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.     

卵巢来源腹膜假黏液瘤临床病理分析

  目的  探讨卵巢来源腹膜假黏液瘤（pseudomyxoma peritonei,PMP）的临床病理学特征及免疫组织化学表型。  方法  回顾性分析2010年1月至2019年1月272例于首都医科大学附属北京世纪坛医院确诊为PMP患者的临床资料,研究PMP肿瘤来源,复阅病理切片并行免疫组织化学标记,标记抗体包括CK7、CK20、CEA、Villin、CDX2、SATB2、CA125、ER、PR、PAX8、MUC1、MUC2等。  结果  272例PMP中阑尾来源245例（90.1%）、非阑尾来源27例（9.9%）。卵巢来源PMP仅5例（1.8%）,其中4例为黏液性囊腺瘤、1例为交界性黏液性囊腺瘤,均发生在单侧,5例中2例合并成熟性囊性畸胎瘤;腹膜播散肿瘤中2例为无细胞性黏液、2例为低级别腹膜黏液癌、1例为高级别腹膜黏液癌。免疫组织化学法检测显示,5例PMP患者组织中CK20、CEA、Villin、CDX2均阳性,2例黏液性囊腺瘤合并畸胎瘤患者的SATB2部分阳性、2例SATB2阴性、1例SATB2灶状阳性。  结论  卵巢来源PMP罕见,需对阑尾全部取材或对可疑组织块行连续切片,以排除阑尾黏液性肿瘤,并结合临床症状体征、影像学表现、手术所见、组织学特征及免疫组织化学法检测进行综合分析。      

A case of pseudomyxoma peritonei successfully treated with multidisciplinary treatment including modified FOLFOX6 regimen

We report a case of pseudomyxoma peritonei caused by carcinoma of the appendix, which was successfully treated with multidisciplinary treatment including modified FOLFOX6 regimen. A 45-year-old man was diagnosed as having peritoneal dissemination associated with cancer of the cecum or appendix. Seven cycles of mFOLFOX6 treatment resulted in a marked decrease in ascites and serum levels of carcinoembryonic antigen and carbohydrate antigen 19-9. At laparotomy, a diagnosis of pseudomyxoma peritonei caused by cancer of the appendix was made. Intraperitoneal lavarge with 10,000 mL 5% glucose was performed after right hemicolectomy, omentectomy and removed of mucinous peritoneal nodules. Intraperitoneal chemotherapy comprised of 3000 mL low molecule dextran and 80 mg cisplatin was added on postoperative days 7 and 14. Modified FOLFOX6 regimen was started again two months postoperatively and reached 28 cycles. The patient does not show any sign of recurrence 12 months postoperatively.     

Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei

BACKGROUND AND OBJECTIVES: Pseudomyxoma peritonei syndrome is a rare disease arising from a perforated appendiceal adenoma. The syndrome is characterized by progressive accumulation of mucinous ascites and tumor within the peritoneal cavity. Direct extension of pseudomyxoma peritonei to the pleural cavity is uncommon and has been associated with surgical penetration of the diaphragm at the time of cytoreduction. METHODS: We review the case of a patient who presented with mucoid peritoneal and pleural fluid consistent with spontaneous pleural spread of pseudomyxoma peritonei. RESULTS: Surgical exploration confirmed direct pleuroperitoneal communication by macroscopic diaphragmatic fenestration. CONCLUSIONS: This is a rare phenomenon. We outline a therapeutic approach to be applied when pleural involvement is suspected in patients with pseudomyxoma peritonei syndrome.     

腹膜假性黏液瘤的临床诊治分析

目的探讨腹膜假性黏液瘤的临床特点及诊治方法,并回顾相关文献。方法收集中国医学科学院肿瘤医院1995年6月至2009年12月所收治的31例腹膜假性黏液瘤患者的临床资料,回顾性分析其临床特点和治疗方式对患者生存状况的影响。结果 31例患者中,男10例,女21例。术前确诊率为6.5%。12例患者行肿瘤细胞减灭术,15例行减瘤术,4例患者行开腹活检术。所有患者的总3,5年生存率分别为76.8%和48.4%。行肿瘤细胞减灭术患者的3,5年生存率分别为90.0%和70.0%;行减瘤术患者的3,5年生存率为54.2%和21.7%。两组患者的生存状况比较存在显著统计学差异（P=0.015）。行活检术的患者均于术后3年内死亡。结论腹膜假性黏液瘤是一种进展缓慢但易复发的疾病,应积极行广泛的肿瘤细胞减灭术以提高患者的生存率。     

鸟苷酸结合蛋白α亚基变异与腹膜假黏液瘤关系的研究进展

鸟苷酸结合蛋白(guanine nucleotide-binding protein,G蛋白)是细胞信号转导的关键调控因子.G蛋白结构和功能异常是诸多疾病的常见分子病理机制.α亚基是G蛋白结构和功能中心.腹膜假黏液瘤(pseudomyxoma peritonei,PMP)是一种以黏蛋白持续分泌为主要病理特征的恶性肿瘤综...     

Pseudomyxoma peritonei due to mucinous cystadenocarcinoma in situ of the urachus presenting as an inguinal hernia

Pseudomyxoma peritonei is generally caused by appendiceal and ovarian tumors. Other primary sites have been rarely reported. We describe herein the second reported case of pseudomyxoma peritonei due to mucinous cystadenocarcinoma of the urachus. A 54-year-old man was admitted with a left inguinal hernia that had developed several months prior to his admission. During herniorrhaphy, we found a large amount of gelatinous mucinous material in the indirect-hernia sac and made a diagnosis of pseudomyxoma peritonei on cytological grounds. At re-operation, the origin of the pseudomyxoma peritonei proved to be a ruptured urachal cyst. The urachal cyst and the dome of the urinary bladder were excised. In addition, we removed as much of the gelatinous material as possible. On histological examination, a unilocular cyst was found to consist of noninvasive mucinous adenocarcinoma. We succeeded in removing the rest of the mucinous material by postoperative intraperitoneal lavage with dextran solution, and have observed no evidence of recurrence for 7 years since the operation.     

Clinically aggressive pseudomyxoma peritonei: a variant of a histologically indolent process

BACKGROUND: Three distinct morphologic types of pseudomyxoma peritonei syndrome have been defined: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinoma (PMCA), and a hybrid morphologic type. Prognosis is best in patients with DPAM; unfortunately, some patients with DPAM succumb to a rapidly progressive disease process. METHODS: We identified a subset of 11 patients with a histopathology of DPAM but a clinical course showing an invasive disease process. As a comparison group, from a database of 501 patients with pseudomyxoma peritonei, we selected 22 age- and sex-matched controls with a DPAM histology who are alive with no evidence of disease. Clinical factors were identified for comparison of case and control groups. Expression of mucin antigens, mucin (MUC)1 and MUC2, were evaluated using immunohistochemistry. RESULTS: The study group consisted of 11 patients (five men and six women), with a median survival of 52.2 months (SD 7.46) and a 31% 5-year survival. All 22 matched control cases (10 men and 12 women) are alive and disease-free. Clinical data on the study and control groups including co-morbidity were similar. No significant difference in the expression of MUC1 (P = 0.74, Fisher's exact test) or MUC2 (P = 0.34, Fisher's exact test) was demonstrated between groups. CONCLUSIONS: Further investigation of pseudomyxoma peritonei at a molecular and genetic level may help to formulate a more comprehensive classification.     

A case of pleural extension of mucinous tumor with pseudomyxoma peritonei

We report a case of pleural extension of mucinous tumor with pseudomyxoma peritonei. A 64-year-old man was diagnosed as pseudomyxoma peritonei according to the findings of abdominal CT scan and cytologic examination of the ascitic fluid. At laparotomy in November 2007, jelly-like ascites and a child-head size mutinous tumor involving the greater omentum, transverse colon, and ascending colon were found. Mucious nodules scattered on the undersurface of the right and left hemidiaphragms. In addition, transhiatal extension of mucinous tumors was also extended through the esophageal hiatus, suggesting that thoracic extension of pseudomyxoma peritonei. To avoid an excessive surgical stress, thracotomy was not performed. Cytoreduction surgery was conducted. Postoperative course was uneventful. Although systemic chemotherapy was performed after surgery, intra-abdominal tumor enlarged gradually. Cytoreduction surgery was performed again in September 2008. Thereafter, intra-abdomial tumor enlarged gradually and his physical condition deteriorated. He died 26 months after the first surgery.     

Peritoneal metastases from colorectal cancer: Patient selection for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Cytoreductive surgery (CRS) in combination with hyperthermic intraperitoneal chemotherapy (HIPEC) is an established treatment modality for patients with pseudomyxoma peritonei. The majority of patients with pseudomyxoma who have complete tumour removal and HIPEC are cured.     

超声和CT对腹膜假性黏液瘤的诊断

目的:探讨超声和CT对腹膜假性黏液瘤(pseudomyxoma peritonei,PMP)的诊断价值。方法:回顾性分析经手术病理证实的18例腹膜假性黏液瘤的超声和CT表现。结果:18例腹膜假性黏液瘤中超声显示肝脾“贝壳症”,彩色多普勒血流显示肿物内部和边缘部有或无彩色血流。CT表现为现肝脾扇贝形压迹;脏器周边的分房黏液团块,可多发或单发。 结论:腹膜假性黏液瘤的CT和超声表现有影像学特点,是诊断腹膜假性黏液瘤有价值的方法。     

Pseudomyxoma peritonei: report of an unusual case.

Pseudomyxoma peritonei, a rare disease, is characterized by accumulation of mucinous material in the peritoneal cavity and is commonly caused by ovarian tumors or appendicular lesions. This report presents necropsy findings of an unusual case of pseudomyxoma peritonei caused by dissemination of mucinous adenocarcinoma of the stomach, hitherto unreported in literature.     

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.     

A case of pseudomyxoma peritonei with a pancreatic cancer treated by the intraperitoneal administration of cisplatinum]

A 55-year-old woman underwent laparotomy with a diagnosis of pseudomyxoma peritonei associated with a pancreatic cancer. The peritoneal cavity was filled with much gelatinous material, which was removed as much as possible by suction and by hand. Distal pancreatectomy, appendectomy and bilateral oophorectomy were performed. The peritoneal cavity was washed by saline and 5% glucose solution followed by dispersion of 100 mg of cisplatinum. The intraperitoneal chemotherapy was performed once every two weeks after the operation through an indwelling catheter. Histological examination revealed a mucinous cystadenocarcinoma of the pancreas, causing pseudomyxoma peritonei. The patient is in a good health 4 years after the operation.     

Comparative analysis of perioperative intraperitoneal chemotherapy regimen in appendiceal and colorectal peritoneal carcinomatosis

Background

Perioperative intraperitoneal chemotherapy (PIC) is delivered by intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and early postoperative intraperitoneal chemotherapy (EPIC). The relative survival benefits of each or both regimens are explored in this large series of patients undergoing cytoreduction at a single institution.

Methods

Patients with a complete (CCR0) or optimal (CCR1) cytoreduction who received intraperitoneal chemotherapy for appendiceal and colorectal peritoneal carcinomatosis were included for analysis. PIC regimens were delivered according to the treatment protocol. Standardized statistical analyses were performed.

Results

Of 262 patients, 98 patients (37%) had colorectal peritoneal carcinomatosis, 108 patients (41%) had low-grade pseudomyxoma peritonei and 56 patients (21%) had appendiceal peritoneal carcinomatosis. For pseudomyxoma peritonei, recurrence-free survival (RFS) did not vary with PIC regimen, 5-year survival was 86% in the HIPEC and EPIC group and 64% in the HIPEC or EPIC group (P = 0.070). For appendiceal peritoneal carcinomatosis, RFS and overall survival (OS) did not vary with PIC regimen. For colorectal peritoneal carcinomatosis, the median RFS was 33 months in the HIPEC and EPIC group, 19 months in the HIPEC alone group and 20 months in the EPIC alone group (P = 0.046). OS did not vary with PIC regimen.

Conclusion

From our experience, without compromising the perioperative morbidity and mortality, PIC consisting of HIPEC and EPIC appears to be associated with potential survival benefits of improved OS in pseudomyxoma peritonei and RFS in colorectal peritoneal carcinomatosis.     

Concomitant mucinous tumors of appendix and ovary. Result of a neoplastic field change?

BACKGROUND. Mucinous tumors of the appendix and ovary are known to occur together in association with pseudomyxoma peritonei. It has been postulated that this association may be attributable to the development of independent tumors or to metastasis from one site to another. METHODS AND RESULTS. This article reports two patients with concomitant mucinous ovarian and appendiceal tumors in the absence of pseudomyxoma peritonei. CONCLUSIONS. The evidence suggests that these tumors are independent primary neoplasms that develop as a result of neoplastic field change that affects colonic-type epithelium.     

Computed tomographic and ultrasonographic findings of pseudomyxoma peritonei

Pseudomyxoma peritonei is an unusual clinical entity in which the peritoneal cavity is filled with a gelatinous material that is mucinous in nature. We present ultrasonographic and computed tomography findings in a patient with pseudomyxoma peritonei secondary to ovarian mucinous cystadenocarcinoma.     

Indikationen für das HIPEC-Verfahren

For decades, there was therapeutic nihilism regarding patients with peritoneal carcinomatosis or primary peritoneal tumors. Development of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemoperfusion (HIPEC) completely changed this attitude. Nowadays, CRS and HIPEC are standards of care for pseudomyxoma peritonei and peritoneal mesothelioma, and are frequently used in secondary peritoneal metastasis. This review article summarizes the currently available publications and results for the different tumor entities, and critically discusses the indications: patients with pseudomyxoma peritonei and peritoneal mesothelioma can be treated with curative intent in cases of complete CRS; they also benefit from incomplete resection and HIPEC. In colorectal cancer, CRS and HIPEC are recommended in patients with a peritoneal cancer index (PCI) < 20. Proactive second-look surgery and HIPEC enable curative control of peritoneal carcinomatosis in patients at a high risk of peritoneal recurrence. In gastric cancer, limited peritoneal metastasis might be treated with CRS and HIPEC in individual patients. Staging laparoscopy is mandatory and has a decisive role in therapy planning.     

<Emphasis Type="Italic">Pseudomyxoma peritonei</Emphasis> secondary to epithelial appendicular neoplasms. Experience in a non-specialised centre

Introduction Pseudomyxoma peritonei is an infrequent entity, defined by collections of gelatinous material in the abdomen and pelvis and mucinous implants on peritoneum, secondary to the rupture of a mucinous lesion, usually of ovarian or appendiceal origin. Materials and methods We present our experience of 11 cases (6 males and 5 females) diagnosed with pseudomyxoma peritonei secondary to epithelial appendicular neoplasms over 27 years. The mean age of the patients was 68 years. Clinical manifestations were abdominal distension (55%), right lower quadrant pain (45%) suggesting acute appendicitis and constitutional syndrome (36%). An abdominal mass was detected at physical examination in 4 patients. CT scan revealed a tumour in right iliac fossa in 4 patients, peritoneal enlargement in 1 and a liquid collection in 1. Preoperative diagnosis was acute abdomen in 5 patients, peritoneal carcinomatosis in 3 and undetermined abdominal mass in 3. Results Surgical findings suggested pseudomyxoma peritonei in 8 patients and peritoneal carcinomatosis in 3. Appendicectomy was performed in 9 patients, and in 3 of them bilateral anexectomy was also performed. One patient underwent ileocaecal resection and another a right hemicolectomy. In all the cases, mucinous material was eliminated as much as possible. Pathology revealed mucinous cystoadenoma in 6 cases, mucinous cystoadenocarcinoma in 3 and epithelial hyperplasia in 2 patients. Median survival was 54 months, with a 5-year survival rate of 40%. The last case we treated was sent to a reference centre for the treatment of pseudomyxoma peritonei. Conclusions There is no consensus on the best treatment for pseudomyxoma peritonei. We recommend avoiding incomplete surgical resections in non-reference centres and submitting patients to a reference centre to undergo adequate treatment.