É um tumor onde as células são bem parecidas com as normais. Ele não é considerado muito agressivo ou tem uma agressividade moderada. Suas células têm características de células nervosas e hormonais, e o tumor surge no canal anal.
Introdução
O que você precisa saber de cara
É um tumor onde as células são bem parecidas com as normais. Ele não é considerado muito agressivo ou tem uma agressividade moderada. Suas células têm características de células nervosas e hormonais, e o tumor surge no canal anal.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
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Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 25 características clínicas mais associadas, ordenadas por frequência.
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🇧🇷 Atendimento SUS — Tumor endócrino anal
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Publicações mais relevantes
Changes in the 6th edition of the World Health Organization classification of tumours of the digestive system.
The 6th Edition of the WHO Classification of Digestive System Tumours represents a significant update to the 5th edition. It integrates pathological, new molecular, and clinical insights to refine the taxonomy of digestive system neoplasms. The revised classification continues to emphasise standardisation in terminology, coding, and diagnostic criteria to facilitate global consistency in diagnosis, treatment, epidemiological reporting and research. Structural reorganisation of book chapters describes epithelial tumours by anatomical site, while separating neuroendocrine, mesenchymal and haematolymphoid tumours into dedicated chapters that are aligned with other WHO tumour volumes. Genetic tumour syndromes are classified by mechanisms, pathways and genes, whereas metastatic disease is comprehensively covered under other tumours and metastases. Key structural and diagnostic refinements include consolidation of gastric dysplasia entities; separation of duodenal/ampullary from jejuno-ileal tumours; clearer categorisation of colorectal serrated polyps and novel carcinoma grading; introduction of small- and large-duct intrahepatic cholangiocarcinoma as separate entities, and redefinition of undifferentiated carcinoma to include 'carcinoma with mesenchymal differentiation'. Several new entities are introduced, including oesophageal epidermoid metaplasia, colorectal intramucosal adenocarcinoma, low-grade tubuloglandular adenocarcinoma and lymphoglandular complex-like adenocarcinoma, intraductal tubulopapillary and intraductal oncocytic papillary neoplasms of the bile ducts and sonic hedgehog hepatocellular adenoma. The concept of amphicrine-like carcinoma (ALC) is distinguished from MiNEN and broadens the understanding of tumours with dual neuroendocrine-non-neuroendocrine differentiation. Grading systems are simplified to two-tier classifications (low/high grade) across precursor lesions, with enhanced criteria for neuroendocrine tumour grading. Anal canal neoplasia terminology is harmonised with human papillomavirus (HPV) related Lower Anogenital Squamous Terminology (LAST) and mass-forming biliary and gallbladder cancer precursors share similar terminology. Finally, carcinoma of unknown primary (CUP) is included in a separate section for the first time, classified by molecular and immunophenotypic profiles to guide therapy. Overall, the 6th edition strengthens tumour diagnostic precision and molecular alignment across the digestive system.
A case of anastomotic recurrence 12 years after intersphincteric resection for anorectal malignant melanoma.
Anorectal malignant melanoma (AMM) is a rare disease with a poor prognosis, accounting for < 1.0% of all malignant melanomas and a 5-year survival rate of 19.2%. The treatment is mainly surgical, and lymph-node dissection is often performed. Cases of recurrence after a prolonged period (> 10 years), as in the present case, are rare. The patient was an 80-year-old woman who underwent laparoscopic intersphincteric resection with bilateral lateral lymph-node dissection for the diagnosis of primary AMM of the lower rectum at X - 12 years. The pathology was pStage III and the resection margins were negative. Twelve years after the initial surgery, in year X, the patient visited our hospital with the chief complaint of discomfort due to a tumor in the anorectal region. Biopsy revealed a recurrence, and laparoscopic abdominoperineal resection was performed. Based on the pathological findings, the patient was diagnosed with local recurrence of the anastomotic anal canal. Both the first and second specimens were negative for BRAF V600E/K mutation. Four months have passed since the surgery, and the patient continued to receive nivolumab without recurrence. Long-term local follow-up is necessary when the anal canal is preserved during AMM surgery.
HER2 aberration is a potential predictive biomarker for extrapulmonary small cell neuroendocrine carcinoma contrary to small cell lung cancer.
In this study, we investigated human epidermal growth factor receptor 2 (HER2) aberrations, including gene amplification and mutation, and protein expression in 123 small cell lung carcinomas (SCLC) and 128 extrapulmonary small cell neuroendocrine carcinomas (EP-SCNC) samples. Among the EP-SCNC cohort, HER2 mutations were found in 5.5% of samples (7/128); urinary bladder (4 cases), and one case each in samples from the colon, anal canal, and uterine cervix. In SCLCs, HER2 mutations were rare, detected in only 0.8% (1/119) of cases. We also identified eight EP-SCNCs and five SCLC cases with HER2 gene variants of uncertain significance (VUS). HER2 gene amplification was detected in 2.3% (3/128) of EP-SCNCs, but no amplification was found in SCLCs. The differences in HER2 mRNA expression were not statistically significant among tumor groups in the EP-SCNCs and SCLCs cohorts. RNA-seq analysis revealed high HER2 mRNA expression in seven EP-SCNCs and four SCLCs. An immunohistochemistry (IHC) analysis of 10 available tumors with high mRNA expression revealed HER2 protein positivity in 8 cases. The prognostic value of HER2 overexpression in EP-SCNC patients was not established in our study. Furthermore, EP-SCNC patients with high HER2 mRNA expression were generally younger, with a mean age of 60 years. These findings highlight the potential of HER2 as a therapeutic target in EP-SCNC, warranting further investigation into its clinical implications.
Primary Neuroendocrine Carcinoma of the Anal Canal with Cancer Genome Profiling.
Primary neuroendocrine carcinoma (NEC) of the anal canal is a rare, highly malignant tumor with a poor prognosis. Despite the standard first-line treatment with etoposide or irinotecan combined with cisplatin, effective second-line therapies are lacking. In 2019, Japan approved cancer genome profiling (CGP) tests for solid tumors to enhance genomic understanding. We present the case of a 79-year-old woman with NEC of the anal canal, treated with etoposide, carboplatin, and amrubicin. As Post-standard therapy, CGP suggested pemigatinib, a tyrosine kinase inhibitor; however, the patient died before receiving it. This case highlights the potential of personalized medicine to improve outcomes in such cases.
Amelanotic Malignant Melanoma With Atypical Divergent Neuroendocrine Differentiation: A Report of an Unusual and Rare Case of Anorectal Bleeding.
Melanocytes located between the anal transition zone and the dentate line of the anal canal can give rise to the uncommon malignant tumor known as anal melanoma. It has a fast-paced clinical course and can masquerade as several common anorectal symptoms, such as hemorrhoids or rectal ulcers. In melanoma, divergent differentiation is a very uncommon phenomenon. The diagnosis of melanoma is difficult with histopathology sections alone (hematoxylin and eosin, H&E). Special stains and ancillary immunohistochemistry investigations are useful in these situations. A 60-year-old female patient presented to the surgical outpatient department with complaints of anorectal bleeding. After clinical evaluation, a growth in the anorectal region was identified, and a biopsy was taken from the growth. Histopathological and subsequent immunohistochemical analysis of the biopsy material was done at the Department of Pathology. A diagnosis of amelanotic melanoma with atypical and divergent neuroendocrine differentiation involving the anorectal region was rendered. Histologically, this tumor showed extremely pleomorphic polygonal to elongated spindle cells that co-expressed neuroendocrine markers and were positive for S100, HMB-45, and Melan-A. This case presented many diagnostic challenges at both the histomorphological level and the immunohistochemical expression profile analysis. We will go into great depth regarding the diagnostic challenges in this instance and provide an outline of our approach. The immunohistochemical and prognostic importance of this case will also be covered.
Publicações recentes
A case of anastomotic recurrence 12 years after intersphincteric resection for anorectal malignant melanoma.
Robotic TAMIS rectal neuroendocrine tumor excision.
Treatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation.
Perianal schwannoma: An unusual location.
Transanal minimally invasive surgery for benign and malignant rectal lesions: midterm outcomes from a tertiary center.
📚 EuropePMCmostrando 100
Changes in the 6th edition of the World Health Organization classification of tumours of the digestive system.
HistopathologyHER2 aberration is a potential predictive biomarker for extrapulmonary small cell neuroendocrine carcinoma contrary to small cell lung cancer.
Virchows Archiv : an international journal of pathologyA case of anastomotic recurrence 12 years after intersphincteric resection for anorectal malignant melanoma.
Clinical journal of gastroenterologyPrimary Neuroendocrine Carcinoma of the Anal Canal with Cancer Genome Profiling.
Internal medicine (Tokyo, Japan)Amelanotic Malignant Melanoma With Atypical Divergent Neuroendocrine Differentiation: A Report of an Unusual and Rare Case of Anorectal Bleeding.
CureusRobotic TAMIS rectal neuroendocrine tumor excision.
Techniques in coloproctologyTreatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation.
Journal of cancer research and clinical oncologyUNVEILING THE REALITIES OF ANAL MELANOMA CARE IN A RESOURCE-CONSTRAINED SETTING.
Journal of Ayub Medical College, Abbottabad : JAMCPerianal schwannoma: An unusual location.
Indian journal of pathology & microbiologySimultaneous laparoscopic liver metastasectomy and intersphincteric resection for neuroendocrine tumor of the rectum by natural orifice specimen extraction surgery.
Journal of minimally invasive surgeryTransanal minimally invasive surgery for benign and malignant rectal lesions: midterm outcomes from a tertiary center.
Annals of Saudi medicineRectal malignant peripheral nerve sheath tumor found after anal canal neurofibroma surgery: A rare case report.
Asian journal of surgeryClinical Updates for Gastrointestinal Malignancies.
Journal of personalized medicineTAMIS Procedure for Rectal Neuroendocrine Tumor: Step by Step.
Diseases of the colon and rectumGoblet cell adenocarcinoma of the anal canal with perianal Paget disease: A rare case report with literature review.
MedicineIs lymphatic invasion of microrectal neuroendocrine tumors an incidental event?: A case report.
World journal of clinical casesOptimal surgical management strategy for treatment of primary anorectal malignant melanoma-a systematic review and meta-analysis.
Langenbeck's archives of surgeryClinical Analysis of Extrapulmonary Neuroendocrine Carcinoma: A Retrospective and Single Institution Experience.
ChemotherapyMerkel cell carcinoma of the anorectum: a case report and review of the literature.
Clinical journal of gastroenterologyOutcomes After Sphincter-Sparing Local Therapy for Anorectal Melanoma: 1989 to 2020.
Practical radiation oncology[A Case of Rectal Neuroendocrine Tumor with a Major Axis of 10.7 mm with Lymph Node Metastasis].
Gan to kagaku ryoho. Cancer & chemotherapy18F-FDG PET/CT of Malignant Presacral Masses in Currarino Syndrome.
Clinical nuclear medicineImplementation of transanal minimally invasive surgery (TAMIS) for rectal neoplasms: results from a single centre.
Techniques in coloproctologyImplementation of robot-assisted curative resection for rare anorectal tumours on the basis of individualised treatment.
The international journal of medical robotics + computer assisted surgery : MRCASAnorectal Mucosal Melanoma: A Case Report and Literature Review.
The American journal of case reportsAnal canal mixed adenoneuroendocrine carcinoma in a young patient misdiagnosed as anal abscess.
BMJ case reportsAnorectal Melanoma-Brownish Black Mass Not Always a Hemorrhoid.
Journal of gastrointestinal cancerSmall cell neuroendocrine carcinoma disguised as an external hemorrhoid.
JAAPA : official journal of the American Academy of Physician AssistantsHigh-grade neuroendocrine small-cell carcinoma of the anal canal: Long-term remission with chemoradiotherapy.
Journal of clinical and translational researchPresacral neuroendocrine tumour in a patient diagnosed with Currarino's syndrome.
Gastroenterologia y hepatologiaCD56 expression in basaloid anal squamous cell carcinoma - A potential diagnostic pitfall.
Annals of diagnostic pathologyMetastatic anorectal malignant melanoma causing ileocaecal junction obstruction due to contiguous spread.
BMJ case reports[A Case of Long-Term Survival after Multidisciplinary Treatment for Neuroendocrine Carcinoma of the Anal Canal].
Gan to kagaku ryoho. Cancer & chemotherapySurgery of the primary tumour in 201 patients with high-grade gastroenteropancreatic neuroendocrine and mixed neuroendocrine-non-neuroendocrine neoplasms.
Journal of neuroendocrinologyPresacral neuroendocrine tumors associated with the Currarino syndrome.
American journal of medical genetics. Part A68Ga DOTATATE PET/CT imaging of Currarino syndrome with neuroendocrine tumor.
Japanese journal of clinical oncologyNeuroendocrine Carcinoma of the Anus and Rectum: Patient Characteristics and Treatment Options.
Clinical colorectal cancerAbject health concern in elderly: Cutaneous metastasis of malignant melanoma of anal canal.
The Indian journal of medical researchCOVID-19-Positive Report Posing a Delay in Treatment Course in a Middle-Aged Metastatic Neuroendocrine Tumor Patient.
Indian journal of palliative care[Retrospective investigation of patients receiving additional surgery after endoscopic non-curative resection for early colorectal cancer].
Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgeryCurrarino Syndrome: A Rare Condition With Potential Connection to Neuroendocrine Tumors.
Pancreas[Efficacy of Amrubicin Monotherapy for Patients with Extrapulmonary Neuroendocrine Carcinomas Refractory to Platinum-Based Chemotherapy].
Gan to kagaku ryoho. Cancer & chemotherapyGoblet cell carcinoid of the rectum: a case report.
Surgical case reportsResectable Anal Melanoma Detected by Rectal Retroflexion.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationA rare case of recurrent primary anorectal melanoma emphasizing the importance of postoperative follow-ups.
BMC surgeryIntensity-modulated radiotherapy (IMRT) in the treatment of squamous cell anal canal cancer: acute and early-late toxicity, outcome, and efficacy.
International journal of colorectal diseaseAnal melanoma, a radically different pathology from cutaneous melanoma with a tragic prognosis.
Cirugia espanolaAnorectal Melanoma as an Unexpected Diagnosis for a Pigmented Mass Resembling a Thrombosed Hemorrhoid.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationMalignant neuroendocrine tumour in an adult female diagnosed with Currarino syndrome.
South African journal of surgery. Suid-Afrikaanse tydskrif vir chirurgieOncologic outcome of mixed adenoneuroendocrine carcinoma (MANEC): A single center case series.
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical OncologyTransanal Minimally Invasive Surgery for Benign and Malignant Rectal Lesions: Operative and Oncological Outcomes of a Single Center Experience.
Journal of laparoendoscopic & advanced surgical techniques. Part APrimary Dedifferentiated Amelanotic Anorectal Melanoma: Report of a Rare Case.
International journal of surgical pathologyAnorectal Melanoma - a Histopathological Case Report and a Review of the Literature.
Folia medicaImmune inflammation indicators in anal cancer patients treated with concurrent chemoradiation: training and validation cohort with online calculator (ARC: Anal Cancer Response Classifier).
Cancer management and researchPerianal Goblet Cell Carcinoid With Pagetoid Spread.
International journal of surgical pathologyPatterns of Care and Survival Outcomes in the Treatment of Anal Melanoma.
Journal of gastrointestinal cancer[A Case of Laparoscopic Abdominoperineal Resection of Anorectal Malignant Melanoma].
Gan to kagaku ryoho. Cancer & chemotherapyPostanal minimally invasive surgery "PAMIS" assisted extra-levator abdominoperineal excision "ELAPE" for cancer: A novel approach in supine position.
Arab journal of gastroenterology : the official publication of the Pan-Arab Association of GastroenterologyMalignant transformation of presacral mass in Currarino syndrome.
Pediatric blood & cancer[Transanal Minimally Invasive Surgery for Rectal Neuroendocrine Tumor].
Gan to kagaku ryoho. Cancer & chemotherapyAnal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease, successfully treated with modified FOLFOX6: a case report.
BMC cancerNivolumab PD-1 inhibitor immunotherapy associated with vulvar, perineal and perianal lichen sclerosus.
Clinical and experimental dermatologyASO Author Reflections: Metastatic Melanoma to the Large Bowel.
Annals of surgical oncologyThe Pedunculated Pretender: A Case of Invasive Anorectal Mucosal Melanoma.
The American surgeonRobotic intersphincteric resection with hand-sewn colo-anal anastomosis for low rectal neuroendocrine tumour - a video vignette.
Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and IrelandEstablishment and Characterization of a Novel Cell Line Derived from a Small Cell Neuroendocrine Carcinoma of the Anal Canal.
NeuroendocrinologyJOURNAL CLUB: Primary Anorectal Melanoma: MRI Findings and Clinicopathologic Correlations.
AJR. American journal of roentgenologyTransanal minimally invasive surgery for local excision of early rectal tumor.
Asian journal of endoscopic surgeryRectal and anal canal neuroendocrine tumours.
Journal of gastrointestinal oncologyGenital marginal failures after intensity-modulated radiation therapy (IMRT) in squamous cell anal cancer: no higher risk with IMRT when compared to 3DCRT.
Medical oncology (Northwood, London, England)Metastatic primary anorectal melanoma developing in a patient treated for multicentric glioblastoma multiforme: two rare malignancies presenting in synchronicity.
BMJ case reportsTransanal Endoscopic Microsurgery for Patients with Rare Rectal Tumors.
Journal of laparoendoscopic & advanced surgical techniques. Part A[Transanal endoscopic microsurgery for treatment of rectal neuroendocrine tumors].
Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgeryMalignant Melanoma of the Anus Found during Routine Colonoscopy in Ulcerative Colitis.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chiCarcinoid transformation of presacral dermoid cyst in patient with currarino syndrome: a case report.
British journal of neurosurgeryAnorectal melanoma: a rare aggressive type of melanoma.
ANZ journal of surgeryAnal Neuroendocrine Tumor Masquerading as External Hemorrhoids: A Case Report.
Gastroenterology research[Mucosal malignant melanomas in the gastrointestinal tract and urogenital organs].
Ugeskrift for laegerPrimary anorectal mucosal melanoma detected by anorectal cytology.
Diagnostic cytopathologySuccessful Restoration of Fecal Continence Using Sacral Nerve Stimulation Following Chemoradiation and Transanal Excision of an Anal Melanoma With Partial Internal Anal Sphincter Resection: A Case Report.
Neuromodulation : journal of the International Neuromodulation Society[A Case Report - A Case of a Rapidly Growing Anal Canal Origin Malignant Melanoma].
Gan to kagaku ryoho. Cancer & chemotherapyPathology of Anal Cancer.
Surgical oncology clinics of North AmericaPolypoid anal melanoma. A case report and review of the literature.
Annali italiani di chirurgiaAnorectal malignant melanoma: curative abdominoperineal resection: patient selection with 18F-FDG-PET/CT.
World journal of surgical oncologyBasaloid Squamous Cell Carcinoma of the Anus Revisited.
The American journal of surgical pathologyAnorectal Cancer: Critical Anatomic and Staging Distinctions That Affect Use of Radiation Therapy.
Radiographics : a review publication of the Radiological Society of North America, IncA case series of anal melanoma including the results of treatment with imatinib in selected patients.
Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and IrelandSomatostatin receptor positron emission tomography/computed tomography imaging in Merkel cell carcinoma.
Journal of the European Academy of Dermatology and Venereology : JEADVChemoradiation for High-grade Neuroendocrine Carcinoma of the Rectum and Anal Canal.
American journal of clinical oncologyA rare cause of constipation: obstructing small cell neuroendocrine carcinoma of the anal canal.
International journal of colorectal diseaseAudiovestibular disorders as autoimmune reaction in patients with melanoma.
Medical hypothesesRobotic colorectal surgery for laparoscopic surgeons with limited experience: preliminary experiences for 40 consecutive cases at a single medical center.
BMC surgeryThe clinicopathologic spectrum of anal cancer in KwaZulu-Natal Province, South Africa: Analysis of a provincial database.
Cancer epidemiologyRobot-assisted intersphincteric resection for rectal submucosal tumour.
The international journal of medical robotics + computer assisted surgery : MRCASMalignant rectal melanoma. Case report.
Annali italiani di chirurgiaRole of fluorine 18 fluorodeoxyglucose positron emission tomography/computed tomography in gastrointestinal cancers.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverPreliminary Suggestion about Staging of Anorectal Malignant Melanoma May Be Used to Predict Prognosis.
Cancer research and treatmentAnal melanoma with neuroendocrine differentiation: report of a case.
International journal of surgical pathologyEvaluation of three types of platforms in single-incision laparoscopic surgery for performing transanal endoscopic microsurgery (SILSTEM).
Hepato-gastroenterologyPrimary anorectal malignant melanoma: a rare but aggressive tumor: report of a case.
World journal of surgical oncologyAssociações
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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.
- Changes in the 6th edition of the World Health Organization classification of tumours of the digestive system.
- A case of anastomotic recurrence 12 years after intersphincteric resection for anorectal malignant melanoma.
- HER2 aberration is a potential predictive biomarker for extrapulmonary small cell neuroendocrine carcinoma contrary to small cell lung cancer.
- Primary Neuroendocrine Carcinoma of the Anal Canal with Cancer Genome Profiling.
- Amelanotic Malignant Melanoma With Atypical Divergent Neuroendocrine Differentiation: A Report of an Unusual and Rare Case of Anorectal Bleeding.
- Robotic TAMIS rectal neuroendocrine tumor excision.
- Treatment of poorly differentiated neuroendocrine carcinomas of rectum and anus with chemoradiotherapy: a single-centre evaluation.
- Perianal schwannoma: An unusual location.
- Transanal minimally invasive surgery for benign and malignant rectal lesions: midterm outcomes from a tertiary center.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:100082(Orphanet)
- MONDO:0015069(MONDO)
- GARD:19757(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55785228(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
