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A case with carcinoid tumor of the appendix was encountered incidentally during an elective cesarean section. The tumor was discovered as a result of the routine exploration of the whole abdomen. The investigation for metastasis proved no evidence of spread and the patient was treated solely by surgery.
OBJECTIVE: The aim of this study was to assess the diagnostic accuracy of appendectomy in predicting lymph node metastases in women undergoing cytoreductive procedures for ovarian cancer. STUDY DESIGN: In 127 consecutive patients with ovarian carcinoma appendectomy was performed in 30 patients over a period of 5 years. Eight of them were found to have metastases to the appendix. Pelvic and paraaortic lymphadenectomy was performed in 34 patients, in 24 of them the appendix was removed during primary surgery. RESULTS: Among 19 patients without metastases to the appendix the lymph nodes were positive in five cases (26.3%) and among five patients with metastases to the appendix the lymph nodes were positive in four cases (80.0%), which is not a significant difference. Evaluation of the appendiceal metastases as a predictor of lymph node metastases in patients with ovarian cancer gives a sensitivity of 44%, a specificity of 93%, a positive predictive value of 80%, a negative predictive value of 74% and an accuracy of 75%. CONCLUSION: The possibility of predicting retroperitoneal lymph node metastases in ovarian cancer on the basis of histological examination of the appendix is limited.
Two cases of carcinoid tumour complicating inflammatory bowel disease (IBD) are presented. Both tumours were located in the appendiceal tip. The first case occurred in a man with Crohn's disease (CD), and the second one in a woman suffering from ulcerative colitis (UC). Histochemical and immunohistochemical studies were not allowed on case 1 because the tumour was not still present on serial sections of the appendix. On case 2, tumour cells were not reactive with Grimelius and Masson-Fontana stainings, but were strongly stained with anti-keratin and anti-chromogranin monoclonal antibodies (MAb), and faintly expressed neuron specific enolase (NSE), and Leu-7. Both cases occurred in inflammatory or damaged mucosa which exhibited Paneth cell metaplasia and hyperplasia and areas indefinite for dysplasia. Along with these lesions, hyperplasia of enteroendocrine cells was pointed out in the neighbouring appendiceal and colonic mucosa by means of anti-chromogranin MAb. These data suggest that the association of carcinoid tumour with IBD, albeit rare, is not coincidental and is the result of hyperplastic and dysplastic troubles that may involve enteroendocrine cells as well as such other derivatives of digestive stem cells as columnar cells, goblet cells and Paneth cells.
Goblet-cell carcinoids are particular mucus-producing tumors combining features of typical carcinoids and adenocarcinomas. The immunoreactivity of five goblet-cell carcinoids of the appendix and one tumor of the ileum for 5-hydroxytryptamine (5-HT, serotonin), glucagon, somatostatin, substance P (SP), neuron-specific enolase (NSE), lysozyme, secretory component (SC) and carcino-embryonic antigen (CEA) was compared with that of the mucosa of the appendix (n = 24) and ileum (n = 12), and of typical carcinoids (appendix: n = 10; ileum: n = 3). The goblet-cell carcinoids were consistently lysozyme-, SC- and CEA-reactive and contained weakly NSE reactive endocrine cells, while typical carcinoids were lysozyme-, SC- and CEA-negative, but strongly NSE- reactive. Two goblet-cell carcinoids were glucagon-reactive, one displayed SP-reactivity, one malignant tumor was reactive to the alpha-chain of glycoprotein hormones; six of ten typical appendix carcinoids were SP reactive, as were the three typical ileum carcinoids. Using the immunogold technique combined with the alcian-blue reaction, the presence of 5-hydroxytryptamine (5-HT) and mucus was demonstrated within the same cell. These findings suggest histogenetic differences between goblet-cell carcinoids and typical carcinoids; the former are possibly derived from undifferentiated stem cells, whereas the latter probably arise from endocrine cells in the mucosal stroma.
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Villous adenoma confined to the distal appendix has not been previously reported in conjunction with acute apendicitis. The presence of an adenoma indicates a need for further investigation due to an association with neoplasia elsewhere.
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Carcinoid tumors are uncommon lesions that arise from the intestinal tract or ovaries. Metastatic carcinoid was found in a 35-year-old woman who underwent laparoscopic excision of pelvic endometriosis. The primary tumor was in the distal appendix, and metastatic carcinoid coexisted with endometriosis in both pelvic sidewall specimens. This unusual situation is another reason to consider excision of presumed pelvic endometriosis. It is important to be aware of other uncommon gastrointestinal and pelvic conditions, such as the occasional carcinoid in the appendix or ovary.
STUDY OBJECTIVE: To evaluate the significance of the abnormal-appearing appendix during gynecologic laparoscopy. DESIGN: Retrospective study (Canadian Task Force classification II-2). SETTING: University-affiliated hospital. PATIENTS: One hundred sixty-six women. INTERVENTIONS: Laparoscopic appendectomy. MEASUREMENTS AND MAIN RESULTS: Five of 166 appendixes removed for abnormal appearance contained a carcinoid tumor. At least one patient was a candidate for additional resection. CONCLUSION: It is important for gynecologists to be aware of the possibility of a carcinoid tumor of the appendix, particularly if an abnormality is seen at the time of surgery. (J Am Assoc Gynecol Laparosc 6(3):303-306, 1999)
BACKGROUND: Interval appendectomy after conservative treatment of appendicitis with tumor formation remains controversial. The purpose of this study was to evaluate the efficacy of interval appendectomy based on a cost-effectiveness analysis. METHODS: This was a retrospective study including appendicitis patients with tumor formation who received conservative treatment at first admission to Taipei Veterans General Hospital between January 1998 and December 2003. Demographic data, rate of recurrent appendicitis, and medical costs were analyzed. RESULTS: Of the 165 patients, 1 died after an acute myocardial infarction. The rate of recurrence of appendicitis after conservative treatment was 25.5%. The median cost of follow-up after conservative treatment was NT24,344 dollars. The median cost of interval appendectomy was NT47,746 dollars. The median cost of appendectomy after recurrent appendicitis was NT62,135 dollars. Routine interval appendectomy in all 164 patients would have cost NT7,830,344 dollars. The follow-up protocol with appendectomy after recurrence cost NT5,655,220 dollars. An additional NT2,175,124 dollars (38%) would have been needed for routine interval appendectomy compared with the follow-up policy, an extra NT13,263 dollars per person. CONCLUSION: Routine interval appendectomy would increase the cost per patient by 38% compared with follow-up and appendectomy after recurrence. Routine interval appendectomy is not a cost-effective intervention.
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A 58-year-old woman underwent surgery for a pelvic mass. Palliative resection was performed despite the presence of multiple retroperitoneal lymph nodes. All pathology specimens exhibited an adenocarcinomatous component associated with carcinoid proliferation related to an appendicular tumor leading to the diagnosis of appendicular adenocarcinoid with ovarian, peritoneal, and nodal metastases. The patient's general status worsened rapidly with widespread nodal metastasis. Chemotherapy (Folfox 4 regimen) was given, and the patient improved within six weeks. Complete response was achieved after three months. Presently, more than three years after the end of the treatment, the patient is still alive and in complete remission. Appendicular adenocarcinoid is exceptional. These tumors exhibit two cellular components. Ovarian metastasis is frequent. Prognosis is intermediate between adenocarcinoma and malignant carcinoid. When given to patients with colonic carcinoma, the Folfox regimen used effectively here, is associated with a 50% objective response but complete response is very exceptional.
Although the use of intraperitoneal mucolytic agents is useful in the management of pseudomyxoma peritonei, effective removal of mucin is difficult even by repeated mucolysis in cases with massive ascites. To establish mucolytic therapy as a further effective procedure, biochemical analysis of mucin is required in greater detail. We reported here a case of pseudomyxoma peritonei caused by primary appendiceal cancer with biochemical analysis of mucin. The abdominal cavity in this case was filled with massive mucinous ascites. Although the mucolytic therapy was performed repeatedly, it was not effective in preventing mechanical ileus which was followed by surgical exclusion. Biochemical study of mucin showed that the mucin contained a small amount of hyaluronic acid, as characterized by electrophoretic study. These data led us to the hypothesis that the hyaluronic acid component plays a role in the pathogenesis of the replacement of mucin in the abdominal cavity.
The diagnosis of adenocarcinoid (mucinous/goblet cell carcinoid) is usually unexpected by both clinicians and pathologists. We report here the case of a 74-year-old man with gastric lymphoma (B-cell MALToma) diagnosed by endoscopy, who was found on exploratory laparotomy also to have extensive intraabdominal involvement by adenocarcinoid, arising from the ileum and/or appendix. The patient died two years after diagnosis with bladder outlet and small bowel obstruction due to diffuse metastases. In addition to mucin positivity, immunohistochemical stains demonstrated the tumor to be positive for chromogranin, synaptophysin, serotonin, gastrin, and glucagon. Of histogenetic interest, some individual neoplastic cells appeared to be positive for both mucin and chromogranin, and this was confirmed by the electron microscopic finding of microvilli, intracytoplasmic mucin droplets, and neurosecretory granules involving the same neoplastic cells. This also appears to be the first reported case of adenocarcinoid associated with lymphoma and demonstration of histochemical/immunohistochemical and ultrastructural evidence of cellular components with dual mucinous adenocarcinoma and neuroendocrine features, and the second reported case to have prostatic metastases.
Malignant tumours of the appendix are rare. They are usually carcinoid tumours that must be distinguished from extremely rare adenocarcinomas. Metastatic mucinous adenocarcinomas of the appendix are only reported as case histories. In clinical terms, the tumours usually manifest themselves as acute appendicitis, as ruptured appendicitis, as a tumour in the right lower abdominal quadrant or as a pelvic tumour, which are generally mistaken for an ovarian tumour with the same sonographic image. Advanced primary adenocarcinomas of the appendix with ovarian metastases cannot be distinguished intraoperatively from a FIGO III ovarian carcinoma. The pathologist makes the definitive diagnosis. These characteristics also apply to the case presented here. Surgical therapy of the isolated primary appendiceal carcinoma consists of a hemicolectomy--an appendectomy in favourable cases--and, in the case of a metastasised carcinoma, according to the guidelines for an advanced ovarian or colon carcinoma. The effect of chemotherapy is insufficiently documented.
Estrogen (ER) and progesterone receptor (PR) expression in primary ovarian mucinous tumors and the utility of these markers for distinguishing metastatic mucinous carcinomas in the ovary from primary ovarian mucinous tumors have not been extensively investigated. Immunohistochemical studies were performed on 124 mucinous tumors, including 52 primary ovarian tumors (30 atypical proliferative (borderline) mucinous tumors of gastrointestinal type, 11 atypical proliferative (borderline) mucinous tumors of seromucinous (endocervical-like) type, and 11 invasive mucinous carcinomas of usual (gastrointestinal) type) and 72 metastatic mucinous carcinomas in the ovary (primary sites: colorectum (24), pancreas (13), endocervix (eight), stomach (four), gallbladder/bile duct (four), appendix (four), and unknown (15)). All atypical proliferative mucinous tumors of gastrointestinal type, primary ovarian mucinous carcinomas, and metastatic mucinous carcinomas were negative for ER and PR with the exception of three metastatic endocervical adenocarcinomas which exhibited only weak expression of ER without PR. All atypical proliferative mucinous tumors of seromucinous type expressed ER to some degree and seven had some expression of PR. Immunohistochemical assessment of hormone receptor expression is of no value in distinguishing the common types of primary ovarian mucinous tumors (atypical proliferative mucinous tumors of gastrointestinal type and mucinous carcinomas of usual type) from the vast majority of mucinous carcinomas metastatic to the ovary. The above observations on hormone receptor expression in primary ovarian mucinous tumors support the concept that atypical proliferative (borderline) mucinous tumors of gastrointestinal and seromucinous (endocervical-like) types are distinctive tumors.
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.