Mantle cell lymphoma involving the appendiceal orifice as an unusual cause of lower gastrointestinal bleeding.
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The regenerating protein (Reg)-like protein (RELP, also known as Reg IV) is a recently characterized fourth member of the human Reg protein family. The Reg proteins are small, about 20-kD-sized, secretory proteins of C-lectin type. The previously known Reg proteins have been functionally implicated in regeneration, proliferation, and differentiation of the pancreas, liver, and gastrointestinal mucosa. To study the tissue expression of RELP, we raised a monoclonal antibody to RELP. By immunohistochemistry and in situ hybridization, we found a robust de novo expression of RELP in the neoplastic goblet cells of appendiceal mucinous cystadenomas and in the epithelial implants of pseudomyxoma peritonei (PMP). Our findings indicate that RELP serves as a marker for appendiceal mucinous cystadenomas and PMP, and that RELP may contribute to the pathogenesis of these disorders.
Small-cell neuroendocrine carcinomas (NECs) of the prostate are believed not to derive from benign orthotopic NE epithelial cells. Instead, an origin from a putative stem cell is actually the most favored concept. Whether this concept can also be applied to neuroendocrine tumors (NETs) of other organs, especially whether there are indications for well-differentiated NET-NEC sequence, is subject of the present study. A double-labeling technique for the proliferation marker MIB-1 and the NE markers chromogranin A (ChrA) and synaptophysin (SNP) was used for the immunohistochemical analysis of 45 well-differentiated NETs, 16 well-differentiated (low-grade) NECs, and 63 high-grade NECs of the esophagus, stomach, small intestine, appendix, colon, lung, prostate, and urinary bladder. The lowest proliferative activity was found in NETs (0.85% of tumor cells), and the highest activity was found in high-grade NECs (72.5%). The expression of ChrA was highest in NETs and lowest in high-grade NECs. None of the NETs and only sporadic cells in low-grade NECs showed double labeling (up to 0.05%). Up to 50% of the tumor cells in high-grade NECs were positive for MIB-1 and SNP. The percentage of double-labeled cells ranged between 0.9 and 39.6 (mean 9.7). No double-labeled cells were found in the normal epithelium adjacent to the tumors. Transitions from NET to NEC could not be observed. NETs and low-grade NECs differ in their proliferative activity from high-grade NECs, suggesting that they may arise from different precursor cell populations.
Leukaemic and lymphomatous infiltration of the appendix is rare and even rarer is acute appendicitis as the initial manifestation. From our routine biopsy material we collected four cases of haematological malignancies presenting as acute appendicitis or acute abdomen, caused or accompanied by tumoral infiltration of the appendix. Appendicitis was the initial manifestation that allowed diagnosis of the underlying disease. The clinical histories and histological examinations of the appendices and of one autopsy are described. We report the first detailed description of acute myeloid leukaemia involving the appendix, and three cases of lymphomatous infiltration of the appendix presenting with appendicitis, and give an overview of the literature. In these days of budgetary cuts in national health services, where one may be tempted not to have seemingly commonplace cases of appendicitis histologically verified, our cases emphasize that careful histopathological examination of all appendectomy specimens should be mandatory. Despite the fact that leukaemia and lymphoma of the appendix are rare, our cases illustrate that these must be included in the differential diagnosis of acute appendicitis and that physicians and surgeons have to be aware of these conditions.
BACKGROUND: Acute appendicitis is the most common abdominal condition necessitating urgent surgical intervention in the United States. The objective of this study was to determine if interval laparoscopic appendectomy after initial nonoperative treatment for late appendicitis presenting as an appendiceal mass is a safe alternative to immediate appendectomy. METHODS: Thirty two consecutive patients (aged 16-74 years) during a 5-year period presented with appendiceal mass. Seventeen received initial nonsurgical treatment followed by interval laparoscopic appendectomy (aged 16-60 years; group 1). Fifteen underwent immediate appendectomy (aged 16-74 years; group 2). RESULTS: All patients in the interval laparoscopic appendectomy group improved with initial therapy and underwent surgery an average of 4.9 months later. Although the operative time and the complication rate were similar between groups 1 and 2, the time to return to baseline activities was significantly less in group 1 after adjusting for age (p = 0.02 or less). CONCLUSIONS: Interval laparoscopic appendectomy is safe in patients with chronic appendicitis and allows for judicious diagnostic evaluation of the appendiceal mass and planned surgery under controlled conditions.
BACKGROUND: Since an increasing number of appendectomies are performed via laparoscopy, it is crucial to determine the impact of this approach on appendix carcinoid (AC) outcome. The goal of this study was to compare results of laparoscopic (LAP) versus open (OP) appendectomy for AC according to intend to treat approach. METHODS: A retrospective review (1991-2003) identified 39 patients (median age, 36 years; range, 12-83) treated by laparoscopy (LAP) or laparotomy (OP) for AC in a single institution. Follow-up was complete for all patients (median, 67 months; range, 4-132). RESULTS: Most cases had associated acute appendicitis (64%). Median carcinoid size was 1.1 cm (range, 0.3-5) and 0.4 cm (range, 0.2-3) in the LAP and OP groups, respectively. LAP and OP were performed in 21 (54%) and 18 (46%) patients, respectively. Surgical margins were positive in two patients in the LAP group and one patient in the OP group (p = 0.6). Right colectomies were performed for AC >2 cm in five patients after LAP and in four patients after OP (p = 0.9). Actuarial 5-year survival rates were 100 and 94% in the LAP and OP groups, respectively (p = 0.2). Two patients died in the OP group, one due to metastatic carcinoid and the other due to metachronous colorectal cancer. Synchronous or metachronous colorectal carcinomas developed in six patients (15%). CONCLUSION: Laparoscopic appendectomy is a safe procedure for AC, with carcinologic and long-term results similar to those of conventional appendectomy. Thus, pre- or per-operative suspicion of AC is not a contraindication to LAP. Prognosis of AC appears more dependent on carcinoid malignant potential or associated tumors. Risk for developing colorectal adenocarcinoma is high in AC patients and warrants follow-up of all patients with colonoscopic screening.
Indications and contraindications to laparoscopic surgery continue to be refined. Laparoscopic appendectomy for acute appendicitis is frequently selected by patients and surgeons, and clinical studies show it to be a reasonable alternative. In this case study, laparoscopic surgery was used to resect an appendiceal mucocele caused by a nonperforated mucinous adenocarcinoma. Implants of mucinous tumor were found widely disseminated on peritoneal surfaces at laparotomy 9 months later. As a result of this case study, the authors suggest that when an appendiceal mucinous tumor is encountered at laparoscopy, a special situation requiring totally atraumatic appendectomy is indicated. This clinical situation should be considered an indication for conversion to open appendectomy. All appendiceal tumors, including the most benign-appearing adenomas, can result in diffuse peritoneal implantation. This is the first report of an appendiceal mucinous tumor resected by laparoscopy associated with subsequent diffuse peritoneal carcinomatosis. This patient presentation reaffirms that dissemination of cancer may be associated with laparoscopic resection of structures containing a malignancy.
A rare case of early colonic adenocarcinoma of the appendix confined to the mucosa is reported. The patient remained well 5 years after simple appendectomy. We also review the Japanese literature on early colonic adenocarcinoma of the appendix. Twenty-seven cases of early colonic adenocarcinoma of the appendix, including ours, have been reported in Japan. In 20 of these patients, right hemicolectomy or ileocecal resection was performed. Eighteen patients were available for lymph node evaluation. Lymph nodes were negative for metastasis in 17 of the 18. Only one patient, with poorly differentiated adenocarcinoma invading the submucosa, had lymph node metastases. Our study shows that well-differentiated adenocarcinoma invading the submucosa, or adenocarcinoma of any differentiation confined to the mucosa, may be feasibly treated with simple appendectomy.
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We report a case of primary appendiceal cancer that metastasized to the ureter. The patient was a 51-year-old woman who complained of persistent left back pain and right lower abdominal pain. After a detailed examination she was diagnosed to have cancer of the appendix, multiple liver metastases, and left hydronephrosis. Since an obstruction of the ileocecum was obvious, she underwent an ileocecal resection and a resection of the spindle-shaped tumor invading the left ureter. Lymph node metastases were found at the root of the superior mesenteric artery. The spindle-shaped tumor was considered to be due to invasion of the appendiceal cancer to the left ureter. The patient died 5 months postoperatively.
We report the case of a 13-year-old girl in whom an appendix carcinoid was found incidentally during excision of a choledochal cyst. Although incidental carcinoid tumors of the extrahepatic bile ducts have been reported, to the best of our knowledge this is the first published case of a choledochal cyst associated with an appendix carcinoid. We present this case to highlight the importance of conducting a thorough exploration of all quadrants of the abdomen before closure.
Mucinous cystadenocarcinoma in the appendix is uncommon. An anomaly in the rotation of the intestine is also uncommon in adults. We herein report a case of mucinous cystadenocarcinoma in the appendix in a patient with nonrotation. To the best of our knowledge, this is the first report of appendiceal carcinoma in a patient with an anomaly of intestinal rotation. A 76-year-old woman was admitted to our hospital with left low abdominal pain. Physical examination revealed tenderness with muscle rigidity in the left lower quadrant. The patient was diagnosed to have intussusception by computed tomography and ultrasonography. An emergency operation showed nonrotation and the top of the appendix situated in the left iliac fossa. An appendectomy was performed because of gangrenous acute appendicitis. However, the cut surface of the appendix showed a mucocele measuring 4 x 4 cm in size. It was diagnosed to be mucinous cystadenocarcinoma histopathologically. A right hemicolectomy with lymph node dissection was performed, and no remaining cancer cells or lymph node metastases were found in the resected specimen pathologically. The patient had an uneventful postoperative course. No signs of recurrence have been observed for 23 months since her last operation.
A laparotomy was performed on a 44-year-old male patient with an acute abdomen who had findings suggestive of acute appendicitis and a periappendicular abscess. A histopathological examination revealed a carcinoid tumor infiltrating the intestinal serosa. The distal ileum and cecum were infiltrated with more than 40 multifocal tumors, with the largest measuring 1 cm in size. The distal ileum and cecum were resected. We failed to find any distant metastases either peroperatively or during postoperative scintigraphic tests. In addition, the patient had no symptoms of carcinoid syndrome either before or after surgery. No recurrence was experienced. We thus conclude that primary multifocal carcinoid tumors may act as a solitary carcinoid tumor, and the largest tumor tends to indicate the overall clinical outcome.
Appendiceal cancer associated with pseudomyxoma peritonei is a relatively low-grade malignancy rarely associated with extraperitoneal metastasis. We report herein the case of a 71-year-old man in whom a metastasis was found in the stomach 2 years after he underwent surgery for pseudomyxoma peritonei of appendiceal cancer. He was referred to our hospital after presenting with anorexia and vomiting. Gastrofiberscopy, abdominal computed tomography, and ultrasound examination all revealed a mass 4 x 4cm in size, containing a small ulcer, in the antrum of the greater curvature of the stomach. The histopathological diagnosis made from a biopsy of the tumor was mucinous cystadenocarcinoma. A distal partial gastrectomy was performed and the resected specimens from the appendiceal cancer resected 2 years earlier showed the same histological pattern as that of the gastric lesion. To the best of our knowledge, this is only the second report of pseudomyxoma peritonei secondary to mucinous cystadenocarcinoma of the appendix that metastasized to the stomach.
Adenocarcinoid of the appendix is a rare tumor with the histological features of both adenocarcinoma and carcinoid tumor. However, its biological behavior and malignant potential are still unclear. We treated two patients with this unusual tumor; a 60-year-old man and a 79-year-old woman. Both patients were initially diagnosed with acute appendicitis followed by an appendectomy. At surgery, the appendix was seen to be acutely inflamed without any macroscopic signs of tumor. Postoperative histological analysis revealed an adenocarcinoid tumor in the appendix, which had spread diffusely into its wall without forming a mass. Immunohistochemical staining with p53, MIB-1, bcl-2, and carcinoembryonic antigen suggested that neither of these tumors were particularly aggressive. Adenocarcinoid of the appendix is a rare tumor, which is very difficult to diagnose preoperatively and even macroscopically, making histological examination essential.
In the past two years two patients with carcinoid tumours have been operated in Chios General Hospital. These two cases gave rise to our review of the literature concerning the therapy and follow-up of colorectal carcinoid tumours. We demonstrate the differences among surgeons concerning the acceptable and relevant indications for a hemicolectomy in appendiceal carcinoids and the prevailing aspects in the therapy of carcinoid of the colon and rectum. Finally we present the imaging techniques and the biochemical examinations that are proven of value and therefore should be used in the post-surgery follow-up.
Peritoneal surface malignancy usually results from implantation of gastrointestinal cancer. In the past, this clinical situation was treated with palliative intent. A definitive approach to peritoneal surface malignancy involves peritonectomy procedures, visceral resections, perioperative intraperitoneal chemotherapy and knowledgeable patient selection. The quantitative prognostic indicators necessary for valid clinical judgements include the cancer histopathology (invasive vs. expansive progression), the preoperative abdominal and pelvic CT, the peritoneal cancer index and the completeness of cytoreduction score. Proper patient selection is mandatory for optimizing the results of treatment. In a series of phase II studies, appendiceal tumors with peritoneal seeding became the paradigm for success with an 85% long-term survival in selected patients. Carcinomatosis from colon cancer had an overall 5-year survival of 45% with selected patients. In all malignancies, early aggressive treatment of minimal peritoneal surface dissemination showed the greatest benefit. The definitive prognostic indicator was the complete cytoreduction. Oncologists must seek new knowledge regarding the management of peritoneal surface dissemination of cancer because a curative approach has been demonstrated in large phase II studies; in contrast all historical controls show 0% long-term survival. Additional adjuvant phase III studies with perioperative intraperitoneal chemotherapy in diseases where peritoneal surface spread occurs are indicated.
Ulcerative colitis (UC) represents a risk factor for colorectal cancer, but the association between UC and appendix cancer is uncommon. A 60-year-old woman with a 5-year history of UC initially received medical treatment with mesalazine and prednisone with no satisfactory response; therefore surgery was indicated. The procedure was a total intersphincteric proctocolectomy with ileostomy. Histopathological analysis indicated adenocarcinoma from the cecal appendix, and chronic-active ulcerative colitis of the colon. In conclusion, surgeons and pathologists should examine every surgical specimen from patients with UC because of the possibility, although remote, of a neoplasic pathology. The appendix adenocarcinoma and ulcerative colitis may or may not be associated, same as colon cancer in patients with UC.