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Granular cell tumour of the appendix in a patient irradiated for a rectal carcinoma.

We report on a 47-year-old man with a granular cell tumour of the appendix, discovered incidentally during surgery for a rectal adenocarcinoma that had been irradiated preoperatively. A detailed immunocytochemical analysis revealed positivity for S-100 and neuron-specific enolase (NSE). Electron microscopically, the cytoplasm of the tumour cells contained numerous pleomorphic lysosomes. In the appendix tissue adjacent to the tumour a neuroma and the histological features of radiation injury were present. Our findings suggest that this granular cell tumour may have originated from a pre-existing appendix neuroma which underwent granular degeneration, possibly as a result of radiation.

Appendiceal Neoplasms↗

Metastatic carcinoid tumor of the appendix. Report of a case and review of the literature.

A case of regional metastatic carcinoid of the appendix is presented and the literature reviewed for indications for right colectomy. Five cases of lymph-node metastasis from primary appendiceal carcinoids less than 2 cm in diameter have been reported. There is no reported case of systemic metastasis occurring after appendectomy for a carcinoid with a diameter of less than 2 cm. Perineural involvement, lymphatic invasion, mesoappendiceal invasion, histologic appearance, and location of the tumor in the appendix seem to be unreliable predictors of clinically significant regional node involvement. It is concluded that a right colectomy is indicated only in patients with carcinoid tumors of the appendix 2 cm or more in diameter, or with residual tumor at the margin of resection.

Adult↗

Retrospective evaluation of carcinoid tumors of the appendix in children.

Carcinoids of the appendix are rare in children and are usually diagnosed incidentally on histologic investigation following appendectomy for appendicitis. To investigate the significance of the diagnosis of appendiceal carcinoid in children, we conducted a retrospective study of the treatment and follow-up of 36 children with histologically confirmed carcinoid tumors of the appendix. Between 1970 and 2000 a total of 36 patients (25 girls, 11 boys) were diagnosed with appendiceal carcinoid. The median age of the patients at diagnosis was 12.3 years (range 6-16 years). The indication for appendectomy was acute lower right quadrant pain in 27 cases and chronic right lower quadrant pain in 9 patients. In 27 specimens the tumor was localized at the apex, in 7 at the midportion, and in 2 at the base of the appendix. The median tumor diameter was 6 mm (range 3-17 mm). Concomitant severe appendicitis was diagnosed in 14 patients 2 with a perforated appendicitis. In only one tumor were mucin-producing cells detectable. After a median follow-up of 10 years (range 2 months to 30 years) all patients were tumor-free. None of the patients had a synchronous or metachronous noncarcinoid malignant tumor. Appendiceal carcinoids are usually asymptomatic, and the indication for surgical intervention is acute or chronic abdominal pains in the right lower quadrant. For most patients the prognosis is excellent after appendectomy. As in adults, appendectomy is the appropriate treatment.

Adolescent↗

Carcinoid tumor of the appendix in the first two decades of life.

Carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in childhood and adolescence. Sufficient long-term follow-up data after surgical treatment are not currently available for patients diagnosed during the first two decades of life. From 1936 to 1988, 23 patients were observed at this institution with histologically confirmed carcinoid tumors involving the vermiform appendix. In contrast to the adult experience, in which the tumor is most commonly encountered as the result of an incidental appendectomy, 18 of these patients presented with signs and symptoms of an acute abdomen directing the surgeon to the appendix. In the other five cases, surgery was performed for other reasons. Median age at presentation was 13.0 years (range, 6 to 20 years). Fourteen patients were female, nine were male. Simple appendectomy was the initial procedure for all patients. Tumor size ranged from "microscopic" to 2.5 cm in largest dimension. Three patients subsequently underwent right hemicolectomy, and one patient had removal of a residual appendiceal stump, but no residual or metastatic tumor tissue was found in any of the resected specimens. Nineteen patients underwent simple appendectomy alone. Eighteen available specimens were reviewed at the time of this study for confirmation of histology and degree of invasion. The tumor invaded to the serosa in nine of 23 (39%). The mesoappendix or periappendiceal fat was involved in seven of 23 (30%). Vessel invasion was not noted in any specimen. Our median follow-up time was very long, being 26 years (range, 9 months to 51 years). No patient has had evidence of recurrent or metastatic disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Mucinous tumors of the vermiform appendix and ovary, and pseudomyxoma peritonei: histogenetic implications of cytokeratin 7 expression.

Cytokeratin 7 (CK-7) has been shown to be uncommonly expressed in colonic epithelial tumors, as opposed to ovarian epithelial tumors, which are always CK-7 positive. The authors investigated the expression of CK-7 in 17 appendiceal cystadenomas and carcinomas, 20 mucinous borderline tumors of the ovary, 10 cases of simultaneous mucinous tumors of the appendix and ovary, three so-called high-stage mucinous borderline tumors of the ovary, and three cases of pseudomyxoma peritonei (PP) of unknown origin. Nine appendiceal cystadenomas were CK-7 negative; two of these were associated with PP, and the peritoneal lesions were negative as well. Three cystadenomas were CK-7 positive. Three appendiceal carcinomas were CK-7 negative, and in one case the metastases were also negative. Two carcinomas were CK-7 positive. All 20 ovarian borderline tumors were CK-7 positive. Six cases of simultaneous mucinous tumors of the ovary and appendix were CK-7 negative, as were their peritoneal mucinous deposits. Four cases showed a positive reaction in both appendiceal and ovarian sites. Two of three so-called high-stage ovarian borderline tumors were CK-7 negative. All three cases of PP of unknown origin were CK-7 negative. In conclusion, appendiceal cystadenomas are often CK-7 negative, whereas ovarian mucinous borderline tumors are always CK-7 positive. The concordant staining pattern for CK-7 of simultaneous mucinous tumors involving the appendix and ovary (60% of which were CK-7 negative) supports an appendiceal origin for these tumors. Our results also support an appendiceal (or colonic) source for any CK-7-negative mucinous tumor involving the ovary or the peritoneum. Furthermore, our findings are in agreement with the assumption that mucinous borderline-like tumors in the ovary associated with PP are not ovarian in origin but are often, if not always, metastatic from an appendiceal (or other) mucinous tumor.

Adult↗

Müllerian-type epithelial tumor arising within a torsed appendix testis.

Because it is a remnant of the müllerian duct system, the appendix testis contains müllerian epithelium that theoretically may produce epithelial tumors similar to those that occur in the female genital tract. Few reports of tumors of müllerian origin arising in the testis exist, and rarely are neoplasms arising from the appendix testis identified. We present a case of a serous cystic neoplasm of low malignant potential derived from müllerian-type epithelium that was located in the torsed appendix testis of a young, otherwise healthy, boy.

Adolescent↗

Carcinoid tumors of the appendix detected at laparoscopy for gynecologic indications.

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)

Adolescent↗

Sonographic diagnosis of a carcinoid tumour of the appendix in a 14-year-old boy.

Carcinoid tumours of the appendix are rare tumours of the bowel which occasionally are found histologically in resected appendices. There are no publications on the sonographic diagnosis of carcinoid tumours of the appendix in children. We report on the sonographic examination of a carcinoid tumour in a 14-year-old boy. Sonography showed a hypoechoic tumour located at the apex of the appendix with eccentric growth. Histology revealed a carcinoid tumour with invasion of the regional lymphatic vessels.

Adolescent↗

Intramucosal adenocarcinoma of the appendix: how to find and how to treat.

A colonoscopic examination of a 58-year-old man revealed a small elevated lesion inside the orifice of the appendix. Using a polypectomy snare, a nodular polypoid lesion with a diameter of ca. 20 mm was removed from the lumen of the appendix. Histopathology showed that it was a well-differentiated adenocarcinoma. This is the first report of an intramucosal adenocarcinoma of the appendix diagnosed preoperatively; laparoscopy-assisted colectomy with a D2 lymph-node dissection was carried out. Endoscopists should consider an appendiceal tumor when an erosion, elevation, or deformity is seen in the head of the cecum.

Adenocarcinoma↗

Laparoscopic appendectomy: three-puncture technique with suspension of the appendix in nineteen cases.

Nineteen laparoscopic appendectomies by the same general surgeon are reported, using three trocar sites and suspending the appendix along the right lower quadrant trocar. This approach provides a fourth access site to the appendix without an additional port. Once the position of the appendix is stabilized, the two available ports are used to dissect around the base of the mesoappendix and place a silk ligature with extracorporeal ligating technique. All 19 procedures were completed by endoscopic means and without the use of endoscopic stapling devices, even when the mesoappendix was markedly swollen and edematous. The technique is presented here as an alterative method when performing laparoscopic appendectomy.

Appendectomy↗

Goblet cell carcinoids and related tumors of the vermiform appendix.

Appendiceal carcinoids with glandular differentiation pose difficulties in classification and prediction of clinical behavior. Sixty-four such cases were divided into three histologic groups on the basis of routine and immunohistochemical stains: (1) Tubular carcinoids were small and confined to the appendix, had small amounts of intraluminal mucin with few or no goblet cells, were nonargentaffin, lacked serotonin, and were diffusely positive for glucagon. All ten with follow-up (mean, 17 months) were without metastasis. (2) Goblet cell carcinoids were confined to the appendix and mesoappendix, circumferentially surrounded the appendiceal lumen, and were often not suspected grossly. Histologically, they were often mixed with small crypt-like glands and were serotonin positive. All 22 with follow-up (mean, 19 months) were without metastasis whether or not right hemicolectomy was performed. (3) Mixed carcinoid-adenocarcinomas showed spread into the cecum or adjacent viscera at the time of diagnosis and had a large carcinomatous pattern with areas of mucinous, signet-ring, or single-file structure, in addition to goblet cell or insular carcinoid. All patients had right hemicolectomies, and all but two with follow-up died of the disease (mean, 16 months). Although a histologic spectrum exists among carcinoid tumors and certain adenocarcinomas of the appendix, it is possible to delineate three biologically distinct groups. Surgical margins should be taken of all appendices because these tumors often do not form discrete masses.

Adenocarcinoma↗

Association of perforation of the appendix with female tubal infertility.

Although perforation of the appendix is considered a risk factor for female tubal infertility, the epidemiologic evidence supporting this relation is inconsistent. Risk factors for tubal infertility were compared for 121 women with documented primary tubal infertility attending in vitro fertilization clinics in Toronto, Canada, from July to December 1998 and 490 controls who were pregnant during the same time period. Self-administered questionnaires and review of medical records were used to assess exposures. The authors found that neither history of acute appendicitis nor perforation of the appendix was a statistically significant risk factor for tubal infertility. The crude odds ratio for perforated appendicitis was 3.4 (95% confidence interval (CI): 0.9, 12.9), and the adjusted odds ratio was 1.4 (95% CI: 0.3, 6.2). In addition to increased age and annual income, cigarette smoking (odds ratio (OR) = 2.0, 95% CI: 1.2, 3.2), history of endometriosis (OR = 6.0, 95% CI: 2.8,12.8), and history of pelvic inflammatory disease (OR = 6.0, 95% CI: 2.8, 12.8) were significantly associated with tubal infertility in multivariate analysis. These data do not provide substantial evidence that perforation of the appendix is an important risk factor for female tubal infertility.

Adult↗

Gastrointestinal stromal tumors in the appendix: a clinicopathologic and immunohistochemical study of four cases.

Mesenchymal tumors of the appendix are very rare, and specific stromal tumors (i.e., gastrointestinal stromal tumors, GISTs) have not been reported in this location to date. Four GISTs were identified in the review of primary mesenchymal tumors of the appendix from the files of the Armed Forces Institute of Pathology from 1970 to 1998. There were also one benign schwannoma, one diffuse neurofibroma with neurofibromatosis 1, one leiomyosarcoma in a child with HIV infection, and one inflammatory fibroid polyp. The four appendiceal GISTs occurred in adult males 56-72 years of age (mean 63 years). Two tumors occurred in patients who had surgery for appendicitis-like symptoms: one was an incidental finding during surgery for a malignant gastric epithelioid GIST and one was an incidental autopsy finding. Only one of the two appendices operated for symptoms had acute inflammation, and a polypoid GIST projected outward from the proximal part of appendix. Three tumors were partially obliterating nodules, eccentrically expanding the appendiceal wall. All four were spindle cell tumors, and three of them contained extracellular collagen globules (skeinoid fibers); none had atypia or mitotic activity (<1/50 high power fields). Immunohistochemically, two tumors studied were positive for CD117 (KIT), and two were positive for CD34. The tumors were negative for alpha-smooth muscle actin and S-100 protein. Follow-up revealed death from cardiovascular disease in one case (4 years after appendectomy) and liver failure because of malignant gastric epithelioid GIST metastatic to liver in another case 15 years after the appendectomy. This report documents the rare occurrence of CD117-positive GISTs as primary appendiceal tumors.

Aged↗

Carcinoid tumors of the appendix.

OBJECTIVE: A retrospective study of 41 patients with histologically confirmed diagnosis of appendix carcinoid tumors was undertaken by reviewing the surgical records at Massachusetts General Hospital. METHODS: There were 8 male and 33 female patients (mean age 32 years). Twenty-two patients (54%) presented with signs and symptoms suggestive of acute appendicitis. In 19 patients (46%) the lesions were discovered incidentally. The tumors were located in 32 patients at the tip, in 6 patients in the middle third, and in 3 patients at the base of the appendix. The tumor was less than 1 cm in diameter in 32 patients, between 1 and 2 cm in 7 patients, and was bigger than 2 cm in 2 patients. In 29 patients, the depth of tumor penetration was confined to the submucosa or to the muscle layers of the appendix, and in 8 patients the serosa was involved. In 4 patients, evidence of tumor extension into the meso-appendicular fat was present, including one patient with a tumor bigger than 2 cm and local lymph-node metastases. Forty patients underwent appendectomy alone. One patient with a tumor size bigger than 2 cm in diameter with positive lymph nodes in the mesoappendix underwent secondary right hemicolectomy. Complete follow-up was achieved in 35 patients, and all patients remained free of tumor recurrence. CONCLUSIONS: The authors conclude that appendiceal carcinoids are rare and most often are asymptomatic. Tumors of less than 1 cm are adequately managed by appendectomy alone. The appropriate treatment for tumors of 1 to 2 cm continues to be controversial. Right hemicolectomy is recommended for all tumors larger than 2 cm, whereas preference for an aggressive approach should be given in young patients.

Adult↗

Primary adenocarcinoma of the appendix in an inguinal hernia.

Primary adenocarcinoma of the appendix and primary carcinomata within hernial sacs are both uncommon clinical problems. A previously undescribed presentation of primary adenocarcinoma of the appendix in a right sided sliding inguinal hernia is presented and discussed. It is important to follow up patients with carcinoma of the appendix in order to exclude other colonic lesions. The St Vincent's Hospital (Melbourne) experience in this condition is limited but parallels that of major series elsewhere.

Adenocarcinoma, Mucinous↗

Level of aggregation for optimal epidemiological analysis: the case of time to surgery and unnecessary removal of the normal appendix.

STUDY OBJECTIVE: To illustrate the concept of "individualised fallacy", the result of improper interpretation and inference about aggregate level associations on the basis of associations at the individual level, in epidemiology. DESIGN: Cohort study. SETTING: Canadian province of Ontario. PATIENTS: All patients who underwent primary appendicectomy in 175 Ontario hospitals from 1989 to 1992. The association between rate of normal appendix removal and time to surgery was analysed at two levels: (1) at individual patient level, in which, for each patient, the exact number of days to surgery was derived, and (2) at hospital level, in which hospital specific proportions of time to surgery was calculated. MAIN RESULTS: Measured at individual level, compared with patients who had an operation on the same day of admission, the odds ratio was 2.41 (95% confidence intervals 2.28, 2.56) for patients who had an operation > 1 day after admission. Measured at hospital level, each 10% increase in the proportion of patients who had an operation > 1 day after admission resulted in a 15% reduction in the odds of normal appendix removal (odds ratio 0.85, 95% confidence intervals 0.82, 0.88) CONCLUSIONS: In this case study, hospital level measure correctly predicted a reduction in the rate of normal appendix removal by delaying surgery, whereas individual level measure biased the direction of the relation to the opposite. This example illustrates that bias in across level inference can occur either at individual or ecological level. The preferred level of analysis is the one that minimises confounding; often, it must be selected on the basis of a priori knowledge of the subject area.

Adolescent↗

Crohn's disease of the appendix: remission obtained by total parenteral nutrition.

A woman, aged 37 years, who had been suffering from advanced Crohn's disease for many years, was treated with long-term total parenteral nutrition (TPN), given in two 2-month periods at an interval of 6 months. Prior to the introduction of TPN there was inflammation of the appendix, the terminal ileum, and the entire colon. During the first period of TPN there was a temporary clinical and radiological improvement in the state of the intestinal tract, including the appendix. Two months after the first period of TPN abdominal pain recurred, and colonoscopy disclosed renewed severe inflammation of the mucosa from the cecum to the rectum. At operation, on day 45 of the second period of TPN, due to remaining stenosis of the terminal ileum, there was no longer any inflammation of the appendix, confirmed by histological examination.

Adult↗

Cancer of the appendix in long-standing ulcerative colitis: a case report.

Cancer of the appendix was found in a 69-year-old female patient affected by long-standing ulcerative colitis (UC). On histological examination the cancer was a typical cystadenocarcinoma of the appendix. The appendiceal mucosa not invaded by the neoplastic process was normal. Histological examination of the colorectal mucosa did not show dysplasia or cancer. These findings suggest that appendiceal cancer and UC may be unrelated diseases. A surveillance program for early detection of cancer of the appendix in patients with long-standing UC does not seem mandatory.

Aged↗