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Management and prognosis of adenocarcinoma of the appendix.

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.

Adenocarcinoma↗

Surgical treatment of appendiceal mucocele.

HYPOTHESIS: Clinical presentation of appendiceal mucocele is related to malignancy and can influence surgical approach. DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: All cases of primary appendiceal mucoceles (simple mucocele, cystadenoma, cystadenocarcinoma) diagnosed between 1976 and 2000 were reviewed. There were 135 patients, 74 of whom were female. Mean age at diagnosis was 59 years. Mean follow-up was more than 6 years. INTERVENTIONS: A total of 129 patients underwent surgery, consisting of appendectomy (22 patients), right hemicolectomy (25 patients), or more extensive procedures (82 patients). MAIN OUTCOME MEASURES: Clinical, diagnostic, and surgical variables were statistically compared with postoperative morbidity and mortality and the presence of malignancy. P<.05 was considered significant. RESULTS: The presence of symptoms was associated with malignancy (58% vs 15%, P<.001), particularly abdominal pain (56% vs 29%, P =.005) and weight loss (77% vs 31%, P =.002). Abdominal mass was also associated with malignancy (86% vs 25%, P<.001). Moreover, pseudomyxoma peritonei and mucocele extravasation were associated with malignancy (95% vs 13%, P<.001, and 83% vs 15%, P<.001, respectively). The lesion size was not associated with malignancy; however, cystadenomas were significantly larger than simple mucoceles (8.1 cm vs 4.1 cm, P<.001), and no cystadenoma was less than 2 cm in largest diameter. CONCLUSIONS: A number of clinical, diagnostic, and intraoperative findings are associated with malignant mucoceles. All mucoceles greater than 2 cm should be excised to remove premalignant lesions.

Appendiceal Neoplasms↗

Is right hemicolectomy for 2.0-cm appendiceal carcinoids justified?

HYPOTHESIS: We believe right hemicolectomy (RHC) is not necessary in patients with an appendiceal carcinoid greater than 2.0 cm. DESIGN: A retrospective review of patients with a histologically confirmed appendiceal carcinoid from April 1, 1980, to February 28, 2005, and an analysis of the literature. SETTING: Tertiary care referral center. PATIENTS: Forty-eight patients (34 females and 14 males) with a histologically confirmed diagnosis of appendiceal carcinoid were included in the study. Appendiceal carcinoid was diagnosed incidentally in all 48 patients. Patient ages ranged from 11 to 86 years (mean age, 41 years). Postoperative follow-up and disease-free survival were confirmed in 33 patients via medical record review. MAIN OUTCOME MEASURES: We assessed the relationship between survival, tumor size, and the role of RHC vs appendectomy alone. RESULTS: Four patients in our series underwent secondary RHC and lymph node dissection for tumors greater than 2.0 cm, and none had positive lymph nodes. Following review of the literature, we were unable to find any recent evidence of distant metastasis from carcinoids in patients already treated by appendectomy. There seem to be no conclusive data to support the notion that RHC confers a survival benefit over appendectomy for carcinoids greater than 2.0 cm. CONCLUSION: Appendiceal carcinoids greater than 2.0 cm can be managed effectively with simple appendectomy, given their low malignant potential and slow growth, obviating the need for RHC in this group of patients without affecting overall survival.

Adolescent↗

Primary adenocarcinoma of the appendix masquerading as a bladder tumor.

Appendiceal adenocarcinoma, a rare lesion, is difficult to diagnose preoperatively. The unusual combination of genitourinary symptoms, a fungating adenocarcinoma of the deeper layers of the bladder wall, and an abnormal barium enema resulted in what seems to be the first reported case to be diagnosed preoperatively. Preoperative radiotherapy and radical exenteration of the tumor with ileotransverse colectomy and ureteroileal cutaneous diversion were performed. The patient is well without evidence of recurrence two years later.

Adenocarcinoma↗

Pseudomyxoma peritonei.

Thirty-eight patients with pseudomyxoma peritonei were treated at the M. D. Anderson Hospital, Houston, from 1954 to 1978. The various treatment regimens used have provided actuarial survival rates of 54% at five years and 18% at ten years. Local or regional disease was the cause of death in 68% of patients, and no patient died of metastatic disease. Initial definitive surgery should consist of effective tumor reduction, omentectomy, appendectomy, and, in the female subject, bilateral oophorectomy. Most patients have been treated adjunctively with either fluorouracil or melphalan (L-phenylalanine mustard) depending on the presumed site of origin, but results in a small number of patients treated with either whole abdominal or strip abdominal radiotherapy suggest that this modality may offer improved survival. Treatment with adjunctive radiotherapy alone has provided a five-year survival rate of 75%, compared with 44% for chemotherapy.

Adolescent↗