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PubMedDate not supplied

Mucinous cystadenocarcinoma of the appendix. The controversy persists: a review.

Mucinous cystic neoplasms of the appendix continue to engender considerable debate in their diagnosis, management, and role in pseudomyxoma peritonei. This review addresses ongoing controversies with these unique neoplasms. Case reports and institutional series of mucinous cystadenocarcinoma were retrieved from the literature using Medline Search (1985-1996) using the following key words: appendix; mucinous cystadenocarcinoma; pseudomyxoma peritonei. 284 patients with mucinous cystadenocarcinoma were identified. Abdominal pain was the most common presenting symptom and objective preoperative diagnosis was difficult. There were 22 patients with concomitant appendiceal and ovarian mucinous neoplasms. Sixty-seven patients presented with mucinous cystadenocarcinoma and pseudomyxoma peritonei. However, a unified definition of pseudomyxoma peritonei was lacking. The recurrence rate was higher when an appendiceal neoplasm was associated with pseudomyxoma peritonei. In the reported literature, about an equal number of patients were subjected to appendectomy alone or right hemicolectomy. However, there was a survival advantage for right hemicolectomy versus appendectomy alone. Management of disseminated pseudomyxoma peritonei remains controversial. Mucinous cystadenocarcinoma appears to be a spectrum of low-grade malignant tumors. For correct diagnosis, a high index of suspicion must be maintained. The appropriate treatment for a patient with mucinous cystadenocarcinoma should be a right hemicolectomy. Aggressive debulking may improve survival when associated with pseudomyxoma peritonei. A better definition of pseudomyxoma peritonei is needed. In evaluation of different treatment modalities and prognosis, patients should be grouped according to underlying primary pathology.

Adult↗
PubMed1990

Sigmoid invasion as a late complication of mucinous cystadenoma of the appendix. Report of a case.

A cystic mass arising from the right iliac fossa was an incidental finding at laparotomy and was treated conservatively after a biopsy had shown no evidence of malignancy. Nine years later the patient presented with shock and colonic bleeding. A large cystic lesion arising from the vermiform appendix and invading the sigmoid colon was found and excised. Histologic examination indicated that the underlying lesion was an inflamed mucinous cystadenoma of the appendix. Frank invasion of viscera by such lesions has not previously been described. Complications of such lesions and their association with other colonic neoplasms are discussed.

Aged↗
PubMed1989

Adenocarcinoma of the appendix.

Patients presenting with adenocarcinoma of the vermiform appendix at the Royal Preston and Chorley District General Hospitals were reviewed for the 15 year period 1972-1986. Eleven cases were identified, representing a rate of 1 in 956 for all appendicectomies performed. A third of the cases presented as acute appendicitis. However, of 8 patients who initially had an appendicectomy, only 4 subsequently underwent further surgery (right hemicolectomy). One patient died of recurrent disease. Five of the patients presenting were under 60 years of age. During the same period, the total number of appendicectomies performed annually declined by 50%. The study highlights the need to subject all appendicectomy specimens to histological examination, and that the disease presents in a younger age group than commonly seen for malignant colonic neoplasms.

Adenocarcinoma↗
PubMed2005

Diagnostic challenge of secondary (metastatic) ovarian tumors simulating primary endometrioid and mucinous neoplasms.

Secondary (metastatic) neoplasms to the ovary often cause diagnostic problems, especially those tumors that produce large, symptomatic ovarian tumors that masquerade clinically and pathologically as primary ovarian tumors of surface epithelial type. Most of these tumors arise from organs of the digestive system. Except for typical Krukenberg tumors, which usually originate in the stomach and generally are easily recognized, the most diagnostically problematic secondary ovarian tumors are those that originate in the large intestine, appendix, and pancreas. Metastases from these sites typically produce histologic patterns resembling primary ovarian endometrioid carcinoma or mucinous epithelial neoplasms of borderline and malignant types. This review focuses on the diagnostic challenge of distinguishing these secondary ovarian tumors from primary ovarian neoplasms. Studies on useful or potentially applicable immunohistochemical stains are also detailed.

Appendiceal Neoplasms↗
PubMed1996

Synchronous mucinous tumors of the appendix and the ovary associated with pseudomyxoma peritonei. A clinicopathologic study of six cases with comparative analysis of c-Ki-ras mutations.

Mucinous tumors of the ovary are often associated with mucinous tumors of the appendix. It has not been clearly determined whether they are independent or metastatic neoplasms. A clinicopathologic study and a comparative analysis of c-Ki-ras mutations were done in six cases of synchronous ovarian and appendiceal tumors. The clinicopathologic features (simultaneous presentation, bilaterality or right-sided predominance, similar histopathologic findings, presence of pseudomyxoma peritonei) suggested that they were primary appendiceal tumors metastatic to the ovaries. DNA was extracted from formalin-fixed, paraffin-embedded tissue, and target sequences were amplified in vitro by the polymerase chain reaction. Mutations were detected by the presence of restriction fragment length polymorphism, artificially introduced by the use of mutant amplimers. The pattern of c-Ki-ras mutations was identical in the ovarian and appendiceal tumors of all patients. Four patients had a GGT --> GAT (Gly --> Asp) transition and one a GGT --> GTT (Gly --> Val) transversion, all detected in codon 12. No mutation was found in the sixth patient in either the ovarian or the appendiceal tumor. Because c-Ki-ras mutations are considered to represent an early event in tumorigenesis, our results support a clonal nature for both tumors and suggest that they are not independent tumors but rather originate one from another.

Adenocarcinoma, Mucinous↗
PubMed1996

Cytologic diagnosis of pseudomyxoma peritonei: common and uncommon causes.

Pseudomyxoma peritonei (PP) refers to accumulation of mucus in the peritoneal cavity, regardless of cause or pathogenesis. We have reviewed 8 cases of PP diagnosed in our institution over the past 5 years. Pertinent cytologic features include a mucinous background with mesothelial cells and histiocytes. Two cases contained rare clusters of neoplastic epithelial cells, with low-grade nuclear features of malignancy. The origin and nature of the parent neoplasms were variable, with no reflection of the cytologic features of PP.

Aged↗
PubMed1993

Review of the pathologic diagnoses of 2,216 appendectomy specimens.

A retrospective review of 2,216 patients surgically treated for a clinical diagnosis of acute appendicitis was performed. In 80% of cases, histologic acute appendicitis was found; 57% of these patients were male. The rate of normal appendectomy was 16%, and females comprised 68% of that group. The remaining 102 cases (4%) were designated as the alternate diagnosis group. Apart from histologic diagnoses that could be considered variants of normal (57) or acute inflammation (20), the alternate diagnosis group included such entities as neoplasm, parasitic infection, mucocele, and diverticulitis.

Adult↗
PubMed1987

Carcinoid tumor of the appendix: treatment and prognosis.

In a long-term study of 150 unselected patients with carcinoid tumors of the appendix, we found that the neoplasms were usually less than 1.0 cm in largest dimension and discovered as an incidental finding during surgery performed for other reasons. Metastases were observed with none of the 127 appendiceal carcinoids less than 2.0 cm in largest dimension, with 3 of the 14 lesions greater than or equal to 2.0 cm but less than 3.0 cm in greatest dimension, and with 4 of the 9 lesions greater than or equal to 3.0 cm. Paradoxically, the patients with the larger tumors and metastases were younger than those with smaller and clinically benign tumors. The median age of patients with tumors greater than or equal to 2.0 cm was 31 years and that of those with metastases was 29 years, as compared with a median age of 42 years in patients with nonmetastasizing tumors less than 2.0 cm. We conclude that simple appendectomy is adequate treatment for patients with apparently localized tumors less than 2.0 cm in largest dimension. We have not observed any recurrences or metastases among 122 such patients followed for a median time of more than 26 years. Simple appendectomy is probably also appropriate treatment for lesions greater than or equal to 2.0 cm in elderly patients or in those at high operative risk. Right hemicolectomy seems justified only in young patients with tumors greater than or equal to 2.0 cm who have a low risk of operative morbidity or mortality. Vascular involvement and invasion of the mesoappendix are features that may favor a more radical approach.

Adolescent↗
PubMed1988

Preoperative colonoscopic diagnosis of villous adenoma of the appendix. Report of a case and review of the literature.

This case of villous adenoma of the appendix reported is unique by virtue of its having been diagnosed preoperatively using colonoscopy. Only 45 such lesions have been described previously, and a review of those cases reveals that 93 percent were discovered at appendectomy performed either incidentally or for acute appendicitis. The malignant potential of this entity is unknown and its treatment is controversial. Because of a report association between adenomas of the appendix and other gastrointestinal neoplasms, long-term surveillance is recommended.

Adenoma↗
PubMed2000

[Morphology, DNA cytophotometry and prognosis in gastrointestinal neuroendocrine tumours (carcinoids). A clinico-pathological study of 95 patients].

A total of 123 manifestations (97 primary tumours and 26 metastases) of neuroendocrine tumours of the gastrointestinal tract observed in 95 patients was investigated for the prognostic value of clinical, histological and DNA cytophotometric parameters. Metastases almost exclusively occurred among ileal carcinoids, which also were responsible for all 14 cases of lethal outcome observed during the follow-up period of mean 42 months. Aneuploid DNA values could be determined significantly more frequently among ileal than in non-ileal carcinoids and showed - upon analysis of the total group of gastrointestinal neuroendocrine tumours - a significant correlation to lethal course of disease. In addition, among 18 cases with primary and secondary carcinoid manifestations available for DNA cytophotometry, an association between the DNA content of metastatic neuroendocrine tumours and prognosis came to light. When applied to the group of ileal neoplasms, however, the parameter DNA content did not allow a better prognostic assessment.

Adult↗
PubMed2001

A case of primary mucus secreting adenocarcinoma of the appendix.

Primary mucus secreting adenocarcinoma of the appendix is a rare finding, one in 2500 cases of intestinal malignancies. This is a report of a young man with vague abdominal symptoms for about two years followed by appendicitis. Interval appendectomy revealed a neoplasm of the appendix. Histopathology confirmed the diagnosis of mucus secreting adenocarcinoma and right hemicolectomy was done.

Adenocarcinoma, Mucinous↗
PubMed2004

Gastrin releasing peptide and gastrin releasing peptide receptor expression in gastrointestinal carcinoid tumours.

AIMS: To establish whether gastrin releasing peptide (GRP) and the GRP receptor (GRPR) are expressed together in gastrointestinal carcinoid tumours. METHODS: Twenty six carcinoid tumours from the stomach, small intestine, appendix, and colorectum were investigated by immunohistochemistry for GRP and GRPR. RESULTS: GRP was detected in nine of 19 tumours and GRPR in 22 of 26. Coexpression of both the ligand and receptor was seen in six of 19 cases. GRPR but not GRP was more strongly expressed in appendix and colonic tumours. CONCLUSIONS: GRP and GRPR are produced by a large number of gastrointestinal carcinoid tumours. An autocrine/paracrine pathway may exist for GRP stimulated cell proliferation in some of these neoplasms, analogous to that seen in small cell anaplastic carcinoma of the lung.

Appendiceal Neoplasms↗
PubMed1998

Multiple primary visceral malignancy--a review with report of two cases.

Multiple primary cancers are being reported with increasing frequency in recent years, the frequency varying from 0.3 to 4.3% in different studies. A combination of primary cancer of larynx and lung is the most common followed by malignant neoplasm involving lip-larynx, skin-larynx, skin-lung, breast-ovary and breast-endometrium. Two interesting cases of rare combinations of primary cancers are being presented. In the first case primary adenocarcinoma of the gall bladder was associated with appendiceal adenocarcinoma. In the second case primary malignant papillary serous cystadenocarcinoma of right ovary and squamous cell carcinoma of cervix uteri were found.

Appendiceal Neoplasms↗