A primary peritoneal pseudomyxoma peritonei arising from the Mullerian tissue: a case report

A primary Pseudomyxoma Peritonei is uncommon. For a long time, ovaries, colon and appendix were proposed like a site origin of these enigmatic lesions. Recent publications show that the majority of cases are due to a rupture of appendicle adenoma. We report a case of a pseudomyxoma peritonei arising in an elderly patient with a previous medical history of appendectomy undergone in infancy. The pseudomyxoma was misdiagnosed as an abdominal abscess related to colitis. In our opinion, metaplasia of the Müllerian system is a possible source of a primary peritoneal pseudomyxoma.     

Ovarian mucinous tumors associated with mature cystic teratomas: morphologic and immunohistochemical analysis identifies a subset of potential teratomatous origin that shares features of lower gastrointestinal tract mucinous tumors more commonly encountered as secondary tumors in the ovary

Most primary ovarian mucinous tumors are of surface epithelial-stromal origin and exhibit diffuse expression of cytokeratin 7 (CK7) combined with variable expression of cytokeratin 20 (CK20); this immunoprofile distinguishes them from most lower gastrointestinal tract tumors secondarily involving the ovaries. The uncommon ovarian mucinous tumors of germ cell (teratomatous) origin have not been extensively evaluated to determine the utility of these markers and other markers of intestinal differentiation for distinguishing these tumors from metastatic gastrointestinal tract mucinous tumors. Immunohistochemical expression of CK7, CK20, CDX2, and villin was assessed in 44 ovarian mucinous tumors associated with a mature cystic teratoma. All cases lacked evidence of a nonovarian primary mucinous tumor. All mucinous tumors were unilateral; 6 cases had bilateral teratomas. All tumors displayed gastrointestinal-type mucinous differentiation, with epithelium that was commonly goblet cell-rich or hypermucinous; 21 were associated with pseudomyxoma ovarii and 3 of these had pseudomyxoma peritonei. Tumor architecture ranged from purely cystadenomatous (n=24), to proliferative (n=13), to carcinomatous (n=6); some tumors had admixtures of these patterns. One tumor had a goblet cell carcinoidlike pattern with pseudomyxoma ovarii. Three carcinomas had a signet ring cell component. Cystadenomatous tumors without pseudomyxoma ovarii (n=15) exhibited all possible CK7/CK20 coordinate expression profiles with nearly equal frequency. All proliferative tumors without pseudomyxoma ovarii (n=8) expressed CK7, most often in combination with CK20 expression. All cystadenomatous and proliferative tumors with pseudomyxoma ovarii (n=9 and n=5) were CK7-/CK20+. All carcinomatous tumors had pseudomyxoma ovarii; 3 were CK7-/CK20+, 2 were CK7+/CK20+, and 1 was CK7+/CK20-. The presence of pseudomyxoma ovarii was significantly associated with a CK7-/CK20+ profile (86% with pseudomyxoma ovarii vs. 13% without, P<0.0001), CDX2 positivity (79% vs. 0%, P<0.0001), and villin positivity (57% vs. 5%, P=0.0009). A subset of mucinous tumors associated with mature cystic teratomas exhibiting morphologic and immunohistochemical features of lower intestinal tract-type mucinous tumors may be teratomatous in origin. In practice, the more common diagnosis of secondary involvement by a lower intestinal tract mucinous tumor should be addressed in the pathology report and in subsequent clinical evaluation; interpretation as a true primary ovarian mucinous tumor of teratomatous origin can be considered as an alternative diagnosis when evaluation and follow-up fail to identify a nonovarian source of the mucinous tumor. Those tumors having CK7 expression with or without CK20 expression may be derived from upper gastrointestinal tract-type or sinonasal-type teratomatous elements but could be independent tumors of surface epithelial-stromal origin.     

Pseudomyxoma peritonei associated with mucinous cystadenoma of the appendix

The authors report a case of a male 86 years old patient with peritoneal pseudomyxoma associated to cystoadenoma of the appendix. Preoperative CT scan showed the presence of a mass in the appendiceal site containing a gelatinous fluid collection and calcifications. Notwithstanding, diagnosis of pseudomyxoma was preoperatively considered less probable due to the rarity of the affection and the advanced age. At laparatomy was revealed the presence of the large mass observed at CT scan, involving the caecum and occupying the retroperitoneal space along the ascending colon. Mucinous ascites was present without invasive mucinous implants in the peritoneum. Operation consisted in a right hemicolectomy with total removal of the retroperitoneal mass and evacuation of the mucinous ascites. Histology confirmed the clinical diagnosis of pseudomyxoma peritonei associated to cystoadenoma of the appendix. The case is of paradigmatic interest as being quite correspondent to the classical Ronnett's definition of pseudomyxoma: a clinicopathological entity characterized by mucinous ascites and non-invasive mucinous implants with a characteristic distribution and containing histologically benign mucinous epithelium derived from an appendiceal mucinous adenoma and having a benign course. According to such definition, the authors stress the importance of an appropriate classification of the observed cases of pseudomyxoma, under whose term is often collected a heterogeneous group of pathological lesions, in order to better evaluate the prognosis and treatment.     

Pleural extension of mucinous tumor in patients with pseudomyxoma peritonei syndrome

Background Pseudomyxoma peritonei syndrome is a rare disease arising from perforation of an adenoma of the appendix. The syndrome is characterized by progressive accumulation of mucinous fluid and tumor within the abdomen and pelvis. Although this tumor is only superficially invasive and does not metastasize, it is a fatal disease. Extra-abdominal spread of pseudomyxoma peritonei is a rare occurrence, with few reports in the medical literature. This review focuses on pleural extension of mucinous tumor in patients with pseudomyxoma peritonei syndrome. Methods From December 1983 to April 1999, all patients who underwent cytoreductive surgery for pseudomyxoma peritonei syndrome were assessed for pleural involvement at the time of the presentation or follow-up. Clinical information on these patients, including chest computed tomographic scan, was retrospectively reviewed. The mechanisms of extension of mucinous tumor from peritoneal cavity to pleural surface and the results of treatment were of special interest. Results Twenty-three of 426 patients (5.4%) showed pleural extension of pseudomyxoma peritonei syndrome. In four patients (17%), extension into the chest occurred before cytoreductive surgery. In 18 patients, the pleural space was entered during a subdiaphragmatic peritonectomy; and, in 12 patients, extension of disease from peritoneal to pleural space occurred. In six patients (26%), surgical interventions were required to excise tumor that had invaded the hemidiaphragm; and, in the six other patients (26%), there was a minor penetration during subphrenic peritonectomy, which was closed immediately. Finally, in seven patients (30%), the mechanism of spread was unknown. Twelve patients were treated for pleural thoracotomy. Eight patients had an attempt to completely eradicate pleural mucinous tumor, and five patients are currently disease free in the chest (22%); four of these five had intrapleural cytoreduction plus intrapleural chemotherapy. The median survival for all 23 patients is 55 months. Conclusion Pleural spread of pseudomyxoma peritonei syndrome may be a direct result of cytoreductive surgery and the subphrenic peritonectomy procedure. In some patients, dissecting mucinous tumor may infiltrate through the diaphragm and result in pleural extension. Pleural extension of pseudomyxoma peritonei syndrome carries a poor prognosis. Intrapleural chemotherapy combined with cytoreductive surgery may be of considerable value in treatment and prevention of disease dissemination; it should be considered when pleural extension of mucinous tumor is feared or confirmed at the time of cytoreductive surgery.     

Möglichkeiten und Grenzen der chirurgischen Therapie des Pseudomyxoma peritonei

Zusammenfassung In einer retrospektiven Analyse bei 7 Patienten wurden die Möglichkeiten und Grenzen der chirurgischen Therapie des Pseudomyxoma peritonei untersucht. Bei allen Patienten war die Appendix der Ursprungsort des Pseudomyxoma. Sämtliche Patienten wurden primär chirurgisch behandelt. Eine R0-Resektion war bei der Primäroperation in lediglich einem Fall mit allerdings benigner Histologie möglich, während bei den übrigen Patienten eine Tumorverkleinerung mit deutlicher Symptombesserung erzielt wurden konnte. Bei Nachweis eines Rezidivs wurden die Patienten einer Relaparotomie zugeführt, um erneut eine Tumorreduktion anzustreben. Die postoperative Morbidität auch nach Wiederholungseingriffen war gering. Die Überlebenszeiten lagen zwischen 2 und 20 Jahren, wobei besonders bei malignem Pseudomyxoma peritonei die additive Chemotherapie mit 5-Fluorouracil einen prognostischen Vorteil erbrachte. Die chirurgische Behandlung ist die Therapie der Wahl beim Pseudomyxoma peritonei, doch ist eine R0-Resektion nur in Ausnahmefällen möglich. Aufgrund der niedrigen Morbidität ist die Indikation zur Relaparotomie beim Rezidiv großzügig zu stellen. Im Vergleich zu anderen Malignomen des Gastrointestinaltrakts sind die Überlebenszeiten, möglicherweise auch durch die Anwendung einer additiven Chemotherapie, deutlich besser.

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The potential and limitations of surgical treatment of pseudomyxoma peritonei

In a retrospective study, the potential and limitations of surgical therapy of pseudomyxoma peritonei were studied in seven patients. In all patients the pseudomyxoma had originated from the appendix. All patients were primarily treated by surgery. An R0 resection at the first operation was possible in only one patient with a benign pseudomyxoma, while significant tumor debulking with improved symptoms was achieved in all other patients. If the tumor recurred relaparotomy was performed to obtain tumor reduction. The perioperative morbidity even after multiple relaparotomies was low. The survival rates ranged between 2 and 20 years with chemotherapy (5 - fluorouracil) which was of particular prognostic benefit in patients with malignant pseudomyxoma peritonei. Surgical therapy is the treatment of choice in pseudomyxoma peritonei, although an R0 resection is hardly feasible. Due to the low morbidity, relaparotomy in cases of tumor recurrence always appears to be indicated. In comparison to other gastrointestinal malignancies, the survival rates in pseudomyxoma peritonei, sometimes treated with additive chemotherapy, are superior.

     

Pseudomyxoma peritonei concomitant with early gastric cancer: Report of a case

The preoperative diagnosis of both appendiceal carcinoma and pseudomyxoma peritonei is difficult because of the nonspecific nature of the associated symptoms. More than 50% of all patients with carcinoma of the appendix are diagnosed with and treated for acute appendicitis. In addition, there have been few reports on patients with an appendiceal carcinoma or pseudomyxoma peritonei in association with other synchronous or metachronous malignancies of the alimentary tract. We herein report the first known patient with synchronous early gastric cancer and pseudomyxoma peritonei, in which the origin was correctly identified preoperatively.     

Intussusception of the appendix by mucinous cystadenoma. Report of a case with an unusual clinical presentation

Appendiceal mucocele is a very rare clinical condition. Associated ascites and an ovarian mass could suggest synchronous ovarian cystadenocarcinoma with pseudomyxoma peritonei. We describe the case of a 36-year-old female with a mucinous cystadenoma of the appendix causing intussusception, diagnosed by CT but not by US scan, since the associated anomalous fixation of the caecum was misleading in defining the precise anatomical site. Although the CT findings were accurate, the synchronous presence of an ovarian cyst and ascites did not allow us to rule out preoperatively a concurrent cystadenocarcinoma of the ovary with pseudomyxoma peritonei. The appropriate surgical treatment was performed on the basis of intraoperative frozen section examination. Surgical treatment depends on the nature of the mucocele: retention forms are effectively treated by appendectomy, while neoplastic conditions require a more extended resection. Treatment of associated ovarian cystadenocarcinoma and pseudomyxoma peritonei includes right colectomy, bilateral ovariectomy and omentectomy. Although a precise preoperative diagnosis of mucocele associated with intussusception of the appendix has been reported as possible, concomitant ascites and ovarian masses, as in the present case, could mimic pseudomyxoma peritonei from concurrent ovarian cystadenocarcinoma. Intraoperative histopathology is required in order to perform the most appropriate treatment.     

腹膜假性黏液瘤11例临床分析

目的探讨腹膜假性黏液瘤的诊断、治疗和预后。方法总结11例腹膜假性黏液瘤的诊治体会并复习文献加以分析。结果11例患者以腹痛、腹胀、腹部包块及不全肠梗阻为主要表现。大多需多次手术治疗。11例均为阑尾黏液囊肿破裂所致。治疗应早期手术治疗,切除原发灶及尽可能切除腹腔内其他脏器上的黏液性病变,必要时联合脏器部分切除。术后辅助治疗包括化疗、局部腹腔内化疗及放疗。我们强调腹腔化疗。术后随访率100%,10年生存率72.73%（8/11）。结论腹膜假性黏液瘤临床少见,部分预后较差。应早期诊断和早期治疗。     

Disseminated Peritoneal Adenomucinosis: A Critical Review

Background: The term pseudomyxoma peritonei has been used in reference to any condition, benign or malignant, in which the peritoneal cavity becomes filled with a gelatinous substance. The term is nonspecific and does not denote therapeutic or prognostic significance. Controversy centers around which patients benefit from and should be treated by aggressive surgical debulking.Methods: A review of the current literature pertaining to the classification, treatment, and prognosis of patients with pseudomyxoma peritonei was undertaken.Results: Disseminated peritoneal adenomucinosis refers to a subset of patients with pseudomyxoma peritonei who derive the greatest long-term benefit from multimodality therapy including aggressive surgical debulking. These patients have a benign form of the disease in which the peritoneal implants are derived from the extrusion of epithelial cells from an adenoma of the appendix. The pathophysiology of mucin deposition is defined by the redistribution phenomenon. The adenomatous cells are distributed according to the fluid flow and gravitational forces within the peritoneal cavity. The small bowel is relatively spared until late in the disease (visceral sparing), and therefore aggressive surgical debulking should be attempted at the first laparotomy by an experienced surgeon. Preoperative computed tomographic imaging can establish the diagnosis and aid in defining which groups of patients are resectable for cure.Conclusions: Attempts at curative treatment should include aggressive debulking and intraperitoneal chemotherapy. Those patients with a high-grade malignant process should be treated symptomatically, because aggressive therapy is associated with high morbidity rates and no long-term improvement in survival.     

Pseudomyxoma peritonei of appendiceal cancer with metastasis to the stomach: report of a case

Appendiceal cancer associated with pseudomyxoma peritonei is a relatively low-grade malignancy rarely associated with extraperitoneal metastasis. We report herein the case of a 71-year-old man in whom a metastasis was found in the stomach 2 years after he underwent surgery for pseudomyxoma peritonei of appendiceal cancer. He was referred to our hospital after presenting with anorexia and vomiting. Gastrofiberscopy, abdominal computed tomography, and ultrasound examination all revealed a mass 4 × 4 cm in size, containing a small ulcer, in the antrum of the greater curvature of the stomach. The histopathological diagnosis made from a biopsy of the tumor was mucinous cystadenocarcinoma. A distal partial gastrectomy was performed and the resected specimens from the appendiceal cancer resected 2 years earlier showed the same histological pattern as that of the gastric lesion. To the best of our knowledge, this is only the second report of pseudomyxoma peritonei secondary to mucinous cystadenocarcinoma of the appendix that metastasized to the stomach. Received: September 12, 2001 / Accepted: January 8, 2002     

Peritoneal pseudomyxoma associated with synchronic malignant mucinous neoplasias of the cecum, appendix and rectum. Case report and review of the literature

Peritoneal pseudomyxoma is a pathological condition that compromises the peritoneum, characterized by the production of large quantities of mucinous liquid, which progressively fills the peritoneal cavity, originating in general from mucinous appendicular or ovarian tumors. We report a peritoneal pseudomyxoma associated with mucinous adenocarcinoma of the appendix synchronic with adenocarcinoma of the rectum in a 44 year old patient, where the initial diagnosis was rectal adenocarcinoma. Tumour of the appendix and peritoneal pseudomyxoma were incidental and found intraoperatively. We focus the histological patterns of the lesions, diagnosis and the treatment, with revision of the literature.     

Swedish Experience with Peritonectomy and HIPEC. HIPEC in Peritoneal Carcinomatosis

Background Peritonectomy with heated intraperitoneal chemotherapy (HIPEC) has shown a survival benefit in selected patients with peritoneal carcinomatosis. This prospective non-randomized study was designed to identify factors associated with postoperative morbidity and survival after peritonectomy HIPEC in patients with this condition. Method Data were prospectively collected from all patients with peritoneal carcinomatosis treated by means of peritonectomy and HIPEC at Uppsala University Hospital between October 2003 and September 2006. Depending on the primary tumor, mitomycin C or a platinum compound was used as a chemotherapeutic agent for perfusion. Results A total of 103 patients were treated. Primary tumors were pseudomyxoma peritonei (47 patients), colorectal cancer (38 patients), gastric cancer (6 patients), ovarian cancer (6 patients) and mesothelioma (5 patients). Postoperative morbidity was 56.3% and was significantly lower in patients treated with mitomycin C for pseudomyxoma peritonei (42%) than in those with another diagnosis treated with platinum compound (71%, P < 0.05). Postoperative mortality was less than 1%. At 2 years, overall survival was estimated to be 72.3%, and disease-free survival was 33.5%. Factors influencing overall and disease-free survival were tumor type and optimal cytoreduction. Conclusion Postoperative morbidity is dependent mainly on a tumor type; however, the chemotherapeutic agent used might also influence morbidity. Survival is determined by optimal cytoreduction and tumor type. Irrespective of age, patients with good performance status benefit from this treatment.     

阑尾黏液囊肿和腹膜假性黏液瘤临床疗效分析

目的探讨阑尾黏液囊肿及腹膜假性黏液瘤的诊断和治疗方法.方法#例阑尾黏液囊肿患者,行单纯阑尾切除术>例;行回盲部切除$例;行右半结肠切除术!例; !例急诊行阑尾切除术后,再次行右半结肠切除术; !例术中发现阑尾囊肿破裂合并腹膜假性黏液瘤,行右半结肠切除术,吸净腹腔内腹水,生理盐水冲洗腹腔,术后全身化疗!次.结果所有患者均手术成功,无明显手术并发症.经随访,均无复发.结论本病临床不多见,但以右下腹痛及包块为主诉的患者应警惕本病.?型超声检查及消化道造影可能是较为简便经济的诊断方法.严格掌握手术适应证,对疑有本病的患者认真探查腹腔会得到最佳的治疗效果.对腹膜假性黏液瘤患者术后化疗或放射治疗可取得较好的疗效.     

Prognostic value of baseline and serial carcinoembryonic antigen and carbohydrate antigen 19.9 measurements in patients with pseudomyxoma peritonei treated with cytoreduction and hyperthermic intraperitoneal chemotherapy

Background Tumor markers are useful for diagnosis and follow-up. We studied the prognostic value of baseline and serial carcinembryonic antigen (CEA) and carbohydrate antigen 19.9 (CA 19.9) measurements in patients with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Methods Sixty-three patients with pseudomyxoma peritonei were treated with cytoreductive surgery and HIPEC. The tumor markers CEA and CA19.9 were collected before therapy and at 3-month intervals during follow-up. Results Preoperative CEA and CA19.9 levels were increased in, respectively, 75% and 58% of the patients. Baseline tumor marker values were related to the extent of tumor. Immediately after HIPEC, both tumor markers decreased markedly (P<.0001). CA19.9 was shown to be a more useful tumor marker than CEA for follow-up. During follow-up, a high absolute CA19.9 level (P=.0005) was predictive for imminent recurrence. Patients who never attained a normal CA19.9 level showed a higher recurrence rate at 1 year (53%; SE, 15%), in comparison to patients with did so (6%; SE 4%). The median lead time of increased CA19.9 to recurrence was 9 months. Conclusions The measurement of the tumor marker CA19.9 is useful in evaluating therapy in patients with pseudomyxoma peritonei treated with cytoreductive surgery and HIPEC. CA19.9 is a prognostic factor for predicting recurrent disease.     

Pseudomyxoma Peritonei Presenting with Femoral Hernias and Peritonitis

Pseudomyxoma peritonei is a rare disease characterized by intraperitoneal accumulation of mucinous ascites produced by neoplastic cells, which mostly originate from an appendiceal adenoma. The clinical presentation of the disease varies, and preoperative diagnosis is often difficult. This report describes a 76-year-old female patient with pseudomyxoma peritonei who presented with lower abdominal pain and bilateral femoral masses. Computed tomography revealed bilateral femoral hernias and fluid collection in the peritoneal cavity. Laparotomy was performed, during which we found extensive diffuse gelatinous material mixed with purulent ascites, and the diagnosis of pseudomyxoma peritonei was confirmed. The disease is rarely associated with femoral hernias or peritonitis. Its clinical presentation, including the characteristic findings on computed tomography, and surgical management are briefly reviewed.     

Pseudomyxoma of the peritoneum. Apropos of 4 cases]

Peritoneal pseudomyxoma is a rare disease (400 published cases). In 60% of all cases, an ovarian tumor is the cause of the disease. The second cause is appendicular mucocele. We report about four personal cases after a longer time lapse. No clinical or biological sign is specific. Ultrasonography associated to CT may allow establishing the preoperative diagnosis. Peritoneal pseudomyxoma has several main features: it is insidious, recurrent, obstinate and severe. Recent work has shown that peritoneal pseudomyxoma is secondary to malignant mucosecretory tumors, mainly of ovarian (cystadenocarcinoma) or appendicular origin, with intraperitoneal cell implants. Surgery is the only treatment with proven effectiveness. The effectiveness of chemotherapy and radiation therapy has not been established.     

Appendiceal mucinous cystadenoma associated with pseudomyxoma peritonei and multicystic peritoneal mesothelioma: Report of a case

An extremely rare case of mucinous cystadenoma developing to pseudomyxoma peritonei together with multicystic peritoneal mesothelioma is herein reported. The patient was a 25-year-old Japanese woman who underwent an appendectomy under the diagnosis of acute appendicitis because of right lower abdominal pain. The patient histopathologically demonstrated appendiceal mucocele with pseudomyxoma peritonei. She underwent a laparotomy in our unit following detailed examinations. Several cystic tumors measuring from 3 to 5 cm in diameter were found in the omentum, and thus omentectomy, partial cecectomy and left oophorectomy were all performed to resect the tumors. Immunostaining and electron microscopy showed the appendiceal lesion to be mucinous cystadenoma, while the peritoneal lesion was multicystic mesothelioma. To our knowledge, this is the first report in the world literature of this rare combination of diseases.     

Pseudomyxoma Peritonei Diagnosed by Peritoneoscopy

A case of pseudomyxoma peritonei diagnosed by peritoneoscopy is reported. The rarity of the tumour and the unfamiliarity of the surgeon with the vagaries of this condition often lead to a pessimistic diagnosis of advanced metastatic mucinous adenocarcinoma. It is suggested that peritoneoscopy may be of help both in the early diagnosis of pseudomyxoma peritonei, thus sparing patients from unnecessary investigations, and in their follow up, especially when the aggressive policy of multiple repeated resections of the recurrent or persistent lesions is to be adopted.     

Pseudomyxoma peritonei diagnosed by peritoneoscopy.

A case of pseudomyxoma peritonei diagnosed by peritoneoscopy is reported. The rarity of the tumour and the unfamiliarity of the surgeon with the vagaries of this condition often lead to a pessimistic diagnosis of advanced metastatic mucinous adenocarcinoma. It is suggested that peritoneoscopy may be of help both in the early diagnosis of pseudomyxoma peritonei, thus sparing patients from unnecessary investigations, and in their follow up, especially when the aggressive policy of multiple repeated resections of the recurrent or persistent lesions is to be adopted.