Editorial: Pseudomyxoma Peritonei: More Questions Than Answers (appendix/ovarian

JCO: Pseudomyxoma Peritonei: More QuestionsThan Answers

"Chances are, if you ask most physicians and surgeons about
pseudomyxoma peritonei (PMP), they will respond with more questions
than answers. The confusion that surrounds PMPis not surprising
because the origin, pathology, treatment, prognosis, and very
definition of PMP are still under debate. PMP is a clinical syndrome
that is characterized by mucinous ascites that result from rupture of a
mucin-producing neoplasm, typically of appendiceal origin....."

"...The appendix is the primary cause of PMP; the ovaries are typically
only secondarily involved...."

paywalled: ncreased risk of neoplasm in appendicitis treated w... [Am Surg. 2012] - PubMed - NCBI

Increased risk of neoplasm in appendicitis treated w... [Am Surg. 2012] - PubMed - NCBI

Am Surg. 2012 Mar;78(3):339-43.

Increased risk of neoplasm in appendicitis treated with interval appendectomy: single-institution experience and literature

Abstract

Appendicitis is a common diagnosis encountered by the acute care surgeon. Management of complicated appendicitis is controversial and often involves initial nonoperative therapy with interval appendectomy. This study reviews single-institutional experience with management of complicated appendicitis with interval appendectomy and addresses an unusually high occurrence of incidental appendiceal malignancies observed with a review of relevant literature. A retrospective review of all diagnoses of appendicitis was performed over 5 years at a tertiary care center. Patient demographics, time to surgery, operative technique, pathologic diagnosis, and clinical outcomes were examined. Three hundred fifteen patients were diagnosed with acute appendicitis. Of these, 24 (7.6%) were deemed complicated and did not undergo immediate appendectomy, and 18 ultimately underwent appendectomy at our institution and were included in analysis. There were no statistical demographic or symptomatic differences between the immediate and interval appendectomy patients. Ninety-nine per cent of the immediate appendectomy patients were treated laparoscopically; 78 per cent of the interval group underwent attempted laparoscopic treatment with 56 per cent completed without conversion to open (P < 0.01). Neoplasms were discovered in 1 per cent of the acute appendectomy group and 28 per cent of the interval appendectomy group (P < 0.0001). Two of the three neoplasms in the acute group were carcinoid, whereas three of the five neoplasms in the interval group were adenocarcinoma. Surgeons should consider appendiceal or colonic neoplasms in cases of complicated appendicitis when nonoperative management is considered. This is most important in patients older than 40 years, in those who forego interval appendectomy, or in those who could be lost to follow-up.

abstract: Incidence, prevalence and risk factors for peritoneal carcinomatosis from colorectal cancer - Segelman - 2012 - British Journal of Surgery - Wiley Online Library

Peritoneal Carcinomatosis (Creighton University) includes a variety of tumors that present with extensive metastasis throughout the peritoneal cavity (inside surface of the abdomen) and can be found with gall bladder, liver, colon, appendix, ovarian, pancreas, mesothelioma, pseudomyxoma peritonei, rectal, small bowel and stomach cancers. It is a broad description in which multiple tumors develop in and line the peritoneal abdominal cavity and linings.

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 Incidence, prevalence and risk factors for peritoneal carcinomatosis from colorectal cancer - Segelman - 2012 - British Journal of Surgery

Abstract

Background:

This was a population-based cohort study to determine the incidence, prevalence and risk factors for peritoneal carcinomatosis (PC) from colorectal cancer.

Methods:

Prospectively collected data were obtained from the Regional Quality Registry. The Cox proportional hazards regression model was used for multivariable analysis of clinicopathological factors to determine independent predictors of PC.

Results:

All 11 124 patients with colorectal cancer in Stockholm County during 1995–2007 were included and followed until 2010. In total, 924 patients (8·3 per cent) had synchronous or metachronous PC. PC was the first and only localization of metastases in 535 patients (4·8 per cent). The prevalence of synchronous PC was 4·3 per cent (477 of 11 124). The cumulative incidence of metachronous PC was 4·2 per cent (447 of 10 646). Independent predictors for metachronous PC were colonic cancer (hazard ratio (HR) 1·77, 95 per cent confidence interval 1·31 to 2·39; P = 0·002 for right-sided colonic cancer), advanced tumour (T) status (HR 9·98, 3·10 to 32·11; P < 0·001 for T4), advanced node (N) status (HR 7·41, 4·78 to 11·51; P < 0·001 for N2 with fewer than 12 lymph nodes examined), emergency surgery (HR 2·11, 1·66 to 2·69; P < 0·001) and non-radical resection of the primary tumour (HR 2·75, 2·10 to 3·61; P < 0·001 for R2 resection). Patients aged > 70 years had a decreased risk of metachronous PC (HR 0·69, 0·55 to 0·87; P = 0·003).

Conclusion:

PC is common in patients with colorectal cancer and is associated with identifiable risk factors.

abstract: Importance of Histologic Subtype in the Staging of Appendiceal Tumors.

Blogger's Note: common feature is mucinous cell type; understudied is familial appendiceal carcinoid

BACKGROUND:

Malignant neoplasms of the appendix have different behavior based on their histologic subtypes in anecdotal series. Current staging systems do not capture the diversity of histologic subtypes in predicting outcomes.

METHODS:

We queried all patients with appendiceal malignancies captured in the Surveillance, Epidemiology, and End Results (SEER) database from 1973 to 2007. Tumors were classified as colonic type adenocarcinoma, mucinous adenocarcinoma, signet ring cell type, goblet cell carcinoid, and malignant carcinoid. We compared incidence, overall survival, and disease-specific survival for these tumors on the basis of patient, tumor, and therapy characteristics. Estimates from Cox proportional hazard modeling were used to predict hazard ratios for differing histologic subtypes with similar tumor, node, metastasis system (TNM) stages.

RESULTS:

Of the 5672 patients identified, we included 5655 (99%) in our analysis. The 5-year disease-specific survival rates were 93% for malignant carcinoid, 81% for goblet cell carcinoid, 55% for colonic type adenocarcinoma, 58% for mucinous adenocarcinoma, and 27% for signet ring cell type. Predicted estimates of adjusted hazard ratios revealed an 8-fold difference between histologic subtypes for similar TNM stages.

CONCLUSIONS:

Histologic subtype is an important predictor of disease-specific survival and overall survival in patients with appendiceal neoplasms. Addition of the histologic subtype to the TNM staging is simple and may improve prognostication.

Mucinous Tumors of the Ovary: Diagnostic Challenges at Frozen Section and Clinical Implications

Conclusions

Our study showed a 34% rate of discordance between FS and final diagnosis. Given that 5 cases (7%) were of GI origin, intraoperative assessment of the appendix should be performed in all mucinous ovarian tumors.

Highlights

► We examined 73 consecutive cases of mucinous ovarian tumors that had frozen section (FS) at the time of surgery.
► The rate of discordance between FS and final diagnosis was 34%.
► Consideration of appendiceal tumors is important as 7% of all cancers were found to be gastrointestinal in origin.

abstract: Indication for oophorectomy during cytoreduction for intraperitoneal metastatic spread of colorectal or appendiceal origin(cytoreductive surgery/HIPEC)

BACKGROUND: The incidence of ovarian metastases at the time of peritoneal carcinomatosis, and the influence of such metastases on survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), are unknown.
METHODS: This retrospective analysis included 194 women subjected to CRS and HIPEC since 2001. The incidence of ovarian metastases, disease-free survival and disease-specific survival were analysed.
RESULTS: The histological diagnosis was colorectal cancer carcinomatosis in 108 patients, peritoneal mucinous carcinomatosis (PMCA) in 23 and disseminated peritoneal adenomucinosis (DPAM) in 63. Ninety-nine patients underwent oophorectomy during the HIPEC procedure. Ovarian metastases were confirmed in at least 52 per cent of the patients. There was a significant difference in disease-free survival between women with or without ovarian metastases in both PMCA and DPAM groups (P = 0·044 and P = 0·010 respectively). No significant differences in survival were found in the group with colorectal cancer carcinomatosis.
CONCLUSION: When peritoneal carcinomatosis of colorectal or appendiceal origin is confirmed, at least 52 per cent of ovaries will have synchronous metastases. Disease-free survival after a HIPEC procedure for PMCA or DPAM is significantly lower in women with ovarian metastases. Oophorectomy during CRS for peritoneal carcinomatosis should be strongly considered.

abstract: Indication for oophorectomy during cytoreduction for intraperitoneal metastatic spread of colorectal or appendiceal origin

BACKGROUND: The incidence of ovarian metastases at the time of peritoneal carcinomatosis, and the influence of such metastases on survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), are unknown.
CONCLUSION: When peritoneal carcinomatosis of colorectal or appendiceal origin is confirmed, at least 52 per cent of ovaries will have synchronous metastases. Disease-free survival after a HIPEC procedure for PMCA or DPAM is significantly lower in women with ovarian metastases. Oophorectomy during CRS for peritoneal carcinomatosis should be strongly considered.

Abstract: Cervical manifestation of a borderline type ovarian cancer with pseudomyxoma peritonei - a case report

Note: mucinous cell type is a cell type found in numerous organs including colon/rectum Abstract: Borderline tumours of the ovary (BOTs) are rare tumour entities that do not show any destructive or invasive growth in the majority of cases, even though they can display characteristics of malignant tumours The mucinous subtype can also originate from the appendix, and ovarian metastases can mimic primary ovarian BOTs, often accompanied by peritoneal manifestation in terms of pseudomyxoma peritonei. In cases where a concomitant appendiceal tumour is present, it may prove difficult to determine the primary tumour. This report describes a special case of BOT with a specific example of the complexity of the differential diagnosis of pseudomyxoma peritonei. Especially the case was simultaneously linked to appendiceal and ovarian cancer. Moreover, this case was exceptional for its unusual manifestation of BOT in the cervix.

Appendiceal mucinous neoplasms: controversial issues - pathology

Abstract:

"...reviewed is the largely resolved controversy about whether ovarian mucinous tumors in this setting are separate primaries or are metastases from the appendiceal tumor."

The role of cytoreductive surgery for non-genital tract metastatic tumors to the ovaries.

Abstract


OBJECTIVE: The aim of this study was to investigate prognostic factors of patients with metastases to the ovaries from non-genital organs.
STUDY DESIGN: From September 1994 to December 2006, 158 patients with pathologically confirmed metastatic tumors to the ovaries at Samsung Medical Center (SMC) were included in this study. The data were obtained from the patients' medical records and pathology reports.
RESULTS: The primary tumor origin was mostly stomach (73 cases) and colon (61 cases). Krukenberg tumor (pathologically proven signet ring cell carcinoma) was found in 34 cases: stomach (25), colon (2), appendix (1), and unknown (6). ....However, age, bilateral tumors, chronology of diagnosis and mass size did not affect survival.
CONCLUSION: Cytoreductive surgery and post-operative adjuvant chemotherapy had a beneficial effect on survival in selected patients.