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Aggressive papillary thyroid carcinoma in a child with type 2 congenital generalized lipodystrophy
Lima, Grayce Ellen da Cruz Paiva; Fernandes, Virgínia Oliveira; Montenegro, Ana Paula Dias Rangel; Carvalho, Annelise Barreto de; Karbage, Lia Beatriz de Azevedo Sousa; Aguiar, Lindenberg Barbosa; Macedo, Mário Sérgio Rocha; Ferreira, Luis Alberto Albano; Montenegro Júnior, Renan Magalhães.
  • Lima, Grayce Ellen da Cruz Paiva; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Fernandes, Virgínia Oliveira; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Montenegro, Ana Paula Dias Rangel; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Carvalho, Annelise Barreto de; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Karbage, Lia Beatriz de Azevedo Sousa; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Aguiar, Lindenberg Barbosa; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Macedo, Mário Sérgio Rocha; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Ferreira, Luis Alberto Albano; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
  • Montenegro Júnior, Renan Magalhães; Universidade Federal do Ceará. Hospital Universitário Walter Cantídio. Grupo Brasileiro para Estudos de Lipodistrofias Herdadas e Adquiridas. Fortaleza. BR
Arch. endocrinol. metab. (Online) ; 63(1): 79-83, Jan.-Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-989300
ABSTRACT
SUMMARY Thyroid carcinoma (TC) is rare in children, particularly in those aged < 10 years. Several studies have demonstrated a correlation between neoplasms and hyperinsulinemia and insulin resistance, which are often associated with a higher risk for and/or aggressiveness of the neoplasm. Congenital generalized lipodystrophy (CGL) with autosomal recessive inheritance is a rare disease and is characterized by the lack of adipose tissue, severe insulin resistance, and early metabolic disturbances. Here, we reported a rare case of a type 2 CGL in a girl who presented with a papillary TC (PTC) at the age of 7 years. She had no family history of TC or previous exposure to ionizing radiation. She had a generalized lack of subcutaneous fat, including the palmar and plantar regions, muscle hypertrophy, intense acanthosis nigricans, hepatomegaly, hypertriglyceridemia, severe insulin resistance, and hypoleptinemia. A genetic analysis revealed a mutation in the BSCL2 gene (p.Thr109Asnfs* 5). Ultrasound revealed a hypoechoic solid nodule measuring 1.8 × 1.0 × 1.0 cm, and fine needle aspiration biopsy suggested malignancy. Total thyroidectomy was performed, and a histopathological examination confirmed PTC with vascular invasion and parathyroid lymph node metastasis (pT3N1Mx stage). This is the first report to describe a case of differentiated TC in a child with CGL. Severe insulin resistance that is generally observed in patients with CGL early in life, especially in those with type 2 CGL, may be associated with this uncommon presentation of aggressive PTC during childhood.
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Full text: Available Index: LILACS (Americas) Main subject: Thyroid Neoplasms / Lipodystrophy, Congenital Generalized / Thyroid Cancer, Papillary Type of study: Diagnostic study Limits: Child / Female / Humans Language: English Journal: Arch. endocrinol. metab. (Online) Journal subject: Endocrinology / Metabolism Year: 2019 Type: Article Affiliation country: Brazil Institution/Affiliation country: Universidade Federal do Ceará/BR

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Full text: Available Index: LILACS (Americas) Main subject: Thyroid Neoplasms / Lipodystrophy, Congenital Generalized / Thyroid Cancer, Papillary Type of study: Diagnostic study Limits: Child / Female / Humans Language: English Journal: Arch. endocrinol. metab. (Online) Journal subject: Endocrinology / Metabolism Year: 2019 Type: Article Affiliation country: Brazil Institution/Affiliation country: Universidade Federal do Ceará/BR