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Primary adenocarcinoma of the vermiform appendix: report of a series of ten cases, and review of the literature.
Ten cases of primary adenocarcinoma of the vermiform appendix are presented. The condition is rare and usually presents after middle age as acute appendicitis. It is seldom recognized during appendicectomy. The tumours resemble colonic adenocarcinomas but, because of peculiarities in the anatomy of the appendix, there is a tendency to early local spread. Right hemicolectomy performed as soon as the true nature of the lesion is recognized is the treatment of choice and has been shown to improve survival. Even after hemicolectomy the prognosis must be guarded, as local spread of disease may not be controlled in many cases.
Carcinoid tumours of the appendix.
Appendicular carcinoid tumours in 147 patients are presented. Two patients had evidence of metastatic spread at the time of presentation. Both patients had primary tumours less than 2 cm in diameter. Size remains the most reliable guide to the malignant potential of these tumours. If, after simple appendicectomy, a carcinoid tumour is found with a post-formalin fixation diameter greater than 1.5 cm then a subsequent right hemicolectomy should be considered.
Carcinoid tumours of the appendix in children 1957-1986: incidence, treatment and outcome.
Forty carcinoid tumours of the appendix were identified in children under 15 years of age between 1957 and 1986 from the records of the West Midlands Regional Children's Tumour Research Group. A significant excess of girls was observed (P = 0.02). All tumours were discovered incidentally and two patients underwent further surgery. Twenty-two of the 29 confirmed locations (76 per cent) were at the tip of the appendix and 34 of the 40 tumours (85 per cent) had invaded beyond the appendiceal submucosa. The reported incidence rate of 1.14 per million children per year remained constant throughout the study period. No recurrences, metastases or deaths from tumour were detected. Appendicectomy alone is confirmed as usually curative, although right hemicolectomy is occasionally necessary. Caution during routine appendicectomy is recommended to avoid possible tumour damage, as is consistent histological examination of all appendices in the paediatric age group. The importance of long-term follow-up is emphasized.
Carcinoid tumours of the appendix in children 1957-1986: incidence, treatment and outcome.
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Neoplastic lesions of the appendix.
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Carcinoid tumour of the appendix.
BACKGROUND: Appendiceal carcinoid tumours are found in 0.3-0.9 per cent of patients undergoing appendicectomy. Controversy exists over the management following appendicectomy, especially with regard to the role of right hemicolectomy in patients with tumours smaller than 2 cm in diameter. METHODS AND RESULTS: The literature pertaining to the behaviour of appendiceal carcinoids was reviewed in order to formulate indications for right hemicolectomy. Metastatic disease from appendiceal carcinoids is a rare occurrence, but is more common when lesions are larger than 2 cm in diameter. The risk-benefit balance of right hemicolectomy needs to be better defined, and an improved understanding of tumour cell biology may aid prognostic accuracy and decision-making. CONCLUSION: There is limited evidence on which to base clear indications for right hemicolectomy in patients with a diagnosis of appendiceal carcinoid. Acceptable indications are carcinoids larger than 2 cm in size, any high-grade malignant carcinoid (including those with a high mitotic index), mesoappendiceal invasion, lesions at the base of the appendix with tumour-positive margins, and goblet cell adenocarcinoid tumours.
Right hemicolectomy does not confer a survival advantage in patients with mucinous carcinoma of the appendix and peritoneal seeding.
BACKGROUND: Traditionally epithelial malignancies of the appendix with or without carcinomatosis have been treated by right hemicolectomy. Recent accumulation of a large number of patients with this disease has enabled a re-evaluation of this surgical judgement. METHODS: Clinical data on 501 patients with epithelial malignancy of the appendix were collected prospectively. All patients had peritoneal seeding at the time of referral and were treated by cytoreductive surgery and perioperative intraperitoneal chemotherapy. The main independent variable for statistical analysis was the surgical procedure used to resect the primary cancer (appendicectomy alone versus right hemicolectomy). Nineteen other clinical and pathological variables were considered as control variables. The endpoint for all analyses was survival. RESULTS: Median follow-up after the initial diagnosis was 4 years. The rate of regional lymph node positivity was 5.0 per cent. When the incidence of lymph node metastasis was determined by histological type, it was statistically significantly higher in intestinal (66.7 per cent) than in mucinous (4.2 per cent) tumours (P < 0.001). The presence of lymph node metastases had no influence on prognosis (P = 0.155). The surgical procedure (appendicectomy alone versus right hemicolectomy) had an influence on patient survival by univariate analysis (P < 0.001), but not by multivariate analysis (P = 0.258). CONCLUSION: Right hemicolectomy does not confer a survival advantage in patients with mucinous appendiceal tumours with peritoneal seeding. These data suggest that right hemicolectomy should be avoided unless metastatic involvement of the appendiceal or distal ileocolic lymph nodes is documented by biopsy, or the resection margin is inadequate.
Treatment of peritoneal carcinomatosis from adenocarcinoid of appendiceal origin.
BACKGROUND: Mucinous peritoneal carcinomatosis arising from appendiceal adenocarcinoid is uncommon and treatment options have been poorly defined. This study examined the effect of combined cytoreductive surgery and intraperitoneal chemotherapy on survival in these patients. METHODS: Of 810 patients with peritoneal malignancy of appendiceal origin treated by cytoreductive surgery and intraperitoneal chemotherapy, 22 patients (mean age 45 years; 17 women) had adenocarcinoid. RESULTS: The overall median survival was 18.5 (range 3.2-95.1) months, with 2- and 5-year survival rates of 39 and 25 per cent respectively. Survival after combined surgery and chemotherapy depended on the Peritoneal Cancer Index (P = 0.008) and the extent of cytoreduction (P = 0.007). CONCLUSION: Peritoneal carcinomatosis from adenocarcinoid of appendiceal origin is as invasive as peritoneal surface malignancy from colorectal adenocarcinoma. Patients in whom complete or near-complete surgical removal is possible should be considered for cytoreduction in combination with intraperitoneal chemotherapy.
Cytology of pseudomyxoma peritonei: report of two cases arising from appendiceal cystadenomas.
Pseudomyxoma peritonei is the clinical term for the diffuse deposition of mucus within the peritoneal cavity secondary to a mucinous tumor of the ovary or appendix. This gelatinous ascites, or "jelly-belly," may result in death from loss of intestinal function and intestinal obstruction caused by peritoneal implants rather than visceral invasion. Microscopic evaluation of peritoneal fluid is frequently an initial diagnostic test; however, in a search of the recent literature we were surprised to find only one case report of the cytologic features. This prompted us to report the cytologic findings in the peritoneal fluid of two cases of pseudomyxoma peritonei arising from appendiceal mucinous cystadenomas.
Goblet-cell carcinoid tumor in peritoneal fluid: a case report.
An unusual case of goblet-cell carcinoid in the peritoneal fluid of a 49-yr-old woman with a prior history of carcinoid tumor of the appendix is presented and discussed. Cytologic findings included clusters of uniform small cells containing nuclei with finely granular chromatin, small prominent nucleoli, and scant cytoplasm admixed with signet-ring cells. Peritoneal biopsies contained acini and small glands lined by malignant cells with features of a carcinoid tumor. Prominent signet-ring cells were interspersed within tumor nests. The signet-ring cells and small glandular lumens showed strong mucin positivity. Endocrine tumor cells were argyrophil- and argentaffin-positive. The histologic findings confirmed the diagnosis of goblet-cell carcinoid involving peritoneal fluid.
Diagnosis of metastatic appendiceal adenocarcinoid in liver by fine-needle aspiration cytology.
A 70-yr-old woman presented with symptoms clinically attributed to acute appendicitis. An adenocarcinoid tumor of the appendix was resected, followed by right hemicolectomy with negative lymph nodes. Seven years later, a fine-needle aspiration biopsy was performed on one of multiple hepatic nodules, revealing metastatic adenocarcinoid. The cytopathologic features of the metastatic tumor are described.
Sonographic appearance of mucocele of the appendix.
Mucocele of the appendix is an uncommon entity infrequently diagnosed prior to surgery or autopsy. Patients may be asymptomatic or present with a palpable right lower quadrant mass and/or pain. Barium enema findings are nonspecific and include nonfilling of the appendix and occasionally mass impression on the cecum. When visualized, calcification of the rim of a right lower quadrant mass may aid in diagnosis. The sonographic appearance in two cases is presented and compared with that in three other case reports in the literature, illustrating a spectrum of sonographic findings. Differential diagnosis is discussed.
Ultrasonographic features of mucocele of the appendix.
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Carcinoid tumor of the appendix: ultrasound findings in two cases.
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Regression of metastatic carcinoid tumors with octreotide therapy: two case reports and a review of the literature.
BACKGROUND: The antiproliferative effect of the somatostatin analogue, octreotide, on metastatic carcinoid tumors is poorly understood. Partial tumor regression seen radiographically has been reported with the use of octreotide therapy for neuroendocrine tumors. Complete regression of carcinoid tumors is rarely reported. RESULTS: Two patients with metastatic midgut carcinoid tumors were treated with subcutaneous octreotide 300 microg/day for symptomatic control of their carcinoid syndrome before debulking palliative surgery. During the laporatomies, both patients were found to have complete macroscopic regression of the metastatic lesions that had been identified radiologically before surgery, including liver metastases in one patient and periportal and retrocaval lymph nodes in the other. After surgery, the patients were evaluated every 3 months, and had no detectable disease at 30 and 43 months, respectively. Thirty cases of partial tumor regression with octreotide administered with or without other treatment modalities have been reported in the literature. Most of the patients involved received other treatment modalities. Only one other case reported in the literature showed complete regression with octreotide monotherapy. CONCLUSIONS: We report two cases of metastatic midgut carcinoid tumors that demonstrated a significant anti-proliferative response to octreotide monotherapy. Review of the literature failed to identify any specific prognostic factors with which the response to octreotide can be predicted. Possible mechanisms for this antiproliferative effect of octreotide on carcinoid tumors are discussed.
Third look surgery and beyond for appendiceal malignancy with peritoneal dissemination.
BACKGROUND AND OBJECTIVES: Second look surgery has been previously studied in patients with recurrent peritoneal dissemination from appendiceal malignancy. However, selection criteria for third, fourth, and subsequent reoperations are not available. METHODS: Five hundred and one patients with epithelial peritoneal surface malignancy of appendiceal origin underwent treatment with cytoreductive surgery and intraperitoneal chemotherapy during an 18-year period. Forty-five of these patients (8.9%) underwent three or more operative interventions. A critical statistical analysis of the impact of selected clinical features on survival was performed from a prospective database. RESULTS: Overall 5-year survival of these 45 patients was 70%. Five- and ten-year survival rates for patients with three interventions were 60 and 48%, for four interventions were 78 and 36%, and for five or more interventions were 100 and 80%. Sites of recurrent disease, histopathologic type of tumor, and lymph node status had no impact on survival. A complete second and third cytoreduction was associated with an improved 5-year survival rate. CONCLUSIONS: Prolonged survival in patients with three or more reoperations was significantly associated with a complete cytoreduction. However, after four or more interventions, the effects of tumor biology may predominate. Although 5-year survival is likely with multiple reoperations, prolonged follow-up shows that cancer cure is a rare event.
Total abdominal colectomy, pelvic peritonectomy, and end-ileostomy for the surgical palliation of mucinous peritoneal carcinomatosis from non-gynecologic cancer.
BACKGROUND AND OBJECTIVES: The optimal management of symptomatic advanced peritoneal carcinomatosis of non-gynecologic origin is not defined. Historic controls of surgical efforts report high postoperative mortality and morbidity rates with equivocal palliation. Novel surgical procedures need to be tested in terms of the impact on survival and quality of life. STUDY DESIGN: We studied 46 consecutive patients who underwent total abdominal colectomy, pelvic peritonectomy with construction of an end-ileostomy for palliation of peritoneal carcinomatosis. RESULTS: Total abdominal colectomy, pelvic peritonectomy, and end-ileostomy was successfully performed in 46 patients of median age of 54.4 years. Overall median survival was 10.7 months, with a mean follow-up period of 12 months. Patients with appendiceal malignancy had a median survival of 19.7 months. Prognosis was poorer for patients with colon cancer, who had a median survival of 7.0 months, while patients with primary peritoneal carcinomatosis had a median of 7.8 months. Postoperative morbidity and mortality rates were 19.5 and 8.6%, respectively. CONCLUSIONS: Total abdominal colectomy, pelvic peritonectomy, and end-ileostomy is a technically feasible procedure and is advocated for the palliation of patients with peritoneal carcinomatosis of appendiceal origin. It is not clear if the procedure should be advocated for more invasive gastrointestinal malignancies.