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Adenocarcinoma mucinoso do apêndice
ORPHA:391723CID-10 · C18.1CID-11 · 2B81.00DOENÇA RARA

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.

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Introdução

O que você precisa saber de cara

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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.

Publicações científicas
104 artigos
Último publicado: 2025 Dec
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SUS: Sem cobertura SUSScore: 0%
CID-10: C18.1
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
7 sintomas
🫁
Pulmão
1 sintomas
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Crescimento
1 sintomas
🩸
Sangue
1 sintomas

+ 2 sintomas em outras categorias

Características mais comuns

Obstrução intestinal
Insuficiência respiratória
Ascite
Constipação
Dor abdominal
Morfologia anormal do peritônio
12sintomas
Sem dados (12)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 12 características clínicas mais associadas, ordenadas por frequência.

Obstrução intestinalIntestinal obstruction
Insuficiência respiratóriaRespiratory insufficiency
AsciteAscites
ConstipaçãoConstipation
Dor abdominalAbdominal pain

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico104PubMed
Últimos 10 anos58publicações
Pico20229 papers
Linha do tempo
2026Hoje · 2026🧪 1993Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

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Diagnóstico

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Tratamento e manejo

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Pipeline de tratamentos
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3Fase 31
2Fase 23
1Fase 11
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 5 ensaios
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Publicações mais relevantes

Timeline de publicações
58 papers (10 anos)
#1

A clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.

Expert review of anticancer therapy2026 Jan

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.

#2

Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.

Journal of surgical case reports2025 Dec

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.

#3

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, India2025

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.

#4

Peritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.

Case reports in oncology2025

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.

#5

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.

International journal of colorectal disease2025 Apr 21

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

Ver todas no PubMed

📚 EuropePMC58 artigos no totalmostrando 57

2025

Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.

Journal of surgical case reports
2026

A clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.

Expert review of anticancer therapy
2025

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

Peritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.

Case reports in oncology
2025

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.

International journal of colorectal disease
2025

Mucinous adenocarcinoma of the appendix invading in the sigmoid colon: Case report.

International journal of surgery case reports
2024

Mucinous adenocarcinoma of the appendix presenting as acute appendicitis: a case report.

Journal of surgical case reports
2024

Mucocoele of the appendix.

Ghana medical journal
2024

Predicting Survival in Mucinous Adenocarcinoma of the Appendix: Demographics, Disease Presentation, and Treatment Methodology.

Annals of surgical oncology
2024

ASO Author Reflections: Shifting Demographics and New Treatment Priorities for Mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2024

ASO Author Reflections: Extent of Resection Not Associated with Disease-Specific Survival in Patients with Localized Mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2023

Cecal-Colon Intussusception due to Appendiceal Mucinous Adenocarcinoma.

The American surgeon
2023

Durable Response after Repeat Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in a Patient with Extensive Mucinous Adenocarcinoma of the Appendix.

Diseases (Basel, Switzerland)
2023

Rare Mucinous Adenocarcinoma of the Appendix Undergoing Multiple Recurrent Surgical Interventions.

Cureus
2022

High-Grade Mucinous Adenocarcinoma of the Appendix With Signet Features and Omental Metastasis.

Cureus
2022

Accidental discovery of appendiceal carcinoma during gynecological surgery: A case report.

World journal of clinical cases
2022

Next generation sequencing targeted detection of somatic mutations in patients with mucinous adenocarcinoma of the appendix.

Annals of diagnostic pathology
2022

Prevention of radiation enteritis by interposition of an intra-abdominal spacer before irradiation of an appendiceal tumour abscess drainage route.

Journal of visceral surgery
2022

Thigh and iliopsoas abscess as a rare presentation of perforated mucinous appendix carcinoma. A case report.

International journal of surgery case reports
2022

Iterative Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2022

Low-Risk Non-mucinous Adenocarcinoma of the Appendix: When Is an Appendectomy Enough?

Annals of surgical oncology
2021

A Rare Case of Psoas Abscess Secondary to Mucinous Adenocarcinoma of the Appendix: A Case Report.

Journal of orthopaedic case reports
2022

ASO Author Reflections: Risk Factors for Lymph Node Metastasis in Non-mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2021

Mucinous Adenocarcinoma of the Appendix: The Challenges of Managing a Complex Surgical Case.

Cureus
2022

Hepatic bridge and round ligament of the liver during cytoreductive surgery: a retrospective cohort.

Langenbeck's archives of surgery
2021

Mucinous adenocarcinoma of the appendix with inguinal node metastases.

Autopsy &amp; case reports
2021

Pseudomyxoma peritonei of appendiceal origin mimicking ovarian cancer - a case report with literature review.

Przeglad menopauzalny = Menopause review
2021

Case 295: Pseudomyxoma from Mucinous Adenocarcinoma of the Appendix Extending from the Retroperitoneum into the Thigh.

Radiology
2021

A Case Report on Life-Threatening Lower Gastrointestinal Bleeding: A Rare Presentation of Mucinous Adenocarcinoma of the Appendix.

Case reports in surgery
2021

Mucinous 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.)
2021

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 Tract
2020

Late stage diagnosis of mucinous adenocarcinoma of the appendix: a case report of an unusual tumor with a rare presentation.

BMC gastroenterology
2020

Mucinous adenocarcinoma of the appendix invading the urinary bladder.

Urology case reports
2019

[A Case of Peritoneal Metastasis of Appendiceal Mucinous Carcinoma Successfully Managed by Multidisciplinary Treatment].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2019

Primary Mucinous Adenocarcinoma of Appendix Invading Urinary Bladder with a Fistula: A Case Report.

Open life sciences
2020

Preoperative Radiographic Assessment Predicts Incomplete Cytoreduction in Patients with Low Grade Mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2019

Bevacizumab Combined With Oxaliplatin/Capecitabine in Patient With Refractory and Recurrent Mucinous Adenocarcinoma of the Appendix: A Case Report.

Frontiers in oncology
2018

Primary Appendicular Malignancy Presenting as Abdominal Wall Abscess with Secondary Tuberculosis Infection: an Interesting Case Report.

Indian journal of surgical oncology
2018

Sister Mary Joseph Nodule as a Cutaneous Manifestation of Metastatic Appendiceal Adenocarcinoma: Case Report and Literature Review.

Cureus
2018

Severe peritoneal sclerosis after repeated pressurized intraperitoneal aerosol chemotherapy with oxaliplatin (PIPAC OX): report of two cases and literature survey.

Clinical &amp; experimental metastasis
2017

[A Case of Appendiceal Mucinous Adenocarcinoma Detected with a Bladder Tumor].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2017

Giant mucinous adenocarcinoma of the appendix: a case report.

Journal of medical case reports
2017

Anesthetic management of Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC): The importance of hydro-electrolytic and acid-basic control.

International journal of surgery case reports
2017

Mucinous Adenocarcinoma of the Appendix: A Case Report.

Mymensingh medical journal : MMJ
2017

Radical appendectomy as an alternative to right colon resection in patients with epithelial appendiceal neoplasms.

Surgical oncology
2017

External Validation of the Simplified Preoperative Assessment for Low-Grade Mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2017

Diarrhea Concealing a Duodenal-Cecal Fistula Secondary to Appendiceal Mucinous Neoplasm.

ACG case reports journal
2016

[A Case of Mucinous Adenocarcinoma of the Appendix with Peritoneal Dissemination Diagnosed by Laparoscopic Abdominal Exploration and Appendectomy].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2016

Limited, 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
2016

[Mucinous adenocarcinoma of the appendix. Report of a case].

Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del Peru
2016

Acute Presentation of a Mucinous Adenocarcinoma of the Appendix.

The American journal of gastroenterology
2016

Pseudomyxoma peritonei with intrathoracic extension: a rare disease with rarer presentation from low-grade mucinous adenocarcinoma of the appendix.

BMJ case reports
2015

Is cholecystectomy and removal of the round ligament of the liver a necessary step in cytoreductive surgery and HIPEC, for peritoneal carcinomatosis?

Annali italiani di chirurgia
2015

Persistent Coughing as the First Symptom of Primary Mucinous Appendiceal Adenocarcinoma.

Journal of clinical medicine research
2015

Mucinous adenocarcinoma of the appendix.

Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences
2015

A Simplified Preoperative Assessment Predicts Complete Cytoreduction and Outcomes in Patients with Low-Grade Mucinous Adenocarcinoma of the Appendix.

Annals of surgical oncology
2015

Cytoreductive surgery and heated intraperitoneal chemotherapy for peritoneal carcinomatosis secondary to mucinous adenocarcinoma of the appendix.

International surgery
Ver todos os 58 no EuropePMC

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Doenças relacionadas

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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.

  1. A clinical visualization model for predicting distant metastasis and prognostic outcomes in mucinous adenocarcinoma of the appendix: a SEER database study.
    Expert review of anticancer therapy· 2026· PMID 41116615mais citado
  2. Giant retroperitoneal mucinous adenocarcinoma of the appendix: a case report.
    Journal of surgical case reports· 2025· PMID 41393058mais citado
  3. 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
  4. Peritoneal Carcinomatosis with Gray-to-Black-Colored Peritoneum and Intestines Treated with Nivolumab: A Case Report.
    Case reports in oncology· 2025· PMID 41063873mais citado
  5. 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.
    International journal of colorectal disease· 2025· PMID 40257575mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:391723(Orphanet)
  2. MONDO:0018330(MONDO)
  3. GARD:21629(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Adenocarcinoma mucinoso do apêndice
Compêndio · Raras BR

Adenocarcinoma mucinoso do apêndice

ORPHA:391723 · MONDO:0018330
CID-10
C18.1 · Neoplasia maligna do apêndice (vermiforme)
CID-11
Início
Adult, Elderly
MedGen
UMLS
C1706832
EuropePMC
Wikidata
Papers 10a
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