Familial papillary thyroid microcarcinoma: a new clinical entity

doi:10.1016/S0140-6736(98)08004-0

The Lancet

Volume 353, Issue 9153, 20 February 1999, Pages 637-639

https://doi.org/10.1016/S0140-6736(98)08004-0 Get rights and content

Summary

Background

Familial, non-medullary thyroid carcinoma is clinically more aggressive than the sporadic form. We wanted to find out whether papillary thyroid microcarcinoma also occurs in a familial pattern, and, if so, to identify specific clinical and prognostic features.

Methods

We reviewed the clinical records of 119 patients with papillary thyroid microcarcinoma. Familial occurrence, together with clinical presentation, surgical treatment, pathological characteristics, and follow-up were recorded.

Findings

We identified a family history of thyroid carcinoma in seven patients. The tumour was multifocal in five patients, bilateral in three, and vascular invasion occurred in three of the seven patients. Lymph-node metastases were found in four patients. Three patients had a recurrence and one patient with pulmonary metastases died within 11 months.

Interpretation

We identified familial occurrence in 5·9% of cases of papillary thyroid microcarcinoma. The unfavourable behaviour in the familal form of papillary thyroid microcarcinoma suggests that radical treatment and careful follow-up are warranted.

Introduction

Carcinoma of the thyroid gland is the most frequent malignant disease of the endocrine system and can occur either as a familial disorder or as a sporadic form. The familial type of medullary thyroid carcinoma is a well-defined clinical entity. However, much less is known about the familial occurrence of differentiated thyroid carcinoma. Two patterns of presentation have been described for familial differentiated thyroid cancer. The first is associated with an inherited tumour syndrome, such as familial adenomatous polyposis, Gardner's syndrome (intestinal adenomatous polyposis, multiple tumours of soft tissue and bone), and Cowden's disease (multiple hamartomas, fibrocystic disease of the breast, and breast cancer). The second pattern occurs in familial aggregates and is not associated with other neoplasms.1, 2, 3

The prevalence of familial nonmedullary thyroid carcinoma (FNMTC) among all thyroid cancers ranges from 3·5% to 6·2%.4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15 Some investigators report that FNMTC has less favourable prognostic features than the sporadic form. Aggressive treatment and careful follow-up are therefore suggested for FNMTC.14, 15

A further classification of thyroid tumours is based on size. WHO defines papillary microcarcinoma of the thyroid as a papillary carcinoma measuring 1·0 cm or less in diameter.¹⁶ This subgroup of patients has been thought to be a specific low-risk category with a favourable prognosis.¹⁷

We investigated whether papillary thyroid microcarcinoma occurred within families—so-called familial papillary thyroid microcarcinoma (FPTMC). We also analysed the clinical and pathological features of FPTMC.

Section snippets

Methods

119 patients seen in our department between January, 1984, and December, 1997, had a diagnosis of papillary thyroid microcarcinoma. In our institution, we ask patients about a family history of thyroid disease when first seen; further information is added to the record when it becomes known. We reviewed the histology of any relatives who had had a thyroidectomy.

Patients who also had a history of one or more first-degree relatives (brother, sister, parents) with a differentiated thyroid

Results

Seven patients (two male) from our group of 119 patients had familial differentiated thyroid microcarcinoma. They were identified from six different families. The median (IQR) age at diagnosis was 32 (26–47) years. Patients 1 and 2 were sisters. Pathological records of the patients' relatives showed non-microcarcinoma differentiated thyroid cancer in nine individuals; multifocal cancer was found in seven (78%) of nine relatives, and bilateral disease in four (44%) of nine relatives.

Clinical and

Discussion

Familial occurrence of tumours is often characterised by specific clinical features. In some instances, such as with prostate adenocarcinoma, a less favourable prognosis and more aggressive clinical behaviour are associated with familial occurrence.¹⁸

Grossman and colleagues¹⁴ found that FNMTC had a high frequency of thyroid carcinoma multiple sites within the thyroid; invasion beyond the thyroid capsule; and metastases to regional lymph nodes or to distant organs. Therefore, total thyroidectomy

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Citation Excerpt :
Local recurrence occurred in three patients and one died of pulmonary metastases. The aggressive nature of these small tumors was striking.142 Similar findings have been reported by other authors.
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Early ReportFamilial papillary thyroid microcarcinoma: a new clinical entity

Summary

Background

Methods

Findings

Interpretation

Introduction

Section snippets

Methods

Results

Discussion

Clin Gastroenterol

Carcinoma of follicular epithelium: pathogenesis

Adenomatous polyposis: an association with carcinoma of the thyroid

Br J Surg

Familial occurrence of differentiated, nonmedullary thyroid carcinoma

World J Surg

Familial nonmedullary thyroid carcinoma—the case for genetic susceptibility

J Clin Endocrinol Metab

Family with nonmedullary thyroid neoplasms

J Surg Oncol

Familial differentiated carcinoma of the thyroid: report of five pairs of siblings

Thyroid

Familial papillary carcinoma of the thyroid

Am J Med Genet

Papillary carcinoma of the thyroid: additional evidence in support of a familial component

Cancer Invest

Familial nonmedullary thyroid cancer

Oncology

Early Report
Familial papillary thyroid microcarcinoma: a new clinical entity