Appendiceal mucinous cystadenoma: a case report and review of the literature

Appendicular mucocele is a low incidence neoplasm with a frequency of 2 cases per 10,000 laparotomies. It is found in 0.2 to 0.3% of all appendectomies, with a higher incidence in women after the fifth or sixth decade of life. It is considered a benign lesion, although in some occasions, approximately 20% of cases it is associated with neoplasms of other intra-abdominal organs. Authors present the case of a 88-year-old patient with clinical findings consistent with acute appendicitis resolved with a simple appendicectomy based on the macroscopic findings during surgery.

Mucocele de apêndice: relato de caso

Introdução: A mucocele do apêndice é um termo utilizado para descrever uma dilatação deste órgão, devido ao acúmulo de secreção mucoide, tornando o apêndice dilatado, de paredes finas e com uma massa cística no seu interior. Na maioria dos casos a mucocele é resultante de neoplasia que obstrui a luz apendicular. Relato de caso: Sexo feminino, 55 anos, branca. Procurou atendimento médico devido à dor abdominal de moderada intensidade há cinco anos. Refere que os sintomas iniciaram com dor em fossa ilíaca direita e região lombar há cinco anos, quando começou a tratar lombalgia. Nesse ano, buscou atendimento graças a piora da dor. O abdome estava globoso, flácido, doloroso a palpação superficial e profunda em fossa ilíaca direita com ruídos hidroaéreos presentes. Devido a sintomatologia foram solicitados um ultrassom de abdome total que evidenciou imagem cística tubuliforme em região anexial direita, de aspecto simples, de contornos regulares e conteúdo anecoico, medindo 13,3 cm x 5,2 cm x 4,3 cm, sem septos, calcificações ou fluxo ao Doppler e ressonância magnética da pelve que demonstrou formação cística alongada em região anexial, mergulhante na região anexial, apêndice cecal não individualizado. Foi realizada uma cirurgia exploradora que resultou em apendicectomia e o produto enviado para análise histopatológica que resultou em mucocele de apêndice, sem critérios histológicos de malignidade. As mucoceles de apêndice foram classificadas em 3 categorias: hiperplasia mucosa sem atipias, mucocele simples; cistadenoma mucinoso, com algum grau de atipia e cistadenocarcinomamucinoso, com invasão estromal ou implantes peritoniais. Conclusão: A sintomatologia é a dor abdominal, de intensidade e duração variáveis, podendo também ser observados a presença de massa palpável em fossa ilíaca direita e emagrecimento. Embora cerca de 25% dos casos sejam assintomáticos, alguns estudos demonstram a correlação entre sintomatologia exuberante e displasia. Aproximadamente 20% dos casos de mucocele de apêndice têm associação com o câncer colorretal, no entanto, outras neoplasias como os carcinomas de ovário, endométrio, mama, próstata, bexiga e rim também podem estar presentes.Descritores: Mucocele; Apêndice; Dor Abdominal.ReferênciasEnsuncho C, Osorio C, Marrugo Á, Herrera F. Obstrucción intestinal parcial producida por mucocele apendicular con fístula a íleon proximal. rev colomb cir. 2016;31(1):61-4.Bichara DSJ, Luz Neto VC, Matuck MJ, Bichara DG. Pseudomixoma retroperitoneal (mucocele apendicular). Relatos Casos Cir. 2015;(1):1-3Moré Cabrera JA, León Aulla SP, Pérez Zavala GA. Presentación inusual de mucocele apendicular. Acta Médica Centro. 2015;9(4):62-7.Morano WF, Gleeson EM, Sullivan SH, Padmanaban V, Mapow BL, Shewokis PA et al. Clinicopathological features and management of appendiceal mucoceles: A systematic review. Am Surg. 2018;84(2):273-81.Park KJ, Choi HJ, Kim SH. Laparoscopic approach to mucocele of appendiceal mucinous cystadenoma: Feasibility and short-term outcomes in 24 consecutive cases. Surg Endosc. 2015;29(11):3179-83.Demetrashvili Z, Chkhaidze M, Khutsishvili K, Topchishvili G, Javakhishvili T, Pipia I et al. Mucocele of the appendix: case report and review of literature. Int Surg. 2012;97(3):266-69.Basak F, Hasbahceci M, Yucel M, Sisik A, Acar A, Kilic A, Su Dur MS. Does it matter if it is appendix mucocele instead of appendicitis? Case series and brief review of literature. J Cancer Res Ther. 2018;14(6):1355-60.Rabie ME, Al Shraim M, Al Skaini MS, Alqahtani S, El Hakeem I, Al Qahtani AS et al. Mucus containing cystic lesions “mucocele” of the appendix: the unresolved issues. Int J Surg Oncol. 2015;2015:139461.Hao S, Lilly R, Bonatti HJR. Laparoscopic resection of an appendix mucocele in a breast cancer patient. Case Rep Surg. 2018;2018:1780342.Dixit A, Robertson JH, Mudan SS, Akle C. Appendiceal mucocoeles and pseudomyxoma peritonei. World J Gastroenterol. 2007;13(16):2381-84.

A case of appendiceal mucinous cystadenoma which presented as painless purulent umbilical discharge

A 57-year-old obese, diabetic woman, presented with 1 day history of purulent umbilical discharge. She was vitally stable and afebrile. Abdominal examination revealed a full abdomen with purulent discharge from the umbilicus, swelling with erythema and induration surrounding the umbilicus. Lab tests were normal. Initial impression was abdominal wall abscess. Ultrasound showed subcutaneous fluid collection. Non-contrast CT showed collection and abdominal wall defect at the umbilicus. On exploration of the abscess cavity, there were two defects (umbilical and supraumbilical) with appendix protruding through the umbilical defect and a part of a small bowel and omentum adherent to the other defect. Wash was given, bowel and omentum were released and appendectomy was performed. Histopathology showed mucinous cystadenoma with periappendicitis. We would like to highlight the rare occurrence of an appendiceal mucinous cystadenoma in such a clinical presentation.

Preoperative Diagnosis and Surgical Approach of Appendiceal Mucinous Cystadenoma: Usefulness of Volcano Sign

We report a case of appendiceal mucinous cystadenoma that was successfully diagnosed preoperatively and treated by laparoscopic resection. We could find volcano sign on colonoscopy and cystic lesion without any nodules at the appendix on computed tomography (CT). Without any malignant factors in preoperative examinations, we performed laparoscopic appendectomy including the cecal wall. We could avoid performing excessive operation for cystadenoma with accurate preoperative diagnosis and intraoperative finding and pathological diagnosis during surgery. Appendiceal mucocele is a rare disease that is divided into 3 pathological types: hyperplasia, cystadenoma, and cystadenocarcinoma. The surgical approaches for it remain controversial and oversurgery is sometimes done for benign tumor, because preoperative diagnosis is difficult and rupturing an appendiceal tumor results in dissemination. Based on our study, volcano sign on colonoscopy and CT findings were important for the preoperative diagnosis of appendiceal mucocele. Furthermore, we think that laparoscopic resection will become a surgical option for the treatment of appendiceal mucocele.