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Original Paper

Hypothalamic Hamartoma: Comparison of Clinical Presentation and Magnetic Resonance Images

Debeneix C.a · Bourgeois M.b · Trivin C.c · Sainte-Rose C.b · Brauner R.a

Author affiliations

aPediatric Endocrinology and bNeurosurgery Departments and cPhysiology Laboratory, Université René Descartes and Hôpital Necker-Enfants Malades, Assistance Publique-Hopitaux de Paris, France

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Horm Res 2001;56:12–18

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Published online: January 31, 2002
Issue release date: 2001

Number of Print Pages: 7
Number of Figures: 1
Number of Tables: 3

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: https://www.karger.com/HRP

Abstract

Background/Aims: Hypothalamic hamartoma (HH) is one of the most frequent causes of organic central precocious puberty (CPP). We compared the clinical presentation and the magnetic resonance images (MRI) of 19 patients with HH aged 5.7 ± 4.1 (SD) years at the first endocrine evaluation. They had isolated CPP (group 1, n = 9), CPP plus gelastic seizures (group 2, n = 5), isolated seizures (group 3, n = 4), and 1 patient was asymptomatic. Methods/Results: All patients without neurological symptoms (group 1 and the asymptomatic patient) had pedunculated lesion (diameter 6.4 ± 3.6 (3–15) mm), suspended from the floor of the third ventricle. All patients with neurological symptoms (groups 2 and 3) had sessile lesion (diameter 18.3 ± 9.6 (10–38) mm, p = 0.0005 compared to the others), located in the interpeduncular cistern with extension to the hypothalamus. Seven patients were overweight. The growth hormone peak, free thyroxine, cortisol and prolactin concentrations, and the concomitant plasma and urinary osmolalities were normal in all the cases evaluated. The mean predicted or adult heights of 10 patients treated 5.2 ± 3.3 years for CPP with gonadotropin hormone releasing hormone (GnRH) analog were –0.3 ± 1.7 SD, similar to their target height –0.1 ± 0.9 SD. Conclusion: The clinical presentation of HH depends on its anatomy: small and pedunculated HH are associated with CPP, while large and sessile HH are associated with seizures. The hypothalamic-pituitary function in these cases is normal, which suggests that the absence of CPP is not due to gonadotropin deficiency. GnRH analog treatment preserves the growth potential in those with CPP.

© 2002 S. Karger AG, Basel


References

  1. Chemaitilly W, Trivin C, Adan L, Gall V, Sainte-Rose C, Brauner R: Central precocious puberty: Clinical and laboratory features. Clin Endocrinol 2001;54:289–294.
  2. Pescovitz OH, Comite F, Hench K, Barnes K, McNemar A, Foster C, Kenigsberg D, Loriaux L, Cutler GB: The NIH experience with precocious puberty: Diagnostic subgroups and response to short-term luteinizing hormone-releasing hormone analogue therapy. J Pediatr 1986;108:47–54.
  3. Judge DM, Kulin HE, Page R, Santen R, Trapukdi S: Hypothalamic hamartoma: A source of luteinizing hormone-releasing factor in precocious puberty. N Engl J Med 1977;296:7–10.
  4. Barral V, Brunelle F, Brauner R, Rappaport R, Lallemand D: MRI of hypothalamic hamartomas in children. Pediatr Radiol 1988;18:449–452.
    External Resources
  5. Sempe M, Pedron G, Roy Pernot MP: Auxologie, méthode et séquences. Paris, Theraplix, 1979.
  6. Rolland-Cachera MF, Cole TJ, Sempé M, Tichet J, Rossignol C, Charraud A: Body mass index variations: Centiles from birth to 87 years. Eur J Clin Nutr 1991;45:13–21.
  7. Marshall WA, Tanner JM: Variations in the pattern of pubertal changes in girls. Arch Dis Child 1969;44:291–303.
  8. Marshall WA, Tanner JM: Variations in the pattern of pubertal changes in boys. Arch Dis Child 1970;45:13–23.
  9. Tanner JM, Goldstein H, Whitehouse RH: Standards for children’s height at ages 2–9 years allowing for height of parents. Arch Dis Child 1970;47:755–762.
  10. Greulich WW, Pyle SI: Radiographic Atlas of Skeletal Development of the Hand and Wrist, ed 2. Stanford, Stanford University Press, 1959.
  11. Bayley N, Pinneau SR: Tables for predicting adult height from skeletal age: Revised for use with Greulich Pyle hand standards. J Pediatr 1952;50:432–441.
  12. Oerter KE, Uriarte MM, Rose SR, Barnes KM, Cutler GB Jr: Gonadotropin secretory dynamics during puberty in normal girls and boys. J Clin Endocrinol Metab 1990;71:1251–1258.
  13. Cacciari E, Fréjaville E, Cicognani A, Pirazzoli P, Frank G, Balsamo A, Tassinari D, Zappulla F, Bergamaschi R, Cristi GF: How many cases of true precocious puberty in girls are idiopathic? J Pediatr. 1983;102:357–360.
  14. Arita K, Ikawa F, Kurisu K, Sumida M, Harada K, Uozumi T, Monden S, Yoshida J, Yoshikazu N: The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. J Neurosurg 1999;91:212–220.
  15. Boyko OB, Curnes JT, Oakes WJ, Burger PC: Hamartomas of the tuber cinereum: CT, MR, and pathological findings. AJNR 1991;12:309–314.
  16. Harada K, Yoshida J, Wakabayashi T, Okabe H, Sugita K: A super long-acting LH-RH analogue induces regression of hypothalamic hamartoma associated with precocious puberty. Acta Neurochir 1995;137:102–105.
    External Resources
  17. Jung H, Carmel P, Schwartz MS, Witkin JW, Bentele KHP, Westphal M, Piatt JH, Costa ME, Cornea A, Ma YJ, Ojeda SR: Some hypothalamic hamartomas contain transforming growth factor α, a puberty-inducing growth factor but no luteinizing hormone-releasing hormone neurons. J Clin Endocrinol Metab 1999;84:4695–4701.
  18. Berkovic SF, Kuzniecky RI, Andermann F: Human epileptogenesis and hypothalamic hamartomas: New lessons from an experiment of nature. Epilepsia 1997;38:1–3.
  19. Mahachoklertwattana P, Kaplan S, Grumbach MM: The luteinizing hormone-releasing hormone-secreting hypothalamic hamartoma is a congenital malformation: Natural history. J Clin Endocrinol Metab 1993;77:118–124.
  20. Feuillan PP, Jones JV, Barnes K, Oerter-Klein K, Cutler GB: Reproductive axis after discontinuation of gonadotropin-releasing hormone analog treatment of girls with precocious puberty: Long-term follow-up comparing girls with hypothalamic hamartoma to those with idiopathic precocious puberty. J Clin Endocrinol Metab 1999;84:44–49.
  21. Tsugu H, Fukushima T, Nagashima T, Utsunomiya H, Tomonaga M, Mitsudome A: Hypothalamic hamartoma associated with multiple congenital abnormalities. Two patients and a review of reported cases. Pediatr Neurosurg 1998;29:290–296.
  22. Fujiwara I, Kondo Y, Iinuma K: Oral-facial-digital syndrome with hypothalamic hamartoma, postaxial ray hypoplasia of the limbs, and vagino-cystic communication: A new variant? Am J Med Genet 1999;83:77–81.
  23. Zuniga OF, Tanner SM, Wild WO, Mosier HD Jr: Hamartoma of CNS associated with precocious puberty. Am J Dis Child 1983;137:127–133.
    External Resources
  24. Uriarte MM, Oerter Klein K, Barnes KM, Pescovitz OH, Loriaux DL, Cutler GB: Gonadotrophin and prolactin secretory dynamics in girls with normal puberty, idiopathic precocious puberty and precocious puberty due to hypothalamic hamartoma. Clin Endocrinol 1998;49:363–368.
  25. Comite F, Pescovitz OH, Rieth KG, Dwyer AJ, Hench K, McNemar A, Loriaux L, Cutler GB: Luteinizing hormone-releasing hormone analog treatment of boys with hypothalamic hamartoma and true precocious puberty. J Clin Endocrinol Metab 1984;59:888–892.
  26. De Brito VN, Latronico AC, Arnhold IJP, Lo LSS, Domenice S, Albano MCC, Fragoso MCBV, Mendonca BB: Treatment of gonadotropin-dependent precocious puberty due to hypothalamic hamartoma with gonadotropin releasing hormone agonist depot. Arch Dis Child 1999;80:231–234.

Article / Publication Details

First-Page Preview
Abstract of Original Paper

Published online: January 31, 2002
Issue release date: 2001

Number of Print Pages: 7
Number of Figures: 1
Number of Tables: 3

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: https://www.karger.com/HRP


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