O adenocarcinoma mucinoso do apêndice é um tipo de câncer muito raro e de crescimento lento, que surge nas células que revestem o apêndice. É considerado "bem diferenciado", o que geralmente significa que é menos agressivo, e se caracteriza pela produção abundante de muco. Na prática, ele pode se manifestar como uma apendicite aguda (com dor na barriga, febre e aumento das células de defesa – glóbulos brancos – no sangue) ou como pseudomixoma peritoneal, que é quando o muco se espalha pela cavidade abdominal (barriga). No entanto, alguns pacientes podem não sentir absolutamente nada no momento do diagnóstico. Em muitos casos, um segundo câncer no sistema digestivo também está presente.
Introdução
O que você precisa saber de cara
O adenocarcinoma mucinoso do apêndice é um tipo de câncer muito raro e de crescimento lento, que surge nas células que revestem o apêndice. É considerado "bem diferenciado", o que geralmente significa que é menos agressivo, e se caracteriza pela produção abundante de muco. Na prática, ele pode se manifestar como uma apendicite aguda (com dor na barriga, febre e aumento das células de defesa – glóbulos brancos – no sangue) ou como pseudomixoma peritoneal, que é quando o muco se espalha pela cavidade abdominal (barriga). No entanto, alguns pacientes podem não sentir absolutamente nada no momento do diagnóstico. Em muitos casos, um segundo câncer no sistema digestivo também está presente.
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 2 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 12 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Adenocarcinoma mucinoso do apêndice
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
0 ensaios clínicos encontrados.
Publicações mais relevantes
A clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.
Mucinous adenocarcinoma of the appendix (MACA) has high metastatic potential and poor prognosis, lacking predictive models. This study aimed to establish models for assessing distant metastasis (DM) risk and survival prognosis of MACA patients with DM. SEER database data of MACA patients diagnosed from 2010 to 2021 were collected. Univariate and multivariate logistic regression identified DM risk factors, while Cox regression determined prognostic factors for DM patients. Two nomograms were created and evaluated via ROC curves, calibration curves, and DCA. Results: A total of 1722 patients with MACA were included, and 832 patients had DM at the time of diagnosis. The independent risk factors for DM in patients with MACA include gender, age, tumor size, T stage, and N stage. For MACA patients with DM, age, N stage, and surgical treatment were independent risk factors for overall survival (OS). The diagnostic and prognostic nomograms showed good discriminatory ability and calibration. The independent prognostic factors for MACA patients with DM are age, N stage, and surgery. The established diagnostic and prognostic models can quantitatively assess the risk and prognosis of MACA with DM, providing more effective guidance for clinical decision-making.
Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.
Giant retroperitoneal mucinous adenocarcinoma of the appendix is a rare, aggressive malignancy characterized by mucin production and significant mass formation, often complicating early diagnosis due to nonspecific symptoms such as abdominal discomfort. Surgical resection, ranging from appendectomy to more extensive procedures like right hemicolectomy, is the primary treatment approach. This case presents a 53-year-old male with a 38 cm retroperitoneal mucinous adenocarcinoma of the appendix. The patient presented with right-sided flank pain, and subsequent imaging revealed a rapidly enlarging mass. Surgical resection was performed successfully via en-bloc resection with a right hemicolectomy. Postoperative care involved monitoring for complications, with the patient recovering uneventfully. This case highlights the challenges in diagnosing and managing large mucinous neoplasms and the importance of a multidisciplinary approach in ensuring optimal patient outcomes.
Metastatic Spread to Breast in a Case of Mucinous Adenocarcinoma Appendix Detected on 18F-FDG PET-CT scan.
Appendiceal adenocarcinomas are rare invasive gastrointestinal neoplasms, with higher propensity to metastasize to lymph nodes and peritoneum, whereas other common sites include liver and lung. Metastases to the breast are exceptionally rare in appendiceal neoplasms, with limited data in literature. We present the case of a recurrent mucinous adenocarcinoma of the appendix with clinically occult breast, skeletal, and nonregional nodal metastases detected on fluorine-18-fluorodeoxyglucose positron emission tomography-computed tomography scan.
Peritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.
Peritoneal carcinomatosis of unknown primary is a life-threatening condition that presents diagnostic and therapeutic challenges with poor prognosis. In 2021, nivolumab was approved for carcinoma of unknown primary in Japan. A 70-year-old male underwent first-line nivolumab treatment for peritoneal carcinomatosis of unknown primary, in accordance with the patient's wishes. Four months after initiating treatment, he developed immune-related adverse event (irAE) hepatitis, which responded to methylprednisolone; however, the patient died a few days later. Autopsy revealed an uncommon presentation of thick, firm, gray peritoneum with globally gray-to-black ischemic intestines, massive adhesions, ascites, and disseminated peritoneal nodules. No evidence of superior mesenteric artery occlusion, segmental necrosis, or typical features of ischemic colitis or nonocclusive mesenteric ischemia was evident. Histological findings revealed fibrous thickening of the peritoneum. Pathological examination collectively suggested that the primary site of the peritoneal carcinomatosis was the non-mucinous adenocarcinoma, not otherwise specified, and mucinous adenocarcinoma of the appendix, which had not been detected on imaging or endoscopy. The final staging was pT4b, pN1a, pM1c, pStage IVC based on the TNM Classification (8th edition). Although irAE colitis is well documented, peritoneal complications associated with immune checkpoint blockades have been scarcely reported in the literature, especially gray-colored peritoneum as in this case. The etiology is still unclear, but might collectively involve inflammation, stromal fibrosis, microscopic tumor invasion, and metabolic dysregulation in the tissue/tumor microenvironment. The unusual peritoneal presentation warrants further case accumulation and investigation into its pathogenesis to improve clinical management strategies.
Influence of the effectiveness of sarcopenia on postoperative major complications after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in patients with peritoneal surface malignancy: a retrospective cohort study.
Sarcopenia has recently been gaining importance due to its role on mortality and mobility in diseases and operations. In this study, we aimed to evaluate the effect of sarcopenia on major postoperative complications in patients undergoing cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for peritoneal surface malignancy. In this longitudinal cohort study included 202 patients treated between January 2018 and September 2023, with 52 undergoing prophylactic procedures. Peritoneal metastases originated from colorectal, gastric, and ovarian cancer; peritoneal mesothelioma; mucinous adenocarcinoma of the appendix; and endometrial cancer. Age, sex, body mass index (BMI), length of hospital stay (LOS), peritoneal cancer index (PCI), competency of cytoreduction (CC), operation time, and primary peritoneal carcinomatosis were recorded. All variables were analyzed according to the presence of major complications and sarcopenia. Significant associations were found between major complications and sarcopenia (p = 0.002), PCI (p = 0.036), operation time (p = 0.015), and LOS (p < 0.001). In sarcopenic patients, significant associations were found with sex (p = 0.035), age (p = 0.025), and BMI (p = 0.001). Multivariate Cox regression analysis identified sarcopenia as an independent risk factor for major complications, tripling the likelihood (p = 0.005). Additionally, PCI score (p = 0.008) and LOS (p < 0.001) were independent risk factors. This study underscores sarcopenia as an independent risk factor for major complications in CRS/HIPEC patients, with PCI and LOS as additional risk factors. In sarcopenic patients, pre-operative evaluation should be done carefully and post-operative risks should be kept in mind.
Publicações recentes
Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.
A clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.
Metastatic Spread to Breast in a Case of Mucinous Adenocarcinoma Appendix Detected on (18)F-FDG PET-CT scan.
Peritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.
Influence of the effectiveness of sarcopenia on postoperative major complications after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in patients with peritoneal surface malignancy: a retrospective cohort study.
📚 EuropePMC58 artigos no totalmostrando 57
Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.
Journal of surgical case reportsA clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.
Expert review of anticancer therapyMetastatic Spread to Breast in a Case of Mucinous Adenocarcinoma Appendix Detected on 18F-FDG PET-CT scan.
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaPeritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.
Case reports in oncologyInfluence of the effectiveness of sarcopenia on postoperative major complications after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in patients with peritoneal surface malignancy: a retrospective cohort study.
International journal of colorectal diseaseMucinous adenocarcinoma of the appendix invading in the sigmoid colon: Case report.
International journal of surgery case reportsMucinous adenocarcinoma of the appendix presenting as acute appendicitis: a case report.
Journal of surgical case reportsMucocoele of the appendix.
Ghana medical journalPredicting Survival in Mucinous Adenocarcinoma of the Appendix: Demographics, Disease Presentation, and Treatment Methodology.
Annals of surgical oncologyASO Author Reflections: Shifting Demographics and New Treatment Priorities for Mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyASO Author Reflections: Extent of Resection Not Associated with Disease-Specific Survival in Patients with Localized Mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyCecal-Colon Intussusception due to Appendiceal Mucinous Adenocarcinoma.
The American surgeonDurable Response after Repeat Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in a Patient with Extensive Mucinous Adenocarcinoma of the Appendix.
Diseases (Basel, Switzerland)Rare Mucinous Adenocarcinoma of the Appendix Undergoing Multiple Recurrent Surgical Interventions.
CureusHigh-Grade Mucinous Adenocarcinoma of the Appendix With Signet Features and Omental Metastasis.
CureusAccidental discovery of appendiceal carcinoma during gynecological surgery: A case report.
World journal of clinical casesNext generation sequencing targeted detection of somatic mutations in patients with mucinous adenocarcinoma of the appendix.
Annals of diagnostic pathologyPrevention of radiation enteritis by interposition of an intra-abdominal spacer before irradiation of an appendiceal tumour abscess drainage route.
Journal of visceral surgeryThigh and iliopsoas abscess as a rare presentation of perforated mucinous appendix carcinoma. A case report.
International journal of surgery case reportsIterative Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyLow-Risk Non-mucinous Adenocarcinoma of the Appendix: When Is an Appendectomy Enough?
Annals of surgical oncologyA Rare Case of Psoas Abscess Secondary to Mucinous Adenocarcinoma of the Appendix: A Case Report.
Journal of orthopaedic case reportsASO Author Reflections: Risk Factors for Lymph Node Metastasis in Non-mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyMucinous Adenocarcinoma of the Appendix: The Challenges of Managing a Complex Surgical Case.
CureusHepatic bridge and round ligament of the liver during cytoreductive surgery: a retrospective cohort.
Langenbeck's archives of surgeryMucinous adenocarcinoma of the appendix with inguinal node metastases.
Autopsy & case reportsPseudomyxoma peritonei of appendiceal origin mimicking ovarian cancer - a case report with literature review.
Przeglad menopauzalny = Menopause reviewCase 295: Pseudomyxoma from Mucinous Adenocarcinoma of the Appendix Extending from the Retroperitoneum into the Thigh.
RadiologyA Case Report on Life-Threatening Lower Gastrointestinal Bleeding: A Rare Presentation of Mucinous Adenocarcinoma of the Appendix.
Case reports in surgeryMucinous Adenocarcinoma of the Appendix With Histologic Response to Neoadjuvant Chemotherapy: Review of Histologic and Clinical Spectrum of Epithelial Neoplastic Mucinous Lesions of the Appendix.
Oncology (Williston Park, N.Y.)The Utility of Preoperative Tumor Markers in Peritoneal Carcinomatosis from Primary Appendiceal Adenocarcinoma: an Analysis from the US HIPEC Collaborative.
Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary TractLate stage diagnosis of mucinous adenocarcinoma of the appendix: a case report of an unusual tumor with a rare presentation.
BMC gastroenterologyMucinous adenocarcinoma of the appendix invading the urinary bladder.
Urology case reports[A Case of Peritoneal Metastasis of Appendiceal Mucinous Carcinoma Successfully Managed by Multidisciplinary Treatment].
Gan to kagaku ryoho. Cancer & chemotherapyPrimary Mucinous Adenocarcinoma of Appendix Invading Urinary Bladder with a Fistula: A Case Report.
Open life sciencesPreoperative Radiographic Assessment Predicts Incomplete Cytoreduction in Patients with Low Grade Mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyBevacizumab Combined With Oxaliplatin/Capecitabine in Patient With Refractory and Recurrent Mucinous Adenocarcinoma of the Appendix: A Case Report.
Frontiers in oncologyPrimary Appendicular Malignancy Presenting as Abdominal Wall Abscess with Secondary Tuberculosis Infection: an Interesting Case Report.
Indian journal of surgical oncologySister Mary Joseph Nodule as a Cutaneous Manifestation of Metastatic Appendiceal Adenocarcinoma: Case Report and Literature Review.
CureusSevere peritoneal sclerosis after repeated pressurized intraperitoneal aerosol chemotherapy with oxaliplatin (PIPAC OX): report of two cases and literature survey.
Clinical & experimental metastasis[A Case of Appendiceal Mucinous Adenocarcinoma Detected with a Bladder Tumor].
Gan to kagaku ryoho. Cancer & chemotherapyGiant mucinous adenocarcinoma of the appendix: a case report.
Journal of medical case reportsAnesthetic management of Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC): The importance of hydro-electrolytic and acid-basic control.
International journal of surgery case reportsMucinous Adenocarcinoma of the Appendix: A Case Report.
Mymensingh medical journal : MMJRadical appendectomy as an alternative to right colon resection in patients with epithelial appendiceal neoplasms.
Surgical oncologyExternal Validation of the Simplified Preoperative Assessment for Low-Grade Mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyDiarrhea Concealing a Duodenal-Cecal Fistula Secondary to Appendiceal Mucinous Neoplasm.
ACG case reports journal[A Case of Mucinous Adenocarcinoma of the Appendix with Peritoneal Dissemination Diagnosed by Laparoscopic Abdominal Exploration and Appendectomy].
Gan to kagaku ryoho. Cancer & chemotherapyLimited, local, extracolonic spread of mucinous appendiceal adenocarcinoma after perforation with formation of a malignant appendix-to-sigmoid fistula: Case report and literature review.
World journal of gastroenterology[Mucinous adenocarcinoma of the appendix. Report of a case].
Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del PeruAcute Presentation of a Mucinous Adenocarcinoma of the Appendix.
The American journal of gastroenterologyPseudomyxoma peritonei with intrathoracic extension: a rare disease with rarer presentation from low-grade mucinous adenocarcinoma of the appendix.
BMJ case reportsIs cholecystectomy and removal of the round ligament of the liver a necessary step in cytoreductive surgery and HIPEC, for peritoneal carcinomatosis?
Annali italiani di chirurgiaPersistent Coughing as the First Symptom of Primary Mucinous Appendiceal Adenocarcinoma.
Journal of clinical medicine researchMucinous adenocarcinoma of the appendix.
Journal of research in medical sciences : the official journal of Isfahan University of Medical SciencesA Simplified Preoperative Assessment Predicts Complete Cytoreduction and Outcomes in Patients with Low-Grade Mucinous Adenocarcinoma of the Appendix.
Annals of surgical oncologyCytoreductive surgery and heated intraperitoneal chemotherapy for peritoneal carcinomatosis secondary to mucinous adenocarcinoma of the appendix.
International surgeryAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Adenocarcinoma mucinoso do apêndice.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Adenocarcinoma mucinoso do apêndice
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- A clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.
- Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.
- Metastatic Spread to Breast in a Case of Mucinous Adenocarcinoma Appendix Detected on 18F-FDG PET-CT scan.Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India· 2025· PMID 41064223mais citado
- Peritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.
- Influence of the effectiveness of sarcopenia on postoperative major complications after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in patients with peritoneal surface malignancy: a retrospective cohort study.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:391723(Orphanet)
- MONDO:0018330(MONDO)
- GARD:21629(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787959(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
