1 / 24

Sindromi paraneoplastiche da dismotilità gastrointestinale

Joint Meeting GISMAD-AIGO-SIED-SIGE DISTURBI DELLA MOTILITA’ GI NELLE PATOLOGIE SISTEMICHE. Verona, martedì 9 Marzo 2010. Sindromi paraneoplastiche da dismotilità gastrointestinale. Rosario Cuomo AOU “Federico II” – Napoli rcuomo@unina.it. Paraneoplastic syndromes.

una
Download Presentation

Sindromi paraneoplastiche da dismotilità gastrointestinale

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Joint Meeting GISMAD-AIGO-SIED-SIGE DISTURBI DELLA MOTILITA’ GI NELLE PATOLOGIE SISTEMICHE Verona, martedì 9 Marzo 2010 Sindromi paraneoplastiche da dismotilità gastrointestinale Rosario Cuomo AOU “Federico II” – Napoli rcuomo@unina.it

  2. Paraneoplasticsyndromes • Paraneoplastic disorders are non metastatic syndromes that are not attributable to toxicity of cancer therapy, infection, or toxic/metabolic causes. • They are clinically important for several reasons: • Paraneoplastic disorders often cause severe and permanent morbidity. • The symptoms are the presenting feature of an otherwise undiagnosed tumor, and so the clinician must be able to recognize and diagnose these syndromes promptly. • The paraneoplastic syndromes are an important part of the differential diagnosis of dysfunction. • Early diagnosis of a paraneoplastic disorder maximizes the likelihood of successful tumor treatment

  3. Paraneoplasticsyndromes • The most common cancersassociatedwithparaneoplasticsyndromes include • Lung carcinoma (most common) • Renal carcinoma • Hepatocellular carcinoma • Leukemias • Lymphomas • Breasttumors • Ovariantumors • Neuralcancers • Gastriccancers • Pancreaticcancers

  4. Paraneoplasticsyndromes • Generalparaneoplasticsymptoms • Cutaneousparaneoplasticsyndromes • Endocrine paraneoplasticsyndromes • GI paraneoplasticsyndromes • Hematologicparaneoplasticsyndromes • Neurologicparaneoplasticsyndromes • Renalparaneoplasticsyndrome • Rheumatologicparaneoplasticsyndromes

  5. Paraneoplastic GI dismotilitysyndromes • A small proportion of patients with occult or established neoplasms develop a gastrointestinal motility disorder, referred to as paraneoplasticdysmotility. • The diagnosis of a paraneoplasticdysmotility requires the onset of gastrointestinal dysmotility associated with the presence of a tumor and presence of specific serum antibodies Kashyap P and Farrugia G, GastroenterolClin North Am. 2008

  6. Clinical presentation of a paraneoplasticdysmotility syndrome • Pseudoachalasia • ParaneoplasticGastroparesis • Paraneoplasticchronicintestinalpseudoobstruction • Chronicconstipation Kashyap P and Farrugia G, GastroenterolClin North Am. 2008

  7. SCLC=small-cell lung cancer; lambert-eaton myastenic syndrome

  8. Enteric Autoantibodies and Gut Motility Disorders • Antibodies associated with paraneoplastic and idiopathic dysmotility • Type 1 antineuronal nuclear antibody (ANNA-1) recognizenuclearproteinHu (in the neurons of the central, peripheral and enteric nervous system) • Calciumchannelantibodies(Antibodies to P/Q and N type calcium channels; less frequently found compared to ANNA-1 antibodies; may coexist with ANNA-1) • Antibodies against neuronal nicotinic acetylcholine receptors (ganglionic antibodies often determine symptoms of gastrointestinal dysmotility) • Purkinje Cell Cytoplasmic Autoantibody, type 1 (PCA1) (Gastrointestinal dysmotility in a minority of PCA-1 seropositive patients +/- cerebellar ataxia in association with gynecological or breast carcinoma Kashyap P and Farrugia G, GastroenterolClin North Am. 2008

  9. Journal ofAutoimmunity (1999)

  10. Autoimmunity Reviews 6 (2007) 162–168

  11. Antineuronal antibodies of the Hu type in a 55-year-old patient with paraneoplastic syndrome characterized by CIPO related to an occult small-cell lung carcinoma GASTROENTEROLOGY 2004;126:1872–1883

  12. A 68-yr-old man developed anorexia, early satiety, nausea, and constipation and lost approximately 20 lb in 3 months. He subsequently developed daily nausea and vomiting with dysgeusia and increasedanorexia. Am J Gastroenterol 2002

  13. Normalhumanjejunaltissue Patient’s jejunalbiopsy MP-ICC = c-Kit positive interstizialcellofCajal in mientericplexus CM = circularmuscle; LM = longitudinalmuscle Am J Gastroenterol 2002

  14. Metastatic small-cell lung carcinoma cells in the biopsied mediastinallymphnode Hematoxylin-eosinstain Immunoreactivity of the Kit protein Am J Gastroenterol 2002

  15. SummaryofPatientsStudied Am J Gastroenterol 2001;96:373–379

  16. Results of Manometric and Radiographic Images in PatientsWith SCLC Results of Serological Tests for Neuronal Autoantibodies Am J Gastroenterol 2001;96:373–379

  17. Upright plain film of the abdomen demonstrating sitz markers throughout the colon 1 month after sitz marker ingestion Supine film of the abdomen taken 1 year after the film shown in Fig 1. Extensive distention of the colon with stool is noted. Nineteen stiz markers ingested a year previously are retained. A gastrostomy tube is present. 63-year-old woman NeurogastroenterolMotil (2005) 17, 16–22

  18. Lymphoplasmacytic infiltrate is noted in the location of the myenteric plexus. NeurogastroenterolMotil (2005) 17, 16–22

  19. Pathogenesis of Malignant Gastroparesis in Various Cancer Types J SupportOncol 2007;5:355–363

  20. J SupportOncol 2007;5:355–363

  21. Medical Management of Gastroparesis J SupportOncol 2007;5:355–363

  22. Enteral tubes for the management of malignant gastroparesis J SupportOncol 2007;5:355–363

  23. Management algorithm for paraneoplasticdysmotility • Insufficient evidence to recommend a paraneoplastic antibody profile on every patient with new onset of a gut motility disorder • The presenceofsignificantweight loss, a rapid onset of the disease, a past or present smoking history should prompt to consider testing for the presence of autoantibodies • ANNA-1 positivity: start with a CT chest and if negative follow up with a PET scan and directed biopsies of any suspicious lymph nodes or massesifindicated (SCLC 13%) • The presence of other autoantibodies without concomitant ANNA-1 positivity is less likely to predict the presence of a malignancy Kashyap P and Farrugia G, GastroenterolClin North Am. 2008

  24. Treatment ofparaneoplasticdysmotility • No treatments have been convincingly shown to alter outcome (steroids, cyclophosphamide, plasmapheresis, immunoglobulin) • Treatment of the underlying primary malignancy • Nutritional support either enterally or parenterally • Prokinetics, treatment of bacterialovergrowth • One additional management strategy is to use high dose IV steroids for 3 days and if there is a clinical response switch to 6-mercatopurine or azathioprine (difficult in the case of chemotherapy) Kashyap P and Farrugia G, GastroenterolClin North Am. 2008

More Related