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A case of McCune-Albright syndrome associated with pituitary GH adenoma: therapeutic process and autopsy
-
Liu Fuyi
und
Chen Gao
Veröffentlicht/Copyright:
24. Mai 2011
Abstract
Background: McCune-Albright syndrome (MAS) is a clinical syndrome with low incidence, and its concurrence with pituitary GH adenoma is rare. Little of the history, treatment and outcome has been studied.
Method: Follow-up of a 37-year-old male patient of MAS associated with pituitary GH adenoma was performed continuously recording the disease development and the treatment process until death, after which an autopsy was performed.
Results: Radiation therapy (RT) efficaciously controlled GH hypersecretion, however, it may have been the cause of the malignant transformation of the dysplastic bone tissue, which eventually caused brain hernia and death; autopsy demonstrated that the cranium had significant thickening (as much as 10 cm), the pathological diagnosis was fibrous dysplasia of bone associated with chondrosarcoma; and undifferentiated chondrosarcoma with malignant fibrous histocytoma subtype in the sellar region; nodular goiter with the thyroid gland, one nodus was pathologically demonstrated as papillary carcinoma.
Conclusion: GH adenoma, present in a patient with MAS, might be cured by RT; but the risk of malignant transformation of the dysplastic bone tissue in the field of irradiation make it controversial. Lessons from the case reported here told us that we should take great caution when recommending RT for patients like this.
Keywords: McCune-Albright syndrome; papillary carcinoma of the thyroid gland; pituitary GH adenoma; radiation therapy
Published Online: 2011-05-24
Published in Print: 2011-06-01
©2011 by Walter de Gruyter Berlin Boston
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Schlagwörter für diesen Artikel
McCune-Albright syndrome;
papillary carcinoma of the thyroid gland;
pituitary GH adenoma;
radiation therapy
Artikel in diesem Heft
- Publisher's Note
- Publisher's Note
- Editorial
- The damage caused by incomplete and uncontrolled medical information
- Images in Pediatric Endocrinology
- Dorsolumbar kyphosis: diagnostic value of hook shaped vertebra
- Original Contributions
- Are adolescent weight-related problems and general well-being essentially an issue of age, gender or rather a pubertal timing issue?
- Comparison of methimazole and propylthiouracil in the management of children and adolescents with Graves’ disease: efficacy and adverse reactions during initial treatment and long-term outcome
- The effect of glucocorticoid replacement therapy on bone mineral density in children with congenital adrenal hyperplasia
- Treatment of symptomatic osteoporosis in children: a comparison of two pamidronate dosage regimens
- Stanozolol regulates proliferation of growth plate chondrocytes via activation of ERα in GnRHa-treated adolescent rats
- A case of McCune-Albright syndrome associated with pituitary GH adenoma: therapeutic process and autopsy
- The role of metabolic syndrome components and adipokines in insulin resistance in prepubertal children
- A new heterozygous mutation (D196N) in the Gs alpha gene as a cause for pseudohypoparathyroidism type IA in a boy who had gallstones
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- Benign transient hyperphosphatasemia in infants and children: a prospective cohort
- The relation between 25-hydroxyvitamin D with peak bone mineral density and body composition in healthy young adults
- Childhood obesity: evidence of an association between plasminogen activator inhibitor-1 levels and visceral adiposity
- Glargine basal-bolus insulin regimen versus insulin pump therapy: comparison of glycemic control
- Patient Report Letters
- A new variant of a known mutation in two siblings with permanent neonatal diabetes mellitus
- HNF1A mutation presenting with fetal macrosomia and hypoglycemia in childhood prior to onset of overt diabetes
- A newborn case with perinatal-lethal Gaucher disease due to R463H homozygosity complicated by C677T homozygosity in the MTHFR gene
- Hyperthyroidism presenting with hyperglycemia in an adolescent female
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