Critical alkalosis following intraperitoneal irrigation with sodium bicarbonate in a patient with pseudomyxoma peritonei

Pseudomyxoma peritonei (PMP) is a rare disease, presenting with large amounts of mucinous ascites, and treatment with intraperitoneal irrigation with mucolytic agents has been tried. We report a patient with PMP who underwent intraperitoneal irrigation with sodium bicarbonate and exhibited marked alkalosis. The patient was a 78-year-old woman who had mucinous ascites, and an appendiceal and an ovarian tumor. Bilateral salpingo-oophorectomy and appendectomy were performed, and she then underwent intraperitoneal irrigation with sodium bicarbonate (7%, 1000 ml). Shortly after the irrigation, blood gas analysis showed critical alkalosis (pH, 7.66; base excess [BE], 24 mEq x l(-1); HCO3 (-), 50 mEq x l(-1)) with electrolyte imbalance (Na+, 153 mEq x l(-1); K+, 2.8 mEq x l(-1); Ca2+, 0.98 mEq x l(-1), Cl(-1), 99 mEq x l(-1)). The alkalosis and electrolyte imbalance were ameliorated with the administration of potassium chloride and calcium chloride intravenously, and the patient was extubated after the 2-h surgical procedure. The patient was discharged home after 15 days without problems. Sodium bicarbonate may be an effective mucolytic agent for PMP. However, during irrigation with sodium bicarbonate, careful evaluation of the acid-base balance and serum electrolytes, and prompt treatment of alkalosis or electrolyte imbalance, should this occur, are of great importance.     

Approach to pseudomyxoma peritonei

Pseudomyxoma peritonei(PMP) is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. The incidence of PMP is believed to be approximately 1-3 out of a million per year. Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and severely impacts quality of life. Curative treatment for PMP is complete cytoreductive surgery(CRS) with hyperthermic intraperitoneal chemotherapy(HIPEC). An extensive literature review was conducted searching EMBASE, MEDLINE, Pub Med, and Google Scholar databases for PMP in aims to delineate a clinical approach to diagnosis and treatment. Literature was limited to the years 2007-2018. We found the 5-year overall survival with CRS and HIPEC estimated to be between 23%-82% and rates of major complications as high as 24%. Therefore, it is important to appropriately stage and select patients that should undergo CRS with HIPEC. Modalities like MDCT radiological scores have been shown to have sensitivity and specificity of 94% and 81%, respectively, in being able to predict resectability and survival. Despite treatment, the disease often recurs. Tumor markers have significant potential for establishing prognosis preoperatively, and this paper will review the most recent evidence in support of them.     

Survival of patients with pseudomyxoma peritonei treated by serial debulking

Aim We evaluated the outcome of patients with pseudomyxoma peritonei (PMP) after traditional debulking. PMP is a clinical condition characterized by disseminated intraperitoneal mucinous tumours often accompanied by mucinous ascites derived usually from an appendiceal neoplasm. Patients with PMP have traditionally been treated by serial debulking, but aggressive cytoreduction followed by hyperthermic intraperitoneal chemotherapy is now advocated as standard treatment in PMP. Method The analysis included 33 consecutive patients with PMP who underwent traditional debulking surgery between June 1984 and August 2008. The patient characteristics and details of the treatment were analysed retrospectively. The primary end‐point was survival. Results The overall 5‐ and 10‐year survival rates were 67% and 31% respectively. The patients underwent an average of 3.2 ± 0.4 operations (range 1–10). Of 33 patients, 23 (70%) underwent only 1–3 operations. The 30‐day operative mortality rate was 2.7%. However, four patients (12%) seemed to have achieved long‐term disease‐free survival of more than 5 years. Conclusions The 5‐year survival is comparable with results achieved in patients receiving a combination of cytoreductive surgery and intraperitoneal chemotherapy, but in the long term, the latter seems superior.     

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.     

阑尾源性腹膜假黏液瘤的治疗

目的 探讨阑尾源性腹膜假黏液瘤的治疗效果.方法 回顾性分析1970年至2010年卫生部北京医院收治的51例阑尾源性腹膜假黏液瘤患者的临床资料,对患者手术及复发再手术情况,辅助治疗及随访情况进行分析.术后是否接受腹腔化疗的患者复发再手术间隔时间的比较采用双尾t检验.结果51例患者中48例接受手术治疗,手术时间(135±72) min.34例患者肿瘤复发,其中16例接受再减瘤手术,再手术次数33次.45例患者中位随访时间49.7个月(3～ 132个月),25例带瘤生存,16例无瘤生存,4例死亡.术后病理检查良性型-弥漫性腹膜腺黏液病19例,中间型6例,恶性型-腹膜黏液癌病26例.3、5、10年生存率分别为75％(38/51)、55％(28/51)和22％(11/51).术后接受化疗患者的生存时间和再手术间隔时间分别为(21±4)个月和(10±6)个月,较未接受化疗患者的(19±7)个月和(7±4)个月有所延长,但差异无统计学意义(t=1.027,0.361,P＞0.05).良性型-弥漫性腹膜腺黏液病、中间型及恶性型-腹膜黏液癌病患者中位生存时间分别为96、63、23个月.良性型-弥漫性腹膜腺黏液病患者与恶性型-腹膜黏液癌病患者肿瘤复发间隔时间分别为(15±5)个月和(7±4)个月,两者比较,差异有统计学意义(=2.193,P＜0.05).结论 应该以积极态度实施阑尾源性腹膜假黏液瘤减瘤手术,复发患者合理适时选择二次或多次的减瘤手术,可以延长复发时间,改善患者的预后.     

History of pseudomyxoma peritonei from its origin to the first decades of the twenty-first century

Pseudomyxoma peritonei(PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term. The word pseudomyxoma derives from pseudomucin, a type of mucin. PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary, a term that has disappeared from today's classifications of cystic ovarian neoplasms. It is known today that in the majority of cases, the origin for PMP is an appendiceal neoplasm, often of low histological grade. Currently, ovarian tumors are wrongly being considered a significant recognized etiology of PMP.PMP classification continues to be under discussion, and experts' panels strive for consensus. Malignancy is also under discussion, and it is shown in this review that there is a long-standing historical reason for that. Surgery is the main tool in the treatment armamentarium for PMP, and the only therapy with potential curative option.     

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。     

腹膜假性黏液瘤减瘤术后腹腔热灌注化疗疗效与安全性的文献复习

目的评估腹腔热灌注化疗（HIPC）对于预防与治疗腹膜假性黏液瘤（PMP）减瘤术后复发的疗效和安全性。方法在PubMed等数据库中检索2010年以前发表的相关文献．根据评估方案综合研究结果。结果通过文献的纳入、排除标准和质量评估。最终有11篇文献入选。入选文献中的患者中位随访时间22。60个月;中位生存期25．6～156．0个月;1、2、3、5和10年的生存率分别为72％～100％、55％～96％、59％～96％、52％～96％和55％～96％。减瘤术后HIPC化疗药物不良反应发生率为2％～15％,围手术期死亡率为0～7％。结论PMP患者减瘤术后予以HIPC治疗安全、有效．     

Morbidity and mortality associated with intraperitoneal chemotherapy for Pseudomyxoma peritonei

BACKGROUND: Many centers include intraperitoneal chemotherapy for treatment of pseudomyxoma peritonei. This study documented the morbidity of intraperitoneal chemotherapy in a single institution. METHODS: A retrospective review of pseudomyxoma peritonei over a 6-year period was undertaken. Treatment, morbidity, and outcome were documented. RESULTS: Eleven patients were identified with an average of 1.9 debulking procedures and 0.8 chemotherapy courses (0.3 complete). Intraperitoneal chemotherapy was not completed in 5 patients because of complications (56%): severe abdominal pain, seizure, neutropenia, and thrombocytopenia (the latter resulted in 1 patient's death). There was no association between incomplete chemotherapy and recurrence. Recurrence was 64% in those without chemotherapy and 44% in those with. Follow-up averaged 26 months and actual 3-year survival was 60%. CONCLUSIONS: Intraperitoneal chemotherapeutic morbidity and mortality were 56% and 11%, respectively. Chemotherapy was associated with decreased recurrence. To optimize outcomes, multicenter prospective trials will likely be required to further refine intraperitoneal chemotherapy protocols.     

Giant mucinous cystadenoma of the appendix

Mucocele of the appendix is a rare entity, characterized by distension of the lumen caused by an accumulation of mucoid substance. We report herein the case of a 73-year-old man with a 3-week history of abdominal pain caused by a large mucinous cystadenoma. The lesion was removed intact, which is the optimal treatment.     

Elevated tumour markers prior to complete tumour removal in patients with pseudomyxoma peritonei predict early recurrence

Objective To assess the potential value of pre‐operative CEA, CA 125 and CA 19–9 in identifying Pseudomyxoma peritonei (PMP) patients at risk of recurrence following complete cytoreductive surgery and intraperitoneal chemotherapy. Method Between March 1996 and October 2001, 35 patients, with documented pre‐operative tumour marker measurements, underwent complete cytoreduction. Outcome in the 32 survivors was recorded. Results Median age was 53 years (range: 32–74). There were 17[53%] females. In 9/32[28%] all tumour markers were normal. The most common abnormality was elevated CEA level in 14/32[44%]. Median follow up was 23 months (range: 11–74). Recurrence was detected in 8/32[25%]. The 2‐year recurrence free interval was 53% in those with elevated CEA compared with 94% with normal CEA. The 2‐year disease free intervals were 100% in 9 patients with normal preop markers compared with 66% for the group with at least one abnormal marker. Conclusion PMP patients with elevated CEA, or more than one abnormal serum tumour maker, are at risk of developing recurrent disease.     

Intussusception of the appendix secondary to mucinous cystadenoma: A rare cause of abdominal pain

A 35-year-old woman was seen as an outpatient with a 3-month history of pain in the right iliac fossa. A CT scan of her abdomen revealed the presence of a mucocele of the appendix. Intra-operatively, an appendico-appendicular intussusception was found. Histology confirmed the presence of a mucinous cystadenoma with the presence of acellular mucin on the serosal surface of the appendix. This association has rarely been described in the literature. Prompt surgical intervention is advocated to prevent the subsequent development of pseudomyxoma peritonei. We present a case of intussusception of the appendix with a mucinous cystadenoma as its lead point.     

Results of Treatment of 385 Patients With Peritoneal Surface Spread of Appendiceal Malignancy

Introduction: In the past, peritoneal carcinomatosis, regardless of primary tumor type, has always been a lethal condition. Recently, special treatments using cytoreductive surgery with peritonectomy procedures combined with perioperative intraperitoneal chemotherapy have resulted in long-term survival. Appendiceal malignancy with a low incidence of liver and lymph node metastases may be especially appropriate for these aggressive local regional treatments.Methods: All patients treated with surgery before January 1999 are included. Patients left with gross residual disease after surgery were not given intraperitoneal chemotherapy, but were later treated with intravenous chemotherapy. The intraperitoneal chemotherapy was given in the perioperative period, starting with mitomycin C at 12.5 mg/m² for males and 10 mg/m² for females. For patients whose pathology showed adenomucinosis, intraperitoneal chemotherapy was limited to treatment in the operating theater with heated mitomycin C. Patients with mucinous adenocarcinoma or pseudomyxoma/adenocarcinoma hybrid had, in addition to mitomycin C, five consecutive days of intraperitoneal 5-fluorouracil at 650 mg/m2 instilled in 1–1.5 liters of 1.5% dextrose peritoneal dialysis solution. A complete cytoreduction was defined as tumor nodules <2.5 mm in diameter remaining after surgery. The histopathology categorized the patients as having adenomucinosis, adenomucinosis/carcinomatosis hybrid, or mucinous carcinomatosis. A previous surgical score was used to estimate the extent of previous surgical procedures.Results: The morbidity of treated patients was 27% and the mortality was 2.7%. In a multivariate analysis, prognostic factors for survival included the completeness of cytoreduction (P < .0001), the histopathological character of the appendix malignancy (P < .0001), and the extent of previous surgical interventions (P = .001). Patients with a complete cytoreduction and adenomucinosis by pathology had a 5-year survival of 86%; with hybrid pathology, survival at 5 years was 50%. Incomplete cytoreduction had a 5-year survival of 20% and 0% at 10 years.Conclusions: Cytoreductive surgery and perioperative intraperitoneal chemotherapy can be used to salvage selected patients with peritoneal surface spread of appendiceal primary tumors. Similar strategies for other patients with peritoneal surface malignancy such as peritoneal carcinomatosis from colon or gastric cancer, peritoneal sarcomatosis, or peritoneal mesothelioma should be pursued.Presented at the 52nd Annual Meeting of the Society of Surgical Oncology, Orlando, Florida, March 4–7, 1999     

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.     

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.