Medline ® Abstracts for References 77-79
of 'Cancer of the appendix and pseudomyxoma peritonei'
The natural history of surgically treated primary adenocarcinoma of the appendix.
Nitecki SS, Wolff BG, Schlinkert R, Sarr MG
Ann Surg. 1994;219(1):51.
OBJECTIVE: The aim of this investigation was to determine the prognostic variables and optimal surgical procedure for patients with adenocarcinoma of the appendix.
SUMMARY BACKGROUND DATA: Primary adenocarcinoma of the appendix is a rare malignancy that constitutes less than 0.5% of all gastrointestinal neoplasms. However, the prognostic factors and the preferred surgical procedure and outcome are poorly understood.
METHODS: The authors reviewed their institutional experience from 1976 to 1992 in treating 94 consecutive patients with primary adenocarcinoma of the appendix. Patients with carcinoid tumors or those in whom the diagnosis of primary cecal cancer could not be ruled out were excluded from the study.
RESULTS: Fifty-two (55%) patients had the mucinous variety, of which 22 had pseudomyxoma peritonei; the other 45% had the colonic and adenocarcinoid types of tumor. The most common presentation was that of acute appendicitis. Interestingly, in no patients was the correct diagnosis made before surgery, and it was entertained intraoperatively in only 30 patients (32%). The cure 5-year survival rate was 55%, but it varied with stage (A, 100%; B, 67%; C, 50%; and D, 6%; p<0.01) and with grade (I, 68%, and III, 7%; p<0.01). Patients with the mucinous type had a better prognosis than those with the colonic type (p<0.01). The survival rate was superior after right hemicolectomy versus appendectomy alone (68% vs. 20%, p<0.001). Right hemicolectomy performed as a secondary procedure resulted in the upstaging of 38% of the patients' tumors. A second primary malignancy occurred in 33 patients (35%), of which 17 were located in the gastrointestinal tract.
CONCLUSIONS: Primary adenocarcinoma of the appendix should be treated by right hemicolectomy, even if it is a secondary procedure. Surveillance for synchronous or metachronous tumors, especially in the gastrointestinal tract, is warranted.
Department of Surgery, Mayo Clinic, Rochester, Minnesota.
Management and prognosis of adenocarcinoma of the appendix.
Cortina R, McCormick J, Kolm P, Perry RR
Dis Colon Rectum. 1995;38(8):848.
PURPOSE: Adenocarcinoma of the appendix is a rare neoplasm, and controversies persist regarding management. The purpose of this study was to identify prognostic factors and define management strategies for patients with adenocarcinoma of the appendix.
METHODS: A retrospective case series was conducted at three medical school teaching hospitals over a 20-year period from 1972 to 1992. Overall survival was determined by the actuarial life table method. Comparisons of prognostic factors were made using exact nonparametric log-rank tests.
RESULTS: Thirteen patients were diagnosed during the study period. Median age was 62 years. There were five males and eight females. The disease was not suspected in any patient preoperatively. Seventy-seven percent of patients had metastatic disease at presentation. Second primary malignancies were found in 15 percent of patients. Thirty-eight percent of female patients had synchronous ovarian lesions. Median survival was 22 months, with an estimated five-year survival of 43 percent (95 percent confidence interval, 22-84 percent). Patients with colonic histology had significantly worse survival than patients with mucinous histology (P = 0.0093). Patients with carcinomatosis had a significantly worse survival than noncarcinomatosis patients (P = 0.0078). Patients who underwent right hemicolectomy had a better prognosis for survival than appendectomy patients, but the difference was not statistically significant.
CONCLUSIONS: Carcinoma of the appendix is very difficult to diagnose preoperatively, and most patients are not identified until disease is advanced. Good prognostic factors include mucinous histology and the absence of carcinomatosis. Right hemicolectomy appears to be a reasonable option, although its superiority to appendectomy alone has not been definitively proven. High frequency of ovarian metastases in women suggests a role for bilateral oophorectomy. In addition, a complete work-up of the patient for a synchronous malignancy, especially in the gastrointestinal tract, should be considered.
Division of Surgical Oncology, Eastern Virginia Medical School, Norfolk 23507-1912, USA.
Mucinous and nonmucinous appendiceal adenocarcinomas: different clinicopathological features but similar genetic alterations.
Kabbani W, Houlihan PS, Luthra R, Hamilton SR, Rashid A
Mod Pathol. 2002;15(6):599.
The genetic alterations of appendiceal carcinomas have not been reported in detail. We studied the clinicopathological factors and genetic alterations including microsatellite instability, p53 overexpression, and mutations of the K-ras proto-oncogene of 30 appendiceal adenocarcinomas, consisting of 23 mucinous and 7 nonmucinous carcinomas. Sixteen (70%) mucinous carcinomas presented with pseudomyxoma peritonei, but 6 of 7 (86%) nonmucinous carcinomas presented with appendicitis (P =.002). All carcinomas were microsatellite stable, and p53 overexpression was present in only 1 of 30 (3%) carcinomas. K-ras mutation was present in 11 of 20 (55%) carcinomas, including 8 of 16 (50%) mucinous and 3 of 4 (75%) nonmucinous carcinomas. The mean survival of patients with mucinous carcinomas was 26 +/- 19 months compared with 13 +/- 9 months for patients with nonmucinous carcinomas (P =.0002). Our findings suggest that mucinous and nonmucinous carcinomas of appendix have similar genetic alterations, but different clinical presentation and prognosis.
Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston, USA.