Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy to Treat Pseudomyxoma Peritonei of Ovarian Origin: A Retrospective French RENAPE Group Study.

BACKGROUND: Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) to treat OPMP. METHODS: Patients from the French National Network for Rare Peritoneal Tumors (RENAPE) database with proven OPMP treated by CRS/HIPEC and with histologically normal appendix and digestive endoscopy were retrospectively included. Clinical and follow-up data were collected. Histopathological and immunohistochemical features were reviewed. RESULTS: Fifteen patients with a median age of 56 years were included. The median Peritoneal Cancer Index was 16. Following CRS, the completeness of cytoreduction (CC) score was CC-0 for 9/15 (60%) patients, CC-1 for 5/15 (33.3%) patients, and CC-2 for 1/15 (6.7%) patients. The median tumor size was 22.5 cm. After pathological review and immunohistochemical studies, tumors were classified as Group 1 (mucinous ovarian epithelial neoplasms) in 3/15 (20%) patients; Group 2 (mucinous neoplasm in ovarian teratoma) in 4/15 (26.7%) patients; Group 3 (mucinous neoplasm probably arising in ovarian teratoma) in 5/15 (33.3%) patients; and Group 4 (non-specific group) in 3/15 (20%) patients. Peritoneal lesions were OPMP pM1a/acellular, pM1b/grade 1 (hypocellular) and pM1b/grade 3 (signet-ring cells) in 13/15 (86.7%), 1/15 (6.7%) and 1/15 (6.7%) patients, respectively. Disease-free survival analysis showed a difference (p = 0.0463) between OPMP with teratoma/likely-teratoma origin (groups 2 and 3; 100% at 1, 5, and 10 years), and other groups (groups 1 and 4; 100%, 66.6%, and 50% at 1, 5, and 10 years, respectively). CONCLUSION: These results suggested that a primary therapeutic strategy using complete CRS/HIPEC for patients with OPMP led to favorable long-term outcomes.

A Case of Low-Grade Appendiceal Mucinous Neoplasm That Led to Surgery After 12 Years of No Treatment.

Low-grade appendiceal mucinous neoplasm (LAMN) is a relatively rare, non-invasive appendiceal tumor. We experienced a case of LAMN that led to surgery after 12 years of no treatment. Until now, LAMN has been reported to progress more slowly than other tumors, but there have been no reports of long-term follow-up of appendiceal tumors without treatment. Although the tumor had grown over the course of 12 years, there was no mixing or migration of other histological types, and it did not lead to pseudomyxoma peritonei. As this course is considered to be relatively rare, we report it along with a literature review.

Significance of laparoscopic cytoreductive surgery for appendiceal pseudomyxoma peritonei with limited disease and low tumor burden.

OBJECTIVE: To investigate the clinical value of laparoscopic cytoreductive surgery (CRS) in treating of appendiceal pseudomyxoma peritonei with limited disease and low tumor burden. METHODS: The clinical data of patients with appendiceal pseudomyxoma peritonei treated by surgery with CRS at the Aerospace Center Hospital from January 2018 to December 2021 were retrospectively analyzed. The patients were divided into laparoscopic or open CRS groups according to the operation method. A propensity score-matched (PSM) analysis (1:1) was performed, the related clinical variables were compared between the two groups, and the effect on progression-free survival (PFS) was also analyzed. RESULTS: One hundred and eight patients were included in this study. After PSM, 33 patients were selected from each group and the age and peritoneal cancer index were matched between the two groups. There were significant differences in operation time (P < 0.001), intraoperative bleeding (P < 0.001), intraoperative blood transfusion (P = 0.007), hospital stay (P < 0.001). The analysis of PFS showed that there was no significant difference between the two operation methods. After multivariate analysis, the pathologic subtype (P = 0.012) was identified as an independent prognostic factor for PFS. CONCLUSION: The curative effect of laparoscopic CRS is like that of open operation, which can significantly shorten the operation time and hospital stay and reduce intraoperative bleeding and blood transfusion event. The laparoscopic CRS is safe and feasible in strictly selected patients. The pathologic subtype is an independent factor affecting the prognosis for PFS.

Appendiceal Adenocarcinoma Presenting As Perforated Appendicitis.

Primary tumors of the appendix, specifically appendicular adenocarcinoma, are a rare malignant neoplasm of the gastrointestinal tract. We present a case of a 64-year-old female who had significant peritoneal adhesions from a previously perforated appendix involving the right ureter, bladder, and anterior abdominal wall after a course of perforated appendicitis, which was managed conservatively with drain placement with interval appendectomy. We are discussing this case in hopes of bringing awareness to the possibility of underlying malignancy in the setting of perforated appendicitis in patients of advanced age.

GNAS mutations as prognostic biomarker in patients with relapsed peritoneal pseudomyxoma receiving metronomic capecitabine and bevacizumab: a clinical and translational study.

BACKGROUND: There is lack of evidence about systemic treatment of pseudomyxoma peritonei (PMP) relapsing after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. There is also lack of biomarkers able to predict outcomes beyond known clinical and pathological prognostic features. METHODS: Fifteen patients with relapsed PMP and progressive disease within the last 6 months were included and received metronomic capecitabine (625 mg/mq/day b.i.d.) and bevacizumab (7.5 mg/Kg three-weekly) until progressive disease/unacceptable toxicity. The primary endpoint was progression-free survival (PFS). Ion Torrent(®) next generation sequencing technology (Hot-spot Cancer Panel) was used to characterize molecular features. RESULTS: At a median follow up of 12 months, median PFS was 8.2 months and 1-year overall survival was 91 %. Partial responses were observed in 20 % of cases, but a significant reduction of tumor markers in up to 79 %. Treatment was very well tolerated without no new safety signals. All tumor samples except one had KRAS mutations. Patients with GNAS mutations had a significantly shorter median PFS as compared to GNAS wild-type ones (5.3 months vs. not reached; p < 0.007). The results were externally validated on our previous series of PMP patients. GNAS mutations were rare in a parallel cohort of 121 advanced colorectal cancers (2.5 %), but were associated with peculiar clinical-pathological features and aggressive course. CONCLUSIONS: Metronomic capecitabine and bevacizumab is an active and well tolerated option in patients with relapsed PMP. The negative prognostic effect of GNAS mutations in gastrointestinal cancers warrants further confirmatory studies and may prompt the development of effective targeted strategies.

Diagnosis and treatment of pseudomyxoma peritonei in accidental discovery: a report of 8 cases / 国际外科学杂志

Objective To investigate the diagnosis and treatment of pseudomyxoma peritonei (PMP) and provide a reference for diagnosis and treatment of PMP.Methods The clinical features,laboratory examinations,treatment and outcomes of 8 PMP misdiagnosed cases were analyzed with recent relevant reference.Results Ultrasonography,CT,peritoneal cytological examination,tumor markers results are helpful for diagnosis of PMP.PMP will be confirmed and classified by pathological examination after operation.Complete cytoreductive surgery (CRS)or major debulking surgery (MDS) of the tumor combined intraperitoneal chemotherapy and systemic chemotherapy,eight patients in seven cases survived 4-71 month range,one patient died of respiratory failure of pulmonary infection after the third operation.Conclusions Ultrasonography,CT,peritoneal cytological examination,tumor markers tests help avoid misdiagnosis of PMP before operations.Intraoperative findings follow after PMP,CRS or MDS should be executed in the operation or the next time.Intraperitoneal chemotherapy and conventional chemotherapy can improve survival in patients with PMP and prolong their survival time.

FOLFOX-4 chemotherapy for patients with unresectable or relapsed peritoneal pseudomyxoma.

PURPOSE: The standard treatment of peritoneal pseudomyxoma is based on cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC). The establishment of newer systemic treatments is an unmet clinical need for unresectable or relapsed peritoneal pseudomyxoma. The aim of our study was to assess the activity of chemotherapy with 5-fluorouracil and oxaliplatin (FOLFOX-4 regimen) in terms of response rate in this subset of patients. MATERIALS AND METHODS: Patients were included in a single-center, observational study and treated with FOLFOX-4 administered every 2 weeks for up to 12 cycles or until progressive disease or unacceptable toxicity. RESULTS: Twenty consecutive patients were reviewed from July 2011 to September 2013. Only partial responses were observed, with an objective response rate of 20%. Median progression-free survival and overall survival were 8 months and 26 months, respectively. Two patients were able to undergo laparotomy with complete cytoreduction and HIPEC in one case. Safety data for FOLFOX-4 were consistent with the literature. By means of a mutant enriched polymerase chain reaction, KRAS mutation was found in 16 of 19 cases (84%), and MGMT promoter methylation was found in 8 (42%, all KRAS mutant). CONCLUSION: FOLFOX-4 chemotherapy is tolerable and active in patients with peritoneal pseudomyxoma when disease is deemed unresectable or relapsed after peritonectomy and HIPEC. The identification of predictive biomarkers, such as KRAS for resistance to anti-epidermal growth factor receptor monoclonal antibodies and MGMT for response to temozolomide, is a priority for the development of evidence-based treatment strategies for peritoneal pseudomyxoma.

Pseudomixoma peritoneal por tumor del apéndice cecal / Peritoneal pseudomyxoma due to cecal appendix tumor

Objetivo: presentar un caso de una patología de muy baja incidencia y realizar una revisión bibliográfica de la misma. Paciente y método: paciente femenina de 87 años de edad que ingresa por guardia por cuadro compatible con hernia inguinal atascada, por lo que se decide cirugía de urgencia. En el acto operatorio se identifica un saco herniario de paredes engrosadas, con contenido intestinal con buena vitalidad, sin signos de necrosis, identificando además abundante secreción mucinosa en el saco herniario y proveniente de la cavidad abdominal. Se decide terminar con la hernioplastia y realizar estudios complementarios diferidos. Luego de los mismos, el comité de tumores decide la realización de nueva cirugía (hemicolectomía derecha con ileotransverso anastomosis por tumor mucinoso de apéndice), durante la cual se constata ascitis gelatinosa. La paciente evoluciona favorablemente y es dada de alta a las 72 horas del post operatorio. La anatomía patológica informa adenocarcinoma mucinoso de apéndice bien diferenciado. Discusión: el Pseudomixoma Peritoneal es una entidad muy poco frecuente, que hace referencia a la diseminación peritoneal de un tumor cuyas células producen gran cantidad de mucina. La supervivencia global de los pacientes es de aproximadamente 75 y 68% durante 5 y 10 años. La clasificación más aceptada es la de Ronnet. Tradicionalmente, el tratamiento ha sido la cirugía de citoreducción, aunque hay autores que proponen procedimientos más agresivos como la peritonectomía más quimioterapia hipertérmica intraoperatoria.

Objective: to present a case of a very low incidence disease and a bibliographic review of it. Patient and method: 87 years old female patient with a compatible history and physical exam for a stuck inguinal hernia, for which we decided to perform emergency surgery. During the surgical procedure, we find a thickened hernia sac, with mucus in it and in the abdominal cavity. The small intestine was vital and not complicated. We finished the hernioplasty, and decided to perform complementary ambulatory studies to the patient. After having them done, we decided, in an interdisciplinary committee, to perform an exploratory surgery, (hemicolectomy with ileum transverse anastomosis for an appendix tumor), during which gelatinous ascites was noticed. The patient evolve favorably and was discharged 72 hours post operation. The surgical biopsy informed an appendix mucinous adenocarcinoma well differentiated. Discussion: the peritoneal pseudomyxoma is a very infrequent entity. The overall survival is 75 and 68% for 5 and 10 years, respectively. Ronnet had published the most accepted histological classification. The accepted treatment is the cytoreductive surgery, although some authors proposed a more aggressive treatment such peritonectomy with hyerthermic intraperitoneal chemotherapy.

[Retroperitoneal mucinous cystadenocarcinoma presenting seven years after appendectomy and right hemicolectomy]. / Cistoadenocarcinoma mucinoso retroperitoneal ocurrido a los 7 años de apendicectomía y hemicolectomía derecha.

Retroperitoneal mucinous cystadenocarcinoma (RMC) with no involvement of neighboring structures is very rare, regardless of whether the lesion is primary or secondary. We report the case of a patient who presented RMC seven years after appendectomy and right hemicolectomy. We discuss both diagnostic possibilities.

Diagnostic Value of Multi-slice Spiral in Pseudomyxoma Peritonei / 实用放射学杂志

Objective To investigate the MSCT characteristics of peritoneal pseudomyxoma. Methods CT findings of peritoneal pseudomyxoma proved by surgery and pathology in 11 cases were retrospectively analyzed. Results On MSCT,the peritoneal pseud-omyxomas were irregular intraperitoneal cystic-solid masses, the CT attenuation was 18.5 ～ 27.6 HU, there were scallop-like, wavy and nodule-like indentations on the liver and/or spleen surface,large amount of mucinous ascites within abdominal, pelvic an-domentum cavity , thickening of peritoneum. On post-contrast administration , mild enhancement of peritoneum,cystic wall and omentum could be revealed. Conclusion MSCT examination has important value in diagnosis and differential diagnosis of peritoneal pseudomyxomas

Clinical Analysis of Appendiceal Mucoceles / 中国医师杂志

Objective To investigate the diagnostic and treatment methods of appendiceal mucoceles and peritoneal pseudomyxoma. Methods Thirteen cases of appendiceal mucoceles were enrolled in this study. Six cases underwent appendectomy, two cases ileocecal resection, two cases right colectomy, and two cases appendectomy in emergency followed by right colectomy because their postoperative pathological diagnosis was appendiceal muco-cystadenocarcinoma. Ruptured appendiceal mucocele and peritoneal pseudomyxoma were found during operation in one case, then right colectomy was performed, ascites was removed and abdominal cavity was washed with normal saline and 5-Fu solution. Systemic chemotherapy was given to this patient after operation. Results Operation was successful in all the patients. Except one case not followed up, the other patients remained free of disease after operation. Conclusion Appendiceal mucoceles is uncommon. The patients should be suspected suffering from this disease when chief complaints are right lower abdominal pain and mass. B-type ultrasonography and other necessary digestive image examinations are economical and inexpensive diagnostic method of appendiceal mucoceles. It is necessary to explore the whole abdominal cavity carefully when this disease is suspected during operation.