|
OJHAS Vol. 9, Issue 4:
Oct-Dec, 2010) |
|
|
| Cortisol Secreting Adrenal Adenoma in a 5 Year Old Child |
|
Gursharan Singh Narang, Associate Professor,
Jivtesh Singh Pahwa, Resident,
Department of Pediatrics, Sri Guru Ram Das Institute
of Medical Sciences and Research, Vallah, Amritsar. |
|
|
| |
|
|
|
|
|
|
Address For Correspondence |
Resident,
Dept., of Pediatrics,
Sri Guru Ram Das Institute
of Medical Sciences and Research,
Vallah, Amritsar.
E-mail:
jivteshpahwa@rediffmail.com |
|
|
|
|
|
Narang GS, Pahwa JS. Cortisol Secreting Adrenal Adenoma in a 5 Year Old Child. Online J Health Allied Scs.
2010;9(4):21 |
|
|
|
|
Submitted: Nov 29,
2010; Accepted: Dec 28, 2010; Published: Jan 20, 2011 |
|
|
|
|
|
|
|
| |
|
| Abstract: |
|
Adrenal adenoma
is a rare endocrinal tumor in children. It can present with features
of Cushing's syndrome. We present a case report of five years old female
child who came with morbid obesity and hypertension. The patient manifested
polyphagia, weight gain, and changes in sleep patterns. During physical
examination we found a full-moon face, bulkiness in the cervico-dorsal
(buffalo-hump) region, high blood pressure.
Key Words: Adrenal
adenoma; Cushing's syndrome; Hypercortisolism
|
|
Adrenocortical
tumours mostly secrete hormones, resulting in Cushing's syndrome, virilisation,
hyperaldosteronism or feminization, while the non-functioning ones are
unusual. Two syndromes have a clear association with adrenocortical
tumour : Li Fraumeni syndrome is associated with mutations of the p53
gene, and Beckwith Wiedemann syndrome which has mutations in the 11p15
region.1 Cushing’s syndrome is defined as the combination
of biological and clinical manifestations resulting from the presence
of abnormally high and sustained circulating concentrations of glucocorticoids,
whether of exogenous or endogenous production.
A 5 years old girl presented
to us in the emergency department with complaints of excessive irritability
and severe headache since 12 hours. History of abnormal body movements
were present since past 4 hours which were associated with frothing
from the mouth and incontinence of urine and stools followed by unconsciousness.
Child was born to 1st gravid mother to a non consanguineous
marriage. Perinatal period was uneventful. Birth weight of the child
was not known to the mother, but child was not overweight as per mothers
observations. Child was fed breast milk till the age of 8 months after
which complementary feeds were started. Milestones were normal for her
age. Child started gaining weight since the age of 3 years. There has
been a gradual increase in the weight of the child till present date.
History of polyphagia and sleep disturbances was present. No H/o intake
of exogenous steroid was present.
Childs Anthropometric measurements
showed weight of 35 kg(>97th percentile), head circumference
of 52.5cm(>50th percentile), height was 106cm(25th
percentile), abdominal circumference of 82.5cm and mid arm circumference
of 27.5cm with BMI > 95th
percentile.
 |
|
 |
|
Fig 1: 5 years old patient weighing 35
kgs. |
|
Fig 2: Cut section of CT abdomen showing
suprarenal mass. |
On examination child was markedly
obese with a BMI of, with BP of 140/90 mmHg. Child had a plethoric moon
face with truncal and subcutaneous and centripetal obesity and abdominal
cutaneous striae. Hirustism was not present. Bone age was corresponding
to the chronological age. Fundus examination, electrocardiograph (ECG)
and other investigations, including blood glucose (and glucose tolerance test), serum cholesterol
and renal function tests were normal. Laboratory investigations revealed
leukocytosis and normal serum electrolytes and normal fasting and post-prandial
glucose levels. Cushings syndrome was suspected and further endocrinological
tests were performed to determine the etiology. Diurnal variation of
plasma cortisol was not observed. The plasma cortisol level was not
suppressed by dexamethasone suppression test. Plasma ACTH levels were
markedly suppressed (less than 10pg/ml). Plasma free T3, free
T4, TSH (thyroid stimulating hormone) were within normal
limits.
Chest X – ray was normal.
Ultrasonography of abdomen didn’t reveal much so Computed tomography
of the abdomen was done which showed a small hypodense (approx 5× 6
mm) lesion in medial limb of left adrenal gland s/o adenoma. MRI brain
was normal.
Exploratory laparotomy through
the left supra umbilical transverse incision was done. There was a left
adrenal mass measuring 6×6 mm. Tumor was excised, it was not adherent
to the surrounding structures.
The cause of hypercortisolism
with clinical manifestations of Cushing’s syndrome is most often as
a result of the administration of synthetic glucorticoids.2,3
Endogenous causes of hypercotisolism may be due to hypersecretion of
adrenocorticotropic hormone (ACTH) by pituitary or by excessive secretion
of the suprarenal glands. Overproduction of cortisol from the suprarenal
tumor is a very uncommon pathology in children < 5 years of age.4
Adrenal cortical tumors (ACTs)
constitutes less than 0.2% of all pediatric neoplasms and account for
6% of all adrenal tumors in children with an estimated incidence of
0.3 million population.5 There is bimodal occurrence by age,
with a peak incidence at less than 5 years and a second peak in the
4th and 5th decades of life. Because of rarity
of these tumors, little is known about their causation and the influence
of genetic factors.
Cushing’s syndrome with the
“full moon” characteristics of the face, hirsutism, acneiform
lesions in the chest and arms, as well as a “hump” and bulging of
the superior segment (trunk) in the cervicodorsal region. All the physical
changes are as a consequence of the persistent and intense action of
the glucorticoids, which favored the accumulation of fat in the abdomen,
chest and face, condition central obesity. With the action of other
compounds such as growth hormone and β- adrenergic receptor antagonists,
these induce lipolysis, facilitating increase of triglycerides and free
fatty acids. Thinning of the extremities is usually observed with proximal
weakness. Decrease in protein synthesis leads to atrophy of the muscle
fibers, a circumstance accentuated by the decrease in potassium.6
Hypertension is usually seen as a consequence of increased renin substrate,
inducing as a consequence the pressor response of angiotensin and catecholamines,
and sodium retention, facilitating the expansion of extracellular volume.
Apart from presence of stria on thighs and abdomen, other skin changes
that tend to be present are capillary fragility, ecchymosis and hematomas.
There are laboratory studies
to establish the diagnosis and help to discern between hypophyseal or
suprarenal origin. These include serum cortisol level, free cortisol
in the urine, determination of 17,hydroxycorticosteroids that has been
substituted by free cortisol in the urine test in order to obtain better
sensitivity, suppression test with nighttime dexamethasone, cardiac
rhythm of cortisol, plasma ACTH.7
Radiological investigations
include Ultrasonography, CT scan(abdomen) and MRI ( brain) etc. CT scan
is more sensitive than ultrasonography in identifying tumor mass and
CT scan seems to be the best for localization of tumor masses.8
Tumors measuring more than
5 cm are usually malignant. Surgical removal of the tumor (Adrenalectomy)
remains the procedure of choice.9
- Ciftci AO, Senocak
ME, Tanyel FC, et al. Adrenocortical tumors in children. J Pediatr Surg
2001;36:549-554.
- Orth DN. Cushing’s
Syndrome. N Engl J Med 1995;332:791-803.
- Lafferty AR, Chorousos
GP. Pituitary tumors in children and adolescents. J Clin Endocrinol
Metab 1999;84:4317-4323.
- Gayer G, Zissin
R, Apter S, Atar E, Portnoy O, Itzchak Y. Pictorial review. CT findings
in congenital anomalies of the spleen. BJR 2001;74:767-772.
- Budhwani KS, Ghritlaharey
K, Debbarma M. Adrenal cortex tumor in a six year girl – A report
and review of literature. Indian Journal of Medical and Pediartric
Oncology 2004;25(3):71-75.
- Trainer PJ, Grossman
A. The diagnosis and differential diagnosis of Cushing syndrome. Clin
Endocrinol 1991;34:317-330.
- Forga L, Anda E,
Martínez de Esteban JP. Síndromes paraneoplásicos. An Sist Sanit
Navar 2005;28:213-226.
- Mayer SK,Oligny
LL, Deal C et al. Childhood Adrenocortical Tumors: Case series &
Reevaluation of Prognosis – A 24 – year experience. J Pediatr Surg
1997;32:911-915.
- Agarwal S, Mitra
DK, Bhatnagar V, Menon PS, Gupta AK. Aldosteronoma in childhood. A review
of clinical features and management. J Pediatr Surg 1994;29:1388-1391.
|
