A case of craniopharyngioma (CP) presenting as precocious puberty associated with growth acceleration after a period of stunted growth is reported in a 7.5-year-old girl. The girl grew along the 25th percentile until the age of 4 years, when her height fell down to 3rd percentile until she was 7 years old. Then, a breast bud occurred, the growth rate increased and a diagnosis of precocious puberty was made. Brain magnetic resonance imaging revealed an oval suprasellar mass pressing and flattening the hypophysis with ventral displacement of the optic chiasm suggesting a CP. No headache or other neurophthalmologic disturbances were documented. After resection of CP, the girl showed panhypopituitarism that was treated with hormonal substitutive therapy. A few months later, the major problem became the exponential increase of the weight due to the hyperphagia not responding to any diet regimen and leading the girl to develop a severe hypothalamic obesity. In fact, at the age of 8.3 years she showed an height of 118.8 cm (-1.53 SDS) and a weight of 37.5 Kg (BMI 2.14 SDS). In conclusion, CP has a wide range of clinical manifestations, depending of the tumour size as well as the extent of endocrine deficiency and the age of the patient. In our patient the initial growth failure was, probably, due to the compressive action of CP. Then, the onset of precocious puberty was related to the stimulating effect of the neoplasia on gonadotropic pituitary cells.

A Case of Craniopharyngioma Presenting as Precocious Puberty after a Period of Stunted Growth

BOZZOLA, MAURO;MEAZZA, CRISTINA;PAGANI, SARA;
2013

Abstract

A case of craniopharyngioma (CP) presenting as precocious puberty associated with growth acceleration after a period of stunted growth is reported in a 7.5-year-old girl. The girl grew along the 25th percentile until the age of 4 years, when her height fell down to 3rd percentile until she was 7 years old. Then, a breast bud occurred, the growth rate increased and a diagnosis of precocious puberty was made. Brain magnetic resonance imaging revealed an oval suprasellar mass pressing and flattening the hypophysis with ventral displacement of the optic chiasm suggesting a CP. No headache or other neurophthalmologic disturbances were documented. After resection of CP, the girl showed panhypopituitarism that was treated with hormonal substitutive therapy. A few months later, the major problem became the exponential increase of the weight due to the hyperphagia not responding to any diet regimen and leading the girl to develop a severe hypothalamic obesity. In fact, at the age of 8.3 years she showed an height of 118.8 cm (-1.53 SDS) and a weight of 37.5 Kg (BMI 2.14 SDS). In conclusion, CP has a wide range of clinical manifestations, depending of the tumour size as well as the extent of endocrine deficiency and the age of the patient. In our patient the initial growth failure was, probably, due to the compressive action of CP. Then, the onset of precocious puberty was related to the stimulating effect of the neoplasia on gonadotropic pituitary cells.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11571/817237
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