MULTIPLE PITUITARY HORMON DEFICIENCY

IN A GIRL WITH PILOCYTIC ASTROCYTOMA

Case Report
ABSTRACT
Background: Multiple Pituitary Hormone Deficiency (MPHD) is most often
caused by a benign tumor of the pituitary gland, or of the brain in the region of
the hypothalamus. Pituitary underactivity may be caused by the direct pressure
of the tumor mass on the normal pituitary or by the effects of surgery or
radiotherapy used to treat the tumor. Pilocytic astrocytoma In the hypothalamic
area may present with pituitary hormonal dysfunctions. Patients should be
made aware of the possibility of hypopituitarism before and after surgery.

Objective: To present a case of multiple pituitary hormone deficiency in a girl

with pilocytic astrocytoma focusing in diagnostic and management.

Case: 10-years old girl was admitted at Soetomo Hospital Surabaya consulted
from Neuro Surgery Department due to intracranial tumor with main complain of
weakness. From physical examination she had short stature and from
laboratory finding Cortisol (morning) 0.43 ng/dL revealed hypocortisol Head MRI
shown mass which compressed chiasma opticum and ventricle III to cavum
orbita enchasing eyes muscle and optic nerve . she got craniectomy and tumor
excision. From biopsy patology anatomy result Pilocytic astrocytoma. After the
surgery she also suffered from hypothyroid. She already treat with hormone
replacement theraphy and waiting for the radiation.

Summary: A case of multiple pituitary hormone deficiency in a girl with Pilocytic

astrocytoma has been presented. Patients should be made aware of the
possibility of hypopituitarism before and after surgery. The clinical presentation
of the MPHD patient can vary from asymptomatic or subclinical cases to life-
threatening. PAs are benign tumors, but some clinicopathological factors, such
as partial resection, optochiasmatic location, invasion of surrounding structures,
have a worse prognosis.

Keyword: pituitary, pilocytic astrocytoma, cortisol, hypothyroid, short stature

1
INTRODUCTION

Multiple Pituitary Hormone Deficiency (MPHD) describe the condition

when the pituitary is not producing two or more of these hormones. The
prevalence of 45/100 000 and an incidence of about 4/100 000/year in the
normal population, with nearly 50% of patients having three to five pituitary
hormone deficits. It is a complex medical condition associated with a higher
mortality than that of the general population. MPHD is most often caused by a
benign tumor of the pituitary gland, or of the brain in the region of the
hypothalamus. Pituitary underactivity may be caused by the direct pressure of
the tumor mass on the normal pituitary or by the effects of surgery or
radiotherapy used to treat the tumor. Less frequently, hypopituitarism can be
caused by infections (such as meningitis) in or around the brain or by severe
blood loss, by head injury, or by various rare diseases such as sarcoidosis. 1
Astrocytoma are tumors that arise from a type of glial cell in the nervous
system. Pilocytic astrocytoma (PA) is the commonest brain tumor in the ages 5-
14 years and comprise of 19.1% of all pediatric brain tumors in the age group 0
– 14 years and have been reported to be the most common gliomas and
cerebellar tumors in children. Pilocytic astrocytoma affects males and females
equally, and the main affected ages are 6 to 13 years-old, with 75% of cases
occurring at this ages. Pilocytic astrocytoma are associated with a relatively
benign clinical course. 2
Pilocytic astrocytoma In the hypothalamic area may present with
hormonal dysfunctions. The involvement of the optical pathway is related to
visual loss or visual-field deficits; proptosis presents only in large, intraorbital
tumors. Occurrence of hypopituitarism after pituitary surgery is influenced by
tumor size, extent of invasion into surrounding structures, remaining viable
normal pituitary, and the skill of the neurosurgeon. Transient postoperative
diabetes insipidus (DI) is seen in 5% of cases but permanent DI occurs less
frequently. At least partial recovery of pituitary function in 40%–65% of patients
has been reported after surgery, but postoperative deterioration has also been
reported. Patients should be made aware of the possibility of hypopituitarism
before and after surgery. Pituitary apoplexy refers to abrupt destruction of

2
pituitary tissue after infarction/ hemorrhage within the tumors/ non-tumors
pituitary gland, clinically characterized by acute onset headache, vomiting,
visual impairment, and meningism. Partial or complete, transient or permanent
hypopituitarism may result. 2,3
The diagnosis from clinical examination, neurological examination, visual
field examination, biochemical, and head MRI. The treatment of MPHD includes
therapies directed at the underlying disease process, and endocrine
replacement therapy. The pituitary tumors may be treated with medical therapy,
surgery, radiotherapy, or a combination of these modalities. They have a fairly
good prognosis with a five-year survival rate of 80 – 90% and a 10-year survival
rate of 81%, depending on the tumor location and extent of resection. This may
reflect the tumor’s slow growth and discrete nature, which allow complete
surgical resection. The patients who underwent partial surgical removal, only
invasion of the surrounding structures was related to prognosis. PAs are benign
tumors, but some clinicopathological factors, such as partial resection,
optochiasmatic location, invasion of surrounding structures, have a worse
prognosis.4.5

The purpose of this case report is to present a case of multiple pituitary

hormone deficiency in a girl with pilocytic astrocytoma focusing in diagnostic
and management.

3
CASE REPORT

N, female, 10 years old consulted to Pediatric Department from neuro

surgery with main complaint weakness since a month before admission her
mother said that she always stay at her bed all day and she got severe
headache since three months before admission. Sometimes she got vomiting
after suffered from severe headache. She never complained about weight loss,
with good appetite, no fever, never got cough before, never gone to pee after
midnight and with good urine production. Her mother complained about her
height was shorter than her friends, but she didn’t noticed since when she got
shorter than her friends. She had history of protruded the left eye got headache
and toothache since a year before admission. She had already consulted to the
doctor given some medication but cannot relieved yet. Six months before
admission the protruded eye getting larger and got blurry vision and she
consulted to the Undaan hospital and finally referred to Soetomo hospital. At
neuro surgery department Soetomo hospital diagnosed as a hydrochepalus
communicant and retrobulbar tumor and underwent VP shunt, she planned to
got excision the tumor but the family refused because of financial problem,. She
came again to the emergency department after a month after the VP shunt and
she already totally lost her vision. She had no history of seizure, tremor or
decreased of consciousness and did not got the menarche yet..

She was born spontaneously by midwife with 9 month of gestational age.

The birth weight was 3000 gram. The mother was in healthy condition. No
history of jaundice and cyanosis during pregnancy and after delivery. It was
recorded good antenatal care and good growth and developmental history.
History of seizure was denied.
Physical examinations at the first admission revealed a weak alert girl,
with body weight 18 kg, height 118 cm, with Body mass index 81% with good
nutrition state. Growth chart shown that she got short stature, with Mid parental
height 144-161 cm. From physical examination revealed: Blood pressure
100/60 mmHg Pulse rate 100 times per minute, respiratory rate 24 times per
minute, and temperature 36.5 º C. There were no anemia, icterus, cyanosis, or
dyspnea. From the head and neck examination found protruded the left eyes.

4
The chest moved symmetrically without any retraction, the breath and heart
sounds were normal. The abdomen was tender, the bowel sound was normal,
the liver and the spleen were not palpable. The extremities were well perfused,
without any edema. From external genitalia revealed prebubertal with no pubic
hair, papilla breast only slight elevation, and no axillar hair yet.
The neurological examination found GCS 456, pupil were isocor 3mm
with positive light reflex, meningeal sign was not found. Upper and lower
extremity muscle strength within normal limit. No Increasing physiologic reflex in
the tendons. Pathological reflexes : Babinski and Chaddock reflexes were
negatives. Other cranial nerves examination was normal. There were no
cerebellar signs.

5
 Figure 1. Patient’s Growth Chart
Source: CDC Growth Chart standart 6

From the eye examination palpebral the left eye got proptosis, did not
found any edema or spasm, from intraocular pressure there were normal
palpation on the lef and right sight. On conjungtiva can not find hyperemia. Left
anad right cornea were clear. Both anterior chamber were deep, with iris radier,
pupil round and isocoric with diameter 5 mm each. Visual field were only light
respone. The lens were clear and from funduscopy were found positive fundal

6
reflex, no hemorrhage retina micoanerysm no exudate and with positive
macula reflex, o the both side, but on the left side found optic nerve head was
blurred margin and hyperemia and on the right side optic nerve head was
blurred margin and the color pale.
Based on the history and physical examination our working diagnosis
were left eye proptosis suspicious intracranial tumor + multiple pituitary
hormone deficiency ( short stature, susp hypocortisol, susp hypotiroid). We
planned to have examined complete blood count, serum electrolyte, cortisol
serum, thyroid function test, urine and blood culture. thorax x-ray, head MRI and
collecting urine 24 hours.
The laboratory examination complete blood count, revealed normal limit:
Hemoglobin: 14.2 g/dL, Leukocytes: 17300/uL, Platelets: 341.000 g/dL. Serum
electrolyte Sodium 137 mmol/L, Potasium 3.8 mmol/L, Chloride 102 mml/L and
Calcium 9.1 mg/dl. BUN 16 mg/dl, creatinin serum 0.5 mg/l, albumin 3.2 g/dl,
ALT 16 U/L, AST 70 U/L, Blood glucose 118 mg/dl, PPT 10.3 second with
control 12 second, APTT 26.8 second with control 33 second. Cortisol
(morning) 0.43 ng/dL ( 4.3 -22.4) . Thyroid function test: FT4 1.33 ng/dL (0.89-
1.76) TSH 0.500 IU/mL (0.35-5.5). CRP 0.7. Chest X-ray evaluation revealed no
abnormalities.

Figure 2. Patient’s Thorax X-ray within normal limit

7
8
 Figure 3. Patient’s Head MRI
The head MRI revealed heterogenous enhanching mass with size 5.31 x 4.69 x
5.08 cm expanded to superior, which compressed chiasma opticum and
ventricle III to anterior to cavum orbita enchasing eyes muscle and optic nerve
suspicious craniopharyngioma.
Based on history, clinical manifestation and laboratorium and radiology
finding ,the patients was diagnosed as left eye proptosis susp.
Craniopharyngioma differential diagnose with hypotalamic glioma + Bilateral
chronic papil edema+ multiple pituitary hormone deficiency ( short stature,
hypocortisol,). The patients planned to get surgery, gave hormone replacement
hydrocortisone therapy and used the crisis dose before started the operation.
Planned to had blood and urine serum electrolyte examination if she got
polyuria . Monitoring vital sign, fluid balance 24 hours and urine production.

9
On 1st day before surgery, she still awake with alert condition. No fever and no
seizure. No vomiting. Vital sign: blood pressure 100/60 pulse 100 tpm,
Respiratory Rate: 24 tpm, temp 37 C . Good urine production with
1.2cc/kg/hour. We planned to gave hydrocortisone before surgery
• 24 hour pre Op  Hydrocortison Na succinate 20 mg po
• 12 hour pre Op  Hydrocortison Na succinate 20 mg po
• 1 hour pre Op  Hydrocortison Na succinate 50 mg iv
• Durante Op  Hydrocortison Na succinate 20 mg iv
• 24hours post Op Hydrocortison Na succinate 8x25 mg iv
• 2 days post Op  Hydrocortison Na succinate 8x12.5 mg iv
• 3 days post Op  Hydrocortison Na succinate 10-10-20mg
• 4days post Op  Hydrocortison Na succinate 5-5-10 mg

During operation the patient got craniotomy tumor excision with osteoplasty.
The freeze coup of the tumor revealed Glioma, from neuro surgery was
success to removed 90% of the tumor and awaiting for the biopsy pathologic
anatomy result. During the operation she got Hydrocortison Na succinate 20
mg injection intravenous. Post operation, patient weak, with stable condition
transferred to ICU GBPT and continued the Hydrocortison Na succinate
injection until 24 hour post operation.

On 2nd day after operation, the patient was alert with GCS 456 blood pressure
110/60 pulse 110 tpm, Respiratory Rate: 24 tpm, temp 37 C. Good urine
production 1.4cc/kg/hour.
The laboratory examination revealed complete blood count, Hemoglobin: 10.10
g/dL, Leukocytes: 19.100/uL, Platelets: 317.000 g/dL. The diagnose was left
eye proptosis + s. hypotalamic glioma + bilateral chronic papil edema + multiple
pituitary hormone deficiency ( short stature, hypocortisol) + post tumor excision
d-2. We gave PRC transfusion 1 day, and Hydrocortison Na succinate 8x12.5
mg iv

10
On 4th day after operation, the patient was decreased of consciousness with
GCS 224 got fever, no vomiting and no seizure, with good urine production
1.7cc/kg/hour. Vital sign: blood pressure 90/60 pulse 110 tpm, Respiratory
Rate: 28 tpm, temp 38 C.
The laboratory examination revealed complete blood count, Hemoglobin: 11.06
g/dL, Leukocytes: 12.400/uL, Platelets: 166.300 g/dL. Serum electrolyte Na:
136mmol/L, K 2.8 mmol/L, Chloride 99 mmol/L. Albumin 2.10 g/dl, BUN 16
mg/dl, Creatinin serum 0.5 mg/l, ALT 23 U/L, AST 44 U/L, Blood glucose 154
mg/dl, CRP 17.40. Blood and urine culture was steril. We gave albumin
transfusion, correction of serum electrolyte, thermoregulation and gave the
Hydrocortison Na succinate 5-5-10 mg

On 6th day after operation, the patient was still decreased of consciousness with
GCS 224 no fever anymore , no vomiting and no seizure with good urine
production 2.0 cc/kg/hour. Vital sign: blood pressure 100/50 pulse 100 tpm,
Respiratory Rate: 24 tpm, temp 37 C.
The laboratory examination revealed Serum electrolyte Na: 121 mmol/L, K 2.6
mmol/L, Cl 81 mmol/L. Albumin 2.6 g/dL, BUN 8 mg/dl, creatinin serum 0.2
mg/l, ALT 32 U/L, AST 65 U/L, Blood glucose 74 mg/dl, CRP 13.40,
procalcitonin 0.43, Cortisol (morning) 9.95 ng/dL ( 4.3 -22.4), Cortisol (evening)
4.07 ng/dL (3.09-16.66) . Thyroid function test: FT4 0.6 ng/dL (0.89-1.76) TSH
0.04 IU/mL (0.35-5.5). The diagnose was left eye proptosis + s. hypotalamic
glioma + bilateral chronic papil edema + multiple pituitary hormone deficiency (
short stature, hypocortisol, hypothyroid) + post tumor excision d-6. We gave
transfusion albumin, correction of serum electrolyte, and gave the
Hydrocortison Na succinate 5-5-10 mg, and L-thyroxin 1x 75mcg

On 8th day after operation, the patient still decreased of consciousness with
GCS 224 no fever anymore , no vomiting and no seizure with good urine
production 1.9 cc/kg/hour. Vital sign: blood pressure 100/60 pulse 112 tpm,
Respiratory Rate: 24 tpm, temp 37 C.

11
The laboratory examination revealed Serum electrolyte Na: 138 mmol/L, K 3.2
mmol/L, Chloride 103 mmol/L. Albumin 3.5 g/dL, complete blood count,
Hemoglobin: 12.03 g/dL, Leukocytes: 8210 /uL, Platelets: 254.000 g/d. The
Pathological anatomy result: Pilocytic Astrocytoma (WHO grade I). The
diagnose was become Pilocytic Astrocytoma (WHO grade I) + bilateral chronic
papil edema + multiple pituitary hormone deficiency ( short stature, hypocortisol,
hypothyroid) + post tumor excision d-8. We planned to consulted to pediatric
neurologic division and we gave the Hydrocortison Na succinate 5-5-10 mg, and
L-thyroxin 1x 75mcg.

On 12th day after operation, the patient still with GCS 225 no fever, no vomiting
and no seizure also with good urine production 1.9 cc/kg/hour. Vital sign: blood
pressure 100/70 pulse 110 tpm, Respiratory Rate: 24 tpm, temp 37 C.
Neurology division advise to had radiotherapy to reduced the tumor. We
planned to got appointment with Radiotherapy department to got preparation
for radiation, and Bone age evaluation.

This patient still under observation because of the unstable condition and we
plan to get Luteinizing hormone (LH), Follicle Stimulating Hormone (FSH),
Progesteron and Growth Hormon (GH) but cannot perform yet because of the
financial problem.

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DISCUSSION
N, female, 10 years old consulted to Pediatric Department from neuro
surgery with main complaint weakness since a month before admission and got
severe headache since three months before admission. Sometimes she got
vomiting after suffered from severe headache.
Weakness has many causes, both neurogical and non neurogical. Non
neurogical causes of weakness include electrolyte abnormalities, severe
infection, or neoplastic with endocrinopaty and neurogical causes are
musculoskletal disorder or cerebrovascular disease. 7
In our case the patient came with main complaint of weakness and got
vomiting when she suffered from severe headache. from the physical
examination she got good vital sign no hypertension, pulse 100 bpm and
respiratory rate 24 tpm, from the laboratorium finding electrolyte was Sodium
137 mmol/L, Potasium 3.8 mmol/L, Chloride 102 mml/L and Calcium 9.1 mg/dl
eithin normal limit so the weakness caused by electrolyte abnormalities could
be excluded.
In our case the patient weaknes but no complaint of fever, no cough,
from physical examination temperature was 36.5 C and complete blood count
revealed Hemoglobin: 14.2 g/dL, Leukocytes: 17300/uL, Platelets: 341.000
g/dL. CRP level 0.7 dan blood and urine culture was steril so the cause severe
infection could be excluded.
In our case the patient no seizure and no decreased of consciousness
with physical examination upper and lower extremity muscle strength and the
physiological reflex were in normal limit, no pathological reflex so neurological
causes include musculoskletal and cerebrovascular disease can excluded.
She suffered from weakness and had the history of protruded left eye,
severe headache, and vomiting. From the head MRI revealed mass on chiasma
opticum enchasing eyes muscle and optic nerve suspicious craniopharyngioma.
So the working diagnosis became suspected craniopharyngioma with the
endocrinopaty

13
 In our case the patient suffered from protruded of the left eye since 1
year before admission, from the eye examination revealed proptosis on the left
eye and bilateral papil edema, no fever, and no history of trauma.
Proptosis is mean a state of protrusion of the globe of the eye between
the lids. diagnosis of a case of proptosis is not always easy. six main types or
categories. These are:
1. Apparent: Under type 1.-the apparent cases of proptosis, the globe is not
really displaced. The cases of high myopia, buphthalmos, megalophthalmos
and also cases of 7th nerve paralysis.
2. Congenital anomaly imperfect development of the orbit, cases of premature
ossification, synostosis of coronary or sagittal sutures in oxycephaly.
3. Traumatic injury cavernous sinus thrombophlebitis, swelling of lids is
followed soon by exophthalmopligia, 3rd, 4th and 6th nerves passing through
the sinus are usually affected.
4. Inflammatory process Inflammation of -nasal sinuses frequently involves the
orbita, especially with ethmoid cells with complaint headache and fever also
Vision is reduced, Other inflammatory conditions witlh proptosis are orbital
periostitis (pneumococcal) which is more frequent and less serious than the
tuberculous form with sequestra, fistulae and possibly tuberculides of the skin.
5. New growth Tumors of intracranial origin which produce exophthalmos when
pressing on a part of the orbit or subsequently penetrating into inferior.
Unilateral proptosis has been observed to occur in about 2 percent. of all
cerebral tumours; uni- and bilateral in 8-10 per cent. of cases. The most
frequent that cause exophthalmos are tumors of the base of the skull, frontal
and temporal lobes, those have a direct connection with the orbit or that can
determine increased tension in the middle and anterior cranial fossae. Pituitary
tumors on the sella and penetrating into the orbit cause proptosis with
symptoms peculiar to that locality, hemianopia
6. Systemic disease. Proptosis connected relation with endocrine activities and
with the sympathetic nervous system. Transient proptosis may be the first sign
of a threatening exophthalmic goitre. Increased thyroid secretion facilitates the
appearance of exophthalmos and vice versa, hypothyroidism induces

14
enophthalmos. For the diagnosis of Graves' disease there are the classical
signs fine tremors of the extended hands occur, with nervousness and
increased pulse rate. 8

In our case from head MRI revealed heterogenous enhanching mass

with size 5.31 x 4.69 x 5.08 cm expanded to superior, which compressed
chiasma opticum and ventricle III to anterior to cavum orbita enchasing eyes
muscle and optic nerve suspicious craniopharyngioma. The working diagnosis
of this patient was suspicious of craniopharyngioma and she did the
cranioplasty and tumor excision, the biopsy of pathology anathomy revealed
pilocytic astrocytoma. The first time we diagnosed this patient with suspicious
craniopharyngioma because of the most common tumor on the sellar or
suprasellar region is craniopharyioma, but we still waiting for the biopsy to
establish the diagnose.
Magnetic resonance (MR) scan is suggestive of the diagnosis but
histology is the gold standard. The differential diagnose of tumor sella tursica
and parasellar region from head MRI are craniopharyngioma, Rathke's cleft
cyst, germinoma, meningioma and optic pathway glioma. 9,10
In our case during the operation from the freeze coup of the tumor
revealed Glioma, and biopsy pathological anatomy result was Pilocytic
astrocytoma (WHO grade I)
Diagnosis of pediatric CNS tumors is traditionally based upon cell
morphology and location, with tumors broadly classified according to World
Health Organization (WHO) criteria. These classes are the neuroepithelial,
cranial and paraspinal nerve, meningeal, lymphoma and haematopoietic, germ
cell, sellar region and metastatic. Each is further sub-classified and graded from
stage I to IV to indicate degree of malignancy and aggressiveness. Astrocytoma
is the most commonly diagnosed type of glioma in children. According to the
WHO classification of brain tumors, gliomas are further classified as low-grade
(grades I and II) and high-grade (grades III and IV) tumors. 10

15
Tabel 1. Major different subtypes of pediatric low-grade gliomas according to
the latest WHO Classification

Source: Berghold G. Pediatric low-grade gliomas: How modern biology reshapes the clinical
field. Biochimica etBiophysica Acta.2014

The main location of PA is the cerebellum, with more than 50% of the
cases occurring in cerebellar hemispheres, however, it can arise all through the
neuraxis, including hypothalamic region, the optic-chiasmatic tract and spinal
cord, with some cases involving the whole spinal cord 2

Figure 4: location of Pilocytic Astrocytoma

Source: Clinical review of pediatric pilocytic astrocytomas treated at a tertiary care hospital in
Pakistan

The neuroendocrine disorder associated with hypothalamic tumor or

chiasmatic gliomas are disorder of hypopituitarism which is defined as either
partial or complete deficiency of anterior or posterior pituitary hormone
secretion, or both.pituitary anterior and posterior hormonal. We should be aware

16
about the pituitary hormone deficiency, it can be isolated if affect single
hormone, multiple if two or three hormone and panpituitary if all the segment
affect. We should check the growth hormone, thyroid, ACTH, ADH and
Gonadotropin hormone.
Occurrence of hypopituitarism after pituitary surgery is influenced by
tumor size, extent of invasion into surrounding structures, remaining viable
normal pituitary, and the skill of the neurosurgeon. Patients should be made
aware of the possibility of hypopituitarism before surgery. Post-surgical
endocrine assessment is mandatory.14
PA arising in sellar and suprasellar regions may determine changes in
the release and/or production of hormones, in as much as the compressive or
even invasive effect of the neoplasm may affect the physiology of the
hypothalamus or pituitary gland. Since any given hypothalamic hormone is
commonly produced in more than one nucleus, and, in many cases, a single
nucleus may express several hormones, different clinical signs may be found in
this group of patients. Structure-function correlations are complex because of
the functional heterogeneity of many hypothalamic nuclei. Alterations in the
suprachiasmatic nucleus of the hypothalamus may be associated with decrease
in gonadotropin release. Lesions of the supraoptic nuclei may decrease the
synthesis of oxytocin and vasopressin. Destruction of the ventromedial nucleus
results in obesity; conversely, destruction of the ventrolateral nucleus causes
anorexia and cachexia. The arcuate (infundibular) nucleus plays a major role in
the modulation of anterior pituitary function. The paired medial and lateral nuclei
are linked with autonomic function, particularly temperature control and
olfaction. The posterior nucleus has been implicated in temperature regulation
and its large neurons are thought to be the source of hypothalamic efferents,
which descend to the reticular formation of the brainstem. 12,13

17
Figure 5: nuclei of hypothalamus
Source: Benjamin Cumming s. Anatomy physiology hypothalamus. 2001

In our case the patient suffered from tumor in optic pathway got
weakness since a month before she only stay at her room all day, with good
appetite, from physical examination vital sign no hypertension, no tachycardia,
and no tachypnea with good urine production equal 1-2 cc/kgBW. From the
laboratory finding Cortisol in the morning 0.43 ng/dL ( 4.3 -22.4) revealed
hypocortisol, she also got the hyponatremia in several days but already
corrected and she got the replacement hormone therapy. We also monitoring
for the fluid balance and urine production for involvement of hormone ADH
deficiency.
There is a Variety of causes for MPHD, such as cranial surgery,
radiotherapy, tumors, hereditary causes, infiltrative disease, infectious diseases
and head trauma. Due to the underlying pathology, clinical symptoms are
related to severity and speed of onset. The clinical presentation of the MPHD
patient can vary from asymptomatic or subclinical cases to life-threatening.
Patient’s symptoms are different, related to which hormone is involved
(including hypothyroidism, hypoadrenalism, hypogonadism, growth hormone
disorder and hypoprolactinemia). Some patients may be referred with
hyponatremia due to adrenal glands dysfunction, in spite of the necessary
studies for the evaluation of patients with hyponatremia, checking the serum

18
cortisol level is needed to rule out hypoadrenalism. Signs and symptoms of the
disease may persist for several years without diagnosis. 14
Hypothalamic causes of ACTH insufficiency include tumors and
radiotherapy. Tumors, such as craniopharyngioma, are associated with ACTH
deficiency in about 25% of patients. The frequency may be higher in tumors
such as germinoma and astrocytoma. Adrenal insufficiency is rarely the
presenting complaint but may contribute to the clinical picture. After surgery
and/or radiation therapy, the great majority of patients with hypothalamic tumors
will have ACTH deficiency as part of their surgical or radiation-induced
hypothalamic-pituitary damage. Therefore, all such patients should receive
glucocorticoid coverage during treatment irrespective of the status of the
hypothalamic-pituitary-adrenal axis at the time the tumor is identified. Because
treatment of secondary adrenal insufficiency can precipitate diabetes insipidus,
close attention to water balance is essential. ACTH insufficiency can also result
following whole-brain irradiation for brain tumors and other central
malignancies. This may involve both hypothalamic and pituitary mechanisms.
The frequency of ACTH insufficiency in such cases is much lower than following
the treatment of hypothalamic tumors, but may manifest some years after
treatment. 15
In most cases, GH secretion is lost first, followed in order by
gonadotropins, TSH, and ACTH. Thus, ongoing vigilance and assessment of
these patients is required for many years. This finding can occur many years
after presentation with GH, TSH, and gonadotropin deficiency, highlighting. the
importance of long-term follow-up of patients with pituitary disorders. 15
In our case the mother complained about her height was shorter than her
friends, but she didn’t noticed the exactly she got shorter. From growth chart
she under P3 and her height under the Mid parental height. Short stature in this
case is about the chronical process, maybe because of the Growth hormone
deficiency or maybe because the short stature it self. We already planned to
check Growth hormone evaluation and bone age but cannot performed yet
because of financial problem and waiting for the stable condition.

19
 Tumors of the CNS are a major cause of hypothalamic insufficiency. This
is especially true for midline brain tumors, such as germinomas, meningiomas,
gliomas, colloid cysts of the third ventricle, ependymomas, and gliomas of the
optic nerve. Many of the tumors that affect hypothalamic function also directly
impact pituitary secretion of GH. Growth disorders have been subdivided into
primary growth abnormalities, and secondary growth disorders example growth
failure resulting from chronic disease or endocrine disorders. The diagnosis of
IGF (insulin- like Growth Factor) deficiency must be auxology, with careful
documentation of serial heights and determination of height velocity. In the
absence of other evidence of pituitary dysfunction, it is necessary to perform
tests of GH secretion. 16
Bone age is a measure of biological age or maturity, rather than actual
age. A significant difference between the actual age and bone age increases
the suspicion of hormone deficiencies. In hypothalamic dwarfism, patients have
more often thyrotroph and growth hormone deficiency, which determine a
shorter height owing to premature fusion of the epiphyseal cartilage. 19
In our case the patient suffered from weakness, and after surgery the
laboratory finding Thyroid function test: FT4 0.6 ng/dL (0.89-1.76) TSH 0.04
IU/mL (0.35-5.5). She suffered from hypothyroid so we gave the hormone
replacement therapy.
Hypothalamic or pituitary disorders (including craniopharyngiomas,
granulomatous disease, and meningitis) may be acquired secondary to head-
trauma tumors. However,the most frequent causes are irradiation of the
neurocranium and chemotherapy in the treatment of malignant disorders
(especially brain tumors). Growth failure due to growth hormone (GH) or TSH
defi ciency is usually the earliest manifestation of pituitary hypofunction, but
other features related to the primary disease, neurologic disorder, or
hypothalamic dysfunction may be prominent.
Isolated central hypothyroidism is an uncommon disorder associated with
subclinical hypothyroidism and short stature in children. In children presenting
with these clinical symptoms and low free T4 and normal or low serum TSH

20
concentrations without other evidence of pituitary disease, the diagnosis of
central hypothyroidism can be considered straightforward. 17

In our case the patient may also suffered from Diabetus incipidus
because of vasopressin of posterior pituitary deficiency. She recorded with good
urine production so we did not check for the vasopressin secretion. But we still
monitoring for further MPHD.
Pilocytic astrocytoma (PA) arising in the hypothalamus corresponds to
approximately 11% of cases of this neoplasm. Hypothalamic/pituitary
dysfunction, including obesity and diabetes insipidus, is often but not invariably
apparent in large hypothalamic examples. Transient postoperative diabetes
insipidus (DI) is seen in 5% of cases but permanent DI occurs less frequently.
At least partial recovery of pituitary function in 40%–65% of patients has been
reported after surgery.2,12
In our case the patient 10 years old , did not got menarche yet and from
physical examination external genitalia revealed prepubertal with no pubic hair,
papilla breast only slight elevation, and no axillar hair yet. This patient still in the
normal range for the pubertal stage, we plan to check Luteinizing hormone
(LH), Follicle Stimulating Hormone (FSH), and Progesteron cannot perform yet
because of the financial problem but we have to evaluate for the Gonadotropin
hormone latter and still monitoring of the pubertal status.
In our case this patient already had a craniectomy tumor excision with
osteoplasty, before the surgery she got hydrocortisone acetate stress dose for
the hormone replacement therapy and also during and after the surgery. A
couple days after the surgery she suffered from hypothyroid so we gave the L-
thyroxin. Up until now she still waiting for the radiotherapy.
Surgical resection remained the mainstay of treatment for the pilocytic
astrocytoma, complete resection led to a better outcome, with most of the
patients showing clinical improvement, and there was no Inpatient or late
mortality. However, from study in Pakistan (42.9%) showed recurrence, even
though there was no evidence of a postoperative residual tumor on imaging.
Adjuvant therapy in the form of chemo- and radiotherapy was not necessary in
PAs that were completely resected. Though radiation therapy is generally

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considered the most effective treatment for unresectable pilocytic astrocytomas
in children, there are few data to support this claim. Although the survival of
these children is excellent, on study Mansur et al almost one third of patients
have progressive disease after definitive radiotherapy. Improvements in tumor
control are needed in this patient population 4,16

Treatment of MPHD includes therapies of underlying cause with

hormone replacement. The main goal of Hormone Replacement Therapy (HRT)
is to achieve to normal levels of circulating hormones to restore the body’s
natural physiology and relief of symptoms with minimal side effects.
Adrenocorticotrophic hormone (ACTH) is one the least common
hormones to become deficient in MPHD. When the body is stressed, such as
during illness or surgery, the body must produce more ACTH and cortisol to
maintain the blood pressure, blood glucose level and generally to be able to
cope with the stress. People with untreated ACTH deficiency have low levels of
cortisol and can experience episodes of dangerously low blood pressure and
low blood sugar levels when the body is stressed. Undiagnosed ACTH
deficiency may lead to slow recovery from operations, injuries and illness.
Synthetic forms of cortisol (hydrocortisone or cortisone acetate) are used as
replacement treatment for people with ACTH and cortisol deficiency. Tablets
are given two or three times a day. The normal release of cortisol from the
adrenal glands is higher in the morning. To mimic the body’s production of
natural cortisol, a larger dose is given first thing in the morning and the
remainder in the afternoon and evening. At times of acute illness, a person with
ACTH deficiency cannot respond to the stress of the illness. It is important that
extra hydrocortisone cortisone is given on these occasions.
In the case of major illness, such as any major fracture or any general
anaesthetic and operation an injection of hydrocortisone must be given. This
should be repeated every 4–6 hours until the patient is better. Thereafter, the
oral dose should be taken again at maintenance dose. 17
1. Before surgery: 24 hour and 12 hour oral Hydrocortisone 20 mg and 1 hour
before operation Hidrocortison Hydrocortisone Sodium Succinate 50 mg
intravenous.

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2. During surgery: Hydrocortisone Sodium Succinate 1-2 mg/kg body weight
intravenous.
3. First day: Hydrocortisone Sodium Succinate 8 X 25 mg intravenous every 4
hours.
4. Second day: Hydrocortisone Sodium Succinate 8 X 12.5 mg intravenous
every 4 hours.
5. Third day : Hydrocortisone Sodium Succinate 10 mg – 10 mg – 20 mg
oraly.
6. Fourth day: Hydrocortisone Sodium Succinate 5 mg – 5 mg – 10 mg oraly.

The treatment of hypothyroidism is to increase the levels of thyroxine in

the body, replacement thyroxine is given in tablet form as a daily dose. The
dose 3-4 mcg/kgBW . Regular blood tests measuring the levels of thyroid
hormones will be necessary usually 2-4 times a year. We will also regularly
assess the rate of growth and bone development as another way to monitor the
effectiveness of replacement hormone therapy. In children with isolated growth
hormone deficiency, commencing growth hormone replacement therapy can
result in a temporary lowering of thyroxine due to suppression of pituitary
hormone release (including TSH). This usually resolves within six months and
long-term treatment with thyroxine may not be necessary. Sometimes the
thyroid hormone level remains low during growth hormone treatment, so the
addition of thyroxine may be required.18
These patients had a weak condition, from the physical examination got
normal proportions between the limbs, trunk, abdomen, and head but in a
growth chart shown short stature. We still planned to got Bone Age examination
to determine whether the bone maturity equal with the actual age or not , but did
not performed yet because still waiting for the stable condition. We did not
performed yet of luteinizing hormone (LH) and follicle stimulating hormone
(FSH) examination becaused of the financial problem.
In our case the patient after surgery she got decreasing of
consciousness, from physical examination she did not got hypertension, normal

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pulse and no dyspneu but the GCS did not getting better. From the laboratory
finding got serum electrolyte abnormality but already correction.
Pilocytic astrocytomas (PA) are Grade I brain tumors characterized by an
excellent prognosis. In some cases, however, the patient has a bad outcome.
The 5-year progression-free survival rate was 75%, and the 5-year survival
rates were 100 and 92% after total and partial removal. Univariate statistical
analysis revealed that partial resection, optochiasmatic PA localization, and
pilomyxoid variant were associated with a worse prognosis. Among the patients
who underwent partial surgical removal, only invasion of the surrounding
structures was related to prognosis. PAs are benign tumors, but some
clinicopathological factors, such as partial resection, optochiasmatic location,
invasion of surrounding structures, and the pilomyxoid variant, have a worse
prognosis.5

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SUMMARY
A case of multiple pituitary hormone deficiency in a girl with Pilocytic
astrocytoma has been presented. Pilocytic astrocytoma In the hypothalamic
area may present with hormonal dysfunctions. Patients should be made aware
of the possibility of hypopituitarism before and after surgery. The clinical
presentation of the MPHD patient can vary from asymptomatic or subclinical
cases to life-threatening. Patient’s symptoms are different, related to which
hormone is involved (including hypothyroidism, hypoadrenalism, hypogonadism,
growth hormone disorder and hypoprolactinemia).
Among the patients who underwent partial surgical removal, only
invasion of the surrounding structures was related to prognosis. PAs are benign
tumors, but some clinicopathological factors, such as partial resection,
optochiasmatic location, invasion of surrounding structures, have a worse
prognosis.

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