Metastasis from argentaffinoma of the appendix.
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Appendiceal Neoplasms↗
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Immunolocalization of prealbumin as a marker for carcinoid tumours.
Using a PAP immunoperoxidase technique, we have demonstrated the plasma protein prealbumin in 92 (87 per cent) out of a series of 106 carcinoid tumours from various sites. The frequency of detection was greater than with the conventional stains; 83 (78 per cent) of the cases were positive for one or more of these stains. The Grimelius technique was by far the most effective of the conventional stains; it was positive in 81 (76 per cent) cases. We were unable to detect prealbumin in a variety of carcinomas. Although prealbumin identification did not appear to have any advantage over the routine stains in midgut-derived carcinoids, it was much more effective in the diagnosis of bronchial (foregut) and rectal (hindgut) carcinoids. We believe that prealbumin staining offers a cheap, reliable and valuable method for the detection of carcinoid tumours in routine histopathology.
Appendiceal Neoplasms↗
Subepithelial neuroendocrine cells and carcinoid tumours of the human small intestine and appendix. A comparative immunohistochemical study with regard to serotonin, neuron-specific enolase and S-100 protein reactivity.
A comparative immunocytochemical study was performed of subepithelial neuroendocrine cells of the human small intestine and appendix and carcinoid tumours of these sites, using a monoclonal antibody to serotonin and polyclonal antisera against neuron-specific enolase (NSE) and S-100 protein. Subepithelial neuroendocrine cells were easily identified in the lamina propria of the appendix. These cells, which sometimes occurred in aggregates, displayed serotonin and NSE immunoreactivity and were surrounded by S-100 protein immunoreactive cells, presumably of Schwann cell origin. In the appendix scattered cells with corresponding morphological features and immunoreactivity were also observed deep in the submucosa. In addition, subepithelial neuroendocrine cells were sparsely present in the lamina propria of the small intestine, occurring only as single cells in the deeper part of the mucosa below or between the epithelial crypts. Most appendiceal carcinoid tumours (11 of 12 examined cases) were biphasic and consisted of neuroendocrine tumour cells with intermingled S-100 protein immunoreactive cells (Schwann cells) with long cytoplasmic extensions. However, small intestinal (11 cases) and caecal (10 cases) carcinoids lacked S-100 protein immunoreactive cells as an integral component. The results indicate that the appendiceal carcinoids are mostly closely related structurally to the subepithelial neuroendocrine and Schwann cell aggregates of the lamina propria and are thus presumed to be histogenetically related to this cell system, while the histogenesis of small-intestinal and caecal carcinoids remains less clear.
Appendiceal Neoplasms↗
Lectin expression in carcinoid tumours of the gastrointestinal tract.
The binding of peroxidase-conjugated Dolichos biflorus (DBA), Triticum vulgaris (WGA), Canavalia ensiformis (Con A), Arachis hypogaea (PNA), Lotus tetragonolobus, and Bandeiraea simplicifolia I (BSAI) to gastrointestinal carcinoid tumours was studied. The results indicate that carcinoid tumour cells express certain carbohydrates similar to those present in the adjacent surface epithelium. The differences in the lectin-binding properties of carcinoid tumours of different sites of the gastrointestinal tract are closely related to the regional differences in the lectin binding of adjacent surface epithelium. These observations therefore form a useful basis for further studies in the application of lectin histochemistry to elucidate the histogenesis of carcinoid tumours.
Appendiceal Neoplasms↗
Endocrine cell hyperplasia and appendiceal carcinoids.
As endocrine tumours in a number of organs may arise in a background of hyperplasia, the density of endocrine cells in appendices from ten patients with carcinoid tumours was compared with that in appendices from ten age- and sex-matched control patients. Crypt and lamina propria endocrine cells were quantified separately. The density of argentaffin endocrine cells in the crypts was significantly higher in appendices with carcinoid tumours when compared with the controls. No difference was found in non-argentaffin endocrine cells, and no difference was found in either argentaffin or argyrophil endocrine cells in the lamina propria. While it is possible that carcinoid tumours induce an increase in the number of enterochromaffin (EC) cells in the background mucosa, it is considered more likely that EC cell hyperplasia predisposes to the development of carcinoid tumours of the appendix.
Adolescent↗
Carcinoid tumours of the appendix are different.
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Appendiceal Neoplasms↗
The topographical and age distributions of neuroendocrine cells in the normal human appendix.
In order to clarify the histogenesis of appendiceal carcinoid tumours, epithelial (ENC) and subepithelial (SNC) neuroendocrine cells were counted at four sites in 50 normal appendices stained by standard argyrophil and argentaffin techniques. In general, ENC were present in similar number at all sites within the appendix, whereas SNC were more numerous at the tip than at the base. The number of ENC was similar throughout life, apart from an increase in one neonate and some elderly patients, whereas SNC were maximal in young adults. Thus, the topographical and age distributions of SNC, but not those of ENC, parallels the topographical and age incidence of appendiceal carcinoid tumours, suggesting that most appendiceal carcinoid tumours arise from SNC rather than ENC.
Adolescent↗
The demonstration of a subset of carcinoid tumours of the appendix by in situ hybridization using synthetic probes to proglucagon mRNA.
Previous studies using immunohistochemistry have shown variable hormone production by carcinoid tumours of the appendix. In order to confirm the existence of a specific subset of these tumours, in situ hybridization using synthetic oligonucleotide probes to detect pre-proglucagon and pre-proinsulin mRNA was performed in formalin-fixed, paraffin-embedded material from eight tubular carcinoids, 12 insulin carcinoids, and two mucinous carcinoids. The results were correlated with standard silver and mucin stains. All tubular carcinoids but none of the insular or mucinous carcinoids contained proglucagon mRNA. Proinsulin mRNA was not detected in any of the tumours. Tubular carcinoids of the appendix constitute a definable subset of appendiceal carcinoids which have a similar distribution and prognosis to typical insular carcinoids and can be diagnosed on haematoxylin and eosin-stained sections confirmed by routine special stains. The main need for recognition is to avoid confusion with mucinous carcinoids, which have a worse prognosis and may require more aggressive treatment.
Appendiceal Neoplasms↗
RASSF1A promoter methylation and 3p21.3 loss of heterozygosity are features of foregut, but not midgut and hindgut, malignant endocrine tumours.
The Ras-association domain family 1A (RASSF1A) tumour suppressor gene is inactivated in a variety of solid tumours, usually by epigenetic silencing of the promoter and/or allelic loss of its locus at 3p21.3. RASSF1A induces cell cycle arrest through inhibition of cyclin D1 accumulation. In this work, 62 endocrine tumours from different sites in the gut were investigated for methylation of the RASSF1A promoter using the polymerase chain reaction, the presence of 3p21.3 deletions by loss of heterozygosity analysis, and cyclin D1 expression by immunohistochemistry. Methylation was found in 20/62 (32%) cases and was restricted to foregut tumours; deletion at 3p21.3 was found in 15/58 (26%) informative cases and restricted to malignant foregut tumours; cyclin D1 hyper-expression was found in 31/58 (53%) cases and correlated with RASSF1A methylation. Our data suggest that RASSF1A is involved in the development of endocrine tumours derived from the foregut only, and that the presence of both RASSF1A methylation and 3p21.3 deletion is associated with malignancy. These results may provide a rationale for foregut-targeted therapy for aggressive endocrine carcinomas entailing the use of demethylating agents.
Adult↗
Complications associated with intraperitoneal 32P.
The charts of all patients having received intraperitoneal 32P in the Indiana University Department of Radiation Oncology were retrospectively reviewed for complications and potentially related factors. Ninety-five patients had received this therapy, with a mean follow-up of 43.6 months. The majority of patients (81) had ovarian cancer. Complications were defined as mild if no intervention was required, moderate if medical intervention was required, and severe if the event was life-threatening or required surgical correction. Twenty patients (21%) had acute side effects recorded, with 15 of them (16%) being mild. The moderate complications (five patients) consisted of three cases of bowel obstruction, and two cases of abdominal pain requiring narcotics. There were no severe acute side effects. Chronic complications were found in 15 patients (20% actuarial 5-year incidence). Seven cases were mild (12% 5-year incidence), one was moderate (1%), and seven cases were classified as severe (7.4% 5-year incidence). All moderate and severe cases were bowel obstructions. Acute side effects were found to be related only to the volume of instillate (P = 0.049). Chronic complications were found to be related only to adjunctive pelvic/abdominal radiotherapy, with a 44% 5-year rate in patients receiving the combination having complications vs 17% (P = 0.04) (or 4.7% if mild complaints are excluded, P = 0.002) of those with 32P only. Comparison is made to other reports in the literature.
Acute Disease↗
Primary appendiceal malignancy mimicking advanced stage ovarian carcinoma: a case series.
BACKGROUND: Primary appendiceal malignancy metastatic to the ovaries is a rare condition that may mimic late stage ovarian cancer. This condition is rarely diagnosed preoperatively. CASES: Three patients referred to our institution from 1994 to 1999 for presumed late stage ovarian cancer were found to have primary appendiceal adenocarcinoma, adenocarcinoid, and mucinous cystadenocarcinoma metastatic to the ovaries at laparotomy. We describe the clinical course of these patients and review the relevant literature. CONCLUSION: It is important for the gynecologic oncologist to be aware of the clinicopathological features and surgical management of these malignancies, as the incidence, prognosis, and recommended treatment vary with histological subtype.
Adenocarcinoma↗
Prognostic features for peritoneal carcinomatosis in colorectal and appendiceal cancer patients when treated by cytoreductive surgery and intraperitoneal chemotherapy.
Peritoneal carcinomatosis from appendical or colorectal cancer has been regarded as a fatal clinical entity. We used cytoreductive surgery and intraperitoneal chemotherapy to treat consecutive patients with peritoneal carcinomatosis. There were 43 colorectal and 104 appendiceal cancer patients. The mean follow-up was 32 months, with a range of 0-140 months. Clinical features that showed prognostic significance included appendiceal versus colorectal primary (p < 0.0001), grade I versus grades II and III histopathology (p < 0.0001), complete versus incomplete cytoreduction (p < 0.0001), lymph node-negative versus lymph node-positive primary tumor (p < 0.0001), volume of peritoneal carcinomatosis present preoperatively for colon cancer (p < 0.0002), and nonmoderate versus heavy prior surgery (p < 0.0043). Features with no statistical prognostic significance include tumor volume for appendiceal cancer, age, sex, number of cycles of chemotherapy, operative time, complications, blood loss, and institution providing treatment. From these prognostic features, four staging groups were identified and 5 year survival was estimated by the product-limit survival method. Group I patients (n = 61) were those with grade I histology, no lymph node metastases, and a complete cytoreduction (survival at 5 years = 90%). Group II (n = 20) patients are those with grade II or III histology, no lymph node metastases, and a complete cytoreduction (62%). Group III patients (n = 22) had any histology, lymph node metastases, and a complete cytoreduction (45%). Group IV patients (n = 44) had an incomplete cytoreduction (12%). Peritoneal carcinomatosis is a treatable condition in selected patients with a possibility for long-term disease-free survival.
Appendiceal Neoplasms↗
Treatment of peritoneal carcinomatosis from colon or appendiceal cancer with induction intraperitoneal chemotherapy.
Colorectal cancer patients may present with peritoneal seeding of the abdominal cavity or develop it as recurrent disease. Peritoneal carcinomatosis has been regarded as a uniformly lethal clinical entity with no specific plan for management. Twenty-six patients with an established diagnosis of isolated spread of adenocarcinoma or cystadenocarcinoma of colorectal or appendiceal origin to peritoneal surfaces were treated with a combination of intraperitoneal 5-fluorouracil and intravenous mitomycin C. Three cycles of chemotherapy of 5 days each in duration were given once a month for 3 months. Two to 4 months after completion of chemotherapy, exploratory surgery and a cytoreductive procedure occurred. Responses to intraperitoneal chemotherapy were recorded at the time of cytoreductive surgery. Four of five patients with low-volume intraperitoneal adenocarcinoma had complete responses to induction chemotherapy. None of 18 patients with moderate- or large-volume cystadenocarcinoma had complete responses. The surgical procedure was facilitated by chemotherapy responses in patients with moderate-volume peritoneal carcinomatosis but not if large-volume disease was recorded. Surgical complications in patients treated by the induction approach were more frequent (p = 0.01) when compared with matched patients without intraperitoneal chemotherapy prior to cytoreductive surgery. This data may suggest that patients with low- or moderatevolume peritoneal carcinomatosis should be treated with induction chemotherapy because of a high rate of responsiveness. Large-volume peritoneal carcinomatosis from grade I cancer should have cytoreductive surgery prior to chemotherapy because of less responsiveness and the frequent surgical morbidity observed with the induction approach.
Adult↗
Patient selection and treatment of peritoneal carcinomatosis from colorectal and appendiceal cancer.
Colorectal cancer is a disease process that disseminates through lymphatic channels, through hematogenous routes, and by invasion through the bowel wall. These mechanisms result in lymph node metastases, liver metastases, and peritoneal seeding. Although lymphatic and venous dissemination requires an invasive local process, peritoneal seeding may occur with both high grade and low grade malignancies. Cancer dissemination that causes liver and lymphatic metastases occurs prior to surgical resection of the primary colorectal cancer. Peritoneal seeding and seeding of the resection site (local recurrence) may also occur as a result of the surgical trauma that accompanies resection of the primary lesion. Leakage of malignant cells from transected lymphatic channels may be the mechanism of this intraoperative intraperitoneal cancer dissemination. To limit the progression of peritoneal seeding and to treat large volume, low grade intraabdominal tumor deposits, combinations of cytoreductive surgery and intraperitoneal chemotherapy have been successfully employed. Selection factors that correlate with long-term benefit are (1) low grade of malignancy, (2) lack of lymph node or liver metastases, and (3) treatment of low volume disease. For patients with moderate or high grade colorectal cancer, only a low volume of disease can be treated successfully. For patients with low grade cancer, peritonectomy procedures are used to achieve minimal residual disease before initiating the intraperitoneal chemotherapy. In properly selected patients, peritoneal carcinomatosis from colorectal and appendiceal cancer is a treatable condition that may result in long-term disease-free survival.
Antineoplastic Agents↗
Appendiceal intussusception due to an appendiceal malignant polyp--an association in a patient with Peutz-Jeghers syndrome: report of a case.
A 40-year-old woman with Peutz-Jeghers syndrome and an appendiceal intussusception is reported. In this patient, the lead point was a large sessile, appendiceal polyp. The invaginated and inverted portion of the appendix resembled the long stalk of a pedunculated polyp on roentgenography and endoscopic examination. Histologically, the appendiceal polyp was a villous adenoma with mild to severe atypia and focal carcinoma in situ. In patients with Peutz-Jeghers syndrome, hamartomatous polyps and colorectal adenomatous polyps with highly malignant potential can coexist and must be managed appropriately. Therefore, when evaluating a polypoid or a pedunculated lesion in the cecal lumen, the possibility of an appendiceal intussusception should also be investigated.
Adenoma, Villous↗
Appendiceal mucinous cystadenoma associated with pseudomyxoma peritonei and multicystic peritoneal mesothelioma: report of a case.
An extremely rare case of mucinous cystadenoma developing to pseudomyxoma peritonei together with multicystic peritoneal mesothelioma is herein reported. The patient was 25-year-old Japanese woman who underwent an appendectomy under the diagnosis of acute appendicitis because of right lower abdominal pain. The patient histopathologically demonstrated appendiceal mucocele with pseudomyxoma peritonei. She underwent a laparotomy in our unit following detailed examinations. Several cystic tumors measuring from 3 to 5 cm in diameter were found in the omentum, and thus omentectomy, partial cecectomy and left oophorectomy were all performed to resect the tumors. Immunostaining and electron microscopy showed the appendiceal lesion to be mucinous cystadenoma, while the peritoneal lesion was multicystic mesothelioma. To our knowledge, this is the first report in the world literature of this rare combination of diseases.
Adult↗
Pseudomyxoma peritonei concomitant with early gastric cancer: report of a case.
The preoperative diagnosis of both appendiceal carcinoma and pseudomyxoma peritonei is difficult because of the nonspecific nature of the associated symptoms. More than 50% of all patients with carcinoma of the appendix are diagnosed with and treated for acute appendicitis. In addition, there have been few reports on patients with an appendiceal carcinoma or pseudomyxoma peritonei in association with other synchronous or metachronous malignancies of the alimentary tract. We herein report the first known patient with synchronous early gastric cancer and pseudomyxoma peritonei, in which the origin was correctly identified preoperatively.
Adenocarcinoma↗
Venous hemangioma of the mesoappendix: report of a case and a brief review of the Japanese literature.
We present herein the rare case of a 48-year-old man in whom an abdominal mass, revealed by celiotomy to be a solid tumor of the mesoappendix, was histologically diagnosed as having a venous hemangioma. To our knowledge, only 18 cases of mesenteric hemangioma have been reported in Japan, including the present case. However, establishing a correct diagnosis preoperatively is extremely difficult despite advanced imaging techniques. In fact, a mesenteric mass was diagnosed preoperatively in only 3 of these 18 cases. Complete excision with or without bowel resection was performed in 16 cases. Interestingly, the histological diagnosis of all the previous cases was cavernous hemangioma, confirming that this report documents the first case of venous hemangioma of the mesentery in the Japanese literature.
Appendiceal Neoplasms↗