal mucinous carcinomatosis, 8 cases (5.2%) of high-grade mucinous carcinoma peritonei with signet ring cells; only 15 cases (9.7%) with lymph node metastasis; 18 cases (11.6%) with tumor emboli in the blood and lymph vessels; 8/126 (6.3%) were positive dMMR; 100 cases (64.5%) had Ki-67 label index <50%, and 56 cases(36.1%) presented with mutant type p53. Univariate analysis revealed 11 survival-related pathological parameters including gender, age, primary tumor site, histopathological type, lymph node metastasis, tumor emboli in the blood and lymph vessels, nerve invasion, cellular density, Ki-67 label index rate, p53 and dMMR. Multivariate analysis identified 4 independent prognostic factors including the histopathological type (HR 59.78, P<0.01), lymph node metastasis (HR 3.74, P=0.028), nerve invasion (HR 7.81, P=0.007) and dMMR (HR 9.82, P<0.01).@*Conclusions@#Histopathological type is the most important prognostic factor of PMP with dMMR as an independent molecular prognostic indicator.

Acute Appendicitis

Acute appendicitis (AA) is one of the most common causes of acute abdominal pain, which can progress to perforation of the appendix and peritonitis. Recently, AA has been classified into uncomplicated (nonperforated, no phlegmon) or complicated (abscess, perforation, phlegmon) appendicitis, for an appropriate initial treatment. With respect to surgical treatment of AA, laparoscopic surgery has been widely accepted worldwide as a safe and feasible first-line treatment. Over the last decade, non-operative treatment has been proposed as an alternative to surgery in uncomplicated AA, and has also played an important role in the management of complicated AA. AA is also the most common cause for abdominal surgery during pregnancy, though an accurate diagnosis of AA during pregnancy is challenging. In this review, the topics being discussed include: 1) Non-operative management for uncomplicated AA, 2) Management for AA in pregnancy, 3) Management for complicated appendicitis (especially immediate laparoscopic surgery for appendiceal abscess), 4) Appendiceal neoplasms related to complicated AA.

Tumor carcinoide del apéndice cecal. Presentación de un caso / Carcinoid Tumor of the Cecal Appendix. Case presentation

Los tumores del apéndice cecal constituyen un grupo heterogéneo de neoplasias con evolución y pronóstico variables; representan una pequeña parte de todas las neoplasias gastrointestinales y de las apendicetomías. Se presenta el caso de un hombre blanco, de 55 años de edad, que ingresó en el Servicio de Cirugía por presentar un cuadro abdominal agudo, interpretado como una apendicitis aguda. Al realizar apendicectomía se obtuvo como resultado un tumor carcinoide tipo clásico (insular) del apéndice cecal, sin que se sospechara de este diagnóstico con anterioridad. Se decidió presentar este caso, debido a la poca frecuencia de su aparición. Se concluye que es importante en estos casos el estudio histopatológico, ya que, incluso en el transoperatorio, es muy difícil sospechar esta entidad, lo que conlleva al diagnóstico tardío y a un peor pronóstico.

Cecal appendix tumors constitute a heterogeneous group of neoplasias with variable evolution and prognosis; they represent a small part of all gastrointestinal neoplasms and appendectomies. A case of a 55 year old, white man, , who was admitted the Surgery Service because he had an acute abdominal condition, interpreted as acute appendicitis. An appendectomy resulted in a classic (insular) carcinoid tumor of the cecal appendix, without previous suspicion of this diagnosis. It was decided to present this case, due to the infrequency of its appearance. It is concluded that histo-pathological study is important in these cases, since even in the trans-operative, it is very difficult to suspect this entity, which leads to a late diagnosis and a worse prognosis.

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.

Plastrón apendicular y manejo clínico actual: una decisión a reconsiderar: a propósito de un caso / Appendicular mass and current clinic management: a decision to reconsiderate: about a case

Introducción: La apendicitis es uno de los principales motivos de consulta e intervención quirúrgica en los servicios de urgencias. Debe tratarse oportunamente dado que puede evolucionar hacia una perforación apendicular y con ello a una peritonitis o un plastrón apendicular, en aproximadamente un 10%, e incrementando las morbilidades, como tiempo de reposo o estadía hospitalaria. Actualmente no existe consenso acerca del tratamiento del plastrón apendicular, por ello, el objetivo del presente trabajo es respaldar y promover la alternativa quirúrgica diferida, versus el manejo médico, como el tratamiento más idóneo del plastrón apendicular. Presentación del caso: Se derivó al Hospital Base Valdivia a una escolar, de 8 años y 11 meses de edad, por dolor en hemiabdomen inferior de 3 semanas de evolución acompañado de sensación febril no cuantificada, vómito y diarrea; se diagnosticó un plastrón apendicular, optándose por el manejo médico; evolucionó favorablemente, dándole el alta. En controles posteriores se planificó una apendicectomía, llevándose a cabo con éxito meses después. La biopsia del apéndice extraído evidenció un carcinoma de apéndice cecal, por lo que se inició el estudio de extensión de neoplasia sin encontrar hallazgos patológicos. Discusión: Actualmente el manejo del plastrón apendicular depende del médico tratante y su criterio. Existen ventajas y desventajas entre la elección de un tratamiento médico o quirúrgico, sea inmediato o diferido; dentro de las ventajas del último destacan la prevención de apendicitis recurrentes y detección temprana del carcinoma apendicular, como en el caso presentado, permitiendo de esta forma un mejor pronóstico para el paciente y evitando el uso de terapias más agresivas.

Introduction: Appendicitis is one of the main reasons for consultation and surgery in the emergency department. It must be treated promptly because it can evolve into a ruptured appendix and thus to peritonitis or an appendiceal plastron, by approximately 10%, and increasing the morbidity, as downtime or hospital stay. There is currently no consensus on the treatment of appendicular plastron, therefore, the objective of this work is to support and promote alternative deferred surgical versus medical management, as the most suitable treatment of appendiceal plastron. Case presentation: A girl of 8 years 11 months old consult for pain in lower abdomen of three weeks of evolution, accompanied by feverish feeling, unquantified, vomiting and diarrhea; Is diagnosed a plastron appendiceal opting for medical management, evolving favorably and giving the discharge. In subsequent controls was planned appendectomy, taking place successfully months later. Biopsy of removed appendix showed a appendix cancer, reason why an extension study of neoplasia was initiated without pathological findings. Discussion: Currently the management of appendicular plastron is dependent on the treating physician and judgment. There are advantages and disadvantages of choosing a medical or surgical treatment, immediate or delayed, inside the advantages of the latter include the prevention of recurrent appendicitis and early detection of appendiceal carcinoma, as in the case presented, thus allowing better prognosis for the patient and avoiding the use of more aggressive therapies.

Segunda citorreducción más quimioterapia hipertérmica intraperitoneal por recaída de seudomixoma peritoneal / Second cytoreduction plus intraperitoneal hyperthermic chemotherapy in recurrence of pseudomyxoma peritonei

Introducción. El seudomixoma peritoneal es una condición rara caracterizada por la presencia de ascitis mucinosa e implantes peritoneales, en la mayoría de los casos, provenientes de tumores mucinosos del apéndice. El tratamiento primario de esta enfermedad es quirúrgico, y la citorreducción más quimioterapia hipertérmica intraperitoneal es el estándar de tratamiento actual, con supervivencia global a 5 y 10 años hasta de 96 y 68 %, respectivamente. No obstante, es una cirugía con alta morbilidad y considerable mortalidad, que apenas se está introduciendo en Colombia y la experiencia es incipiente. El porcentaje de recaída es de 28 a 44 % y existen pocos reportes sobre su manejo; la segunda citorreducción más quimioterapia hipertérmica intraperitoneal parece tener resultados aceptables en cuanto a supervivencia, morbilidad y mortalidad. Resultados. No hay estudios de segundas intervenciones por recaída peritoneal del seudomixoma en Colombia y, por esta razón, se decidió reportar la experiencia de dos casos del Instituto Nacional de Cancerología, donde, después de una primera citorreducción más quimioterapia hipertérmica intraperitoneal, los pacientes presentaron recaída peritoneal diagnosticada con imágenes durante el seguimiento y fueron sometidos a una nueva cirugía con buen resultado quirúrgico. Conclusión. La citorreducción secundaria más quimioterapia hipertérmica intraperitoneal es un procedimiento complejo con morbilidad considerable, que debe practicarse en lugares con experiencia y que proporciona al paciente un tratamiento radical y, posiblemente, se convierta en el manejo estándar de la recaída

Introduction: Peritoneal pseudomyxoma is a rare condition characterized by mucinous ascitis and peritoneal implants, originating from mucinous tumors of the apendix in the majority of cases. Primary treatment is surgical resection, with cytoreduction surgery plus hiperthermic intraperitoneal chemotherapy as the current standard of care, with 96% and 68% 5 and 10 year overall survival rates. Nonetheless, it is a surgical procedure associated with high morbidity and considerable mortality, with initial experience in Colombia. Recurrence is estimated between 28% and 44% and few reports address the management of recurrence peritoneal pseudomyxoma; second cytoreduction surgery plus hiperthermic intraperitoneal chemotherapy (CRS + HIPEC) seems to have acceptable results in terms of survival, morbidity and mortality. No studies of second CRS + HIPEC have been reported in Colombia; this is why we decided to publish two cases treated at the Instituto Nacional de Cancerología, Bogotá, Colombia, who had recurrence after a fist CRS + HIPEC, diagnosed by follow up images and who were taken to a second surgical treatment with good results. Conclusion: Second CRS + HIPEC is a technically challenging procedure with considerable morbidity that should only be performed in specialized and experienced centers, a radical form of treatment that could possibly become the standard of choice for recurrence.

Tumor mucoso apendicular / Appendiceal mucous tumor

Los tumores mucosos apendiculares tienen baja incidencia y comúnmente se diagnostican en el estudio anatomo-patólogico después de la apendicectomía. Se reporta el caso de una mujer de 41 años de edad, con un cuadro clínico de ocho meses de evolución, caracterizado por dolor abdominal de tipo opresivo, difuso y de gran intensidad en el hemiabdomen inferior, acompañado de náuseas. Después de cinco meses de iniciado este cuadro clínico, se evidenció una masa en la fosa iliaca derecha; el dolor se agudizó e intensificó, y las náuseas continuaron, por lo cual fue remitida al hospital. En los exámenes practicados se observó una masa quística compleja abdomino-pélvica de origen indeterminado, y la tomografía computadorizada de abdomen fue sugestiva de mucocele apendicular. Con estos hallazgos, se optó por el tratamiento quirúrgico por laparotomía, consistente en hemicolectomía derecha, con resección parcial de íleon, epiplectomía, histerectomía y salpigooforectomía bilateral.

The clinical manifestations of an appendicular mucous tumors are non specific. The vast majority are associated with complications of intrabdominal rupture causing acute abdomen, while the spectrum associated while the associated with extrinsic compression of adjacent organic structures is exceptional. We report a case of partial intestinal obstruction caused by an appendiceal mococele fistulized to the proximal ileum.

Obstrucción intestinal parcial producida por mucocele apendicular con fístula a íleon proximal / Partial intestinal obstruction caused by appendiceal mucocele with proximal ileum fistula

Las manifestaciones clínicas de un mucocele apendicular son inespecíficas. La gran mayoría de las veces se asocian con las complicaciones de su ruptura intraabdominal, al ocasionar un cuadro clínico de abdomen agudo, mientras que el espectro de manifestaciones clínicas asociadas con la compresión extrínseca de estructuras orgánicas vecinas, es excepcional. Se presenta el caso de un síndrome de obstrucción intestinal parcial producida por un mucocele apendicular con fístula al íleon proximal.

The clinical manifestations of an appendiceal mucocele are nonspecific. The vast majority are associated with the complications of intrabdominal rupture causing an acute abdomen, while the spectrum associated with extrinsic compression of adjacent organic structures is exceptional. We present the case of a partial bowel obstruction syndrome caused by an appendiceal mucocele fistulized to the proximal ileum.

Pseudomyxoma Peritonei in a Patient with History of Breast Cancer / 대한소화기학회지

Pseudomyxoma peritonei is a very rare condition, and even rarer in patients with history of cancer. A 70-year old woman with a history of breast cancer was admitted with abdominal pain and distention. Abdominal computed tomography revealed ascites collection, diffuse engorgement and infiltration of the mesenteric vessel, suggesting peritonitis or peritoneal carcinomatosis. Diagnostic paracentesis was attempted several times, but a sufficient specimen could not be collected due to the thick and gelatinous nature of the ascites. Therefore, the patient underwent diagnostic laparoscopy for tissue biopsy of the peritoneum, which indicated pseudomyxoma peritonei. However, the origin of the pseudomyxoma peritonei could not be identified intraoperatively due to adhesions and large amount of mucoceles. Systemic chemotherapy was performed using Fluorouracil, producing some symptomatic relief. After discharge, abdominal pain and distention gradually worsened, so at 18 months after initial diagnosis the patient received palliative surgery based on massive mucinous ascites and palpable mass at the omentum. The patient expired after surgery due to massive bleeding.

Appendiceal Neuroendocrine, Goblet and Signet-Ring Cell Tumors: A Spectrum of Diseases with Different Patterns of Presentation and Outcome / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Appendiceal tumors are a heterogeneous group of diseases that include typical neuroendocrine tumors (TNET), goblet cell carcinoids (GCC), and atypical GCC. Atypical GCC are classified into signet-ring cell cancers (SRCC) and poorly differentiated appendiceal adenocarcinoids. The prognosis and management of these diseases is unclear because there are no prospective studies. The aim of this study is to assess the characteristics and outcome of appendiceal TNET, GCC, and SRCC patients. MATERIALS AND METHODS: Appendiceal TNET, GCC, and SRCC patients diagnosed between 1973 and 2011 were identified in the Surveillance Epidemiology and End Results (SEER) database. Demographics, type of surgery, and clinicopathologic characteristics were collected. Survival functions were estimated by the Kaplan-Meier method, and log-rank test was used to assess the difference in overall survival (OS) among the three histologies. RESULTS: The SEER database yielded 1,021 TNET patients, 1,582 with GCC, and 534 SRCC patients. TNET presented at a younger age (p < 0.001). Patients with SRCC presented with advanced stage disease (p < 0.001). The median OS (mOS) for GCC and TNET patients was not reached; mOS for SRCC was 24 months. Multivariate analysis stratified for stage revealed significantly longer survival for TNET and GCC than SRCC (p < 0.001). CONCLUSION: This is the largest report to date for appendiceal neuroendocrine tumor patients, suggesting a spectrum of diseases with different characteristics and outcomes. In this report, we present a treatment approach for this complex spectrum of disease, based on the experience of Ohio State and Emory Universities investigators.