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Prise en charge des tumeurs endocrines
| Content Provider | Semantic Scholar |
|---|---|
| Author | Baudin, Elisabeth |
| Copyright Year | 2003 |
| Abstract | Classification and treatment of Endocrine Tumors (ET) Endocrine tumors (ET) are defined by positive immunostaining for chromogranine A, synaptophysin and less specific markers such as neuron-specific enolase and N-CAM. This definition includes various tumors scattered within the body and which share common characteristics : hormone secretion, association as part of inherited syndrome, common prognostic parameters and therapeutic management. Several points challenge the clinical management of these tumors : confusion regarding previous definition (apudoma, carcinoid...), their rarity, insufficient knowledge regarding prognostic parameters of relapse and survival and poor chemosensitivity. ETs stem from two main embryological tissues : the neuroectoderm (medullary thyroid carcinoma and pheochromocytoma mainly) and the endoderm (also called gastro-enteropancreatic (GEP) tumors). In the latter group, ETs are subdivided into three subgroups with various behavior regarding biological activity, association as part of inherited syndrome frequency, prognosis : foregut-derived (head and neck, thymus, bronchus, pancreas mainly), midgut-derived (ileum), hindgut-derived (rectum) ET. Classification aims at recognizing tumors with common clinical presentation, prognostic or therapeutic behavior. Initial clinical Correspondance : Dr BAUDIN Service de Cancérologie Endocrinienne et Médecine Nucléaire Institut Gustave Roussy 94805 Villejuif, France management of these tumors has two main goals, to look for associated hormone secretions and inherited syndromes, both being dependent on the primary. The great majority of ETs secrete at least one hormone with or without clinical consequences. Morbidity induced hormone secretion (metanephrine, serotonine, insulin, gastrin) should be early recognized and treated. Also, hormone secretion may constitute helpful biological markers which high specificity but various sensitivity. The recommended biological work-up has been standardized according to the primary (Baudin et al, Ann Oncol 2001). Of note, secretions in foregut-derived ETs, in contrast with midgut-derived ETs are characterized by their biological diversity. Screening for association as part of inherited syndrome is restricted to neuroectoderm and well-differentiated endoderm-derived ET. Familial and personal patient history, ET primary and tumor presentation (multifocality, natural history) are major points to look for a genetic screening. Main consequences of such a diagnosis are : early diagnosis of associated tumors, and familial screening with a various impact on cure rate depending on each syndrome. Main inherited syndromes to be screened for are : multiple endocrine neoplasia type 1 and 2, Von-Hippel-Lindau disease, type 1 neurofibromatosis, mitochondrial complex type II disease. Genes responsible for these syndromes are known and genotype-phenotype correlations have been described for some. ET prognosis mainly depends on pathological differentiation and tumor stage. Primary impact on prognosis is still questioned, as well as ET association as part of an inherited syndrome. Hormone secretion is no more a major prognostic parameter. The best prognostic approach has been brought by studying lung ETs. Travis demonstrated that 4 subgroups of lung ETs can be distinguished according to their survival by taking into account the mitotic count and necrosis. Typical carcinoid is characterized by a low mitotic count (< 2 / 10 HPF), atypical carcinoid or well differentiated endocrine carcinoma by mitosis ranging between 2 to 10 / HPF and or punctiforme necrosis, large cell (poorly differentiated ) endocrine carcinoma by a high rate of mitosis above 10 / HPF , large necrosis area and lost of the endocrinoid pathological pattern. For intestinal ETs, the WHO 2000 classification defines benign or uncertain well differentiated endocrine tumors depending on size, depth invasion and angioinvasiveness. For pancreatic ETs, the Ki67 positive percentage of cells and the mitotic count is taken into account instead of depth invasion. Patients with locoregional extension or distant metastases are classified as well differentiated endocrine carcinoma. Poorly differentiated endocrine carcinoma may be encountered mainly in pancreatic, colon and rectum ET. Therapeutic management aims at reducing both hormone secretion (always first) and tumor burden. The only curative therapy is sure-mémoires de l'Académie Nationale de Chirurgie, 2003, 2 (3) : 39-46 40 Siège embryologique Intestin antérieur Intestin moyen Intestin postérieur sièges anatomiques principaux larynx, bronche, thymus, pancréas estomac iléon, appendice, colon droit rectum sécrétions hormonales fréquentes, multiples fréquentes rares syndrome de prédisposition héréditaire <1-30% exceptionnelle exceptionnelle tumeurs peu différenciées fréquentes exceptionnelles rares métastases extra-hépatiques en l’absence de méta hépatique fréquentes exceptionnelles rares présentation métastatique 10-60% 80% (sauf appendice < 5%) 5% Tableau 1 : Caractéristiques cliniques, biologiques et histoire naturelle des tumeurs endocrines gastro-entéro-pancréatiques en fonction de leur origine embryologique gery in patients presenting with localized ET mainly, appendix, rectum, bronchus and insulinoma. In the majority of metastatic ET patients treatment remains palliative and should therefore take into account the natural history of the disease. Poorly differentiated ET and pancreatic ET are chemosensitive but complete responses are rare. In no or slowly progressive ET, a wait -and-see policy and/or locoregional therapeutic approaches should be considered. Protocols should include patients with morphologically progressive ET. |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://www.academie-chirurgie.fr/ememoires/005_2003_2_3_39x46.pdf |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
