A family in which several members have medullary carcinoma and phaeochromocytoma is described. Four of the 5 patients in this family who had a phaeochromocytoma have died; in one of these the medullary carcinoma may have contributed to death but in general the thyroid cancers were slow growing. In our experience successful diagnosis and treatment of the phaeochromocytomas have presented the greatest challenge. The welfare of future generations with this syndrome would seem to depend upon regular clinical and biochemical surveillance of the individuals and expeditious diagnosis and treatment of their tumours, particularly the phaeochromocytomas.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1002/bjs.1800620404 | DOI Listing |
Virchows Arch
January 2025
Department of Oncology, University of Turin, Orbassano, Turin, Italy.
In non-papillary follicular cell-derived thyroid carcinomas, prognostic factors are scarce. Intratumoral fibrosis was identified as an adverse factor in papillary and medullary carcinomas, but it has not been investigated in other subtypes. We aimed at exploring the presence of intratumoral fibrosclerosis in a cohort of 132 non-papillary follicular cell-derived thyroid carcinomas (53 follicular and 31 oncocytic carcinomas, including 10 high grade differentiated thyroid carcinomas and 48 poorly differentiated carcinomas) and correlating its presence and extent with clinical and pathological features and survival.
View Article and Find Full Text PDFActa Endocrinol (Buchar)
January 2025
AIIMS Mangalagiri - Pathology, Mangalagiri, Guntur, Andhra Pradesh, Vijayawada, India.
Surg Pract Sci
December 2024
Faculty of Medicine University of Tasmania, Tasmania, Australia.
Aims: Medullary carcinoma of the colon is a rare subtype of adenocarcinoma, first described in 1999. Clinically known to have a favourable prognosis in comparison to poorly differentiated cancers, it is associated with deficient mismatch repair. This is an observational single center study of patients with medullary cancer, and comparison with the current literature.
View Article and Find Full Text PDFAnn Endocrinol (Paris)
January 2025
CHU Angers, Service d'Endocrinologie-Diabétologie-Nutrition, Angers, 49100, France. Electronic address:
Background: RET variants affecting codon 804 are part of the low-to-moderate risk group in the ATA classification, with indications for prophylactic thyroidectomy beyond age of 5 years. However, aggressiveness seems to be variable. The objective of this study was to report a large cohort of French carriers of a pathogenic variant at codon 804 in the RET proto-oncogene.
View Article and Find Full Text PDFFront Oncol
January 2025
Endocrinology Unit, Garibaldi-Nesima Hospital, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Background: Multiple endocrine neoplasia type 2 syndrome (MEN2) is a hereditary disease resulting from mutations of the rearranged during transfection (RET) protooncogene subclassified into MEN2A [medullary thyroid carcinoma (MTC), pheochromocytoma, and primary hyperparathyroidism] and MEN2B (MTC, pheochromocytoma, Marfanoid habitus, mucous neuromas, and intestinal ganglioneuromatosis). Prophylactic thyroidectomy is recommended in RET-mutated patients. The age at which it should be performed depends on the type and aggressiveness of the mutation.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!