repost: Re: ThyCa & Hurthle hereditary -->

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Subject: Hurthle hereditary?

Title A gene predisposing to familial thyroid tumors with cell oxyphilia maps

to chromosome 19p13.2.

Author Canzian F; Amati P; Harach HR; Kraimps JL; Lesueur F; Barbier J;

Levillain P; Romeo G; Bonneau D

Address Unit of Genetic Cancer Susceptibility, International Agency for Research

on Cancer, Lyon, France.

Source Am J Hum Genet, 63(6):1743-8 1998 Dec

Abstract

Familial nonmedullary thyroid cancer (FNMTC) is a clinical entity

characterized by a phenotype more aggressive than that of its sporadic

counterpart. Families with recurrence of nonmedullary thyroid cancer

(NMTC) have been repeatedly reported in the literature, and epidemiological data

show a very high relative risk for first-degree relatives of probands with

thyroid cancer. The transmission of susceptibility to FNMTC is compatible with

autosomal dominant inheritance with reduced penetrance, or with complex

inheritance. Cases of benign thyroid disease are often found in FNMTC kindreds.

We report both the identification of a new entity of FNMTC and the mapping of

the responsible gene, named " TCO " (thyroid tumors with cell oxyphilia), in a

French pedigree with multiple cases of multinodular goiter and NMTC. TCO was

mapped to chromosome 19p13.2 by linkage analysis with a whole-genome panel of

microsatellite markers. Interestingly, both the benign and malignant thyroid

tumors in this family exhibit some extent of cell oxyphilia, which, until now,

had not been described in the FNMTC. These findings suggest that the relatives

of patients affected with sporadic NMTC with cell oxyphilia sh

oul

d be carefully investigated.

timo

acidophilic(oxyphilic)oncocytic(acidophilic)hurthle

SFO

++++++++++++++++++++++++++

- Here's another from Germany suggesting a therapeutic possibility

(at the bottom of the abstract).

UI - 99142250

AU - Gorges R

AU - Kahaly G

AU - Muller-Brand J

AU - Macke H

AU - Walgenbach S

AU - Bruns C

AU - s J

AU - Brandt-Mainz K

AU - Bockisch A

TI - [somatostatin receptor status in non-medullary thyroid carcinoma]

PT - JOURNAL ARTICLE

TA - Nuklearmedizin

YR - 1999

EM - 199904

AB - AIM: Recent in-vitro and in-vivo studies demonstrated a

somatostatin

receptor expression in some non-medullary thyroid carcinomas. In

this

study we investigated the somatostatin receptor status for this

particular tumor entity in a larger patient group. SUBJECT AND

METHODS: We compared 131-iodine with 111-In-pentetreotide scans in

24

patients with metastasizing, non-medullary thyroid cancer. The

findings were correlated with other imaging modalities.

Additionally,

we performed receptor autoradiography in one patient, octreotide

therapy in another patient and administration of 90-Y- and

111-In-DOTATOC in 2 consecutive patients. RESULTS: In the 15

patients

with papillary or follicular carcinoma, 111-In-pentetreotide was

inferior to 131-I in 8/15, equal in 1/15, and superior in 6/15

patients. In 8/9 of the patients with Hurthle cell carcinoma,

metastases showed a 111-In-pentetreotide accumulation of various

intensity, while 131-iodine scans were negative except for one

patient. 111-In-pentetreotide was equal or superior compared to

201-Tl

or 99m-Tc-sestamibi, but for the most part inferior in comparison

with

18-F-FDG-PET. The findings of 111-In-pentetreotide scintigraphy

correlated well with the receptor autoradiography and the accumulation of

DOTATOC, but not with the therapeutic effect of " cold " octreotide on the thyroid

cancer metastases. CONCLUSIONS: Several metastases of papillary and follicular

carcinoma, and the majority of Hurthle cell cancer metastases can express

somatostatin receptors. 111-In-pentetreotide scintigraphy is a promising

tool for

localization of metastases especially in Hurthle cell cancer or if PET is not

available, and may be useful for selection of possible candidates, if

therapeutic effective beta-emitting somatostatin analogues will be available for

routine application.

AD - Klinik und Poliklinik fur Nuklearmedizin, Universitatskliniken

Essen,

Deutschland.

SO - Nuklearmedizin; 38(1):15-23 1999

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