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Adenocarcinoid of the vermiform appendix.

Four cases of adenocarcinoid of the appendix were studied. Two tumours were found among 28 cases primarily diagnosed as appendiceal carcinoids. They showed characteristic histological structures with features of both a conventional carcinoid tumour and a mucinproducing adenocarcinoma with goblet cells. All tumours were small and ill-defined; three were associated with fibrous obliteration of the appendiceal lumen. All were diagnosed incidentally by the pathologist in appendices removed "en passant" or because of acute appendicitis. Three of the tumours appeared well differentiated with a low degree of malignancy similar to that of the conventional carcinoid tumour. In one case however, the tumour was less differentiated with atypical foci and a high mitotic count and had metastasised to peritoneum and both ovaries.

Adenocarcinoma↗

Extraepithelial intraneural endocrine cells as starting-points for gastrointestinal carcinoids.

Endocrine cells can be demonstrated by light- and electron microscopy in the lamina propria of the mucosa of the appendix. They are always in direct contact with a nerve fibre. The endocrine cell (type EC1 and EC2 cell) and the polyaxonal non-myelinated nerve fibre are separated from the interstitial connective tissue by a common continuous basal lamina. The term "ECC-NF complex" ("EC cell-nerve fibre complex") is suggested by the authors to describe this morphological unit. The intraneural endocrine cells may be derived from neuroendocrine-programmed ectoblasts (Pearse, 1977). The electron microscopic demonstration of these "ECC-NF complexes" in carcinoid tumours of the appendix and of similarly structured "eC-NF complexes" ("endocrine cell-nerve fibre complexes") in carcinoids of the rectum allows us, following the demonstration of small nerve fibres within carcinoids of the appendix, caecum and rectum (and bronchus) to propose a hypothetical pathogenesis of gastrointestinal carcinoid tumours from these intraneural endocrine cells. Carcinoid tumours may develop by proliferation of the intraneural endocrine cells with microcarcinoids as intermediate stages. In this way the histogenesis of the carcinoids is located a priori in the subepithelial stroma. Nerve fibres are morphological markers of this proposed mechanism. Assuming a neuroectodermal cytogenesis for the intraneural endocrine cells we therefore also postulate a histogenesis of the carcinoids from the neuroectoderm.

Appendiceal Neoplasms↗

A case of appendiceal cancer metastatic to the stomach with pseudomyxoma peritonei.

Appendiceal cancer associated with pseudomyxoma peritonei is a low grade malignancy and its extraperitoneal metastasis is extremely rare. We report a case of gastric metastasis of this tumor in a 76-year-old man. Two metastatic gastric tumors, which appeared after a 1-year interval, were successfully resected endoscopically. The patient was well for more than 3 years after the onset of the disease. To our knowledge, gastric metastasis from appendiceal cancer with pseudomyxoma peritonei has not been previously reported.

Aged↗

Synchronous adenocarcinoma of the transverse colon, the gallbladder and the vermiform appendix.

According to the literature synchronous adenocarcinomas of both, the colon and the gallbladder or the colon and the appendix are extremely unusual. To our knowledge the simultaneous occurrence of all three tumors has never been published before. Preoperative diagnosis of early stage tumors of the gallbladder and the vermiform appendix is also made very seldom. The following case report records an instance of synchronous adenocarcinomas of the gallbladder, the transverse colon, in a colonic polyp and the appendix. Owing to the patient's advanced age simultane cancer occurrence may be mere coincidence. Nevertheless, this extremely rare entity could also be ascribed to a genetic defect or an unknown carcinogenic agent.

Adenocarcinoma↗

Lymphoma of the appendix.

A rare case of appendiceal lymphoma in a patient who presented with lower gastrointestinal bleeding is reported. The findings on double-contrast barium enema, computed tomography (CT), and ultrasound are described and the clinical features of the few previously reported cases of appendiceal lymphoma are discussed.

Aged↗

Endometriosis of the cecum and appendix: two case reports.

Two cases of endometriosis of the cecal area are reported. In one case, the endometriosis was located in the appendix causing intussusception into the cecum. Both cases were associated with localizations of endometriosis in the rectosigmoid.

Adult↗

Peritoneal carcinomatosis from appendiceal cancer: results in 69 patients treated by cytoreductive surgery and intraperitoneal chemotherapy.

Sixty-nine patients presenting over a 10-year period with peritoneal carcinomatosis from appendiceal cancer were treated with cytoreductive surgery combined with intraperitoneal chemotherapy. The three-year survival is 89.5 percent in patients (38/69) with pseudomyxoma peritonei, 34.5 percent in patients (25/69) with cystadenocarcinoma, and 38.1 percent in patients (6/69) with adenocarcinoma (P < 0.01). In this study, a classification of residual disease following the cytoreductive surgery was used. The prognosis of the patients with minimal residual disease was better than that of those with moderate or gross disease, showing a 91.6 percent three-year survival compared with 47.8 percent and 20 percent, respectively (P < 0.01). The patients without lymphatic or hematogenous metastases had a better three-year survival than those with metastases (75.1 percent vs. 28.6 percent; P < 0.01). These findings suggest that peritoneal carcinomatosis from appendiceal cancer can be treated with long-term disease-free survival. The patients with low malignant potential cancer, complete cytoreduction, and no metastases showed the most effective disease control.

Adult↗

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↗

Femoral neuropathy: unusual presentation for recurrent large-bowel cancer.

PURPOSE: The recurrence of colon or rectal cancer may be signaled by serial carcinoembryonic antigen assays, patient symptoms, or radiologic tests such as abdominal and pelvic computed tomographic scans. There are unusual clinical presentations of recurrent disease. METHODS: Retrospectively, six patients with recurrent appendix, colon, or rectal cancer had a femoral neuropathy. Their clinical features and results of reoperative surgery were reviewed. RESULTS: All six patients had thigh weakness and atrophy, and four complained of leg pain. Each of the six patients underwent a reoperation, with a complete cancer resection in four. Pain control was excellent, but only one of the six patients, a patient with appendix cancer, demonstrated prolonged survival. CONCLUSIONS: Leg pain, thigh weakness, and atrophy are compatible with femoral neuropathy and are symptoms and signs compatible with recurrent large-bowel cancer. These findings may alert the clinician to the need for further investigation and treatments.

Adenocarcinoma↗

Peutz-Jeghers syndrome manifesting complete intussusception of the appendix and associated with a focal cancer of the duodenum and a cystadenocarcinoma of the pancreas: report of a case.

The unusual occurrence of an "inside-out" appendix reported here is a case of complete intussusception of the appendix of a 45-year-old woman with Peutz-Jeghers syndrome in whom the diagnosis of intussusception was made preoperatively. At laparotomy, the lead point of intussusceptum was revealed to be a Peutz-Jeghers syndrome polyp of the appendix. There was also a cystic lesion in the pancreas, and subsequent distal pancreatectomy revealed a cystadenocarcinoma of the pancreas. Two jejunal Peutz-Jeghers syndrome polyps and two duodenal Peutz-Jeghers syndrome polyps were found via intraoperative endoscopies. The duodenal polyps were endoscopically removed, whereas a jejunal wedge resection was performed for the adjoining jejunal polyps. One of the two duodenal polyps possessed an adenocarcinoma focus. To our knowledge, this is the first report of complete intussusception of the appendix caused by a Peutz-Jeghers syndrome polyp.

Appendiceal Neoplasms↗

Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies.

BACKGROUND: Appendiceal tumors are rare and often unexpectedly discovered in an acute situation, in which decision-making is difficult. To help define the most appropriate management, a retrospective analysis was undertaken to describe the clinicopathologic behavior of appendiceal tumors, and the literature was reviewed of the management of the different types of appendiceal tumors. METHOD: From a single center, a histopathologic database of 7,970 appendectomies, all appendiceal tumors, were identified and case notes reviewed. Analysis of clinical presentation, histopathology, operation, and outcome is presented. RESULTS: During a 16-year period (7,970 appendectomies), 74 patients (0.9 percent) with appendiceal tumors were identified: 42 carcinoid, 12 benign, and 20 malignant. Acute appendicitis was the most common presentation (49 percent), and 9.5 percent were incidental findings. Primary malignant tumors of the appendix were found in 0.1 percent of all appendectomies. Secondary malignant disease was identified in the appendix of 11 patients, most commonly (55 percent) from patients with primary colorectal disease. There was a high incidence of synchronous and metachronous colorectal cancer in all appendiceal tumors: carcinoids, 10 percent; benign tumors, 33 percent; secondary malignancies, 55 percent; primary malignancies, 89 percent. CONCLUSION: Appendiceal tumors are uncommon and most often present as appendicitis. Most are benign and can be managed by appendectomy, except adenocarcinomas and carcinoids larger than 2 cm, which are most appropriately managed by right hemicolectomy. A suggested management algorithm is provided. Controversy exists over the management of carcinoids 1 to 2 cm in size and adenocarcinoids. All types of appendiceal tumors have a high incidence of synchronous and metachronous colorectal cancer.

Adenocarcinoma↗

Mucinous cystadenoma of appendix concomitant with perityphlic granuloma.

We describe a case of mucinous cystadenoma of the appendix with perityphlic granuloma. The patient developed a hard palpable mass in the right lower quadrant and barium enema induced irregular bowel wall at the cecum. Based on a preoperative diagnosis of carcinoma of the appendix, we performed a laparotomy. On operation, we found a tumor mass in the region of the appendix; the mass adhered strongly to the retroperitoneum and cecum. Right hemicolectomy was carried out on a diagnosis of carcinoma of the appendix. Microscopic examination revealed mucinous cystadenoma of the appendix with perityphlic granuloma. Mucinous cystadenoma in the appendix is a rare tumor. In this patient, the tumor was accompanied by granuloma formation in adjacent tissues because of mucin expelled from the appendix. This case emphasizes that granuloma formation can make of difficult to differentiate mucinous cystadenoma in the appendix from cancer.

Appendiceal Neoplasms↗