+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

A Novel Syndrome of Generalized Lipodystrophy Associated With Pilocytic Astrocytoma

, : A Novel Syndrome of Generalized Lipodystrophy Associated With Pilocytic Astrocytoma. Journal of Clinical Endocrinology and Metabolism 100(10): 3603-3606

A rare presentation of hypothalamic tumors in infants and young children is profound emaciation and generalized loss of sc adipose tissue, also known as "diencephalic syndrome." Similar loss of sc fat can be observed in children with acquired generalized lipodystrophy or congenital generalized lipodystrophy. Precise diagnosis may be challenging early in the course of the disease, especially in the absence of metabolic abnormalities. We report three males who presented with poor weight gain and generalized loss of sc fat at birth to 3 years of age consistent with generalized lipodystrophy, with subsequent development of pilocytic astrocytoma. Two of them had hypothalamic tumors, and one had a multicentric tumor with a large right parietal mass. Our patients are unique because the onset of lipodystrophy occurred 2.5 to 7.3 years before the diagnosis of brain tumor, and all of them gained body fat or weight after surgical removal and/or chemotherapy. One patient had hepatosplenomegaly and impaired glucose tolerance, and another patient had severe hyperglycemia and hypertriglyceridemia during the course of the disease. Two patients presented with central precocious puberty and advanced bone age at the chronological age of 6 years. It is likely that pilocytic astrocytoma may induce generalized lipodystrophy by paraneoplastic antiadipocyte antibody formation or by excessive hormones or cytokine secretion resulting in excess lipolysis from adipocytes. We conclude that young children presenting with idiopathic acquired generalized lipodystrophy or atypical congenital generalized lipodystrophy, with or without metabolic abnormalities, should prompt investigation for brain tumors.


PMID: 26252356

DOI: 10.1210/jc.2015-2476

Other references

Ilyas, A.M., 2010: Proximal row carpectomy with a dorsal capsule interposition flap. Arthritis of the wrist can be a debilitating condition that presents with progressive pain and loss of motion. A proximal row carpectomy (PRC) is indicated for the management of wrist arthritis and is designed to improve pain while maintaining mot...

Natapoff, J.N., 1995: Pediatric nursing: progress and perspectives. Journal of the New York State Nurses' Association 26(1): 10-11

Schmitt, J.; Goellner, M.; Lohbauer, U.; Wichmann, M.; Reich, S., 2013: Zirconia posterior fixed partial dentures: 5-year clinical results of a prospective clinical trial. This study aimed to evaluate three- and four-unit posterior fixed partial dentures (FPDs) with zirconia frameworks after 5 years of function. Of the initial 30 subjects, 25 patients with 25 FPDs were examined after a mean follow-up period of 62.1...

White, David A., 1993: Vascular plant community development on mudflats in the Mississippi River delta, Louisiana, USA. Plant community development and phytosociology on mudflats on two inner deltaic splays in the Mississippi River delta, Louisiana, are described. The splays supported three communities based upon plant species occurrence, soil, and plant biomass da...

Zborowska-Sluis, D.T.; Mildenberger, R.R.; Klassen, G.A., 1977: The role of coronary flow and pressure as determinants of myocardial oxygen consumption in the presence or absence of vasomotor tone. When coronary flow was kept constant and coronary pressure increased by angiotensin or decreased by dipyridamole, myocardial oxygen consumption (MVO2) remained unchanged. When vasomotor tone was abolished by dipyridamole or was intact, changing co...

Kiefer, J., 1987: UV response of the temperature-conditional rad 54 mutant of the yeast Saccharomyces cerevisiae. The survival of the yeast mutant rad 54-3, which is temperature-conditional for the repair of double-strand breaks, was measured after exposure to UV-light (254 nm) and incubation at 23 degrees C and 36 degrees C. It was found that survival was dr...

Nicholson, P.; Devitt, A.; Stevens, M.; Mahalingum, K., 1999: Acute exertional peroneal compartmental syndrome following prolonged horse riding. Injury 29(8): 643-644

Mosca, Anna M., 1957: Researches on the fungus flora of the soil of a snow valley in the Gran Paradiso National Park. From the soil of a snow valley in the Italian Alps (Aosta) at about 8, 750 ft. only 37 spp. were isolated by the soil suspension method. They included Beauveria bassiana, B. globulifera, and an unusual insect parasite (Synnematium sp.). The fungal...

Kim, J-Yong.; Moon, K-Deog.; Seo, K-Il.; Park, K-Wuk.; Choi, M-Sook.; Do, G-Min.; Jeong, Y-Kee.; Cho, Y-Su.; Lee, M-Kyung., 2009: Supplementation of SK1 from Platycodi radix ameliorates obesity and glucose intolerance in mice fed a high-fat diet. This study investigated the beneficial effects of SK1 on obesity and insulin resistance in C57BL/6 mice, which were fed a high-fat diet (37% calories from fat). SK1 is an edible saponin-rich compound from Platycodi radix. The mice were supplemente...

Paulin, D.D.cazy, B.C.ulibaly, N., 1985: Seasonal variations in pollination and pod production in a cacao smallholding in the Ivory Coast. Proceedings Lome Togo 12-18 Feb Fev 1984 9 International Cocoa Research Conference = Actes Lome Togo 12-18 Feb Fev 1984 9 Conference internationale sur la recherche cocaoyere: 556