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PubMed2001

Goblet cell carcinoids of the appendix: immunophenotype and ultrastructural study.

BACKGROUND: Goblet cell carcinoids of the appendix are rare neoplasms with uncertain biological behavior. OBJECTIVE: The aims of our study were to evaluate the immunophenotype of this neoplasm with cell cycle/cell proliferation markers and to understand their histogenesis with ultrastructural analysis using conventional carcinoids as a frame of reference. METHODS: Clinical data and archival pathologic material of all goblet cell carcinoids of the appendix recorded by the Saskatchewan Cancer Registry between 1970 and 1998 were reviewed and evaluated by light microscopy, histochemistry, immunohistochemistry, and electron microscopy. RESULTS: Seven cases of goblet cell carcinoids were identified among 110 cases of conventional carcinoids of the appendix. Histopathology revealed widespread infiltration of the periappendiceal fat in all cases, with extensive perineural invasion. The cells stained strongly positive for mucicarmine, periodic acid-Schiff, periodic acid-Schiff diastase, Alcian blue, cytokeratin, and carcinoembryonic antigen. Most cases were positive for synaptophysin. Increased expression of cell proliferation markers and cell cycle markers was observed. Expression of p53 was strong in one case. Electron microscopy demonstrated the presence of mucinous vacuoles of varying sizes and occasional membrane-bound neuroendocrine granules. CONCLUSIONS: Goblet cell carcinoids of the appendix arise from a pluripotent cell with divergent neuroendocrine and mucinous differentiation. These neoplasms are widely invasive; they demonstrate a high cellular proliferation rate and dysregulation of the cell cycle with up-regulation of cyclin D1 and p21, and down-regulation of p16. Complete removal of the tumor is recommended because of the unpredictable biological behavior of this tumor, which includes delayed local recurrences and lung metastases.

Adult↗

PubMed1976

Appendicular mucocele and peritoneal pseudo-myxoma.

From 2 personal cases the authors present a pathological appraisal of appendicular mucoceles. They distinguish between a benign kind with an atrophic mucous membrane and a malignant type with a hyperplasic epithelium arranged in papillary and vegetating structures; this is a neoplasm with local malignancy. This variety remains the most liable to become a peritoneal pseudo-myxoma representing the most serious complication of the appendicular mucecele. Treatment of the mucocele, which consists in an appenicectomy or a right bemi-colectomy, usually yields good results provided operative rupture can be avoided. Contrarily the peritoneal pseudo-myxoma remains a serious disease with a disappointing treatment.

Aged↗

PubMed1994

Villous adenoma of the vermiform appendix with cecal intussusception: a case report and review of literature.

Villous adenoma of the appendix is an uncommon neoplasm. Intussusception of the appendix is a rare pathological entity. We report here an intussusception of the appendix, the lead point being a villous adenoma, presenting in a 62-year-old male whose initial complaint was a progressive enlargement of a right inguinal hernia. The endoscopic, surgical, and pathologic data are presented. A review of the literature of this uncommon entity is also presented.

Adenoma, Villous↗

PubMed2002

Epithelial neoplasms of the appendix and colorectum: an analysis of cell proliferation, apoptosis, and expression of p53, CD44, bcl-2.

CONTEXT: Carcinomas of the appendix are usually well-differentiated mucinous adenocarcinomas that tend to produce pseudomyxoma peritonei and do not show metastatic spread until late in the disease process. In contrast, adenocarcinomas of the colon and rectum rarely result in pseudomyxoma peritonei and frequently metastasize, even if mucinous and well differentiated. These differences in behavior may be reflected by differences at the molecular level. OBJECTIVES: To examine adenocarcinomas and their precursor lesions (adenomas) of the appendix and colorectum and to determine whether differences exist in the numbers of proliferating and apoptotic cells or in expression of p53, bcl-2, and the standard form of CD44 (CD44s). DESIGN: Retrospective analysis of surgical specimens. SETTING: Multicenter study. PATIENTS: Individuals treated surgically for tumors of the appendix or colorectum. INTERVENTIONS: Sections were cut from formalin-fixed surgical specimens and immunohistochemical tests were performed for Ki-67 (as a marker of proliferating cells), M30 (as a marker of apoptotic cells), p53, CD44s, and bcl-2. MAIN OUTCOME MEASURES: Expression of Ki-67, M30, p53, CD44s, and bcl-2 in tumor cells. RESULTS: The appendiceal adenomas showed significantly lower Ki-67 counts, p53 expression, and bcl-2 expression. When compared with adenocarcinomas of the colorectum in general (mucinous and nonmucinous), the appendiceal adenocarcinomas showed significantly lower Ki-67 counts, M30 counts, and CD44s expression. However, when the analysis was confined to well-differentiated mucinous adenocarcinomas, only the M30 count was significantly different. CONCLUSIONS: The lower proliferative and apoptotic activity of appendiceal carcinomas and the lower CD44s expression are in keeping with their more indolent behavior compared with adenocarcinomas of the colorectum. However, when only the subset of well-differentiated mucinous adenocarcinomas was compared, only the apoptotic activity was different, suggesting that the other differences were related to the morphologic structure of the lesions.

Adenocarcinoma↗

PubMed1986

[Goblet cell carcinoid of the appendix].

A 50-year-old man was admitted with a diagnosis of acute appendicitis on January, 5, 1984 and an appendectomy was performed on the same day. The acutely inflamed appendix measured 7 cm in length, and a gross tumor, was seen at the apex. Histologic examination of the gross tumor, which was 1.7 cm in diameter, revealed a neoplasm representing a goblet cell carcinoid. Silver impregnation by both the Grimelius and Masson-Fontana methods disclosed a positive reaction in some neoplastic cells. Electron microscopically, endocrine granules with both limiting membranes and ahlos, 100-210 nm in diameter, were present in the neoplastic cells, Because of the relatively large size of the tumor and neoplastic invasion to the mesoappedix in this case and, in addition, the possible malignant nature of carcinoid tumor, ileoascendocolectomy was performed as an additional operation.

Appendectomy↗

PubMed1990

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↗

PubMed2001

Laparoscopic cryoablation of a metastatic carcinoid tumor.

Carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that can cause a carcinoid syndrome. The majority of carcinoid syndromes are the result of multiple hepatic metastases and are usually unresectable. Medical therapy has not proven effective and often causes intolerable side effects. Cryoablation has emerged as a promising treatment for various hepatic lesions when resection is not an option and medical therapy has been exhausted. With the addition of laparoscopy, surgeons now possess a new technique to treat primary and various secondary liver lesions. We report for the first time laparoscopic cryoablation for the treatment of a carcinoid metastatic to the liver.

Aged↗

PubMedDate not supplied

Primary adenocarcinoma of the appendix and pseudomyxoma peritonei.

Two cases of primary adenocarcinoma of the appendix are reported. Both patients presented with a clinical picture resembling acute appendicitis. One case was complicated by a pseudomyxoma peritonei. The other patient additionally had several colonic adenomatous polyps and a malignant neoplasm of the ascending colon. Primary adenocarcinoma of the appendix is rare and only some 250 cases have been reported. It usually presents as acute appendicitis in the 6th or the 7th decade. Prognosis and histology closely resemble that of colonic adenocarcinoma. Resection of the ileocecal segment is the first choice treatment in all stages except Dukes A. In Dukes A appendectomy alone is sufficient treatment. After ileocecal resection the 5 year survival is better than after appendectomy alone for Dukes' stages B and C. Clinical presentation and treatment of the adenocarcinoma and a remarkable complication, the pseudomyxoma peritonei, will be discussed.

Adenocarcinoma↗

PubMed1990

Adenocarcinoma of the vermiform appendix.

Primary adenocarcinoma of the appendix is rare. Eleven patients with this rare neoplasm have been evaluated at our institution over a 50-year period. We have reviewed the presentation and clinical course of these patients, and have compared them with those described in the literature. The presenting signs and symptoms, physical findings, and treatment were similar to those described in the literature. However, the 5-year survival of 20% is lower than most series, and reflects the advanced stage of disease at the time of diagnosis in this group of patients. Despite the low 5-year survival, we feel that aggressive therapy (right hemicolectomy) is necessary to obtain long-term survival.

Adenocarcinoma↗

PubMed1991

A case of cecocolic intussusception with complete invagination and intussusception of the appendix with villous adenoma.

Villous adenoma of the appendix is a rare neoplasm and intussusception of the appendix is a rare pathologic condition. A very rare case seen in a 35-year-old male with pain in the right lateral abdomen is reported. In this patient, the appendix along with the villous adenoma intussuscepted and invaginated into the cecal lumen, and presented as cecocolic intussusception. A polypoid lesion was diagnosed in the cecum by fiberoptic colonoscopy. Unlike polypoid lesions at other sites in the large intestine, polypoid lesions of the cecum may accompany intussusception and invagination of the appendix. Consequently, caution is required in performing endoscopic polypectomy in cases of polypoid lesions of the cecum.

Adenoma↗

PubMed1976

Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). I. Adenocarcinoma of the appendix.

A study of primary epithelial neoplasms of the appendix (with the exception of classical carcinoids), produced 24 adenocarcinomas and 42 benign lesions from the files of the Laboratory of Surgical Pathology of Columbia University. Invasive adenoarcinomas were often complicated by appendicitis and were then unexpectedly discovered during appendectomy. In one third of the cases the carcinomas were found in juxtaposition to adenomatous lesions of the appendix. Well-differentiated adenocarcinomas of the colonic type generally had a better prognosis than signet-ring cell (or microglandular) tumors. The most reliable criterion for correlating prognosis was the Duke's method of staging. Since invasive adenocarcinomas of the appendix can metastasize to regional lymph nodes, treatment should ideally consist of ileocolectomy.

Adenocarcinoma↗

PubMed1998

Goblet cell carcinoid of the appendix endoscopically diagnosed and examined with p53 immunostaining.

We report a case of a 62-year-old woman with goblet cell carcinoid of the appendix. She was admitted to our hospital in September 1994 after the discovery of liver tumors. After admission, a tumor in the right kidney and multiple tumors in the liver were found. She was diagnosed with renal cell cancer and metastasis to the liver and underwent excision of the kidney and enucleation of the largest liver tumor. Histological examination revealed that the liver tumor was a metastatic carcinoid tumor. As carcinoid tumors have frequently been found in the appendix, endoscopic examination was performed and a lesion was found in the appendix by colonoscopy. As predicted, the biopsy specimen was a carcinoid tumor, and she underwent an appendectomy. Histologically, the tumor was a goblet cell carcinoid. Goblet cell carcinoid is a rather rare neoplasm that has the histologic features of both carcinoids and adenocarcinoma. Forty-two cases of goblet cell carcinoid of the appendix have been reported thus far in Japan. However, few were diagnosed via endoscopic examination before surgical operation. We also carried out an immunohistochemical study with anti p53 antibody on the goblet cell carcinoid tumor of the appendix. Most tumor cells were strongly positive, while in three benign carcinoid tumors investigated simultaneously they were negative. These findings suggest that goblet cell carcinoid has an aggressive phenotype compared with benign carcinoid tumors.

Appendiceal Neoplasms↗

PubMed1999

[Tumors of the appendix].

Neoplasms involving the vermiform outgrowth are among the rarely met with conditions, accounting for 0.1 to 0.5 per cent of all gastrointestinal tract tumors. This is a report on personal observation of three patients presenting tumors of the appendix, diagnosed over the period 1987 to 1995. As shown by the results, tumors of the appendix run a clinical course characterized by two distinct forms: clinical picture of acute appendicitis--two cases, and clinical picture of tumor of the colon--one case. It is underscored that intraoperative diagnosis is extremely difficult. The diagnosis is usually made by pathoanatomist, while the therapeutic approach and operative tactics are determined on the ground of histomorphological study results and local operative status. The scope of operative intervention includes appendectomy and right hemicolectomy.

Adult↗

PubMed1980

Primary lymphoma of the gastrointestinal tract.

Primary gastrointestinal lymphoma represents approximately 1% of all gastrointestinal neoplasms. Gastric involvement is more common than small or large intestine and carries a better prognosis. Abdominal pain and weight loss may be the only manifestations and may be present for months or years before the diagnosis is made. Perforation and obstruction occur infrequently. Multiple tumors constitute 8% of cases. Although barium studies and endoscopy reveal the lesion in a high percentage of cases, exploratory celiotomy is not infrequently required for diagnosis. Only one-third of lymphomas are confined to the bowel at laparotomy. Histologically one-third are reticulum cell sarcomas and the remainder lymphosarcoma or lymphocytic lymphoma. Five year survival overall was 38%. Curative resections yielded a survival of 60% regardless of site while palliative resections offered only a 17% chance of cure. As expected, survival was inversely proportional to extent of nodal spread. Postoperative radiotherapy is recommended for residual disease.

Adolescent↗

PubMed2003

Mucous cystadenoma of the appendix: is it safe to remove it by a laparoscopic approach?

Mucinous cystadenoma is an uncommon neoplasm of the appendix usually discovered intraoperatively. Its clinical significance lies in the possible rupture and consequent spillage of mucin into the peritoneal cavity, leading to pseudomyxoma peritonei. Even if laparoscopy has been successfully used to perform appendectomy, some concerns exist regarding its use in dealing with mucinous secreting lesions because of possible spillage of mucin during surgery. We report a case of mucous cystadenoma of the appendix, which was successfully removed using a laparoscopic approach. At a 12-month follow-up assessment, the patient was free of disease. We believe that the laparoscopic approach is safe if surgery can be performed without grasping the lesion, and if the specimen is removed through the abdominal wall using a bag. Conversion to laparotomy should be considered if the lesion must be traumatically grasped, or if the tumor clearly extends beyond the appendix.

Appendiceal Neoplasms↗

PubMed2002

[Primary adenocarcinoma of the appendix. Case report and review of the literature].

A rare case of primary adenocarcinoma of the vermiform appendix (less than 250 cases described in the literature) in a 36 year-old female patient presenting signs and symptoms of an acute appendicitis is reported. Adeno-carcinoma of the vermiform appendix is a rare neoplasm of the gastrointestinal tract with an incidence of about 0,01-0,2%. Usually the diagnosis of adenocarcinoma of the vermiform appendix is difficult because symptoms and signs are not pathognomonic. In the case described, the diagnosis was intra and postoperative and confirmed by the pathological examination of the surgical specimens. At laparotomy, performed under suspicion of an acute appendicitis, disseminated disease was discovered, characterized by the involvement of the two ovaries, the left colon, the cecum, the vermiform appendix, with a peritoneal carcinosis and a hydroureteronephrosis. According to the dissemination of the disease, the surgical treatment was right hemicolectomy, anterior resection of left colon, bilateral oophorectomy and omentectomy. The post-operative course was regular. Adjuvant therapy was performed for 6 cycles, with 5FU and oxaliplatinum. The patient is still alive after 6 months and there is no sign of progression of the disease. A mild left hydroureteronephrosis is persistent.

Adenocarcinoma↗

PubMed2005

Carcinoid tumor of the appendix in childhood: the experience of two Italian institutions.

OBJECTIVES: Although rare, carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in children and adolescents. It is usually an incidental finding after a laparotomy for appendectomy, with a frequency of 2 to 5 cases per 1000 appendectomies. The experience with 14 cases of carcinoid reported in the appendix is described. METHODS AND RESULTS: In six patients the tumor measured 1 cm or less; only in one patient did it measure 2 cm. In three patients the tumor measured between 1 and 2 cm and in four the size was not known. In five cases the lesion had invaded the mesoappendix and periappendiceal fat. All tumors were discovered by chance, and three patients underwent further surgery as a result of suspected involvement of the margins. All the patients were alive with no evidence of disease at 24 to 214 months from diagnosis. CONCLUSIONS: Traditionally, local invasiveness and size have been considered prognostic factors. In our experience, both patients with local invasiveness and the patient with a tumor larger than 2 cm had good outcomes. Ileocolectomy performed in the patient with a 2-cm tumor and in another two patients with smaller tumors did not demonstrate residual disease. Although the need for right hemicolectomy still remains controversial for tumors measuring more than 2 cm, the approach may be nonaggressive in case of tumors invading the serosa and the periappendiceal fat. Nonaggressive treatment has been suggested by some authors in cases of tumors larger than 2 cm; however, larger series need to be evaluated.

Adolescent↗

PubMed1994

The natural history of surgically treated primary adenocarcinoma of the appendix.

OBJECTIVE: The aim of this investigation was to determine the prognostic variables and optimal surgical procedure for patients with adenocarcinoma of the appendix. SUMMARY BACKGROUND DATA: Primary adenocarcinoma of the appendix is a rare malignancy that constitutes less than 0.5% of all gastrointestinal neoplasms. However, the prognostic factors and the preferred surgical procedure and outcome are poorly understood. METHODS: The authors reviewed their institutional experience from 1976 to 1992 in treating 94 consecutive patients with primary adenocarcinoma of the appendix. Patients with carcinoid tumors or those in whom the diagnosis of primary cecal cancer could not be ruled out were excluded from the study. RESULTS: Fifty-two (55%) patients had the mucinous variety, of which 22 had pseudomyxoma peritonei; the other 45% had the colonic and adenocarcinoid types of tumor. The most common presentation was that of acute appendicitis. Interestingly, in no patients was the correct diagnosis made before surgery, and it was entertained intraoperatively in only 30 patients (32%). The cure 5-year survival rate was 55%, but it varied with stage (A, 100%; B, 67%; C, 50%; and D, 6%; p < 0.01) and with grade (I, 68%, and III, 7%; p < 0.01). Patients with the mucinous type had a better prognosis than those with the colonic type (p < 0.01). The survival rate was superior after right hemicolectomy versus appendectomy alone (68% vs. 20%, p < 0.001). Right hemicolectomy performed as a secondary procedure resulted in the upstaging of 38% of the patients' tumors. A second primary malignancy occurred in 33 patients (35%), of which 17 were located in the gastrointestinal tract. CONCLUSIONS: Primary adenocarcinoma of the appendix should be treated by right hemicolectomy, even if it is a secondary procedure. Surveillance for synchronous or metachronous tumors, especially in the gastrointestinal tract, is warranted.

Actuarial Analysis↗