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[Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999].
Bull Acad Natl Med. 2003; 187(7):1249-58; discussion 1259-60.BA

Abstract

About 25% of patients with ZES have MEN 1. Except diarrhoea, less frequent in patients with ZES-MEN 1 than in sporadic ZES, and specific MEN 1-related signs, clinical characteristics are similar in both ZES types. Acid output and gastrin levels are also similar whether in the basal state or after secretin stimulation. Primary hyperparathyroidism (PHPT) exists in the majority of ZES-MEN 1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25 to 30% have EC-Lomas; bronchial and thymic carcinoids have probably been underevaluated. Gastrinomas are multiple predominantly located in the duodenal wall, but also in the pancreas in association with clinically silent endocrine tumors. The spread of the disease: metastases to the liver (LM), mediastinum, bones, is evaluated at best by Octreoscan. Endoscopic ultrasonography evaluates the number, size and anatomical characteristics of gastrinomas. Patients without LM have an excellent prognosis. Surgery never cures ZES, but is necessary in case of associated life-threatening condition such as insulinoma and has been advocated to prevent LM development in patients with large pancreatic tumor(s). However although, indeed, the size of the tumor, when located in the pancreas > 3 cm, favours metachronous LM occurrence, surgery, in our experience, has not been able to prevent LM development. Hepatic malignancies remain however the most pejorative prognostic determinant for survival and raise the most difficult therapeutic challenge. Surgery is the best option whenever feasible; specific chemotherapy and chemo-embolisation have not conclusively achieved definite successes. Long-term octreotide treatment, however, has been shown recently to obtain tumour stabilisation. Internal irradiation with 90 Ytrium-labelled octreotide is a new promising option, presently under evaluation (Novartis European trial). Preliminary results are promising.

Authors+Show Affiliations

Service d'Hépato-gastroentérologie, Hôpital Bichat-Claude Bernard et INSERM (U 10 et U 410)-46 rue Henri Huchard, 75877 Paris.

Pub Type(s)

English Abstract
Historical Article
Journal Article
Review

Language

fre

PubMed ID

15146602

Citation

Mignon, Michel. "[Diagnostic and Therapeutic Strategies in Zollinger-Ellison Syndrome Associated With Multiple Endocrine Neoplasia Type I (MEN-I): Experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]." Bulletin De l'Academie Nationale De Medecine, vol. 187, no. 7, 2003, pp. 1249-58; discussion 1259-60.
Mignon M. [Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. Bull Acad Natl Med. 2003;187(7):1249-58; discussion 1259-60.
Mignon, M. (2003). [Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. Bulletin De l'Academie Nationale De Medecine, 187(7), 1249-58; discussion 1259-60.
Mignon M. [Diagnostic and Therapeutic Strategies in Zollinger-Ellison Syndrome Associated With Multiple Endocrine Neoplasia Type I (MEN-I): Experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. Bull Acad Natl Med. 2003;187(7):1249-58; discussion 1259-60. PubMed PMID: 15146602.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. A1 - Mignon,Michel, PY - 2004/5/19/pubmed PY - 2004/6/2/medline PY - 2004/5/19/entrez SP - 1249-58; discussion 1259-60 JF - Bulletin de l'Academie nationale de medecine JO - Bull. Acad. Natl. Med. VL - 187 IS - 7 N2 - About 25% of patients with ZES have MEN 1. Except diarrhoea, less frequent in patients with ZES-MEN 1 than in sporadic ZES, and specific MEN 1-related signs, clinical characteristics are similar in both ZES types. Acid output and gastrin levels are also similar whether in the basal state or after secretin stimulation. Primary hyperparathyroidism (PHPT) exists in the majority of ZES-MEN 1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25 to 30% have EC-Lomas; bronchial and thymic carcinoids have probably been underevaluated. Gastrinomas are multiple predominantly located in the duodenal wall, but also in the pancreas in association with clinically silent endocrine tumors. The spread of the disease: metastases to the liver (LM), mediastinum, bones, is evaluated at best by Octreoscan. Endoscopic ultrasonography evaluates the number, size and anatomical characteristics of gastrinomas. Patients without LM have an excellent prognosis. Surgery never cures ZES, but is necessary in case of associated life-threatening condition such as insulinoma and has been advocated to prevent LM development in patients with large pancreatic tumor(s). However although, indeed, the size of the tumor, when located in the pancreas > 3 cm, favours metachronous LM occurrence, surgery, in our experience, has not been able to prevent LM development. Hepatic malignancies remain however the most pejorative prognostic determinant for survival and raise the most difficult therapeutic challenge. Surgery is the best option whenever feasible; specific chemotherapy and chemo-embolisation have not conclusively achieved definite successes. Long-term octreotide treatment, however, has been shown recently to obtain tumour stabilisation. Internal irradiation with 90 Ytrium-labelled octreotide is a new promising option, presently under evaluation (Novartis European trial). Preliminary results are promising. SN - 0001-4079 UR - https://www.unboundmedicine.com/medline/citation/15146602/[Diagnostic_and_therapeutic_strategies_in_Zollinger_Ellison_syndrome_associated_with_multiple_endocrine_neoplasia_type_I__MEN_I_:_experience_of_the_Zollinger_Ellison_Syndrome_Research_Group:_Bichat_1958_1999]_ L2 - http://www.diseaseinfosearch.org/result/7600 DB - PRIME DP - Unbound Medicine ER -