A case of Sipple's syndrome is reported, in which the full phenotype was expressed. The patient had the typical marfanoid habitus, with thickened lips and alae nasi, neuromas on lips and tongue, medullary carcinoma of the thyroid gland, phaeochromocytomas, and medullated corneal nerve fibres. The plasma calcitonin level was initially elevated, rose on calcium infusion before thyroidectomy, and was undetectably after thyroidectomy. The urinary catecholamine excretion was elevated. The plasma parathyroid hormone, adrenocorticotrophin and growth hormone levels and the serum calcium level were normal.

Download full-text PDF

Source
http://dx.doi.org/10.5694/j.1326-5377.1976.tb134453.xDOI Listing

Publication Analysis

Top Keywords

medullary carcinoma
8
carcinoma thyroid
8
marfanoid habitus
8
sipple's syndrome
8
thyroid multiple
4
multiple phaeochromocytomas
4
phaeochromocytomas mucosal
4
mucosal neuromas
4
neuromas marfanoid
4
habitus abnormalities
4

Similar Publications

Background: Metastatic colon cancer (MCC) is a debilitating condition with a poor prognosis. Currently, there is limited data that investigates MCC in relation to mismatch repair (MMR) status. The aims of this study are to compare sociodemographic and clinicopathologic features and mortality between patients with MMR-proficient (MMR-P) and MMR-deficient (MMR-D) MCC.

View Article and Find Full Text PDF

Can ACR TI-RADS predict the malignant risk of medullary thyroid cancer?

J Clin Transl Endocrinol

March 2025

Department of Ultrasound, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, PR China.

Objectives: This study aimed to evaluate the diagnostic performance for medullary thyroid cancer (MTC) based on the 2017 Thyroid Imaging Reporting and Data System by the American College of Radiology (ACR TI-RADS) guideline, and the ability to recommend fine needle aspiration (FNA) for MTC.

Methods: Fifty-six MTCs were included, and 168 benign thyroid nodules (BTNs) and 168 papillary thyroid nodules (PTCs) were matched according to age. Ultrasound (US) features were reviewed according to ACR TI-RADS.

View Article and Find Full Text PDF

A hyalinizing trabecular tumor (HTT), characterized by a trabecular growth pattern and notable hyalinization within the trabeculae, occurs at a rate of ~1%. As patients with HTT may be asymptomatic, accurate diagnosis is a challenge. Due to its resemblance to other tumors, such as papillary thyroid carcinoma and medullary thyroid carcinoma, a precise diagnosis necessitates both pathological and molecular examinations.

View Article and Find Full Text PDF

Purpose: Pancreatic ductal adenocarcinoma (PDAC) is an aggressive malignancy with poor response to chemotherapy. High-frequency microsatellite instability (MSI-H) is a rare biological phenomenon in conventional PDAC, being more frequently described in tumors with medullary or mucinous features.

Methods And Results: In this manuscript, we report the case of a patient with an MSI-H pancreatic carcinoma with medullary features (medullary carcinoma of the pancreas-MCP) that achieved a complete pathological response after neoadjuvant modified FOLFIRINOX.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!