Adenocarcinoma of the appendix: an unusual disease

Hananel, N; Powsner, Eldad; Wolloch, Y

doi:10.1080/110241598750005282

Cited by 51 publications

(29 citation statements)

References 6 publications

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“…Appendiceal carcinoma is an uncommon malignancy of the gastrointestinal tract with a prevalence ranging from 0.2 to 0.3% of appendectomy specimens (1,2). Most appendiceal carcinomas arise from an adenomatous polyp or serrated adenoma (3)(4)(5) and present clinically with pseudomyxoma peritonei.…”

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confidence: 99%

Mucinous and Nonmucinous Appendiceal Adenocarcinomas: Different Clinicopathological Features but Similar Genetic Alterations

et al. 2002

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The genetic alterations of appendiceal carcinomas have not been reported in detail. We studied the clinicopathological factors and genetic alterations including microsatellite instability, p53 overexpression, and mutations of the K-ras proto-oncogene of 30 appendiceal adenocarcinomas, consisting of 23 mucinous and 7 nonmucinous carcinomas. Sixteen (70%) mucinous carcinomas presented with pseudomyxoma peritonei, but 6 of 7 (86%) nonmucinous carcinomas presented with appendicitis (P ‫؍‬ .002). All carcinomas were microsatellite stable, and p53 overexpression was present in only 1 of 30 (3%) carcinomas. K-ras mutation was present in 11 of 20 (55%) carcinomas, including 8 of 16 (50%) mucinous and 3 of 4 (75%) nonmucinous carcinomas. The mean survival of patients with mucinous carcinomas was 26 ؎ 19 months compared with 13 ؎ 9 months for patients with nonmucinous carcinomas (P ‫؍‬ .0002). Our findings suggest that mucinous and nonmucinous carcinomas of appendix have similar genetic alterations, but different clinical presentation and prognosis. KEY WORDS: Adenocarcinoma, Appendix, K-ras, Microsatellite instability, p53. Mod Pathol 2002;15(6):599 -605Appendiceal carcinoma is an uncommon malignancy of the gastrointestinal tract with a prevalence ranging from 0.2 to 0.3% of appendectomy specimens (1, 2). Most appendiceal carcinomas arise from an adenomatous polyp or serrated adenoma (3-5) and present clinically with pseudomyxoma peritonei. Most colorectal cancers develop from adenomatous polyps, and morphological and genetic progression in an adenoma-adenocarcinoma sequence and in hereditary colorectal cancer syndromes are well described (6 -9). The majority of colorectal cancers have truncating mutations or deletions of the adenomatous polyposis coli (APC) gene on chromosome 5q or mutations of the ␤-catenin gene. Point mutations of the K-ras protooncogene and mutations and/or deletions of the p53 gene on chromosome 17p are also common. In a second pathway to colorectal neoplasia, microsatellite instability (MSI; also termed DNA replication errors and ubiquitous somatic mutations) is caused by mutations in a nucleotide mismatch repair gene, including hMSH2, hMLH1, PMS1, PMS2, and GTBP (6 -9). MSI is characterized by additions and deletions of nucleotides in numerous repeated nucleotide sequences (microsatellites). MSI is frequent in the right-sided colon carcinomas and mucinous colorectal carcinomas (6 -9).The genetic alterations in appendiceal carcinoma have not been reported in detail. We therefore studied MSI, p53 overexpression, and K-ras mutations in appendiceal carcinomas and compared these genetic alterations with the clinicopathologic findings. MATERIALS AND METHODS Case MaterialA computer search of MD Anderson Cancer Center surgical pathology diagnoses from 1995 through 2000 was performed. Primary appendiceal carcinomas were identified using the World Health Organization classification of appendiceal carcinomas (10). There were 30 patients with primary appendiceal carcinomas and available paraffin-embedded bloc...

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Mucinous and Nonmucinous Appendiceal Adenocarcinomas: Different Clinicopathological Features but Similar Genetic Alterations

et al. 2002

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“…For several reasons, due to obstacle to emptying from the mucus-secreting tumors, the appendix undergoes a progressive accumulation of mucus in the lumen with dilation until it can tear. Appendicular mucocele may also be associated with other cancers, such as cystadenoma and cystadenocarcinoma, colon and ovarian mucin-secreting cells cancers (2,(5)(6)(7).…”

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confidence: 99%

“…In 50-65% of cases symptoms and signs of acute inflammation of the appendix, in 10-20% of cases intestinal obstruction or palpable mass in the right lower abdominal quadrant are present (1)(2)(3)9). Melena (8), intussusception (13,14), infiltration of the bladder (15), vaginal penetration with colporragia (16), and pionephrosis by compression of the right ureter (17) are rarer occurences.…”

Section: Discussionmentioning

confidence: 99%

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Appendicular mucocele: two case reports and literature review

Anania¹,

Giaccari²,

G³

et al. 2015

Gchir

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L.R., a 45-years-old woman, Caucasian race. Past medical history: tonsillectomy in childhood; Laparoscopic cholecystectomy in 1993; in 2004 ATC-HCV positivity (transaminases always remained normal and HCV-RNA quantitative research always negative). Menarche at 12 years; regular menstrual cycles; abortion 18 years; abortion 36 years for fetal genetic disease; then subsequent pregnancy terminated in the first quarter; 3 attempts at artificial insemination with ovarian stimulation, without success.She comes to our attention in October 2012 for relapsing pelvic effusion in the absence of other symptoms of note. The general physical examination does not reveal any pathologies of organs or systems. The periodic ultrasound scans performed in the fall of 2011 to March 2012 showed hypoechoic fluid in the pelvis (50x25 mm) posterior to the uterus, and attached to the uterus towards the space with the bladder; hypoechoic area in the content of superimposed layers of different densities compatible with psudomyxoma peritonei or pseudocyst adhesions; hypoechoic collection in Douglas 6 cm in content more dense with small marginal hyperechoic area. Blood tests and tumor markers (CA 19.9, CA 125) were still in the normal range.In October 2014 the patient underwent diagnostic laparoscopy. Explorating the abdominal cavity we found an important effusion of gelatinous material, which also involved other abdominal organs, and an appendicular neoplasm, which was removed. We also appreciated peritoneal soft-consistency nodes; some of them were removed and sent for histology.Cytology on gelatinous intraperitoneal substance showed fibrous tissue containing mucoid material and inflammatory cells; histology of peritoneal nodes showed hyperplastic mesothelium, with chronic inflammatory infiltrate and focal deposits of mucoid material. Definitive histology showed appendicular mucinous neoplasm of low grade, extensive tissue and serous.Due to the results of the histological results, the patient was sent to a specialized center for the treatment of the peritoneal cavity diseases.During the follow up, the patient underwent CT abdomen (December 2014), that revealed the presence of small flap of abdominal effusion disposed around the li-SUMMARY: Appendicular mucocele: two case reports and literature review.

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“…It accounts for 0.2–0.3% of appendectomy specimens [7]. Acute appendicitis from luminal obstruction is the most common manifestation for most tumour types.…”

Section: Discussionmentioning

confidence: 99%

Primary Carcinoma of the Appendix – Hull Series

Iwuagwu

Jameel²,

Drew³

et al. 2005

Dig Surg

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Background: Appendiceal carcinoma (AC) is a rare entity that does not have a well-defined treatment strategy. At presentation, most patients are clinically thought to have appendicitis and the diagnosis is made only by formal histology. Once the diagnosis of AC is made, patients are treated by various strategies including surgery, chemotherapy depending on nodal status of the disease. Aim: To review the Hull hospitals’ experience with AC. Methods: Between 1982 and 2002, 10 patients with primary AC were seen. The histopathology reports of all appendiceal specimens removed were traced. Follow-up was by chart review or patient follow-up as appropriate. We did not include patients with primary carcinoid tumours or secondary adenocarcinoma. Results: There was an equal sex distribution. All patients underwent surgery, 3 had post-operative chemotherapy. Complete follow-up information was available with a median follow-up time of 56 months, with a range of 12–168 months. Five patients survived at least 4 years from the time of diagnosis. Conclusion: Long-term survival in patients with AC is possible.

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Adenocarcinoma of the appendix: an unusual disease

Abstract: Right hemicolectomy is the treatment of choice for adenocarcinoma of the appendix.

Cited by 51 publications

References 6 publications

Mucinous and Nonmucinous Appendiceal Adenocarcinomas: Different Clinicopathological Features but Similar Genetic Alterations

Mucinous and Nonmucinous Appendiceal Adenocarcinomas: Different Clinicopathological Features but Similar Genetic Alterations

Appendicular mucocele: two case reports and literature review

Primary Carcinoma of the Appendix – Hull Series

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