Pseudomyxoma peritonei in a pediatric patient: A case report and literature review / Pseudomixoma peritoneal em paciente pediátrico: relato de caso e revisão de literatura

Summary Introduction: Pseudomyxoma peritonei (PMP) is a rare clinical condition, with an incidence of 1-2 cases per million, characterized by the dissemination of mucinous implants on the peritoneal surface and progressive gelatinous ascites. Although it usually presents an indolent behavior, its non-specific clinical presentation contributes to many cases remaining undiagnosed until a laparotomy is performed. With late diagnosis, performed after a long period of clinical deterioration and disease progression, it is common to find complications such as the formation of intestinal fistulas and obstruction. Method: Review of the medical record and search for references in the Medline, Lilacs, SciELO and MD Consult databases. Results: There are rare case reports found in the literature demonstrating atypical PMP presentations. Our report is that of a 17-year-old adolescent with a sporadic tumor diagnosed in a primary site in the transverse colon, contrary to data commonly found in the literature that mention a more frequent occurrence in women in the fifth decade of life and with a primary site in the ovary and appendix. The development of mucinous adenocarcinoma is rare in the pediatric population, and topography in the transverse colon and non-familial sporadic pattern are unusual. Conclusion: The case reported not only raises awareness about the atypical presentations of the disease, but also emphasizes the use of imaging examinations for diagnosis, which has an important impact on prognosis and survival if performed timely.

Resumo Introdução: O pseudomixoma peritoneal (PMP) é uma condição clinica rara, com incidência de 1-2 casos por milhão, caracterizada pela disseminação de implantes de natureza mucinosa pela superfície peritoneal e acúmulo progressivo de ascite gelatinosa. Embora apresente geralmente um comportamento indolente, a apresentação clínica inespecífica contribui para que muitos casos permaneçam sem diagnóstico até a realização de laparotomia. Com o diagnóstico tardio, realizado após um longo período de deterioração clínica e progressão de doença, é comum encontrar complicações, como a formação de fístulas e obstruções intestinais. Método: Revisão do prontuário médico e pesquisa bibliográfica nas bases de dados Medline, Lilacs, SciELO e MD Consult. Resultados: São raros os relatos de caso encontrados na literatura que demonstram apresentações atípicas do PMP. O presente estudo apresenta o caso de um adolescente com 17 anos ao diagnóstico e sítio primário no colón transverso com tumor esporádico, contrário aos dados comumente encontrados na literatura, que referem acometimento mais comum em mulheres na quinta década de vida e com sítio primário em ovário e apêndice. O desenvolvimento de adenocarcinoma mucinoso é raro na população pediátrica e a topografia no cólon transverso e padrão esporádico não familial também são pouco usuais. Conclusão: O caso relatado alerta para as apresentações atípicas da doença e enfatiza o uso de exames de imagem para o diagnóstico, que, se realizado precocemente, impacta de maneira importante o prognóstico e a sobrevida.

Progression of peritoneal adenomucinosis to the scrotum: a rare occurrence treated with cytoreductive surgery and hyperthermic chemoperfusion of the scrotum in two patients / Progresión de adenomucinosis peritoneal en el escroto de dos pacientes y tratamiento con cirugía citorreductora y quimioperfusión hipertérmica

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

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

Pseudomyxoma peritonei: a review of 17 cases

To evaluate the various presentations, causes and management of Pseudomyxoma Peritonei [PMP], and to create awareness among young surgeons regarding its importance. Retrospective study from Jan. 1999 to Dec. 2005. Surgical Unit of Khyber Teaching Hospital, Peshawar and Gynaecology and Obstetrics Unit, Hayatabad Medical Complex, Peshawar. All patients who had Pseudomyxoma Peritonei during the study period. The clinical records of all patients undergoing laparotomy for various causes were reviewed and cases of PMP separated. Their biodata, clinical presentation, clinical diagnosis, investigation results, operative findings, histopathology report and outcome were recorded. Out of 23,573 cases that underwent laparotomy 17 cases of PMP were on record. All were diagnosed incidentally per-operatively. Most [47.05%] cases were seen in the age group of 30-40 years and 58.82% patients were females. Abdominal pain, mass, abdominal distension and intestinal obstruction were the common presenting features. Ultrasound and CT scan reported three cases as ovarian cysts, three as multiple encysted fluid collections with thin cyst walls, two as ascites, two as ascites with peritoneal thickening, but none as PMP. Surgery was the mainstay of treatment. Appendicectomy with clearing of mucin in 11[64.70%], oophorechtomy and appendicectomy in 3[17.64%], bilateral oophorechtomy and hysterectomy in 2[11.76%], right hemi-colectomy in 1[5.88%] and second look surgery for complications were done in 2[11.76%] cases. Mortality was 11.76% and due to complication including one due to intestinal obstruction and another due to septicemia following second look surgery. Histopathological tissue diagnosis was available in only 13 cases and included mucinous adenoma appendix in five, mucinous cystadenoma ovary in three, mucinous epithelial cells of unknown origin in four and mucinous cystadenoma of borderline malignancy in one case. PMP is under reported in our setup. Pre-operative diagnosis is difficult and incidental findings usually go unnoticed due to lack of awareness and standard management protocol. There is a need to create awareness among surgeons, radiologists, pathologist and oncologist regarding this condition for the better outcome

Relation entre mucocele appendialaire, tumeurs mucineuses ovariennes et malade gilatineuse du peritoine

Pseudomyxoma peritonei is a rare pathology, which posesa number of questions about its physiopatholoogy. We report two cases of appendiceal mucocele and mucinous ovarian tumor with pseudomyxoma peritonei. We discuss the morphological aspect of this affection. A mucinous metaplasia of mesothelial cells caused by local diffusion of the mucus is proposed in order to give an explanration to this microscopically benign but very aggressive due affection to its numerous recurrences

Pseudomyxoma peritoneii.

A middle aged male patient presented with gradual distension of the abdomen. Imaging modalities showed classical features of pseudomyxoma peritoneii which was confirmed by aspiration cytology. Details of the case are described and relevant literature is reviewed.

Pseudomixoma peritoneal maligno: relato de caso / Malignant peritoneal pseudomyxoma peritonei. A case report

É relatado um caso de pseudomixoma peritoneal maligno. Esta rara entidade caracteriza-se por ampla disseminaçäo na cavidade abdominal e comportamento biológico de baixa malignidade. O estado geral costuma estar supreendentemente preservado. O diagnóstico pode ser sugerido pela tomografia computadorizada, mas säo basicamente os achados cirúrgicos que determinam e orientam a conduta. O melhor tratamento tem sido a ressecçäo de tecido tumoral, mas a extensäo deste procedimento ainda näo está bem definida