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      Mucocele of the appendix: appendectomy or colectomy?

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          Abstract

          Mucocele of the appendix is a rare disease. It can be triggered by benign or malignant diseases, which cause the obstruction of the appendix and the consequent accumulation of mucus secretion. The preoperative diagnosis is difficult due to non-specific clinical manifestations of the disease. Imaging tests can suggest the diagnosis. The treatment is always surgical and depends on the integrity and size of the appendix base and on the histological type of the original lesion. The prognosis is good in cases of integrity of the appendix. The perforation of the appendix and subsequent extravasation of its contents into the abdominal cavity may lead to pseudomyxoma peritonei, which has very poor prognosis if not treated properly.

          Translated abstract

          A mucocele do apêndice cecal é uma doença rara. Pode ser causada por doenças benignas ou malignas que provocam a obstrução da luz do apêndice e, consequente, acúmulo de secreção mucoide. O diagnóstico pré-operatório é difícil devido ao quadro clínico inespecífico da doença. Exames de imagem sugerem o diagnóstico. O tratamento é sempre cirúrgico e é determinado pela integridade do órgão, dimensões da base e tipo histológico da lesão. O prognóstico é bom nos casos com integridade do apêndice cecal. A perfuração do apêndice e o consequente extravasamento do seu conteúdo para a cavidade abdominal pode ocasionar o pseudomixoma peritoneal, cujo prognóstico é bastante desfavorável se não tratado adequadamente.

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          Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases.

          The classification of appendiceal mucinous tumors is controversial and terminology used for them inconsistent, particularly when they lack overtly malignant features but are associated with extra-appendiceal spread. We reviewed 107 appendiceal mucinous neoplasms and classified them as low-grade appendiceal mucinous neoplasm (LAMN) (n = 88), mucinous adenocarcinomas (MACAs) (n = 16), or discordant (n = 3) based on architectural and cytologic features. LAMNs were characterized by a villous or flat proliferation of mucinous epithelium with low-grade atypia. Thirty-nine tumors were confined to the appendix, but 49 had extra-appendiceal tumor spread, including 39 with peritoneal tumor characterized by mucin pools harboring low-grade mucinous epithelium, usually dissecting in a hyalinized stroma. Eight of the 16 MACAs lacked destructive invasion of the appendiceal wall and eight showed an infiltrative pattern of invasion. Extra-appendiceal tumor spread was present in 12 MACAs (four peritoneum, seven peritoneum and ovaries; one ovaries only). In MACAs with an infiltrative pattern, peritoneal tumor consisted of glands and single cells in a desmoplastic stroma. The peritoneal tumor in the remaining cases consisted of mucin pools that contained mucinous epithelium with high-grade atypia and, in some cases, increased cellularity compared with that seen in peritoneal spread in cases of LAMN. Three cases were classified as discordant because the appendiceal tumors were LAMNs but the peritoneal tumors were high-grade. Follow-up was available for 49 LAMNs, 15 MACAs, and 2 discordant cases. None of the patients with LAMNs confined to the appendix experienced recurrence (median follow-up 6 years). LAMNs with extra-appendiceal spread were associated with 3-, 5-, and 10-year survival rates of 100%, 86%, and 45%, respectively. Patients with MACA had 3- and 5-year survival rates of 90% and 44%, respectively (p = 0.04). The bulk of peritoneal disease correlated with prognosis among patients with MACA (p = 0.04) and, to a lesser degree, among patients with LAMNs (p = 0.07). We conclude that: 1) appendiceal mucinous neoplasms can be classified as either low-grade mucinous neoplasms or mucinous adenocarcinoma based on architectural and cytologic features; 2) tumors that can be confidently placed in the low-grade group (which requires rigorous pathologic evaluation of the appendix) and are confined to the appendix are clinically benign in our experience to date; 3) low-grade tumors confined to the appendix are morphologically identical to those with extra-appendiceal spread (except for the usual identification of breach of the wall in the latter cases) and the same designation is appropriate for the appendiceal neoplasia in each situation; 4) the long-term outlook for patients with low-grade tumors and peritoneal spread is guarded with just over half dying of disease after 10 years; 5) appendiceal mucinous tumors with destructive invasion of the appendiceal wall, complex epithelial proliferations, or high-grade nuclear atypia generally pursue an aggressive clinical course and should be classified as mucinous adenocarcinomas; 6) peritoneal tumor can be classified as involvement by LAMN or MACA, and this distinction is of prognostic significance; 7) bulky peritoneal tumor worsens prognosis; and 8) LAMNs associated with high-grade peritoneal tumor behave as adenocarcinoma.
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            Mucinous and nonmucinous appendiceal adenocarcinomas: different clinicopathological features but similar genetic alterations.

            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.
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              The volcano sign of appendiceal mucocele

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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Journal
                jcol
                Journal of Coloproctology (Rio de Janeiro)
                J. Coloproctol. (Rio J.)
                Sociedade Brasileira de Coloproctologia (Rio de Janeiro )
                2317-6423
                September 2011
                : 31
                : 3
                : 276-284
                Affiliations
                [1 ] Secretaria de Estado da Saúde do Distrito Federal Brazil
                Article
                S2237-93632011000300008
                10.1590/S2237-93632011000300008
                1b98f4f7-2487-4031-9821-fe7f22f4bad0

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=2237-9363&lng=en
                Categories
                GASTROENTEROLOGY & HEPATOLOGY
                SURGERY

                Gastroenterology & Hepatology,Surgery
                mucocele,appendix,pseudomyxoma peritonei,treatment,apêndice cecal,pseudomixoma peritoneal,tratamento

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