Pituitary adenomas in children
Prolactinomas
pubertal development, no significant tumor mass is
present and visual fields are intact. In the other pa-
tients hypersecretion of PRL still persists in 2 cases
(cases 3 and 4), partial or complete hypopituitarism
is present in 4 (cases 3, 4, 6, 10), relevant tumor rem-
nant is evident in 4 (cases 3, 4, 9, 10) and visual field
is impaired in 2 patients (cases 3, 4).
Cabergoline caused a quick improvement in the
two female patients (cases 1 and 2); prolactin levels
are normal, tumor shrinkage has been impressive
and visual fields are normal; their sexual character-
istics are fully expressed and they have regular
menses. In male patients the results of therapy were
less successful apart from case 5. In this patient
bromocriptine had not improved bilateral hemi-
anopsia, he was operated on and then treated with
cabergoline 0.5 mg/day: PRL is normal, the size of
the residual tumor has decreased and the visual
field has markedly improved. Patient 3 had a giant
prolactinoma; a transcranial-transsphenoidal oper-
ation was performed, followed by radiotherapy.
Complete hypopituitarism is present and, despite
bromocriptine 20 mg/day and a decrease in the tu-
mor size, PRL is 142 ng/ml and significant deran-
gement of visual field is still present. Patient 4 was
treated by transsphenoidal surgery followed by
bromocriptine with doses up to 20 mg/day and lat-
er cabergoline 3 mg/week, but PRL did not norma-
lize, the tumor went on growing and visual field
worsened so a second transsphenoidal operation
was performed. Despite bromocriptine 15 mg/day,
PRL levels keep increasing, a significant tumor rem-
nant is still present and visual field is worsening,
therefore radiotherapy has been scheduled.
DISCUSSION
In childhood and adolescence pituitary adenomas
constitute 2.1-6% of adenomas removed by surgery
in all age groups (1-6). All our patients were older
than 10 years of age, confirming that most pituitary
adenomas in pediatric age occur in the pubertal
period (2, 4-6). Males accounted for 60% of our pa-
tients. A slightly higher prevalence of males (52-
60%) had been reported in a few series (2, 3, 9),
while others reported a higher prevalence (70-83%)
of females (4-6). The female/male ratio is higher for
prolactinomas, ranging from 1.6:1 to 4.8:1 (3, 5, 6,
10, 11) but perhaps less striking than in adults; in
GH and mixed GH/PRL tumors, female/male ratio is
lower ranging from 1:3 to 1:4.5 (3, 5, 6, 12); clini-
cally non-functioning adenomas are predominant-
ly male (6, 13).
Half our patients came to medical attention for tu-
mor mass symptoms, namely visual defects and
headache. It should be emphasized that clinical
evaluation at diagnosis disclosed that most of our
patients had unrecognized or misinterpreted en-
docrine dysfunctions, in particular 40% of the pa-
tients had inappropriate pubertal development for
their age: a more careful investigation of these en-
docrine signs would probably have allowed an ear-
lier diagnosis. The initial complaints of pediatric pa-
tients are generally reported to be endocrine symp-
toms or signs, particularly failure of growth or sex-
ual maturation (5, 6), but headache was present in
61% of patients in the series of Kane (5) and
headache and/or visual defects were the presenting
symptoms in half of 26 prolactinomas (11), too.
Perhaps in our series the large prevalence of symp-
toms due to tumor mass can be accounted for by
the fact that 90% were macroadenomas, sometimes
of great size. In pediatric age macroadenomas en-
compass 54-69% of prolactinomas (4-6, 10, 11), 50-
92% of GH/PRL-secreting tumors (3, 5, 6, 12) and
92-100% of clinically non-functioning adenomas (6,
13): this is in sharp contrast with ACTH-secreting
adenomas which are almost always microadenomas
(6, 14-16).
GH-secreting adenomas
Patient 6 was operated on by transsphenoidal
route, but the operation was complicated by in-
trasellar hemorrhage causing complete hypopitu-
itarism. No residual tumor is present, basal GH is
less than 0.5 ng/ml and IGF-I is 93 ng/ml. Patient
7 underwent selective transsphenoidal adenomec-
tomy. Despite no evidence of residual tumor, acro-
megaly was still active. He is currently treated with
octreotide LAR 30 mg/month and cabergoline 1.5
mg/week: GH is 2.5 ng/ml and IGF-I is normal.
Clinically non-functioning adenomas
Two patients (cases 8 and 9) were successfully op-
erated on by transsphenoidal approach: visual fields
are normal and they both have normal sexual de-
velopment with regular menses; no residual tumor
was evident after surgery but in case 9 an asymp-
tomatic recurrence was found 20 months later. Pa-
tient 10 was operated on by transcranial route, fol-
lowed by radiotherapy. The patient suffers from hy-
popituitarism, but his visual field is normal and a
small remnant in the cavernous sinus is unchanged.
Therefore, treatment was completely successful in
5 patients (cases 1, 2, 5, 7, 8) (50%): they now show
normal hormone secretion, have achieved complete
On the basis of clinical and immunohistochemical
characteristics 50% of our cases were PRL-secret-
ing, 20% pure GH-secreting and 30% non-func-
95