ineal gland secretes melatonin
whose regulatory role on puberty is hypothesized but ill defined. Evidence
exists that disorders of the hypothalamic-pituitary-gonadal axis are
linked to altered plasma melatonin profile, demonstrating the role of the
pineal gland in reproductive endocrinology(1).
Pineal gland lesions present mostly as tumors and
rarely as cystic lesions. They affect children and adolescents and the
clinical presentation is usually prolonged history of head ache and
vomiting. Germ cell tumors are the commonest tumors of pineal gland and
the elevated human chorionic gonadotropin results in sexual precocity in
these patients. Pineal gland cysts are usually asymptomatic and are rarely
reported to be associated with central precocious puberty(2). We present
an interesting case of a young boy with true sexual precocity and an
associated pineal cyst to highlight the role of pineal gland in pubertal
A 5 year old boy was brought by parents with history of
rapid increase of height, enlargement of the external genitalia, and
appearance of pubic hair, voice change and presence of acneiform lesions
over face for last two years. The boy was the last sibling amongst four (2
girls and 2 boys), born out of a third degree consanguineous marriage with
all other siblings reportedly normal. The birth weight was 2.8 kg and his
motor and mental milestones were normal. The parents denied history of
headache, vomiting, seizure episodes, loss of consciousness and any other
feature to suggest CNS illness. They also denied any family history of
precocious puberty or exposure to medications by the child.
Anthropometric examination revealed: height 125 cm
(>95th centile), weight 23 kg (>95th centile), upper/lower segment ratio
1.2 and arm span of 122 cm. Pubertal assessment by Tanner grading revealed
G3P3A2 with bilateral scrotal testes of volume 8-10 mL and stretched
penile length of 7 cm. Detailed clinical examination revealed acne over
face, with no evidence of neurocutaneous markers, midline defects, goiter
or evidence of systemic disease. Neurological examination revea-led normal
visual fields and fundi with no clinically detectable abnormality.
Estimated bone age was 12 years by the Greulich-Pyle
method. His hormonal evaluation revealed a basal LH (1.1 IU/L) and FSH
(0.7 IU/L) and a high level of serum testosterone (4.3 nmol/L). Other
hormonal evaluation was normal. T1- and T2-weighted and FLAIR magnetic
resonance imaging (MRI) images showed a 1.1 × 0.8 × 0.7 cm cyst in the
pineal region. The content of the pineal cyst was homogeneous and
isointense relative to cerebrospinal fluid. The wall of the cyst was
slightly thickened with focal irregularity. Based on history, findings of
precocious puberty and pubertal hormonal profile, we started the patient
on depot preparation of GnRH-analogue (Inj Leuprolide 3.75 mg subcutaneous
every 4 weeks). In view of asymptomatic pineal gland cystic lesion, no
neurosurgical intervention was planned immediately and the patient is kept
under regular follow up.
Our patient, a case of true precocious puberty had an
associated pineal cyst. Literature search revealed only three similar case
reports earlier(2,3). Pineal cysts are benign, asymptomatic and are
detected incidentally with a population prevalence of 1-4%. Our case had
typical features of true precocious puberty without neurological symptoms
or signs attributable to pineal cyst. Hence, the pineal cyst was
considered as an associated finding and the boy was started on medical
therapy with Leupro-lide. Pineal cysts of less than one cm and
asymptomatic should be followed with serial neuroimaging and symptomatic
large cysts are subjected for surgical resection. There is no role of
chemotherapy or radiotherapy for benign pineal cysts(4). In our case,
surgical resection was not contemplated in view of small size and
The pineal gland influences human reproductive function
at hypothalamic-pituitary level, by inhibition of the gonadotropin
releasing hormone (GnRH) pulse and also at the gonadal level(2,5). Pineal
gland germ cell tumors cause sexual precocity by release of human
chorionic gonadotropin but the mechanism of precocity is unknown with
other lesions. Data from animal models and human studies suggest that
there is removal of the gonadotropin inhibition by mela-tonin leading to
precocity or stimulation of hypothalamo-pituitary-gonadal axis via a
secretory product analogous to GnRH. Other proposed mechanisms are loss of
inhibitory effect of the pineal gland on gonadotropin release. The
contributing factors are size of the cyst and alterations in the
functional capacity of the gland due to cystic nature of pineal gland(6).
To conclude, we present an unusual association between
precocious puberty related to pineal cyst. The underlying etiological
association is unclear and this case exemplifies the enigmatic role of the
pineal gland in puberty.
Contributors: KVS, AV worked up the patient
clinically. KVS, KDM reviewed the literature and drafted the manuscript
initially. KVS, RSR co drafted and revised the manuscript. KDM will act as
the guarantor and the final manuscript was approved by all authors.
Competing interests: None stated.
1. Aleandri V, Spina V, Morini A. The pineal gland and
reproduction. Hum Reprod Update 1996; 2: 225-235.
2. Dickerman RD, Stevens QE, Steide JA, Schneider SJ.
Precocious puberty associated with a pineal cyst: is it disinhibition of
the hypothalamic-pituitary axis? Neuro Endocrinol Lett 2004; 25: 173-175.
3. Benítez Fuentes R, Velázquez de Cuéllar, Paracchi M,
Blanco Rodríguez M, Soriano Guillén L. Central precocious puberty and
pineal gland cyst: an association or incidental finding? Ann Pediatr (Barc)
2008; 68: 72-73.
4. Wishoff JH, Epstein F. Surgical management of
symptomatic pineal cysts. J Neurosurg 1992; 77: 896–900.
5. Macchi MM, Bruce JN. Human pineal physiology and
functional significance of melatonin. Front Neuroendocrinol. 2004; 25:
6. Rivarola G, Belgorosky A, Mendilaharzu H, Vidal G.
Precocious puberty in children with tumours of the suprasellar and pineal
areas: organic central precocious puberty. Acta Paediatr 2001; 90: