Medline ® Abstracts for References 61,72,73
of 'Cancer of the appendix and pseudomyxoma peritonei'
Goblet cell carcinoid of the appendix.
Butler JA, Houshiar A, Lin F, Wilson SE
Am J Surg. 1994;168(6):685.
BACKGROUND: Over the past 15 years, a variant of appendiceal carcinoid has been recognized which is not widely known to the general surgeon. This tumor, variously labeled adenocarcinoid or goblet cell carcinoid, has a more aggressive natural history than classic appendiceal carcinoids and requires a different surgical approach.
PATIENTS AND METHODS: A survey of the tumor registries of 10 hospitals identified 7 female and 2 male patients with goblet cell carcinoid of the appendix between 1983 and 1993. The clinical data of these patients were reviewed.
RESULTS: Median age was 58 years (range 31 to 73). Clinical presentation followed two distinct patterns: acute onset suggestive of appendicitis or chronic symptoms associated with a pelvic mass. In no case was the diagnosis suspected preoperatively or established intraoperatively. Four patients with discrete distal appendiceal tumors treated with appendectomy are alive without evidence of disease 2 to 11 years since their diagnoses. The 5 patients with diffuse appendiceal involvement all had intra-abdominal metastases. Four are dead of disease at intervals from 5 months to 2 years postdiagnosis.
CONCLUSIONS: Goblet cell carcinoids are a distinct clinical and pathologic entity with variable malignant potential. Patients with diffuse appendiceal involvement require aggressive surgery as part of multimodal therapy.
Department of Surgery, University of California, Irvine.
Treatment of peritoneal carcinomatosis from adenocarcinoid of appendiceal origin.
Mahteme H, Sugarbaker PH
Br J Surg. 2004;91(9):1168.
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.
Department of Surgical Sciences, University Hospital of Uppsala, Sweden. Haile.Mahteme@Surgsci.uu.se
Mixed carcinoid and adenocarcinoma of the appendix: report of 4 cases with immunohistochemical studies and a review of the literature.
Lin BT, Gown AM
Appl Immunohistochem Mol Morphol. 2004;12(3):271.
Four cases of mixed carcinoid and adenocarcinoma of the appendix were reported. All cases presented with a dominant cecal-appendiceal tumor mass and local metastasis. Two patients had multiple peritoneal implants mimicking primary peritoneal serous adenocarcinoma or carcinomatosis. Histopathologic features of the tumors are similar, with infiltrating microglandular and cribriform patterns of tumor nests, and variable numbers of goblet cells. A literature review of "goblet cell carcinoid" that included nonlocalized cases revealed a significant percentage (>14%) of tumor-associated death, in contrast to the classic carcinoid tumor. Immunohistochemical stains were helpful to separate these tumors from carcinoid tumors and primary peritoneal serous adenocarcinoma. Mixed carcinoid and adenocarcinomas were cytokeratin (CK)-20 positive, and negative or weakly positive for chromogranin A and synaptophysin. Carcinoid tumors were CK20 negative and diffusely positive for chromogranin A and synaptophysin. Peritoneal serous adenocarcinomas were CK20 negative. These cases were clinically aggressive, and 1 patient had multiple recurrences and responded partially to chemotherapy.
Division of Surgical Pathology, Encino Tarzana Regional Medical Center, 18321 Clark Street, Tarzana, CA 91356, USA. firstname.lastname@example.org