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Results for “APPENDICEAL NEOPLASMS”

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Appendiceal adenocarcinoma: long-term outcomes after surgical therapy.

PURPOSE: Appendiceal adenocarcinomas are very rare. We analyzed contemporary outcomes associated with surgical therapies for these malignancies. METHODS: Retrospective outcomes for patients treated at a tertiary academic medical center from 1981 through 2001 were analyzed. RESULTS: A total of 36 patients (22 females (61 percent) mean age, 52 years) with appendiceal adenocarcinoma were treated. Eighty-eight percent of patients presented with symptoms of acute appendicitis. Eighteen (50 percent) patients underwent curative resection (7 primary right hemicolectomies, 10 appendectomies + subsequent right hemicolectomy, and 1 appendectomy alone). Mean length of follow-up was 55 months. Overall five-year survival rate was 46 percent. The five-year survival rate after curative resection was 61 percent and after palliative surgery was 32 percent ( P < 0.05). Among patients who underwent curative resection, factors associated with improved five-year survival rates included histologic type (79 vs. 32 percent for colonic vs. mucinous types, respectively; P < 0.05), T stage (75 vs. 47 percent for T1 and 2 vs. T3 and 4, respectively; P < 0.05), and tumor grade (100 vs. 46 percent for well-differentiated tumors vs. moderately or poorly differentiated tumors, respectively; P < 0.05). CONCLUSIONS: Patients undergoing surgery for appendiceal adenocarcinoma can be stratified according to prognostic variables. The role of adjuvant therapies for patients with poor prognostic factors needs to be evaluated in a multi-institutional setting.

Abdominal Pain↗

Malignancies of the appendix: beyond case series reports.

PURPOSE: A comprehensive analysis was performed for five histologic types of appendiceal tumors to compare incidence, clinicopathologic features, survival, and appropriateness of surgery. METHODS: All patients diagnosed with mucinous adenocarcinoma (n = 951), adenocarcinoma (n = 646), carcinoid (n = 435), goblet (n = 369), and signet-ring cell (n = 113) in the Surveillance, Epidemiology, and End Results database (1973-2001) were analyzed. Evaluation of incidence, stage, and five-year relative survival were determined for each histology. The appropriateness of the operative procedure (i.e. , appendectomy vs. colectomy) was examined by tumor type and size. RESULTS: Tumor incidence, patient demographics, survival outcomes, and appropriateness of surgery varied significantly among the different appendiceal tumor histologies. The most common appendiceal tumors were mucinous. With regard to patient demographics, carcinoids presented at an earlier mean age of 41 years and 71 percent were female (P < 0.001 for both). Overall five-year survival was highest for carcinoid (83 percent) and lowest for signet ring (18 percent). Although current guidelines specify that a right hemicolectomy (rather than an appendectomy) be performed for all noncarcinoid tumors and carcinoid tumors >2 cm, we found that 30 percent of noncarcinoids underwent appendectomy. Similarly, 28 percent of carcinoids >2 cm under-went appendectomy, which is a lesser resection than is indicated. CONCLUSIONS: This study provides a population-based analysis of epidemiology, tumor characteristics, survival, and quality of care for appendiceal carcinomas. This characterization provides a novel description of the presentation and outcomes for malignancies of the appendix and highlights that a substantial number of patients with appendiceal tumors may not be receiving appropriate surgical resection.

Adenocarcinoma, Mucinous↗

Goblet cell carcinoid tumors (adenocarcinoid) of the appendix.

PURPOSE: Goblet cell appendiceal carcinoids represent rare tumors that exhibit histologic features of both adenocarcinomas and neuroendocrine tumors. We present the long-term results of a series of 15 patients, focusing on clinical manifestations, diagnosis, and management. METHODS: Eight male and seven female patients (median age, 52.8 years) were included. Final diagnosis was confirmed by histology. Patients were evaluated clinically, biochemically, and radiologically every four months. Median follow-up was 30 months. RESULTS: The majority of patients (7/15) presented with symptoms compatible with acute appendicitis. Right hemicolectomy was performed in all except one, who subsequently developed metastases. Three patients had metastases at previous diagnosis. Plasma chromogranin-A was slightly elevated in two of them, while urinary 5-hydroxy-indol-acetic acid was normal. (111)Indium-labeled octreotide scintigraphy was positive only in two of the four patients with metastases. Ki67 index was greater than 20 percent in all of them, while in only one with local tumor. Combination chemotherapy with either cisplatin plus etoposide or with 5-fluorouracil, cisplatin, and streptozotocin was administered to all patients with metastases resulting in temporary stabilization of disease. Twelve patients are alive, while three died of their disease 9, 13, and 14 months after diagnosis. CONCLUSIONS: The diagnostic value of chromogranin-A, urinary 5-hydroxy-indol-acetic acid, and (111)Indium-labeled octreotide scintigraphy seems to be limited in these tumors. Ki67 index appears to predict tumor behavior. Right hemicolectomy may reduce the risk of developing metastases. Chemotherapy may have efficacy in metastatic disease, however, more data are required to determine this and the optimal regimen.

Adult↗

Surgical debulking and intraperitoneal chemotherapy for established peritoneal metastases from colon and appendix cancer.

BACKGROUND: Aggressive treatment of peritoneal metastases from colon cancer by surgical cytoreduction and infusional intraperitoneal (IP) chemotherapy may benefit selected patients. We reviewed our institutional experience to assess patient selection, complications, and outcome. METHODS: Patients having surgical debulking and IP 5-fluoro-2'-deoxyuridine (FUDR) plus leucovorin (LV) for peritoneal metastases from 1987 to 1999 were evaluated retrospectively. RESULTS: There were 64 patients with a mean age of 50 years. Primary tumor sites were 47 in the colon and 17 in the appendix. Peritoneal metastases were synchronous in 48 patients and metachronous in 16 patients. Patients received IP FUDR (1000 mg/m2 daily for 3 days) and IP leucovorin (240 mg/m2) with a median cycle number of 4 (range, 1-28). The median number of complications was 1 (range, 0-5), with no treatment related mortality. Only six patients (9%) required termination of IP chemotherapy because of complications. The median follow-up was 17 months (range, 0-132 months). The median survival was 34 months (range, 2-132); 5-year survival was 28%. Lymph node status, tumor grade, and interval to peritoneal metastasis were not statistically significant prognostic factors for survival. Complete tumor resection was significant on multivariate analysis (P = .04), with a 5-year survival of 54% for complete (n = 19) and 16% for incomplete (n = 45) resection. CONCLUSIONS: Surgical debulking and IP FUDR for peritoneal metastases from colon cancer can be accomplished safely and has yielded an overall 5-year survival of 28%. Complete resection is associated with improved survival (54% at 5 years) and is the most important prognostic indicator.

Adenocarcinoma↗

Lymph node metastasis in epithelial malignancies of the appendix with peritoneal dissemination does not reduce survival in patients treated by cytoreductive surgery and perioperative intraperitoneal chemotherapy.

BACKGROUND: Peritoneal dissemination of appendiceal malignancy combined with regional lymph node metastasis is an unusual combination of patterns of cancer dissemination. METHODS: A database of 501 appendiceal malignancy patients, all with documented peritoneal seeding, was used to identify 25 patients with involvement of the regional lymph nodes. All patients were uniformly treated with cytoreductive surgery plus perioperative intraperitoneal chemotherapy with mitomycin C and 5-fluorouracil. The clinical and pathologic features of the lymph node-positive patients were compared with those of the lymph node-negative patients. The effect of regional lymph node involvement on survival was determined. Within the group of lymph node-positive patients, clinical and pathologic features were tested for their effect on survival. RESULTS: When compared with patients with no apparent lymph node positivity, patients with positive lymph nodes were more likely to have an acute abdomen as the initial presentation (P < .001). The intestinal (nonmucinous) histological type was more common (P < .001), and the disseminated peritoneal adenomucinosis histology was less common (P < .001). Survival with the aggressive treatment strategy used in these patients was not different for lymph node-positive as compared with lymph node-negative patients (P = .15 by univariate and P = .38 by multivariate analysis). CONCLUSIONS: Appendiceal malignancy with dissemination to the lymph nodes has a more acute onset and a more frequent nonmucinous histology. With aggressive treatment strategies, lymph node-positive patients did not show a statistically significantly diminished survival.

Acute Disease↗

Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy.

INTRODUCTION: In the past, peritoneal carcinomatosis, regardless of primary tumor type, has always been a lethal condition. Recently, special treatments using cytoreductive surgery with peritonectomy procedures combined with perioperative intraperitoneal chemotherapy have resulted in long-term survival. Appendiceal malignancy with a low incidence of liver and lymph node metastases may be especially appropriate for these aggressive local regional treatments. METHODS: All patients treated with surgery before January 1999 are included. Patients left with gross residual disease after surgery were not given intraperitoneal chemotherapy, but were later treated with intravenous chemotherapy. The intraperitoneal chemotherapy was given in the perioperative period, starting with mitomycin C at 12.5 mg/m2 for males and 10 mg/m2 for females. For patients whose pathology showed adenomucinosis, intraperitoneal chemotherapy was limited to treatment in the operating theater with heated mitomycin C. Patients with mucinous adenocarcinoma or pseudomyxoma/adenocarcinoma hybrid had, in addition to mitomycin C, five consecutive days of intraperitoneal 5-fluorouracil at 650 mg/m2 instilled in 1-1.5 liters of 1.5% dextrose peritoneal dialysis solution. A complete cytoreduction was defined as tumor nodules <2.5 mm in diameter remaining after surgery. The histopathology categorized the patients as having adenomucinosis, adenomucinosis/carcinomatosis hybrid, or mucinous carcinomatosis. A previous surgical score was used to estimate the extent of previous surgical procedures. RESULTS: The morbidity of treated patients was 27% and the mortality was 2.7%. In a multivariate analysis, prognostic factors for survival included the completeness of cytoreduction (P < .0001), the histopathological character of the appendix malignancy (P < .0001), and the extent of previous surgical interventions (P = .001). Patients with a complete cytoreduction and adenomucinosis by pathology had a 5-year survival of 86%; with hybrid pathology, survival at 5 years was 50%. Incomplete cytoreduction had a 5-year survival of 20% and 0% at 10 years. CONCLUSIONS: Cytoreductive surgery and perioperative intraperitoneal chemotherapy can be used to salvage selected patients with peritoneal surface spread of appendiceal primary tumors. Similar strategies for other patients with peritoneal surface malignancy such as peritoneal carcinomatosis from colon or gastric cancer, peritoneal sarcomatosis, or peritoneal mesothelioma should be pursued.

Appendiceal Neoplasms↗

Unusual cause of obstructive uropathy.

We report a case involving a 55-year-old healthy man that presented with urinary retention and a lower abdominal mass. His evaluation revealed a large mucocele arising from the appendix that caused severe bilateral ureteral obstruction and renal failure. Appendiceal mucoceles are rare and insidious entities that can present with urologic manifestation. Despite their rarity, this case highlights the importance of including mucoceles in the differential diagnosis in cases involving ureteral obstruction caused by external compression.

Appendiceal Neoplasms↗

Controversial origin of Pseudomyxoma peritonei.

Pseudomyxoma peritonei describes the accumulation of mucinous material in the abdominal cavity. The main diagnostic problem appears when the primary site of origin could be appendix or ovary. In this paper describe clinicopathological features and biological markers that support appendiceal origin.

Adenocarcinoma, Mucinous↗