A Rare Papillary Thyroid Carcinoma Variant; How Aggressive We Should Be? Case Report

Case Report

Annals Thyroid Res. 2017; 3(1): 92-94.

A Rare Papillary Thyroid Carcinoma Variant; How Aggressive We Should Be? Case Report

Alex Gonzalez Bossolo¹*, Michelle Mangual Garcia¹, Yanerys Agosto Vargas¹, Miosotis Garcia¹, Victor J Carlo-Chevere², Guillermo Villarmarzo¹ and Jose Hernan Martinez¹

¹Endocrinology Department, San Juan City Hospital, USA

²Puerto Rico Pathology, USA

*Corresponding author: Gonzalez A, Endocrinology Department, San Juan City Hospital, San Juan, Puerto Rico, USA Internal Medicine Training Program, Department of Medicine, P.O. Box 70344, USA

Received: January 27, 2016; Accepted: March 09, 2017; Published: March 24, 2017

Abstract

Cribriform Morular Variant (CMV) of Papillary Thyroid Carcinoma (PTC) is a rare type of thyroid papillary neoplasm. It is more prevalent in patients with Familial Adenomatous Polyposis syndrome (FAP), however sporadic CMV-PTC has been described. Due to a low prevalence of this condition, recent consensus guidelines do not state a specific therapeutic approach for this variant. We present a 20-year-old woman with PTC-CMV treated with a total thyroidectomy alone, with no evidence of recurrence at the one year follow up. More aggressive treatments that include lymph node dissection and adjuvant radio ablation have been described. This case shows that patients with this variant can be treated with a more conservative approach similar to that of variants that are at low risk for recurrence.

Keywords: Thyroid cancer; Cribriform modular variant; Management

Introduction

Cribriform Morular Variant (CMV) of Papillary Thyroid Carcinoma (PTC) is a rare type of thyroid papillary neoplasm, which is more prevalent in patients with Familial Adenomatous Polyposis syndrome (FAP), however CMV-PTC without FAP has been described [1]. Although the relation between both conditions is strong, the incidence of PTC has remained low (0.4-1.3%) [2,3]. CMV-PTC is characterized by its cribriform pattern with solid areas and spindle cell component [4]. Long term management is unclear. The latest American Thyroid Association [ATA] guidelines for differentiated thyroid cancer do not give any recommendation regarding therapeutic approaches for this specific variant [5]. This case report describes a 20-year-old woman with PTC-CMV treated as a low risk carcinoma.

Case Presentation

This is the case of a 20-year-old woman with past medical history of FAP. She came to our endocrinology clinics due to discomfort upon swallowing liquids and solids and sensation of neck fullness since 3 months ago. She had been diagnosed with FAP 6 months before our evaluation. Upon initial encounter, the patient denied any symptoms suggestive of thyroid dysfunction. Physical examination was remarkable for a mildly enlarged thyroid gland and a palpable non-tender nodule in the right side of the neck. Thyroid function tests were normal. Thyroid ultrasound revealed a solid hypoechoic nodule on the right thyroid lobe measuring 1.2cm x 1.0cm x 0.9 cm without calcifications, irregular borders, extra thyroidal extension. A Fine Needle Aspiration Biopsy (FNAB) was performed and showed a Papillary Thyroid Carcinoma with cytoplasmic and nuclear positive stain to b-catenin, suggestive of CMV-PTC (Figure A,B,C,D,E). Due to these findings and FAP history, a total thyroidectomy was performed. Surgical pathology report showed a well-delimited, unifocal, 1.5cm x 1.0cm x 0.9cm CMV-PTC, without angiolymphatic or perineural invasion. Postoperatively, the patient was treated with levothyroxine suppression with a target TSH of 0.5-2.0 ng/mL. Due to prior reports that support less invasive treatment, no lymph node dissection or radioactive iodine treatment was given. One year after her surgery, the patient remains without evidence of loco regional recurrence.

Discussion

Familial adenomatous polyposis syndrome is an autosomal dominant condition caused by a mutation in the Adenomatous Polyposis Coli gene (APC). It can present with several extra-intestinal manifestations in the thyroid, pancreas, liver and central nervous system among others. CMV-PTC is an uncommon variant and is highly associated with FAP syndrome [1]. It was first described since 1949 by Crail et al. [6]. Since then, roughly 200 cases have been reported in the literature. In contrast to other PTC variants, data is scarce regarding therapeutic approaches.