Mutations homozygotes du gène de la desmogléine 1 et syndrome dermatite sévère, allergies multiples et fuite métabolique (syndrome SAM) - Université de Montpellier
Article Dans Une Revue Annales de Dermatologie et de Vénéréologie Année : 2015

[Homozygous desmoglein 1 gene mutations and severe dermatitis, multiple allergies and metabolic wasting (SAM syndrome)].

Mutations homozygotes du gène de la desmogléine 1 et syndrome dermatite sévère, allergies multiples et fuite métabolique (syndrome SAM)

Résumé

Pelvic exenteration (PE) is a radical surgical procedure used for the past 6 decades to treat locally advanced malignant diseases confined to the pelvis, particularly persistent or recurrent gynecologic cancers in the irradiated pelvis. The traditional surgical technique known as total PE consists of resection of all pelvic viscera followed by reconstruction. Depending on the tumor extent, the procedure can be tailored to remove only anterior or posterior structures, including the bladder (anterior exenteration) or rectum (posterior exenteration). Conversely, more extended pelvic resection can be performed if the pelvic sidewall is invaded by cancer. Preoperative imaging evaluation with magnetic resonance (MR) imaging and fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is central to establishing tumor resectability and therefore patient eligibility for the procedure. These imaging modalities complement each other in diagnosis of tumor recurrence and differentiation of persistent disease from posttreatment changes. MR imaging can accurately demonstrate local tumor extent and show adjacent organ invasion. FDG PET/CT is useful in excluding nodal and distant metastases. In addition, FDG PET/CT metrics may serve as predictive biomarkers for overall and disease-free survival. This pictorial review describes different types of exenterative surgical procedures and illustrates the central role of imaging in accurate patient selection, treatment planning, and postsurgical surveillance.

Domaines

Dermatologie
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Dates et versions

hal-01975720 , version 1 (09-01-2019)

Identifiants

Citer

Olivier Dereure. Mutations homozygotes du gène de la desmogléine 1 et syndrome dermatite sévère, allergies multiples et fuite métabolique (syndrome SAM). Annales de Dermatologie et de Vénéréologie, 2015, 142 (12), pp.798-799. ⟨10.1016/j.annder.2015.06.001⟩. ⟨hal-01975720⟩
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