Clin Pediatr Endocrinol 1994;3(Suppl 4): 169-173Copyright(C)1994 by The Japanese Society for Pediatric Endocrinology

A Boy with Optic Glioma

Taisuke Okada, Sumitaka Dohno, Yousei Shimasaki, Takashi Tomoda, Makiko Koga,Kumiko Araki and Takanobu Kurashige

Department of Pediatrics, Kochi Medical School, Kohasu, Oko-cho, Nankoku-city, Kochi, Japan

Abstract. A 6-month-old boy had nystagmus. Hydrocephalus and a large hypothalamic tumor

were found by MRI. After partial resection of the tumor, diabetes insipidus (DI) and ACTH

deficiency had developed. The tumor was diagnosed as optic astrocytoma, histologically.

Although he had been treated with dexamethasone, DI was not well controlled. At 1.5 years old,

he was admitted to our hospital with polyuria, obesity and hypertrichosis. The growth chart

showed that he had grown rapidly from birth. The serum level of GH was normal, but insulin like

growth factor (IGF)-I value was high. Serum levels of insulin and prolactin were normal. Ontreatment with low doses of hydrocortisone and DDAVP, the urine volume and serum level of

IGF-I decreased. He was diagnosed as having partial GH deficiency by intravenous insulin

stimulation test. He has blindness and slight mental retardation now. The etiology of his

overgrowth is not known clearly, but some other unknown growth factor may have stimulated

his growth.

Key words: optic glioma, overgrowth, diabetes insipidus, ACTH deficiency, IGF- I

Introduction

Gliomas of the optic nerve and chiasm arerare, and occur most commonly in childhood.

The majority of these tumors are low-grade

astrocytomas histologically, but the clinicalcourse of these tumors is not benign. Tumorsinvolving the optic chiasm and particularly

extending outside of the chiasm into thehypothalamus tend to be more aggressive

than tumors involving the optic nerve alone

(1). Chiasmatic-hypothalamic gliomas oftencause endocrine abnormalities in addition tovisual symptoms and have been associated

with the diencephalic syndrome of emaciation

(2). We report here a case of optic glioma inwhom overgrowth in both height and weight

developed from birth.

Case Report

The patient had a normal, full-term, spon-

taneous delivery. The growth chart showed

that he had grown rapidly from birth. He was

first noted to have macrocephaly and slightly

retarded head control at the age of 4 months.

Correspondence: Dr. Taisuke Okada, Department of Pediatrics,

Kochi Medical School, Kohasu, Oko-cho, Nankoku-shi, Kochi783 Japan

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OKADA et al.

Fig. 1. Magnetic resonance T1-weighted image, coronalprojection, obtained after administration ofgadolinium. Note the enhancing mass in thesupra- sellar area.

He was admitted to another hospital because of nystagmus at the age of 6 months. Hydroce-phalus and hypothalamic tumor were foundby MRI and he was referred to our hospital foroperation. MRI on admission (Fig. 1) showeda 5cm diameter suprasellar tumor which wasenhanced homogenously by administration ofgadlinium. The V-P shunt operation and par-tial resection of the tumor were performed;thereafter, diabetes insipidus (DI) and ACTHdeficiency appeared. The diagnosis ofastrocytoma was made histologically.Although he had been treated with dex-amethasone and 1-deamino-8-D-arginine vaso-pression (DDAVP) or indomethacin, DI wasnot well controlled. At age 16 months he wasreadmitted to our hospital with polyuria, obe-sity, and hypertrichosis. On admission, hisheight was 84cm (+ 2.0SD), weight 15.0kg (+3.5SD) and his head circumference was 55cm(+3.8SD) (Fig. 2). Bone age was slightlyretarded. The urine volume was about 3liters/day and the specific gravity was 1.003.Serum osmolarity was 298mOsm/kg, urine

Fig. 2. Growth Chart

osmolarity was 171mOsm/kg, and antidiuretic

hormone (ADH) was 0.8pg/ml. The level of

growth hormone (GH) was normal, but IGF-I

value was high. Urinary GH secretion was not

detected. Serum prolactin level and TSH were

within normal limits. ACTH and cortisol

levels were low (Table. 1). With administra-

tion of hydrocortisone (30mg/day) and

DDAVP (1.3pg twice a day), urine volume and

serum IGF-I level gradually decreased. At age

19 months, the dosage of hydrocortisone and

DDAVP was 17mg/day and 2.5ƒÊg twice a day

respectively. The urine volume was about 1.2

liters/day and serum values of ACTH and

cortisol were low (Fig. 3). Serum insulin value

was normal, but serum prolactin level slightly

increased. ACTH, cortisol, and GH showed

low responses to hypoglycemia by intravenous

insulin stimulation test (0.05U/kg). Rapid

ACTH test revealed low adrenal function

(Tabled). Therefore, he was diagnosed as

having partial GH deficiency. However, his

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A Boy with Optic Glioma

Table 1. Laboratory Data (1993, May)

overgrowth has continued in both height andweight. He has blindness and slight mental

retardation. The optic glioma has not shownregrowth as followed by MRI examination,

without chemotherapy or radiotherapy (Fig.4).

Discussion

Optic pathway/hypothalamic astrocytomasin childhood have long been believed to followa highly unpredictable course (3). Someremain static for many years, while othersincrease in size and can lead to significantmorbidity and even to death. Observationonly, or treatments by radiotherapy, chemo-therapy, and surgical resection have all beenproposed. Consequently, there is no uniformmethod of management. In this case, the largetumor was placed posteriorly, resulting inhydrocephalus and endocrinological dysfunc-tion without neurofibromatosis. Harold et al.say that anteriorly placed tumors restricted toone orbit and causing unsightly proptosis andsignificant visual loss had better be resected(3). Posteriorly placed tumors causinghydrocephalus and/or endocrinological

Fig. 3. Clinical Course

Fig. 4. No evidence of tumor regrowth compared to

preoperation.

dysfunction are treated with shunting andresection, with medical therapy for the endo-crinological dysfunction. As these posteriorlyplaced optic pathway tumors cannot, how-ever, undergo total surgical excision, radiationtherapy and/or chemotherapy may be needed.Radiation therapy is sure to be effective inpatients with optic glioma with involvement

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OKADA et al.

of the chiasm and posterior structures (4)(5),

but the side effects of irradiation can be seri-ous (6), so chemotherapy should be consideredin the initial management following resection

(7). In our patient the symptoms did notprogress on clinical and radiographic evalua-tion without chemotherapy and radiationtherapy. He has to be followed carefully byMRI and further treatment may be needed if

the tumor should progress.Clinical manifestations of optic glioma

may be decreased vision, headache or endo-

crine dysfunction. A variety of endocrinedysfunctions are reported such as precocious

puberty, GH deficiency, delayed puberty, DI,hypothyroidism, and hypopituitarism. Our

case had DI, ACTH deficiency, and probably

partial GH deficiency. However, he had over-

growth from birth. Overgrowth in patientswith optic glioma has not previously beenreported, to our knowledge. The etiology ofthis overgrowth is unknown. Overgrowth

already developed before administration ofhydrocortisone and contined during therapy.As he had partial GH deficiency, it was sug-

gested that some growth factor, which wassecreted by the tumor, might have acceleratedhis growth from birth. The level of IGF-I washigh in spite of GH deficiency, but it de-.

creased by reducing the dose of hydrocor-tisone. Therefore, administration of hydrocor-

tisone was thought to induce the high level ofIGF-I. Although IGF-I producing astrocytomahas been reported (8), there is no report of

optic glioma that produced some growth fac-tor. On the other hand, it has been reported

that some patients with brain tumor hadnormal or accelerated growth in spite of GH

deficiency (9). The mechanisms of the growthin these cases have not been clarified. These

patients had hyperphagia and severe obesity.A high level of serum insulin or hyper-

prolactinemia can be associated with a normalIGF-I value in GH deficient patients. In our

case, the serum value of insulin was normal,but prolactin was at a slightly high level. As

overgrowth appeared when the serum

prolactin level was normal, prolactin might

have no direct relation to his overgrowth.

Therefore, some other growth factor secreted

by the tumor may have acted as a stimulating

factor. We must evaluate the bioactivity of

IGF-I to clarify the mechanism of his over-

growth.

Conclusion

1. We have reported here a case of optic

glioma who had DI, ACTH deficiency,

partial GH deficiency and overgrowth.2. DI and ACTH deficiency was well

controlled by replacement therapy, but the

etiology of overgrowth has not been deter-

mined. Some other unknown growth factor

may act as a stimulating factor.

3. 8 months after operation, the residual

tumor had not regrown by MRI and he was

watched, without chemotherapy or radio-

therapy. If the tumor progresses further

treatment may be needed.

References

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3. Harold JH, Robin PH, James MD, LaurenceEB, Derek J, et al. Optic pathway/hypoth-alamic gliomas: a dilemma in manage-ment. Pediatr Neurosurg 1993; 186-95.

4. Bataini JP, Delanian S, Ponvert D. Chias-mal gliomas: results of irradiation manage-ment in 57 patients and review of litera-ture. Int J Radiation Oncology Biol Phys1991; 21: 615-23.

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