Possible therapeutics for Pseudomyxoma peritonei: a rare, lethal, and the least investigated disease

Pseudomyxoma peritonei (PMP) refers to a growth disorder characterized by glycoprotein neoplasm in the peritoneum, where mucin oversecretion occurs. The tumors of the appendix region are well associated with PMP; however, ovarian, colon, stomach, pancreas, and urachus tumors have also been linked to PMP. Other mucinous tumors in the pelvis, paracolic gutters, greater omentum, retrohepatic space, and Treitz ligament can be the reason for PMP. Despite being rare and having a slow growth rate, PMP can be lethal without treatment. It is treated with neoadjuvant chemotherapy with the option of cytoreductive surgery and intraperitoneal chemotherapy. In the current study, we hypothesize that there may be novel gentle ways to inhibit or eliminate the mucin. Dr. David Morris has used mucolytics - such as bromelain and N-acetyl cysteine to solubilize mucin. In the present review, we aimed to study the regulation of mucin expression by promoter methylation, and drugs that can inhibit mucin, such as boldine, amiloride, naltrexone, dexamethasone, and retinoid acid receptors antagonist. This review also explored some possible pathways, such as inhibition of Na + , Ca2+ channels and induction of DNA methyltransferase along with inhibition of ten-eleven translocation enzymes, which can be good targets to control mucin. Mucins are strong adhesive molecules that play great roles in clinging to cells or cell to cell. Besides, they have been greatly involved in metastasis and also act as disease markers for cancers. Diagnostic markers may have exclusive roles in disease initiation and progression. Therefore, the present review explores various drugs to control and target mucin in various diseases, specifically cancers. (AU)

Clinical and ultrasound features of pseudomyxoma peritonei and its histopathological subtypes among women seen at a Philippine tertiary hospital: A 10‑year review / Philippine Journal of Obstetrics and Gynecology

Objectives@#Pseudomyxoma peritonei (PMP) in women arises from an appendiceal or ovarian pathology and presents either of two histological subtypes of differing prognosis, disseminated peritoneal adenomucinosis (DPAM), or peritoneal mucinous carcinomatosis (PMCA). This study aimed to evaluate the demographic, clinical, and sonographic features among affected women and the differences between the two histological subtypes.@*Methods@#A retrospective study was conducted involving 36 women with histopathological diagnosis of PMP who had preoperative ultrasound and underwent surgery at the department of obstetrics and gynecology in a tertiary hospital. Demographic and clinical data, ultrasound images and reports, and final histopathology were reviewed. To compare the subtypes, one‑way analysis of variance for continuous data and Chi‑square/Fisher exact test for categorical data were used, with P < 0.05 indicating statistical significance.@*Results@#Patients were mostly >50 years of age, multigravid, and presented with abdominal distention. Ultrasound examinations consistently showed amorphous, mixed echo or echogenic ascites, peritoneal thickening, and omental caking. Adnexal/ovarian masses were detected in 66.7% of cases. Omental caking was significantly more prevalent in PMCA (83.3%; P = 0.0002), whereas larger ovarian tumors (>20 cm) and papillarities were more common in DPAM (both 92.9%; P = 0.0005). Most patients underwent gynecologic surgery (n = 31; 86.1%), and 14 (38.9%) required readmission due to recurrence. The final histopathology revealed largest tumor involvement of the appendix (n = 13; 36.1%), the ovaries (n = 11; 30.5%), or undetermined (n = 12; 33.3%).@*Conclusions@#Preoperative diagnosis of PMP is possible based on its clinical and ultrasound features. Although the subtypes are similar in most of these features, certain ultrasound findings may aid in distinguishing them.

Cistoadenoma volumoso associado a teratoma ovariano maduro, com evolução para pseudomixoma peritoneal: relato de caso / Giant cystadenoma associated with mature ovarian teratoma with progression to pseudomyxoma peritonei: a case report

Massas anexiais representam grande desafio diagnóstico, devido a seu caráter geralmente assintomático¹. Traz-se caso peculiar de paciente que evoluiu com sintomas exuberantes de rápida evolução, situação característica de cistoadenomas, tumores benignos de origem epitelial²,³. Realizada laparotomia, com achados macroscópicos que revelaram conteúdo mucinoso e presença de pelos e neovascularização em seu interior. O anatomopatológico confirmou o diagnóstico de cistoadenoma associado a teratoma ovariano, sendo assim considerado um tumor de colisão ovariana.

Adnexal masses represent a major diagnostic challenge due to its usually asymptomatic characteritcs¹. We present a peculiar case of a patient who evolved with exuberant symptoms of rapid evolution, characteristic of cystadenomas, benign tumors of epithelial origin²,³. Laparotomy was performed, with macroscopic findings that revealed mucinous content and the presence of hairs and neovascularization inside. Pathology confirmed the diagnosis of cystadenoma associated with ovarian teratoma, thus being considered an ovarian collision tumor.

Clinical evaluation of tumor-stroma ratio in pseudomyxoma peritonei from the appendix / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the effect of tumor-stroma ratio (TSR) on disease progression and prognosis of pseudomyxoma peritonei (PMP) from the appendix.@*METHODS@#The study included 30 PMP patients with complete individual patient data, who underwent cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) in Beijing Shijitan Hospital. Image-Pro Plus was used to quantitatively analyze the proportion of tumor and stromal areas in hematoxylin-eosin staining pathological images, from which TSR was derived. Correlation studies were conducted to evaluate the relationships between TSR and clinicopathological features, immunohistochemical characteristics, and prognosis of PMP.@*RESULTS@#Among 30 PMP patients, there were 16 males (53.3%) and 14 females (46.7%), with the mean age of (54.9±2.3) years. There were 15 cases (50.0%) of low-grade mucinous carcinoma peritonei (LMCP) and high-grade mucinous carcinoma peritonei (HMCP), respectively, with vascular tumor emboli occurring in 4 cases (13.3%), nerve invasion occurring in 3 cases (10.0%), and lymphatic metastasis occurring in 4 cases (13.3%). The median peritoneal cancer index (PCI) score was 36 (range: 3-39). The median TSR was 8% (range: 2%-24%), with TSR≤10% in 19 cases (63.3%) and TSR>10% in 11 cases (36.7%). Immunohistochemistry showed that 16 cases (53.3%) had Ki67 label index ≤ 50% and 14 cases (46.7%) > 50%. The mutation rate of p53 was 56.7% and the loss rate of MMR protein was 11.8%. In addition, the expression rates of MUC2, MUC5AC, CDX2, CK7, and CK20 were 66.7%, 100.0%, 82.6%, 56.0%, and 92.3%, respectively. There were significant correlations between TSR and histopathological types, nerve invasion, Ki67 label index, and p53 mutation (P<0.05 for all). At the end of the last follow-up, 21 patients (70.0%) died and 9 patients (30.0%) survived, including 6 patients survived with tumor. The median overall survival (OS) was 12.7 months (95%CI: 10.4-11.5 months), and the 1-, 2-, and 3-year survival rates were 60.5%, 32.3%, and 27.7%, respectively. The median OS was 19.4 months (95%CI: 3.0-35.9 months) in the TSR≤10% group, versus 12.6 months (95%CI: 0.7-24.5 months) in the TSR>10% group (χ2=3.996, P=0.046).@*CONCLUSION@#TSR is correlated with histopathological types, tumor proliferation, invasion behaviors and prognosis of PMP, thus could be a new prognostic indicator for PMP.

Transumbilical endoscopic surgery in the diagnosis of ascites of unknown origin / 中南大学学报(医学版)

To explore whether transumbilical endoscopic surgery (TUES) can effectively and safely elucidate the causes of ascites of unknown origin.
 Methods: A total of 23 consecutive patients with ascites of unknown origin who undertook TUES procedures in the Department of Gastroenterology of The Third Xiangya Hospital of Central South University between January 2014 and January 2016 were retrospectively investigated. Clinical manifestations, laboratory examinations and findings from radiological examinations and endoscopic investigations were noted before the procedure. Conditions of the abdominal cavity under endoscope, final diagnosis and outcome of patients were carefully recorded.
 Results: TUES procedure was successfully performed in all 23 patients with an operation time of (58.2±13.9) min. Twenty-two patients were undertaken biopsy on the nodules or masses that found in the abdominal cavity. Definite diagnoses were established in the overwhelming majority of patients (22/23). The most common causes of ascites for the 23 cases was tuberculosis (8 cases), followed by peritoneal carcinomatosis (6 cases), and pseudomyxoma peritonei (5 cases). Operation-related complications, such as postoperative bleeding, perforation, peritonitis, intraabdominal chronic abscesses, were not observed, except one case showed a transient moderate fever in 24 hours after operation. No mortality related to TUES occurred. We concluded that TUES was a feasible, economic and minimally invasive approach to access the peritoneal cavity.
 Conclusion: TUES combinated with biopsy can effectively elucidate the causes of ascites of unknown origin.

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.

Peritoneal carcinomatosis treated with cytoreductive surgery and intraperitoneal chemotherapy / Carcinomatose peritoneal tratada com cirurgia citoreductiva e quimioterapia intraperitoneal

ABSTRACT Introduction: To evaluate the combined treatment with cytoreductive surgery and intraperitoneal chemotherapy for peritoneal carcinomatosis arising from colorectal cancer, pseudomyxoma peritonei and mesothelioma. Methods: Data were obtained from 73 patients with peritoneal carcinomatosis arising from colorectal cancer (52.1%), pseudomyxoma peritonei (41.1%) or mesothelioma (6.8%) between 2002 and 2011. We reported the morbidity grade (II, III and IV), mortality and survival rates of the candidates after cytoreductive surgery and intraperitoneal chemotherapy. Results: 41 (56.2%) women participated, and the median age was 50 years. Thirty-nine patients (53.4%) underwent complete cytoreductive surgery and intraperitoneal chemotherapy. Patients who underwent a complete cytoreduction received intraperitoneal chemotherapy with mitomycin C, from which only 16/39 (41%) had hyperthermic intraperitoneal chemotherapy (41-42 °C). The overall morbidity rate was 23.3% and the grade III/IV complication rate was 12.3%. The overall mortality rate was 5.5%. The univariate analysis showed that cytoreductive surgery and intraperitoneal chemotherapy (p = .029), a blood transfusion (p = .002) and the operative time (p = .001) were significant for the occurrence of postoperative complications. Patients with peritoneal carcinomatosis from colorectal cancer who underwent complete cytoreductive surgery and intraperitoneal chemotherapy had overall survival rates of 81.3%, 12.5% and 12.5% at 1, 3 and 5 years, respectively. Patients with peritoneal carcinomatosis from pseudomyxoma peritonei who underwent complete cytoreductive surgery and intraperitoneal chemotherapy had overall survival rates of 84.2%, 77.7% and 77.7% at 1, 3 and 5 years, respectively. Conclusion: The combined treatment for peritoneal carcinomatosis may be performed safely with acceptable morbidity and mortality in a specialized unit setting. Although over half of patients underwent normothermic intraperitoneal chemotherapy, our results were comparable to results from others centers.

RESUMO Introdução: O objetivo foi avaliar o tratamento combinado da cirurgia citorredutora e quimioterapia intraperitoneal em pacientes com carcinomatose peritoneal secundária ao câncer colorretal, pseudomixoma peritoneal e mesotelioma. Métodos: Foram obtidos dados de 73 pacientes com carcinomatose peritoneal secundária ao cirurgia citorredutora (52.1%), pseudomixoma peritoneal (41,1%) ou mesotelioma (6,8%). Foram avaliados o grau de morbidade, a taxa de mortalidade e as taxas de sobrevida após a cirurgia citorredutora e quimioterapia intraperitoneal. Resultados: 41 (56,2%) pacientes do sexo feminino participaram, com média de idade de 50 anos. 39 pacientes (53,4%) foram submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal. Todos esses receberam Mitomicina C, sendo 16/39 (41%) quimioterapia intraperitoneal hipertérmica (41-42°C). A morbidade global foi 23,3%, com taxa de mortalidade global de 5,5%. A análise univariada mostrou que câncer colorretal e quimioterapia intraperitoneal (p = .029), transfusão sanguínea (p = .002) e tempo operatório (p = .001) foram associados com complicações pós-operatórias. Pacientes com carcinomatose peritoneal secundária ao cirurgia citorredutora submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal tiveram sobrevida global de 81,3%; 12,5% e 12,5% em 1, 3 e 5 anos, respectivamente. Os pacientes com pseudomixoma peritoneal que foram submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal tiveram sobrevida global de 84,2%; 77,7% e 77.7% em 1, 3 e 5 anos, respectivamente. Conclusão: O tratamento combinado para carcinomatose peritoneal é seguro quando realizado em centros terciários com experiência no procedimento. Embora mais da metade dos pacientes tenham sido submetidos a quimioterapia intraperitoneal normotérmica após a cirurgia citorredutora completa, os resultados podem ser comparados a de outros centros que utilizam exclusivamente a quimioterapia hipertérmica.

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.

Pseudomyxoma peritonei: Revisited / Philippine Journal of Obstetrics and Gynecology

@#Bleeding after menopause raises suspicion of malignancy; more

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.

Single Center Experience With Hyperthermic Intraperitoneal Chemotherapy

PURPOSE: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been proposed for controlling peritoneal seeding metastasis in some kinds of cancers, including those of colorectal origin, but their safety and oncological benefits are subjects of debate. We present our early experience with those procedures. METHODS: Data were retrospectively collected from all patients with peritoneal carcinomatosis (PC) and pseudomyxoma peritonei (PMP) treated using CRS and HIPEC at Yonsei Cancer Center between July 2014 and July 2015. Short-term outcomes and risk factors for postoperative complications were analyzed. RESULTS: Twenty-three patients with PC (n = 18) and PMP (n = 5) underwent CRS and HIPEC. Median follow-up and age were 2 months and 54 years, respectively. The median peritoneal carcinomatosis index score was 15, and CC0-1 was achieved in 78.3% of all patients. The median operation time and bleeding loss were 590 minutes and 570 mL, respectively. Grade-IIIa/grade-IIIb complications occurred in 4.3% (n = 1)/26.1% (n = 6) of the patients within 30 days postoperatively, and no 30-day mortalities were reported. Factors related to postoperative complications with CRS and HIPEC were number of organ resection (P = 0.013), longer operation time (P < 0.001), and amount of blood loss (P = 0.003). All patients treated with cetuximab for recurred colorectal cancer had grade-III postoperative complication. CONCLUSION: Our initial experience with CRS and HIPEC presented about 30% grade-III postoperative complications. Therefore, expert surgeons need to perform those procedures with great caution in selected patients who might benefit from it.

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.

Experience in transumbilical endoscopic surgery diagnosis for 3 cases of pseudomyxoma peritonei / 中南大学学报(医学版)

Three patients of pseudomyxoma peritonei who were diagnozed by transumbilical endoscopic surgery (TUES) were reviewed retrospectively from September 2014 to November 2014. Three cases of ascites patients underwent TUES were diagnozed as pseudomyxoma peritonei. All operations were successful. No open surgery or laparoscopic surgery was required. The mean operative time was (45±16) min; the mean intraoperative blood loss was 510 mL; the mean hospital stay time was 3 days. During the follow up of 911 months, no obvious scar was observed. Cosmetic results appear to be excellent. All patients were treated with intraperitoneal hyperthermia and chemotherapy. The survival rate was 100%. As a novel scarless endoscopic invasive abdominal surgery, TUES has high clinical value with the advantages such as small trauma, no scars, small risk and low cost in the diagnosis of unexplained ascites.

Surgical treatment for high-grade pseudomyxoma peritonei originated from appendix: Analysis of clinical outcomes of repeated debulking surgery

PURPOSE: To evaluate the effect of repeated debulking surgery for high-grade pseudomyxoma peritonei (PMP) originating from the appendix.METHODS: Between January 1998 and December 2014, fifty patients, who underwent debulking surgery for high-grade PMP originating from the appendix, were obtained from a prospectively collected database and retrospectively analyzed. Two groups according to the number of operations were divided and analyzed.RESULTS: A total of 118 operations were performed. Thirty-one patients received more than two operations. The median interval between operations was 18.2 months (range, 2–170 months). Complications developed after 26 operations (22.0%), including ileus (n=10), intra-abdominal fluid collection (n=7), surgical site infection (n=5), and others. There were two mortalities within 30 days after operation. Between two groups of patients who received one operation only and patients who received more than two operations, transfusion, diversion operation, and postoperative complication rate showed statistically significant differences. Two groups of patients had no differences in overall survival rates.CONCLUSION: Our results indicate that the number of operations does not affect the survival rate of high-grade appendiceal PMP, in which repeated debulking surgery is vital to relieve symptoms of the tumor burden.

A rare pseudomyxoma peritonei with a MSH2 variation of unknown significance and two mutation carrier family members

Pseudomyxoma peritonei (PMP) is a rare tumor that usually originates in the appendix, but a small number of cases originate in the ovary. Lynch syndrome (LS) is an autosomal dominant hereditary condition that increases the risk of cancer, particularly in the colon and endometrium. Mutations in the mismatch repair genes (MSH2, MLH1, MSH6, and PMS2) increase the risk of LS. Reported PMP cases with hereditary gene mutations of unknown significance are also rare. Here, we investigated a PMP patient and her family members, who have an MSH2 variant of unknown significance. Physicians have an important role in counseling, management, and surveillance based on genetics and pathogenicity.

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.

Seudomixoma peritoneal como causa infrecuente de ascitis mixta masiva / Peritoneal Pseudomyxomata tumor as uncommon cause of massive mixed ascites

Se presenta el caso clínico de una paciente de 32 años de edad con historia de aumento progresivo de volumen del abdomen, que le dificultaba la respiración y la alimentación, quien ingresa en el Servicio de Medicina Interna del hospital del instituto ecuatoriano del seguro social de la ciudad de Ibarra, con diagnóstico de ascitis para estudio. Al realizar la paracentesis se extrae líquido de aspecto gelatinoso, por lo cual se plantea la hipótesis de un seudomixoma peritoneal. Teniendo en cuenta estas características del líquido se recupera el informe de biopsia de la apendicectomía efectuada 2 años antes, donde se halló la presencia de un tumor mucinoso de apéndice. Se efectuó cirugía citorreductora sin hipertermia intraperitoneal y se confirmó la citada hipótesis.

The case report of a 32 years patient with history of progressive increase in abdomen volume making difficult breathing and feeding is presented. She is admitted in the Internal Medicine Service of the Ecuatorian institute hospital of social insurance in Ibarra city, with ascites diagnosis to be studied. When carrying out the abdominal tap, a jelly-like liquid is extracted, reason why the hypothesis of a peritoneal pseudomyxomata tumor is stated. Taking into account these characteristics the biopsy report from an appendectomy made 2 years earlier is recovered, where the presence of an appendix mucinous tumor was found. Cytoreduction surgery was performed without intraperitoneal hyperthermia and the mentioned hypothesis was confirmed.

Neoplasias mucinosas del apéndice / Mucinous Neoplasm of the Appendix

Se presenta el caso de una paciente femenina de 52 años de edad que consulta por dolor abdominal al servicio de urgencias, donde se le realizan estudios imaginológicos y se sospecha de mucocele. Se confirma el diagnóstico por patología de mucocele del apéndice por neoplasia mucinosa apendicular de bajo grado. En este artículo se describe la presentación clínica de la paciente y se analizan los hallazgos en los estudios por imágenes, en cirugía y en patología. Posteriormente se hace una revisión de la literatura de los tumores mucinosos del apéndice, con énfasis en los hallazgos imaginológicos. Estos tumores son raros, presentan diferente histología y no tienen predilección de género. Se presentan principalmente como abdomen agudo, aunque tienen otras formas de presentación que pueden confundir al clínico. Como se describe, los estudios por imágenes cumplen un papel importante en el diagnóstico, seguimiento y detección de complicaciones.

We present the case of a 52- year- old woman who complains of abdominal pain to the emergency room, ultrasound and CT scan suggested the diagnosis of mucocele and was confirmed by pathology as mucocele of the appendix due low grade appendix mucinous neoplasm. In our review we describe clinical presentation of the patient and discuss imaging findings as well as findings in surgery and pathology. Subsequently, there is a literature review and the mucinous tumors of the appendix, with emphasis in the imaging techniques. These tumors are rare, have different histology and no gender predilection. They occur mainly as acute abdomen, although they have other forms of presentation which may mislead the clinician. As described, imaging techniques play an important role in the diagnosis, follow-up, and detection of complications

Necrotizing Fasciitis Arising From an Enterocutaneous Fistula in a Case of an Appendiceal Mucocele

An appendiceal mucocele (AM) is a rare tumorous condition of the appendix. Many patients with AM are admitted to the hospital with abdominal pain or discomfort, and many cases are found incidentally. Although the rate of complications in patients with AM is very low, if left untreated, a mucocele may rupture and produce a potentially fatal entity known as pseudomyxoma peritonei. In this paper, we report a case of an 80-year-old man with necrotizing fasciitis arising from an enterocutaneous fistula caused by AM.