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Exp Clin Endocrinol Diabetes 2003; 111(3): 162-167
DOI: 10.1055/s-2003-39788
Article

J. A. Barth Verlag in Georg Thieme Verlag Stuttgart · New York

Endocrine Findings in Patients with Optico-Hypothalamic Gliomas

R. Martínez[*] 1 , J. Honegger[*] 1 , R. Fahlbusch 1 , M. Buchfelder 1
  • 1Department of Neurosurgery, University of Erlangen-Nürnberg, Erlangen, Germany
Further Information

Publication History

Received: March 19, 2002 First decision: May 6, 2002

Accepted: August 13, 2002

Publication Date:
04 June 2003 (online)

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Abstract

Objectives

Optico-hypothalamic gliomas (OHG) are an important differential diagnosis in suprasellar tumors. Visual impairment and hydrocephalus are the most frequent findings at presentation. However, only limited data are available about endocrine disturbances in these lesions.

Patients and Methods

38 consecutive patients (19 children) were studied retrospectively. Clinical assessment and endocrinological evaluation of pituitary and hypothalamic functions (combined pituitary stimulation test, ITT, CRH-GRH test) were performed before and after (1 week and 3 months) the neurosurgical procedure.

Results

Only three patients showed clinical features of endocrine deficiency initially. 16 children and 15 adults out of the 38 patients (31/38, 81.6 %) showed no abnormality in their hormone values when assessed with the combined pituitary stimulation test alone or using more sophisticated investigations such as ITT and CRH-GRH test (72.2 %). Patients with hypothalamic disturbances (8 out of 38, 21 %) had endocrine abnormalities more frequently than those without (57.1 % vs. 9 %, p < 0.05), as assessed by ITT and CRH-GRH-test.

Conclusions

In contrast to other suprasellar tumors, endocrine deficiencies are surprisingly rare in optico-hypothalamic gliomas despite their large size, especially in the absence of hypothalamic disorders. This may be used as a criterion in the differential diagnosis of these lesions, in addition to the radiological findings.

Key words

Optico-hypothalamic glioma - endocrine deficiency - pituitary - cachexia

References

  • 1 Allen J C. Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1.  Pediatr Neurosurg. 2000;  32 154-162
    CrossrefPubMedSearch in Google Scholar
  • 2 Alshail E, Rutka J T, Becker L E, Hoffman H J. Optic chiasmatic-hypothalamic glioma.  Brain Pathol. 1997;  7 799-806
    PubMedSearch in Google Scholar
  • 3 Attie K M, Ramirez N R, Conte F A, Kaplan S L, Grumbach M M. The pubertal growth spurt in eight patients with true precocious puberty and growth hormone deficiency: evidence for a direct role of sex steroids.  J Clin Endocrinol Metab. 1990;  71 975-983
    PubMedSearch in Google Scholar
  • 4 Baba H, Ryu N, Mori K, Yoshimoto M, Yamashita A, Tsuji Y. Hypothalamic glioma with diencephalic syndrome and following precocious puberty. A case report.  No To Shinkei. 1989;  41 1029-1035
    PubMedSearch in Google Scholar
  • 5 Buchfelder M, Fahlbusch R, Walther M, Mann K. Endocrine disturbances in suprasellar germinomas.  Acta Endocrinol (Copenh). 1989;  120 337-342
    PubMedSearch in Google Scholar
  • 6 Carella C, Rotondi M, Del Buono A, Sinisi A M, Del Basso de Caro M L, Mone C M, Vizioli L, Sorvillo F, Mazziotti G, Bellastella A. Diabetes insipidus and increased serum levels of leptin and lactate-dehydrogenase (LDH) in an adolescent boy with a primary intracranial germinoma. Case report and an endocrinological revaluation of literature.  J Endocrinol Invest. 1999;  22 558-561
    PubMedSearch in Google Scholar
  • 7 Collet-Solberg P F, Sernyak H, Satin-Smith M, Katz L L, Sutton L, Molloy P, Moshang Jr T. Endocrine outcome in long-term survivors of low-grade hypothalamic/chiasmatic glioma.  Clin Endocrinol (Oxf). 1997;  47 79-85
    CrossrefPubMedSearch in Google Scholar
  • 8 Constine L S, Woolf P D, Cann D, Mick G, McCormick K, Raubertas R, Rubin P. Hypothalamic-pituitary dysfunction after radiation for brain tumors.  N Eng J Med. 1993;  328 87-94
    PubMedSearch in Google Scholar
  • 9 Dolenc V V. Hypothalamic gliomas.  Adv Tech Stand Neurosurg. 1999;  25 161-194
    PubMedSearch in Google Scholar
  • 10 Erkal H S, Serin M, Cakmak A. Management of optic pathway and chiasmatic-hypothalamic gliomas in children with radiation therapy.  Radiother Oncol. 1997;  45 11-15
    CrossrefPubMedSearch in Google Scholar
  • 11 Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M. Surgical treatment of craniopharyngiomas: experience with 168 patients.  J Neurosurg. 1999;  90 237-250
    Search in Google Scholar
  • 12 Fröhlich A. Ein Fall von Tumor der Hypophysis cerebri ohne Akromegalie.  Wien Klein Rundschau. 1901;  1 883-906
    PubMedSearch in Google Scholar
  • 13 Ganz J C, Smievoll A I, Thorsen F. Radiosurgical treatment of gliomas of the diencephalon.  Acta Neurochirur Suppl (Wien). 1994;  62 62-66
    PubMedSearch in Google Scholar
  • 14 Garvey M, Packer R J. An integrated approach to the treatment of chiasmatic-hypothalamic gliomas.  J Neurooncol. 1996;  28 167-183
    PubMedSearch in Google Scholar
  • 15 Grabenbauer G G, Schuchardt U, Buchfelder M, Rodel C M, Gusek G, Marx M, Doerr H G, Fahlbusch R, Huk W J, Wenzel D, Sauer R. Radiation therapy of optico-hypothalamic gliomas (OHG)-radiographic response, vision and late toxicity.  Radiother Oncol. 2000;  54 239-245
    CrossrefPubMedSearch in Google Scholar
  • 16 Gropman A, Packer R, Nicholson H, Vezina L, Jakacki R, Geyer R, Olson J, Phillips P, Needle M, Broxson E, Reaman G, Finlay J. Treatment of diencephalic syndrome with chemotherapy. Growth, tumor response and long-term control.  Cancer. 1998;  83 166-172
    CrossrefPubMedSearch in Google Scholar
  • 17 Honegger J, Buchfelder M, Fahlbusch R. Surgical treatment of craniopharyngiomas: endocrinological results.  Neurosurg. 1999;  90 251-257
    PubMedSearch in Google Scholar
  • 18 Kaplan S L, Grumbach M. Pathophysiology and treatment of sexual precocity.  J Clin Endocrinol Metab. 1990;  71 785-789
    PubMedSearch in Google Scholar
  • 19 Kato T, Sawamura Y, Tada M, Ikeda J, Ishii N, Abe H. Cisplatin/vincristine chemotherapy for hypothalamic/visual pathway astrocytomas in young children.  J Neurooncol. 1998;  37 263-270
    CrossrefPubMedSearch in Google Scholar
  • 20 Packer R J. Chemotherapy: low-grade gliomas of the hypothalamus and thalamus.  Pediatr Neurosurg. 2000;  32 259-263
    CrossrefPubMedSearch in Google Scholar
  • 21 Perilongo G, Carollo C, Salviati L, Murgia A, Pillon M, Basso G, Gardiman M, Laverda A. Diencephalic syndrome and disseminated juvenile pilocytic astrocytomas of the hypothalamic-optic chiasm region.  Cancer. 1997;  80 142-146
    CrossrefPubMedSearch in Google Scholar
  • 22 Poussaint T Y, Barnes P D, Nichols K, Anthony D C, Cohen L, Tarbell N J, Goumnerova L. Diencephalic syndrome: clinical features and imaging findings.  Am J Neuroradiol. 1997;  18 1499-1505
    PubMedSearch in Google Scholar
  • 23 Ramelli G P, von der Weid N, Stanga Z, Mullis P E, Buergi U. Suprasellar germinomas in childhood and adolescence: diagnostic pitfalls.  J Pediatr Endocrinol Metab. 1998;  11 693-697
    PubMedSearch in Google Scholar
  • 24 Rodriguez L A, Edwards M S, Levin V A. Management of hypothalamic gliomas in children. An analysis of 33 cases.  Neurosurg. 1990;  26 242-246
    PubMedSearch in Google Scholar
  • 25 Russell A. A diencephalic syndrome of emaciation in infancy and childhood.  Arch Dis Child. 1951;  26 274
    PubMedSearch in Google Scholar
  • 26 Souweidane M M, Hoffman H J. Current treatment of thalamic gliomas in children.  J Neurooncol. 1996;  28 157-166
    PubMedSearch in Google Scholar
  • 27 Steiger H J, Götz C, Schmid-Elsaesser R, Stummer W. Thalamic astrocytomas: surgical anatomy and results of a pilot series using maximum microsurgical removal.  Acta Neurochir (Wien). 2000;  142 1327-1337
    CrossrefPubMedSearch in Google Scholar
  • 28 Stewart L, Steinbok P, Daaboul J. Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty. Report of six cases.  J Neurosurg. 1998;  88 340-345
    PubMedSearch in Google Scholar
  • 29 Sutton L N, Molloy P, Sernyak H, Goldwein J, Phillips P, Rorke L, Moshang T, Lange B, Packer R. Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery.  J Neurosurg. 1995;  83 583-589
    PubMedSearch in Google Scholar
  • 30 Tanabe M, Watanabe T, Hori T. Von Recklinghausen's disease with diencephalic syndrome in an adult.  J Neurosurg. 1994;  80 556-558
    PubMedSearch in Google Scholar
  • 31 Tanner J M, Whitehouse R H. Clinical longitudinal standards for height, weight, height velocity, weight velocity and stages of puberty.  Arch Dis Child. 1976;  51 170-179
    PubMedSearch in Google Scholar

1 These authors contributed equally to this paper.

M. D. R. Martínez

Neurochirurgische Klinik, Klinikum Fulda

Pacelliallee 4

36043 Fulda

Germany

Phone: +49661

Fax: +49661845802

Email: rmartinez3108@hotmail.com