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At least 19 recordsLinked to original sources
PubMed2002

MUC2 is a molecular marker for pseudomyxoma peritonei.

Pseudomyxoma peritonei, a syndrome first described by Rokitansky in 1842, is an enigmatic, often fatal intra-abdominal disease characterized by dissecting gelatinous ascites and multifocal peritoneal epithelial implants secreting copious globules of extracellular mucin. Although much past interest in the syndrome has focused on the question of whether the disease arises from primary appendiceal or ovarian mucinous tumors of varying malignant potential, the accumulation of extracellular mucin with its resulting obstruction of abdominal viscera and adhesion formation is one major cause of this disease's morbidity and mortality irrespective of the origin or transformed status of the epithelium secreting it. Because of this and because of the recent discovery and cloning of a series of specific mucin genes responsible for mucin secretion and extracellular deposition, we decided to analyze cases of pseudomyxoma peritonei with specific mucin cDNAs and corresponding antibodies to identify a characteristic marker for this disease which ultimately might be targeted therapeutically. Our study specifically investigated MUC2 and MUC5AC because these two mucins possessed the physicochemical property of being gel-forming, a property exhibited by pseudomyxoma peritonei grossly. Expression of MUC2 and MUC5AC in pseudomyxoma peritonei and in accompanying and non-accompanying appendiceal and ovarian mucinous neoplasms were analyzed by in situ hybridization, immunocytochemistry and digital image analysis. A striking overexpression of both MUC2 and MUC5AC was observed in nearly all cases of pseudomyxoma peritonei of unknown and appendiceal origin. In these cases, however, MUC2 gene expression was more prominent. The mucin:cell ratio averaged 10 to 1 in these cases. The primary ovarian mucinous tumors, some of which exhibited pseudomyxoma ovarii and/or peritoneal implants but not classic pseudomyxoma peritonei, in contrast, expressed only MUC5AC and gave rise to implants where the mucin:cell ratio averaged only 1 to 1. MUC2 overexpression then supported an intestinal rather than ovarian origin for true pseudomyxoma peritonei, irrespective of whether an appendiceal primary was documented. In all cases studied, the fidelity of MUC2 and MUC5AC expression held irrespective of the degree of malignant transformation which was present. MUC2 is therefore a reliable molecular marker for pseudomyxoma peritonei.

Appendiceal Neoplasms↗
PubMed2000

Unusual origins of Pseudomyxoma peritonei.

Pseudomyxoma peritonei is a rare neoplastic condition in that gelatinous intraperitoneal fluid collections and mucinous implants on the peritoneal surfaces and omentum are found. The pathological origin is usually an adenoma or well-differentiated adenocarcinoma of the appendix. A smaller number of cases arises from ovarian tumors. We report two unusual cases of pseudomyxoma peritonei. As a child, the first patient underwent several surgical procedures of the large bowel to relieve the consequences of Hirschsprung disease that were complicated by recurrent enteric fistulae. Colonic epithelial cells, with neoplastic changes due to chronic inflammation in the presence of enteric fistulae, were probably dislocated during these episodes, causing pseudomyxoma peritonei. In the second patient, pseudomyxoma was caused by intraperitoneal seeding of a mucinous urachal adenocarcinoma. We hypothesize that seeding of mucus producing epithelial cells into the abdominal cavity may result in this rare entity called pseudomyxoma peritonei, regardless of the source.

Adenocarcinoma, Mucinous↗
PubMed1999

[Intraperitoneal administration of a combined CDDP, 5-FU and MMC therapy for pseudomyxoma peritonei].

Pseudomyxoma peritonei, which is the seeding of the peritoneum by mucin-secreting metastatic deposits and the filling of the peritoneal cavity by these secretions, is a malignancy that even after excision of the metastatic areas has a poor prognosis, since no effective therapy has yet been established. Herein, we report five cases of pseudomyxoma peritonei that responded to a combined postsurgical therapy consisting of CDDP, 5-FU and MMC. A 59-year-old women had a complete response who underwent an appendectomy, a right ovariectomy, and an omentectomy due to pseudomyxoma peritonei, and was postoperatively given a 50 mg intraperitoneal administration of CDDP. Further, a subcutaneous implant-type reservoir was positioned intraperitoneally for postoperative chemotherapy. At 14 days after surgery, therapy was begun which consisted of CDDP (70 mg/m2/day 1), 5-FU (350 mg/m2/day 1) and MMC (6 mg/m2/day 1) administered intraperitoneally, and 5-FU (350 mg/m2/day 1-3) that was continuously infused by an intravenous drip. She received 4 courses of postoperative chemotherapy. As a result of this therapy, the serum CEA values, which had elevated postoperatively, fell to within their respective normal ranges, and at 44 months postoperatively, this patient remains alive. Given the results above, intraperitoneal administrations of this combined CDDP, 5-FU, MMC therapy may be effective for patients with pseudomyxoma peritonei.

Adult↗
PubMed2004

Imaging findings of urachal mucinous cystadenocarcinoma associated with pseudomyxoma peritonei.

Pseudomyxoma peritonei is an uncommon neoplastic condition in which gelatinous fluid-like materials are observed in the peritoneal cavity caused by the dissemination of mucinous adenocarcinoma. Although ruptured appendiceal mucocele is the most common cause, tumors arising from other organs may also cause pseudomyxoma peritonei. We report the imaging findings of an extremely rare case of urachal mucinous adenocarcinoma associated with pseudomyxoma peritonei on computed tomography and magnetic resonance imaging with histopathologic correlation.

Aged↗
PubMedDate not supplied

Gray-scale sonographic findings in a patient with pseudomyxoma peritonei.

Pseudomyxoma peritonei is a rare condition in which the abdominal cavity fills with thick mucoid material as a result of either a benign or malignant disease. We report the findings with gray-scale sonography and laparoscopy in a 74-year-old man with pseudomyxoma peritonei. After paracentesis and drainage of the ascites, sonography visualized hypoechoic nodules in the peritoneum at the surface of the liver and between bowel loops. Laparoscopy revealed mucoid material diffusely throughout the abdominal cavity. The diagnosis of pseudomyxoma peritonei was confirmed by cytologic examination of peritoneal fluid and histologic examination of laparoscopically obtained specimens.

Aged↗
PubMed1996

Pseudomyxoma peritonei.

Pseudomyxoma peritonei is a clinical entity that has lead to much confusion about its etiology, clinical manifestations, treatment, and prognosis. Pseudomyxoma peritonei is currently defined as a grade I mucinous adenocarcinoma that arises from a primary appendiceal adenoma. The clinical entity is defined by a redistribution phenomenon. This means that cancer cells from the appendix tumor are found localized at predetermined sites within the abdomen and pelvis but that the primary tumor may be small and inconspicuous. The small bowel is spared of mucinous tumor, while spaces beneath the hemidiaphragms and within the pelvis are filled by disease. The omentum is massively replaced by tumor in most patients. The disease, when treated by multiple surgical procedures, presents a median survival of approximately 2 years. Good results depend on early diagnosis and treatment before large volumes of disease and multiple surgical procedures lead to small bowel entrapment by tumor. In modern therapy using peritoneotomy procedures and intraperitoneal chemotherapy with mitomycin C and 5-fluorouracil, the long-term survival at 10 years approaches 80 percent.

Humans↗
PubMed1997

[Pseudomyxoma peritonei].

Pseudomyxoma peritonei was diagnosed in 3 men aged 38, 66 and 54 years with weight loss and distension of the abdomen. Pseudomyxoma peritonei results from seeding of the peritoneal cavity with mucus-producing epithelium. The disease is traditionally characterized by accumulation of huge amounts of mucinous ascites, relatively long survival and absence of distant, extraperitoneal metastases. Mostly, the primary tumour is an appendicular adenoma or adenocarcinoma. Sometimes, the primary tumor is localized in the ovaries. Extensive surgical debulking with postoperative intraperitoneal chemotherapy appears to be the treatment of choice.

Adult↗
PubMed2006

Pseudomyxoma peritonei.

Pseudomyxoma peritonei is very rare, and its exact pathogenesis is unknown. It is characterized by intra-abdominal extracellular gelatinous fluid collections. We report a case of pseudomyxoma peritonei in a 38-year-old Saudi male who presented with right iliac fossa mass and weight loss. He was treated initially as an appendicular mass and computed tomography was helpful in making the diagnosis. He was treated by laparotomy, right hemicolectomy and omentectomy, but no perioperative intraperitoneal chemotherapy was instilled. He received postoperative chemotherapy and remained alive with no recurrence at 18-month follow-up.

Adenocarcinoma, Mucinous↗
PubMed2003

[Prognostic difficulties in pseudomyxoma peritonei].

Pseudomyxoma peritonei is a rare disease leading to the accumulation of gelatinous masses in peritoneal cavity, usually secondary to neoplasmatic process of appendix or ovary. The authors describe a case report of 50 years old man with pseudomyxoma peritonei. Literature based discussion on prognostic difficulties and operative therapy perspectives in patients with this disease is presented.

Abdomen, Acute↗
PubMedDate not supplied

[Conservative treatment of pseudomyxoma peritonei].

Pseudomyxoma peritonei is a clinical entity characterized by gelatinous ascites. It is most often due to a mucinous cystadenocarcinoma of the appendix or ovary. These patients die frequently of bowel obstruction after multiple surgical procedures. A case of pseudomyxoma peritonei treated successfully with intraabdominal infusion of a 2.5% solution of dextrose is reported.

Aged↗
PubMed2003

Pseudomyxoma peritonei.

Pseudomyxoma peritonei is a rare form of mucinous ascites associated with peritoneal and omental implants. The origin is controversial, and recent immunohistochemical and molecular genetic evidence suggests the appendix to be the likely site. The condition often presents as an incidental finding at laparotomy. Ultrasonography, computed tomography and magnetic resonance imaging aid in preoperative diagnosis. Treatment remains controversial, surgery being the main stay. The role of intraperitoneal and systemic chemotherapy is poorly defined. We review the literature on the pathology, clinical features and treatment options in pseudomyxoma peritonei.

Appendiceal Neoplasms↗
PubMed1995

[Pseudomyxoma peritonei].

Pseudomyxoma peritonei is an infrequent clinical entity, characterized by peritoneal implantations organized in cyst of mucine or free peritoneal mucine. Most arise from cystadenoma or cystadenocarcinoma of the ovary and appendix, and fewer from other tumours and locations. It is more common in females, and it is usually diagnosed over the fifth decade. Three cases of pseudomyxoma peritonei are presented, two associated to appendicular carcinoma and one to carcinoma of the ovary, and the data published about this disease are reviewed.

Aged↗
PubMed1996

Clinical determinants of treatment failure in patients with pseudomyxoma peritonei.

Pseudomyxoma peritonei is a mucinous cancer of low biologic aggressiveness that disseminates widely throughout the abdominopelvic cavity prior to diagnosis. Complete control of the disease process on peritoneal surfaces should translate into long-term disease-free survival. In a series of 120 patients with pseudomyxoma peritonei, 46 were defined as treatment failures after cytoreductive surgery and regional chemotherapy. Clinical features that correlated significantly with treatment failure were tumor site (colon vs. appendix), histopathology grade (grade II vs. grade I), preoperative cancer volume, and completeness of cancer removal by cytoreductive surgery. For grade I histopathology, treatment failure was 10 times more common after incomplete versus complete cytoreduction. For grade II histopathology treatment failure was three times more common with incomplete cytoreduction. Death from other causes was more common over the age of 65, and stroke was the most common diagnosis. The major causes of morbidity and mortality were related to progressive disease in the abdomen causing intestinal obstruction and biliary obstruction. When treatment failures were categorized as surgical (failure to cytoreduce) versus medical (failure of chemotherapy to sustain a response), there were 27 surgical and 10 medical treatment failures. Improvements in the cytoreductive approach await the development of surgical technologies to increase the total clearance of cancer from the abdominal cavity and chemotherapy treatments that are complete enough to sustain control of small-volume residual disease on all peritoneal surfaces.

Humans↗
PubMed1993

Pseudomyxoma peritonei.

Pseudomyxoma peritonei results from implantation of malignant tumors or irritation from ruptured benign cysts. This disease is traditionally characterized by accumulation of mucinous ascites, relatively long survival period and absence of extraperitoneal metastases. Disease progression is difficult to predict because of the spectrum of underlying pathologic entities. Four unusual instances of pseudomyxoma peritonei are presented. An instance of the neoplasm confined to the splenic parenchyma suggests potential for hematogenous dissemination. The tumor can be limited to and extend along the retroperitoneum. Retained rectal tissue after proctocolectomy may be a possible origin of disease. Enterobronchial fistula formation is a serious long term complication. Aggressive surgical approach with resection of the bulk of disease offers the optimal palliation and prognosis.

Female↗
PubMed2002

Pseudomyxoma peritonei.

Pseudomyxoma peritonei is a relatively rare and poorly understood condition in which mucus accumulates within the peritoneal cavity. The presence of cells in the mucin, either inflammatory or neoplastic, distinguishes it from simple acellular mucus ascites caused by mucinous spillage. There is widespread seeding of the peritoneal and omental surfaces with a heavy cancerous glaze. This is principally a complication of borderline or malignant neoplasm of the ovary and/or appendix. This paper describes two cases of previously healthy women who both presented with an acute abdomen, and were diagnosed postoperatively with pseudomyxoma peritonei. In addition, literature on the clinical presentation, diagnostic procedures, and treatment options has been briefly reviewed.

Abdomen, Acute↗
PubMedDate not supplied

[Cytoreductive surgery (peritonectomy) and intraperitoneal hyperthermic chemotherapy: an innovative and effective approach to the treatment of pseudomyxoma peritonei].

Pseudomyxoma peritonei is a rare disease characterized by a complete redistribution of mucin into the peritoneal cavity. Thirty three PMP patients referred to National Cancer Institute of Milan were submitted to 34 consecutive procedures of peritonectomy and intra peritoneal hyperthermic perfusion (IPHP). Five yrs overall survival has been 96% while 3 yrs PFS 68%. Morbility, mortality and toxicity were 18, 3 and 9% respectively. Peritonectomy associated with IPHP is the most indicated approach to cure patients with DPAM and IFG, subgroup of pseudomyxoma peritonei.

Adult↗
PubMed2006

A specific cadherin phenotype may characterise the disseminating yet non-metastatic behaviour of pseudomyxoma peritonei.

Pseudomyxoma peritonei (PMP) is a rare neoplasm of mainly appendiceal origin, characterised by excess intra-abdominal mucin production leading to high morbidity and mortality. While histological features are frequently indolent, this tumour disseminates aggressively throughout the abdominal cavity, yet seldom metastasises. This study determined the expression of several markers of colorectal differentiation (carcinoembryonic antigen (CEA), cytokeratins (CK20 and CK7), epithelial membrane antigen), mucin production (MUC-2, interleukin-9 (IL-9), IL-9 receptor (IL-9Ralpha)), and cell adhesion (N- and E-cadherin, vimentin) in PMP tissue (n=26) compared with expressions in normal colonic mucosa (n=19) and colorectal adenocarcinoma (n=26). Expressions of CEA and cytokeratins were similar for PMP as those in colorectal adenocarcinomas with the exception that the CK20-/CK7- pattern was rare in PMP (Fisher's exact test: P=0.001). Similarly, expressions of mucin-related proteins were comparable for adenocarcinoma and PMP, with the exception that IL-9 expression was uncommon in adenocarcinoma (P=0.009). Pseudomyxoma peritonei demonstrated a specific pattern of adhesion-related protein expressions of increased N-cadherin, reduced E-cadherin, and increased vimentin (P=0.004), a phenotype suggesting a possible epithelial-mesenchymal transition state. Primary PMP cell cultures were successfully maintained and demonstrated marker expressions similar to those seen in in vivo tissues. These early characterisation studies demonstrate similarities between PMP and colorectal adenocarcinoma, but also reveal a specific cadherin phenotype that may characterise the distinct non-metastasising behaviour of PMP, and form the basis for future mechanistic and therapy-targeting research.

Adult↗