¿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)

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.

Cistoadenoma mucinoso apendicular / Appendiceal mucinous cystadenoma

El cistoadenoma apendicular es una neoplasia poco frecuente, que tiene una incidencia de 0,2 % a 0,3 % en todas las apendicetomías; esta afección predomina en pacientes féminas y su presentación es poco específica en cuanto a los síntomas, los cuales pueden compararse a un cuadro de apendicitis aguda, una masa abdominal, un cuadro obstructivo o ginecológico, o manifestaciones urológicas, que son las menos frecuentes. Se presentó una paciente femenina de 59 años de edad con dolor abdominal localizado en la fosa ilíaca derecha de 4 meses de evolución; se realizaron varios exámenes complementarios, una laparoscopia diagnóstica, una laparotomía exploratoria y también una apendicetomía. Después de estos exámenes se realizó un diagnóstico histológico de cistoadenoma mucinoso apendicular.

Appendiceal cystadenoma is a rare neoplasm, with an incidence of 0.2% to 0.3% among all appendectomies; this condition predominates in female patients and its presentation is unspecific in terms of symptoms, which can be compared to acute appendicitis, an abdominal mass, obstructive or gynecological symptoms, or urological manifestations, which are the least frequent. We present a 59-year-old female patient with an abdominal pain over 4 months located in the right iliac fossa; several complementary tests were performed such as a diagnostic laparoscopy, an exploratory laparotomy and an appendectomy. After these examinations, a histological diagnosis of appendiceal mucinous cystadenoma was made.

Abordaje laparoscópico de la neoplasia mucinosa apendicular de bajo grado / Laparoscopic approach of low-grade appendiceal mucinous neoplasm

RESUMEN Las neoplasias mucinosas apendiculares son infrecuentes y se clasifican en neoplasias mucinosas de bajo grado, de alto grado y adenocarcinoma mucinoso. Se consideran precursoras del pseudomixoma peritoneal, caracterizado por la acumulación de mucina dentro de la cavidad abdominal. Informamos tres casos de neoplasia mucinosa apendicular de bajo grado, dos varones y una mujer, que presentaron dolor abdominal agudo, diagnosticados preoperatoriamente mediante tomografía y que fueron manejados con un abordaje laparoscópico (apendicectomía, resección parcial de ciego y colectomía derecha, respectivamente). Los tres casos tuvieron una buena evolución posoperatoria y no hubo complicaciones. Se concluye que las neoplasias mucinosas apendiculares son raras y no tienen una presentación clínica específica. El abordaje laparoscópico es una opción segura y factible. La frecuencia y la duración del seguimiento de estos pacientes es motivo de controversia.

ABSTRACT Appendiceal mucinous neoplasms are rare and are classified in low-grade mucinous neoplasm, high-grade mucinous neoplasm and mucinous adenocarcinoma. They are considered precursors of pseudomyxoma peritonei, characterized by the accumulation of mucin within the abdominal cavity. We report three cases of low-grade appendiceal mucinous neoplasm, two men and one woman, who presented with acute abdominal pain. The diagnoses were made preoperatively by computed tomography scan and the tumors were managed through laparoscopic approach (appendectomy, partial resection of the cecum and right colectomy, respectively). The three patients evolved with favorable postoperative outcome without complications. Appendiceal mucinous neoplasms are rare without specific clinical presentation. The laparoscopic approach is a safe and feasible option. The frequency and duration of surveillance in these patients are still controversial.

Analysis of perioperative efficacy and safety of cytoreductive surgery in the treatment of colorectal cancer peritoneal metastases / 中华胃肠外科杂志

Objective: To analyzed perioperative safety of cytoreductive surgery (CRS) for patients with colorectal cancer peritoneal metastasis (CRPM) and to construct a predictive model for serious advese events (SAE). Methods: A descriptive case-series study was conducted to retrospectively collect the clinicopathological data and treatment status (operation time, number of organ resection, number of peritoneal resection, and blood loss, etc.) of 100 patients with peritoneal metastases from colorectal cancer or appendix mucinous adenocarcinoma who underwent CRS at the Sixth Affiliated Hospital of Sun Yat-sen University from January 2019 to August 2021. There were 53 males and 47 females. The median age was 52.0 (39.0-61.8) years old. Fifty-two patients had synchronous peritoneal metastasis and 48 had metachronous peritoneal metastasis. Fifty-two patients received preoperative neoadjuvant therapy. Primary tumor was located in the left colon, the right colon and the rectum in 43, 28 and 14 cases, respectively. Fifteen patients had appendix mucinous adenocarcinoma. Measures of skewed distribution are expressed as M (range). Perioperative safety was analyzed, perioperative grade III or higher was defined as SAE. Risk factors associated with the occurrence of SAEs were analyzed using multivariate logistic regression. A nomogram was plotted by R software to predict SAE, the efficacy of which was evaluated using the area under the ROC curve (AUC) and correction curves. Results: The median peritoneal cancer index (PCI) score was 16 (1-39). Sixty-eight (68.0%) patients achieved complete tumor reduction (tumor reduction score: 0-1). Sixty-two patients were treated with intraperitoneal hyperthermic perfusion chemotherapy (HIPEC). Twenty-one (21.0%) patients developed 37 SAEs of grade III-IV, including 2 cases of ureteral injury, 6 cases of perioperative massive hemorrhage or anemia, 7 cases of digestive system, 15 cases of respiratory system, 4 cases of cardiovascular system, 1 case of skin incision dehiscence, and 2 cases of abdominal infection. No grade V SAE was found. Multivariate logistic regression analysis showed that CEA (OR: 8.980, 95%CI: 1.428-56.457, P=0.019), PCI score (OR: 7.924, 95%CI: 1.486-42.259, P=0.015), intraoperative albumin infusion (OR: 48.959, 95%CI: 2.115-1133.289, P=0.015) and total volume of infusion (OR: 24.729, 95%CI: 3.956-154.562, P=0.001) were independent risk factors for perioperative SAE in CRS (all P<0.05). Based on the result of multivariate regression models, a predictive nomogram was constructed. Internal verification showed that the AUC of the nomogram was 0.926 (95%CI: 0.872-0.980), indicating good prediction accuracy and consistency. Conclusions: CRS is a safe and effective method to treat CRPM. Strict screening of patients and perioperative fluid management are important guarantees for reducing the morbidity of SAE.

Cirurgia Citorredutora com Quimioterapia Intraperitoneal Hipertérmica em Pacientes com Adenocarcinoma Mucinoso de Apêndice: Série de 43 Casos / Cytoreductive Surgery with Hyperthermic Intraperitoneal Chemotherapy in Patients with Appendix Mucinous Adenocarcinoma: Series of 43 Casess / Cirugía Citorreductiva con Quimioterapia Intraperitoneal Hipertermal en Pacientes con Adenocarcinoma Mucinoso de Apéndice: Serie de 43 Caso

Introdução: O carcinoma primário de apêndice é uma condição rara. Muitas revisões retrospectivas internacionais delineiam a experiência de diferentes centros em neoplasias apendiculares. Por sua vez, o tratamento do câncer nessa localização é complexo e depende do subtipo histológico e da extensão da doença. Um dos tratamentos mais promissores é a cirurgia citorredutora (CCR) associada à quimioterapia intraperitoneal hipertérmica (HIPEC). No Brasil, não há descrição de séries de casos que tiveram essa abordagem terapêutica. O objetivo desta série de casos é analisar as características sociodemográficas e o tipo de intervenção terapêutica em pacientes com doenças malignas de apêndice em um Centro de Assistência de Alta Complexidade em Oncologia III (Cacon III). Relato dos casos: Foram incluídos 43 casos de tumores primários de apêndice. O adenocarcinoma do apêndice do tipo mucinoso de baixo grau foi a neoplasia mais diagnosticada. O principal protocolo utilizado foi de uma a duas cirurgias e aplicação de mitomicina C em temperatura média de 40 graus. Os casos apresentaram grande heterogeneidade quanto ao uso do protocolo. Conclusão: O presente relato de 43 casos é importante por se tratar de um tumor raro nessa localização. A modalidade terapêutica descrita é promissora, mas não há protocolo definido para essa finalidade. É necessário atualizar as diretrizes terapêuticas para normatizar a conduta internamente, especialmente em se tratando de uma unidade de referência nacional

Introduction: Primary appendix carcinoma is a rare condition. Many international retrospective reviews outline the experience of different centers in appendicular neoplasms. The cancer treatment in this location is complex and depends on the histological subtype and the extent of the disease. One of the most promising treatments is cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). In Brazil, there is no description of series of cases with this therapeutic approach. The purpose of this case series is to analyze the sociodemographic characteristics and the type of therapeutic intervention in patients with malignant diseases of the appendix in a High Complexity Care Center in Oncology III (Cacon III). Case reports: 43 cases of primary appendix tumors were included. Low-grade mucinous adenocarcinoma was the most diagnosed neoplasm. The main protocol used was 1 to 2 surgeries and application of mitomycin C at an average temperature of 40 degrees. There was great heterogeneity regarding the use of the protocol. Conclusion: The present report of 43 cases is important because it is a rare tumor with this location. The therapeutic modality described is promising, but there is no defined protocol for this purpose. It is necessary to update the therapeutic guidelines to standardize the conduct internally, especially in the case of a national reference unit

Introducción: El carcinoma primario de apéndice es una entidad poco frecuente. Numerosas revisiones retrospectivas internacionales describen la experiencia de diferentes centros en neoplasias apendiculares. A su vez, el tratamiento de esta localización del cáncer es complejo y depende del subtipo histológico y la extensión de la enfermedad. Uno de los tratamientos más prometedores es la cirugía citorreductora (CCR) asociada a quimioterapia intraperitoneal hipertérmica (HIPEC). En Brasil, no hay descripción de series de casos que tuvieran este abordaje terapéutico. El propósito de esta serie de casos es analizar las características sociodemográficas y el tipo de intervención terapéutica en pacientes con enfermedades malignas del apéndice en un Centro Asistencial de Alta Complejidad en Oncología III (Cacón III). Reporte de los casos: Se incluyeron 43 casos de tumores primarios de apéndice. El adenocarcinoma mucinoso de bajo grado tuvo la mayor incidencia. El protocolo principal utilizado fue de una a dos cirugías y aplicación de mitomicina C a una temperatura promedio de 40 grados. Los casos mostraron gran heterogeneidad en cuanto al uso del protocolo. Conclusión: El presente informe es importante porque es un tumor raro. La modalidad terapéutica descrita es prometedora, pero no existe un protocolo definido para tal fin. Es necesario actualizar las pautas terapéuticas para normalizar la conducta internamente, especialmente en el caso de una unidad de referencia nacional

Neoplasias apendiculares incidentales / Incidental appendicular neoplasms

Las neoplasias apendiculares se presentan hasta en el 50 % de los casos como un episodio de apendicitis aguda. Existen características demográficas, clínicas y radiológicas que aumentan las posibilidades de un tumor apendicular subyacente, sin embargo, en la mayoría de los casos, son los hallazgos intraoperatorios los que alertan al cirujano. A pesar de que el tipo histológico determina la radicalidad del manejo quirúrgico de estos pacientes, algunas características macroscópicas pueden orientar a las patologías específicas y a una conducta adecuada. En general, los objetivos del manejo quirúrgico inicial se cumplen con una resección limitada al apéndice cecal, asociada a la citología de mucina y biopsia de los implantes peritoneales si están presentes, reservando las resecciones extendidas, como hemicolectomía derecha oncológica, para los pacientes con compromiso extenso de la base o del mesenterio apendicular ante la sospecha de neoplasias neuroendocrinas o adenocarcinoma del apéndice cecal.

Appendicular neoplasms present in up to 50% of cases as an episode of acute appendicitis. There are demographic, clinical and radiological characteristics that increase the chances of an underlying appendicular tumor; however, in most cases are the intraoperative findings that alert the surgeon. Although the histological type determines the radical nature of the surgical management of these patients, some macroscopic characteristics can guide specific pathologies and appropriate behavior. In general, the objectives of initial surgical management are met with a limited resection of the cecal appendix, associated with mucin cytology and biopsy of peritoneal implants if present, reserving extended resections such as oncological right hemicolectomy for patients with extensive compromise of the base or appendicular mesentery when neuroendocrine neoplasms or adenocarcinoma of the cecal appendix are suspected.

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.

Appendiceal Neuroendocrine Tumours - Experience of One Service / Tumores neuroendócrinos do apêndice - Experiência de um serviço

Abstract Tumours of the appendix are rare and tend to be diagnosed incidentally, in cases of acute appendicitis. For some authors, appendiceal neuroendocrine tumours (ANETs) are the most frequent neoplasm of the appendix, observed in 0.3% to 0.9% of cases acute appendicitis. The present is a unicentric retrospective study conducted between January 2005 and March 2017. Out of a total of 3,007 surgeries for appendiceal pathologies performed in the adult population at the hospital where the present study was conducted, there were 70 (2.33%) malignant cases, 20 (28.6%) of which were ANETs. The patients had a median age of 44 years (range: 18 to 85 years), and were predominantly women (there were 1.9 times more women than men). In 16 cases (80%), a simple appendicectomy was performed (1 patient was submitted to a right hemicolectomy later). The cases of ANETs had a good prognosis in our series: 85% of the patients are either alive today or were alive after 5 years of follow-up. Despite the fact that ANETs are described as the most frequent tumor of the appendix, this was not confirmed in our series, in which they only represented 28.6% of the cases; adenocarcinoma was the most frequent tumor (65.7%) among our sample.

Resumo Tumores do apêndice são raros, e tendem a ser diagnosticados de forma incidental, em casos de apendicite aguda. Para alguns autores, tumores neuroendócrinos do apêndice (TNEAs) são as neoplasias mais frequentes do apêndice, e são observados em 0,3 a 0,9% de todos os casos agudos de apendicite. Este é um estudo monocêntrico e retrospectivo realizado entre janeiro de 2005 e março de 2017. Entre umtotal de 3.007 cirurgias para patologias do apêndice realizadas na população adulta no hospital em que o estudo foi conduzido, houve 70 (2,33%) casos de malignidade, 20 (28,6%) dos quais eram TNEAs. Os pacientes tinham uma idade média de 44 anos (gama: 18-85 anos), e eram predominantemente mulheres (havia 1,9 vezes mais mulheres do que homens). Em 16 casos (80%), realizou-se uma simples apendicectomia (1 paciente foi submetido a uma hemicolectomia direita mais tarde). Os casos de TNEAs tiveram um bom prognóstico em nossa série: 85% dos pacientes estão vivos hoje, ou estavam após um seguimento de 5 anos. Apesar de os TNEAs serem descritos como os tumores mais frequentes do apêndice, isso não foi confirmado nesta série, na qual eles representaram apenas 28,6% dos casos; adenocarcinoma foi o tumor mais frequente (65,7%) emnossa amostra.

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)

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.

Fibromatosis tipo desmoide de apéndice cecal: reporte de caso inusual y revisión de la literatura / Appendicular desmoid tumor: unusual case and review of the literature

RESUMEN Antecedente : La fibromatosis tipo desmoide es un proceso neoplásico benigno no encapsulado localmente invasivo y agresivo, que se origina de la proliferación de fibroblastos y miofibroblastos aparentemente normales. La localización más frecuente de la fibromatosis es extra-abdominal (60%), pared abdominal (25%) e intra-abdominal (8-15%), en raras ocasiones puede originarse en las vísceras (0,73%), como el páncreas, unión gastroesofágica, diafragma y apéndice. La incidencia anual de tumor desmoide se estima de 2 a 5 casos por millón. En el presente artículo, reportamos un caso de presentación inusual, originado en el apéndice cecal. Caso : Paciente de sexo femenino de 41 años con dolor pélvico agudo, que ingresa a sala de operaciones con diagnóstico clínico y ecográfico de probable tumor de ovario a pedículo torcido. En el intraoperatorio se evidenció una tumoración sólida de 15 cm de diámetro que dependía del apéndice cecal, correspondiendo el estudio anatomopatológico a tumor desmoide de apéndice cecal. Conclusiones : El tumor desmoide puede originarse en diversas localizaciones extra e intra-abdominales, siendo esta última la más rara y agresiva. El diagnóstico preoperatorio exacto es muy difícil y casi siempre los pacientes ingresan al quirófano con sospecha diagnostica de tumoración intraabdominal de etiología desconocida. Los factores de riesgos asociados a su aparición aún no se encuentran caracterizados, siendo la resección quirúrgica completa del tumor -con márgenes quirúrgicos libres (R0)- el tratamiento de elección; sin embargo, el riesgo de recurrencia es alto incluso con la remoción óptima del tumor.

ABSTRACT Background : Desmoid type fibromatosis is a benign neoplastic process non-encapsulated locally invasive and aggressive, which arises from a proliferation of bland-looking fibroblasts and myofibroblasts. The most frequent location of fibromatosis is extra- abdominal (60%), abdominal wall (25%) and intra-abdominal (8-15%), rarely can originate in the viscera (0.73%), such as the pancreas, gastroesophageal junction, diaphragm and appendix. The annual incidence of desmoid tumor is estimated at 2 to 5 cases per million. In this article, we report a case of unusual presentation, originated in the cecal appendix. Case : A 41-year-old female patient with acute pelvic pain, admitted to surgical ward with a clinical and ultrasound diagnosis of probably ovarian pedicle tumor. An explorative laparotomy revealed a solid mass of 15 cm in diameter arising from the cecal appendix, with the anatomopathological study corresponding to a desmoid tumor of the cecal appendix. Conclusions : The desmoid tumor can arise from several extra and intra-abdominal locations, the latter being the rarest and most aggressive. Accurate preoperative diagnosis is very difficult and almost always patients enter to surgery with suspected diagnosis of intraabdominal tumor of unknown etiology. The risk factors associated to its appearance are not yet characterized. The complete surgical resection of the tumor -with free surgical margins (R0)- is the treatment of choice; however, the risk of recurrence is high even with optimal tumor removal.

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.

Appendiceal neuroendocrine tumors: approach and treatment / Tumores neuroendócrinos do apêndice: abordagem e tratamento

ABSTRACT The incidence of tumors in the appendix has increased over the years, and they are mainly found in the anatomical and pathological examination of appendices operated due to acute appendicitis. The annual incidence of neuroendocrine tumors of the appendix, also called carcinoid tumors, is 0.15-0.16 per 100,000 people. In absolute terms, the incidence of these tumors has increased in the last decade by 70-133%. Appendiceal carcinoid tumors occur more often in women, and are found in 0.3-0.9% of the appendices removed in appendectomies. They appear in the subepithelial neuroendocrine cells and have an indolent course, with the symptoms being indistinguishable from an acute appendicitis. There are two classifications, one presented by the European Neuroendocrine Tumor Society and the other by the American Joint Committee on Cancer. Both classifications use tumor size as a predictor of tumor burden. The classification used by European Neuroendocrine Tumor Society also uses the invasion of the mesoappendix to select the best surgical treatment. However, these classifications require the inclusion of more criteria to define the selection of surgical treatment of tumors between 1 and 2 cm. Thus, along with the size of the tumor and the invasion of the mesoappendix, other factors such as vascular invasion, ki67 index, mitotic index and tumor location should be considered at the time of classification, for a better selection of the treatment and prognostic evaluation.

RESUMO A incidência de tumores no apêndice tem aumentado ao longo dos anos, principalmente encontrados no exame anatomopatológico dos apêndices operados por apendicite aguda. A incidência anual de tumores neuroendócrinos do apêndice, também designados por tumores carcinoides é de 0,15 a 0,16 por 100.000 pessoas. Em termos absolutos, a incidência destes tumores tem aumentado na última década em 70% a 133%. Os tumores carcinoides do apêndice ocorrem mais em mulheres e são encontrados em 0,3%‒0,9% dos apêndices removidos em apendicectomias. Têm origem nas células neuroendócrinas subepiteliais e apresentam um curso indolente, sendo os sintomas indistinguíveis de uma apendicite aguda. Existem duas classificações, a apresentada pela ENETS (European Neuroendocrin Tumor Society) e da AJCC (American Joint Committee on Cancer). Ambas as classificações utilizam o tamanho do tumor como preditor de carga tumoral. A classificação utilizada pela ENETS recorre ainda à invasão do mesoapêndice para selecionar o melhor tratamento cirúrgico. Contudo, estas classificações necessitam incluir mais critérios para definir a escolha do tratamento cirúrgico de tumores entre 1‒2 cm. Assim, para além do tamanho do tumor e da invasão do mesoapêndice, outros fatores como a invasão vascular, o ki67, o índice mitótico e a localização do tumor devem ser considerados no momento da classificação, para uma melhor seleção do tratamento e avaliação prognóstica.

Pseudomyxoma peritonei originating from appendix tumors / Pseudomixoma peritoneal com origem em tumores do apêndice

ABSTRACT Background: Appendix tumors represent about 1% of all gastrointestinal neoplasia, in other words they are quite rare. However, there is a specific type of appendiceal neoplasms (mucinous adenocarcinoma) that spreads to the peritoneum and in almost 20% of the cases, resulting in a disease called pseudomyxoma peritonei. Although, it is a very rare condition, it is nonetheless a very severe one and therefore it is crucial to know how to correctly diagnose and treat it. Objective: This study provides updated data on how to diagnose, classify and treat pseudomyxoma peritonei that originates from appendix tumors. Methods: A bibliographic research was performed on PubMed database, including articles published since 2000, as well as, cross-referencing with the initial research. Discussion: In the past, patients diagnosed with pseudomyxoma peritonei would only undergo palliative measures, so their overall survival rate was greatly reduced. Over the years pseudomyxoma peritonei treatment has evolved and patients are now undergoing treatment which is a combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. This new therapy has allowed an increase of survival chances of up to 5 years in those patients with values between 53% and 88%, depending on the type of tumor. Conclusion: Despite the great progress we have witnessed in recent years, which have led to a large increase in survival rates, more research needs to be done, on what to do when the disease is in an unresectable stage. Finding a less aggressive therapy than cytoreductive surgery + hyperthermic intraperitoneal chemotherapy will be an important step forward.

RESUMO Introdução: As neoplasias do apêndice são bastante raras, representando atualmente cerca de 1% de todas as neoplasias gastrointestinais. O adenocarcinoma mucinoso é um dos subtipos de neoplasia do apêndice e caracteriza-se por metastizar para o peritoneu, em 20% dos casos, facto que se manifesta sob a forma de uma doença designada por Pseudomixoma Peritoneal. Apesar de ser uma condição muito rara, a sua extrema gravidade justifica a importância de a saber diagnosticar e tratar corretamente. Métodos: Foi realizada uma pesquisa bibliográfica na base de dados PubMed, incluindo artigos publicados desde 2000 bem como artigos de pesquisa cruzada com os iniciais. Discussão: No passado, os doentes diagnosticados com Pseudomixoma Peritoneal eram apenas submetidos a medidas paliativas, pelo que a sua sobrevida era muito reduzida. Ao longo dos anos o tratamento do Pseudomixoma Peritoneal foi evoluindo sendo agora os doentes submetidos a uma combinação de cirurgia citoredutiva e quimioterapia hipertérmica intraperitoneal. Esta nova terapêutica tem permitido aumentar a sobrevida aos 5 anos destes pacientes para valores entre os 53% e os 88%, dependendo do tipo de tumor. Conclusões: Apesar dos grandes avanços que se têm verificado, e que culminaram com um grande aumento das taxas de sobrevivência, devem ser feitos mais estudos que encontrem novas abordagens para quando o tumor já é diagnosticado num estado irressecável, bem como terapêuticas menos invasivas.

Prognostic analysis and clinicopathological features of 20 patients with appendiceal neuroendocrine neoplasms / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To investigate clinicopathological features and prognostic factors of appendiceal neuroendocrine neoplasms(a-NEN).</p><p><b>METHODS</b>Clinical data of 20 patients diagnosed with a-NEN at Zhongshan Hospital of Fudan University between January 2000 and December 2016 were retrospectively analyzed. Pathological diagnosis was based on the WHO classification criteria of digestive system tumors (2010 edition). Based on the mitotic count and Ki-67 index, a-NENs were divided into grade 1 neuroendocrine tumor (NET G1), grade 2(G2) NET G2) and grade 3 (neuroendocrine carcinoma, NEC). Some special types of a-NEN (e.g. goblet cell carcinoid) and mixed adenoneuroendocrine neoplasms were classified as mixed adenoneuroendocrine carcinoma (MANEC). Follow-up was conducted by telephone or return visits. Univariate analysis was carried out using the Kaplan-Meier method, and the log-rank test was used to draw survival curves.</p><p><b>RESULTS</b>Of 20 patients, 14 were male and 6 were female with median age of 54 years. Seventeen cases presented acute right lower quadrant abdominal pain, 1 chronic right lower quadrant abdominal pain, 1 persistent abdominal discomfort with outburst whole abdominal pain and 1 was found during body check without symptoms. Twenty cases comprised 8 G1 patients, 4 G2 patients, 3 G3 patients, and 5 MANEC patients. When diagnosed, there was 1 patient with liver metastasis, 1 patient with abdominal and pelvic metastases, and 2 patients with postoperative pathological findings of lymph node metastasis. Six patients underwent appendectomy, 12 underwent right hemicolectomy, 1 underwent right hemicolectomy plus small intestine resection, and 1 underwent partial hepatectomy plus right hemicolectomy. The follow-up time was 7-187 months(average, 36 months). The total 1- and 3-year survival rates were 94.7% and 60.2%, respectively. Univariate analysis showed that age >50 years (χ=7.036, P=0.008), pathology grade as MANEC (χ=5.297, P=0.021), and metastasis (χ=6.558, P=0.010) indicated lower 5-year survival rate.</p><p><b>CONCLUSIONS</b>Most a-NEN patients have no typical symptoms, and the main complaint at consultation is acute right lower quadrant abdominal pain. Prognosis is poor for patients with age >50 years, MANEC pathology grade and metastasis.</p>