Lymph node positive pseudomyxoma peritonei.

BACKGROUND: Appendiceal mucinous neoplasms are routinely accompanied by peritoneal metastases at the time of diagnosis of the primary appendiceal tumor. In contrast, liver metastases and lymph node metastases are unusual. METHODS: From an extensive database, patients with lymph node metastases identified at the time of primary appendiceal cancer resection were selected for special study. The clinical, treatment-related and histologic variables of this group of patients were statistically analyzed for their impact on overall survival. RESULTS: From a prospectively maintained database of 685 patients with a complete cytoreduction of a mucinous appendiceal neoplasm with peritoneal dissemination, 39 patients (5.6%) had lymph node metastases at the time of primary diagnosis. The median follow-up was 5.0 years and overall median survival was 6.0 years. Histologically, 6 of these patients (15.4%) had an appendiceal mucinous neoplasm - Intermediate type (MACA-Int). In 5 patients, the involved lymph nodes were not within the ileocolic lymph node group. The 7 patients (17.9%) who had a complete or near complete response to neoadjuvant chemotherapy prior to definite cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) showed prolonged survival (HR 4.8 (1.1, 20.5) p = 0.0323). A prior right colon resection required repeat resection in 87% of patients. CONCLUSION: Long-term survival is unusual but occasionally seen in this group of patients. Response to neoadjuvant chemotherapy is an important determinant of a favorable outcome.

Clinicopathologic parameters and outcomes of mucinous neoplasms confined to the appendix: a benign entity with excellent prognosis.

Appendiceal mucinous neoplasms (AMNs), characterized by expansile or "pushing" growth of neoplastic epithelium through the appendix wall, are sometimes accompanied by peritoneal involvement, the extent and grade of which largely determine clinical presentation and long-term outcomes. However, the prognosis of tumors entirely confined to the appendix is still debated and confusion remains regarding their biologic behavior and, consequently, their clinical management and even diagnostic nomenclature. We evaluated AMNs limited to the appendix from 337 patients (median age: 58 years, interquartile range (IQR): 47-67), 194 (57.6%) of whom were women and 143 (42.4%) men. The most common clinical indication for surgery was mass or mucocele, in 163 (48.4%) cases. Most cases (N = 322, 95.5%) comprised low-grade epithelium, but there were also 15 (4.5%) cases with high-grade dysplasia. Lymph nodes had been harvested in 102 (30.3%) cases with a median 6.5 lymph nodes (IQR: 2-14) per specimen for a total of 910 lymph nodes examined, all of which were negative for metastatic disease. Histologic slide review in 279 cases revealed 77 (27.6%) tumors extending to the mucosa, 101 (36.2%) to submucosa, 33 (11.8%) to muscularis propria, and 68 (24.4%) to subserosal tissues. In multivariate analysis, deeper tumor extension was associated with older age (p = 0.032; odds ratio (OR): 1.02, 95% confidence intervals (CI): 1.00-1.03), indication of mass/mucocele (p < 0.001; OR: 2.09, CI: 1.41-3.11), and wider appendiceal diameter, grossly (p < 0.001; OR: 1.61, CI: 1.28-2.02). Importantly, among 194 cases with at least 6 months of follow-up (median: 56.1 months, IQR: 24.4-98.5), including 9 high-grade, there was no disease recurrence/progression, peritoneal involvement (pseudomyxoma peritonei), or disease-specific mortality. These data reinforce the conclusion that AMNs confined to the appendix are characterized by benign biologic behavior and excellent clinical prognosis and accordingly suggest that revisions to their nomenclature and staging would be appropriate, including reverting to the diagnostic term mucinous adenoma in order to accurately describe a subset of them.

Peritoneal metastases found in routinely resected specimens after cytoreductive surgery and heated intraperitoneal chemotherapy.

INTRODUCTION: Peritoneal metastases (PM) originating from colorectal cancer (CRC) and pseudomyxoma peritonei (PMP) can be treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Certain sites in the peritoneal cavity are prone to contain PM and are, therefore, routinely resected. The aim of this study is to investigate the frequency of disease in CRS specimens routinely resected. Secondly, to investigate if the risk of finding PM in routinely resected specimen is associated with involvement of anatomic related peritoneal areas. MATERIALS AND METHODS: This study investigated 433 patients diagnosed with PMP (n = 119) or PM from CRC (n = 314) and operated with CRS + HIPEC between June 2006 and November 2020 at a national center. Baseline data were prospectively registered. Pathology reports were reviewed for the presence of metastases in the routinely resected umbilicus, ligamentum teres hepatis, ovaries and greater omentum. Tumor extent was estimated using the Dutch region count. RESULTS: PM was found in 14.7% of umbilical resections, in 17.4% of the resected ligamentum teres hepatis, in 48.2% of the resected ovaries and in 49.5% of the greater omentum specimens. We found an association between macroscopic disease involvement of the nearest region and risk of PM found in the related resections. Seven of 31 women with no macroscopically visible disease in the pelvis had PM diagnosed in the resected ovaries. CONCLUSIONS: A substantial proportion of routine resections held histologic verified PM. Our results may advocate for a routinely performed resection of the umbilicus, ligamentum teres hepatis, ovaries and greater omentum.

Acute Chylopericardium With Tamponade and Cardiac Arrest With Pseudomyxoma Peritonei.

A 51-year-old woman with pseudomyxoma peritonei developed cardiac arrest 5 days after surgery. Acute echocardiography demonstrated pericardial tamponade. Emergency pericardiocentesis evacuated milky fluid and circulation was re-established. Analysis of the pericardial fluid suggested chylopericardium. In conclusion, this case demonstrates that chylopericardium may be life-threatening and underlines the importance of acute echocardiography in critical management of patients with unexplained shock.

Mucinous ascites secondary to peritoneal pseudomyxoma of an uncertain origin.

Peritoneal pseudomyxoma (PP) is a rare malignant tumor, which is characterized by the presence of mucinous ascites. Abdominal ultrasound as the first diagnostic method of this entity allows the visualization of ascites on the one hand and its analysis through a diagnostic paracentesis on the other.

Pseudomyxoma Peritonei-An Unusual Cause of Ascites: A Case Report.

INTRODUCTION: Ascites in patients with cardiac disease can be multifactorial. Serum ascitic albumin gradient (SAAG) helps in identifying the etiology of ascites. High SAAG ascites is related to hepatic or posthepatic causes. The causes of low SAAG ascites results include tuberculosis, peritoneal malignancy, or pancreatitis. CASE PRESENTATION: We report an unusual cause of low SAAG ascites in a 48-year-old woman with valvular heart disease. The patient presented with ascites, and cross-sectional imaging revealed a right iliac fossa mass with omental deposits. The patient was finally diagnosed as having pseudomyxoma peritonei on the basis of clinicoradiopathological features. DISCUSSION: Pseudomyxoma peritonei is a rare cause of low SAAG ascites. It is characterized by the deposition of mucinous material on the peritoneal surfaces. The most common site of origin is the appendix, although it can arise from other intraabdominal organs as well. Excision of the tumor combined with intraperitoneal chemotherapy is the preferred modality of treatment.

Liver scalloping: Pseudomyxoma peritonei liver compression.

Pseudomyxoma peritonei is a rare tumor characterized by the presence of mucous disseminated throughout the peritoneal cavity generally arising from the rupture of an appendicular mucocele. Liver scalloping is a highly suggestive image of pseudomyxoma and corresponds to the indentation of the liver compressed by the gelatinous mucin.

[Pseudomyxoma peritonei : a cause of ascites that should not be underestimated]. / Pseudomyxome péritonéal : une cause d'ascite à ne pas négliger.

Pseudomyxoma peritonei (PMP) is a rare condition that refers to a clinical syndrome resulting from the accumulation of mucin in the peritoneal cavity. It results from the intraperitoneal rupture of a mucinous epithelial neoplasm which is classically appendiceal. The prognosis of a limited appendiceal tumor is favourable. Nevertheless, in the case of peritoneal dissemination, the prognosis could be different according to the histological type and therapeutic management. We report the case of a 71-year-old female patient who developed a pseudomyxoma peritonei originating from an appendiceal adenocarcinoma.

Le pseudomyxome péritonéal (PMP) est une condition rare qui se réfère à un syndrome clinique résultant d'une accumulation de mucine dans la cavité péritonéale. Il est secondaire à la rupture intrapéritonéale d'une néoplasie épithéliale mucineuse dont l'origine est classiquement appendiculaire. Le pronostic d'une tumeur appendiculaire limitée est favorable. Toutefois, en cas de dissémination péritonéale, il est variable selon le type histologique et la prise en charge thérapeutique. Nous rapportons le cas d'une patiente de 71 ans ayant développé un pseudomyxome péritonéal secondaire à un adénocarcinome appendiculaire.

HIV patient with pseudomyxoma peritonei of extra-appendiceal origin is disease-free and alive 9 years after complete cytoreductive surgery.

OBJECTIVE: Pseudomyxoma peritonei (PMP) is a clinical syndrome that is mainly characterized by the presence of mucinous ascites that results from the rupture of a mucin-producing neoplasm. No reports exist so far regarding the management of this syndrome in HIV patients. CASE REPORT: A 54-year old male patient with a diagnosed atypical colitis developed additionally over time a complicated diverticulitis which was initially treated conservatively with antibiotics. Due to septic complication, the patient received a Hartmann resection. Six months after the first surgery a Hartmann reversal was tried; it, however, revealed peritoneal implants and a significant stenosis of the colon stump. Resection of these lesions confirmed histopathologically the presence of a low-grade pseudomyxoma peritonei. Five months later, a second try for a Hartmann reversal was performed. In the view of the slightly enlarged paracolic lymph nodes, a low anterior resection was performed with a primary descendorectostomy. Histopathological examination revealed no more signs of the tumor, which confirmed the completeness of the cytoreductive surgery by the first try for a Hartmann reversal. CONCLUSIONS: Completeness of cytoreductive surgery and low grade histology seem to be the most important factors for the prognosis in pseudomyxoma peritonei in immunocompromised patients as suggested by the long overall and progression free survival of the present patient.

Management of an inguinal hernia in patients with pseudomyxoma peritonei.

BACKGROUND: Pseudomyxoma peritonei is a disease that results from a perforated mucinous neoplasm of the appendix so that mucinous ascites and mucin-producing tumor cells are widely disseminated in a characteristic pattern throughout the abdomen and pelvis. The intraabdominal mucus can accumulate in the inguinal canal and by physical examination be indistinguishable from the usual inguinal hernia. METHODS: A database of patients with pseudomyxoma peritonei was used to identify patients who had an inguinal hernia prior to or at the time of cytoreductive surgery (CRS) and perioperative hyperthermic chemotherapy (HIPEC). At the time of CRS, care was taken in all patients to remove the peritoneal lining of the inguinal canal. Patients who had the inguinal hernia repaired prior to definitive treatment with CRS and HIPEC had all tissue and mesh associated with prior herniorrhaphy resected. RESULTS: In 178 pseudomyxoma peritonei patients, 17 had a new onset or previously repaired inguinal hernia that required extraction of mucus and mucinous tumor from the hernia site. No repair of the open inguinal canal was attempted at the time of CRS. No recurrent inguinal hernias were recorded and no patients required an inguinal incision at a later time to resect progressive disease within the inguinal canal. CONCLUSIONS: Inguinal hernias caused by mucinous ascites and tumor were definitively treated by cytoreductive surgery plus HIPEC. Extraction of tumor and peritoneum from the inguinal canal facilitates fibrous closure of the hernia defect so that hernia recurrence was not observed.

Pseudomyxoma peritonei of a mature ovarian teratoma caused by mismatch repair deficiency in a patient with Lynch syndrome: a case report.

BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare disease with an estimated incidence of 1-2 cases per million individuals per year. PMP is characterized by the accumulation of abundant mucinous or gelatinous fluid derived from disseminated tumorous cells. Most of the tumorous cells are originated from rupture of appendiceal neoplasms, but some are from the metastasis of cancer of the colon, ovary, fallopian tube, urachus, colorectum, gallbladder, stomach, pancreas, lung and breast. Although frequent mutations in KRAS and/or GNAS genes have been reported, precise molecular mechanism underlying PMP remains to be elucidated. It is of note that mucinous tumour is one of the frequent histological features of colorectal cancer (CRC) in Lynch syndrome (LS), an autosomal dominantly inherited disease caused by a germline mutation of the DNA mismatch repair (MMR) genes including human mutL homolog 1 (MLH1), human mutS homolog 2 (MSH2), human mutS homolog 6 (MSH6), and postmeiotic segregation increased 2 (PMS2). Therefore, typical LS-associated tumours show mismatch repair instability. Although LS patients are most strongly predisposed to CRC, PMPs from mucinous CRC have not been reported in LS patients. CASE PRESENTATION: In this report, we report a case of PMP originating from an ovarian teratoma in a LS patient. The patient had surgical treatment of PMP arising from an ovarian teratoma at the age of 38 years, and later developed a transverse colon cancer at the age of 40. The patient's family history fulfilled the Amsterdam criteria, and genetic analysis of the peripheral leukocytes identified a germ line mutation in the MLH1 gene (MLH1 c.1546dupC p.Q516PfsX3). Interestingly, immunohistochemical staining showed that the expression of MLH1 was lost in the colon cancer as well as the ovarian teratoma. Consistent with the loss of MLH1 expression, both tumours showed high microsatellite instability (MSI-H). CONCLUSION: This case suggested that LS patients may develop various types of tumours including ovarian PMP, and that mismatch repair deficiency may play a role in the development of PMP derived from, at least, a part of ovarian teratomas.

[Anesthetic Management of Peritonectomy and Extended Operation for Peritoneal Pseudomyxoma Combined with Massive Ascites].

We report successful anesthetic management of extended operation for peritoneal pseudomyxoma combined with massive ascites (16.5 l). A 66-year-old man (weight 76 kg) who could not keep a supine position due to massive ascites associated with peritoneal pseudomyxoma was scheduled for elective peritonectomy and extended surgery. With the patient in the semisitting position, we administered 3% sevoflurane to induce loss of consciousness while preserving spontaneous ventilation. We then performed crush induction with propofol and rocuronium after topical anesthesia to the tongue base. Tracheal intubation with the Macintosh laryngoscope was successful. During the operation, we aspirated about 16.5 l of ascites over 20 l normal saline irrigation. To maintain the body temperature, we performed both active and passive extracorporeal warming. The operation included not only total peritonectomy but also distal gastrectomy, right colon resection, splenectomy, and cholecystectomy. To perform effective postoperative analgesia, we placed two epidural catheters from the upper and lower thoracic epidural spaces. The patient was extubated 3 days after operation, and discharged uneventfully on the 18th postoperative day.

Palliative parenteral nutrition use in patients with intestinal failure as a consequence of advanced pseudomyxoma peritonei: a case series.

Pseudomyxoma peritonei (PMP) is a rare condition complicated by intra-abdominal spread that can cause multilevel gastrointestinal (GI) obstruction. Parenteral nutrition (PN) use in the context of palliative care and malignancy remains controversial. We describe the use of palliative PN in three patients with progressive PMP causing multilevel GI obstruction and intestinal failure. All patients received > 90 days of PN. PN was safe in this cohort of patients. However, patient selection and the timing of intervention are important factors when considering the initiation of PN.

A weeping umbilical hernia: bilateral ovarian mucinous cystadenoma with disseminated peritoneal adenomucinosis.

A young woman was referred to us for the management of an umbilical hernia with macerated overlying skin through which massive ascites was leaking. On examination we found a jelly-like substance seeping out of the opening. A contrast-enhanced CT scan of the abdomen revealed bilateral ovarian cystadenocarcinoma with pseudomyxoma peritonei. The patient underwent cytoreductive surgery along with hysterectomy and bilateral oophorectomy followed by hyperthermic intraperitoneal chemotherapy with mitomycin C. Histopathological examination showed bilateral borderline cystadenoma with peritoneal adenomucinosis. Follow-up at 1 year showed no signs of recurrence.