Transcript Adrenocortical tumors in children: a case report

5th ARAB RADIOLOGY CONGRESS
25th - 28th April 2012
H.SAKLY1, MA. JELLALI1, A. ZRIG1, W.MNARI,
M.MAATOUK1, W.HARZALLAH1, R. SALEM1,
I.KRICHENE, A.NOURI , M. GOLLI1.
1
2
Radiology Department, CHU F.B Monastir.
Pediatric surgery Department, CHU F.B Monastir
PEDIATRICS : PD 8
INTRODUCTION
 Primary neoplasms of the adrenal cortex are
rare in pediatric population. They merit
separate discussion from their counterparts
in adults because they have distinctive
epidemiologic and clinical features.
Objectives
Be familiar with the spectrum of clinical,
pathologic and radiologic findings in children
with adrenocortical neoplasms.
Understand the role of imaging studies in
diagnosis, staging and guiding biopsy of
adrencortical neoplasms in children.
CASE REPORT
 Age and sex
3 years old girl;
 consanguinity
 Family history of cancer
mother with a breast cancer, 2 maternal uncles
with respectively colon and hepatic cancer. A
paternal uncle with brain cancer.
Physical examination:
 Mass occuping the entire abdomen
 Cushing’s syndrome
manifestations mixed with
virilization manifestations
including deepening of the voice,
acne, hirsutism, and increasing
of muscle mass.
Computed tomography
Contrast-enhanced
CT
scan of the abdomen
reveals
a
bulky,
circumscribed, lobulated,
heterogeneous
intraabdominal
mass
measuring 18*13*17cm.
Curvilinear foci of high
attenuation,
consistent
with calcification, delimit
tumor lobules. The origin
of this mass were
impossible to determine.
 Biology: plasma cortisol , testosterone
αFP, βHCG, CA125 normal
 Biopsy + histological examination:
adrenocortical carcinoma
* Metastasis:
- Pulmonary metastasis
- Cerebral metastasis:
Treatment:
chemotherapy was proposed to the
parents however they refused all
treatment.
DISCUSSION
Adrenocortical carcinoma (ACC) comprises only
0.002% of all childhood malignancies and is
potentially lethal.
Occur more frequently between the age of 3 and 5.
These hormone-secreting neoplasms are manifested
by virilization, Cushing’s Syndrome, aldosteronism,
or feminization.
Clinical Features
 Occur more frequently between the age of 3 and 5.
 It’s now recongnized that most children with an
adrenocortical neoplasm show clinical evidence of an
endocrine abnormality, in contrast to the behaviour of
adrenocortical tumors in adults.
 These hormone-secreting neoplasms are manifested by
virilization, Cushing’s Syndrome, aldosteronism, or
feminization
 Virilization signs include an increase in muscular
mass, rapid growth, acne, pubic and facial hair,
hirsutism, and an increase in the size of the penis or
clitoris.
these signs appeared in our patient
Diagnosis imaging of adrenocortical
neoplasms
Children
Typically guided by
clinical
presentation
Adults
Incidentally
discovered in
asymptomatic
adults
Predisposing constitutional genetic factors have
been found in approximately 50% of children with
ACC. Two genetic syndromes are clearly associated
with ACC:
Beckwith-Wiedemann syndrome: alteration of the
11p15 region.
Li-Fraumeni syndrome: alterations of the tumor
suppressor gene p53 on chromosome 17p
In families with the Li-Fraumeni syndrom the
frequency of adrenocortical tumors is 100 times
that in the general population.
• Metastasis:
- The lung is the most common site of metastasis
followed in frequency by the liver.
- Other metastatic sites:
* The peritoneum ( 29%)
* pleura or diaphragm (24%)
* abdominal lymph nodes (24%)
* Kidney ( 18%)
* Venous extention
* Cerebral metastasis are extremely rare
+++
Radiologic Features
 Cross-sectional imaging studies including US, CT,
and magnetic resonnance (MR) imaging, have
largely suppleated use of invasive procedures:
 US and CT : principal diagnosis modalities used.
 They are useful to suggest the diagnosis, and
define local and distant extension as well as they
guide biopsy.
Cross-sectional imaging studies typically
demonstrate a large, circumscribed,
predominantly solid suprarenal mass with
variable heterogeneity due to hemorrhage and
necrosis. Calcification is not uncommon.
Local invasion and metastases to the lungs,
liver, and regional lymph nodes may be
present at diagnosis. When friable tumor
thrombus extends into the inferior vena cava,
it poses a high risk of pulmonary
embolization.
Treatment and Prognosis
 Surgery is the only mode of therapy documented as
effective for treating paediatric ACT
Radiotherapy has not yielded good results.
The role of chemotherapy has not been systematically
evaluated in childhood ACC.
 Currently, ortho-para-DDD, also known as
MITITANE, is the chemotherapeutic agent used to
treat ACC in adults.
Little information is available about the use of
mitotane in children but response rates appear to be
similar to those seen in adults with ACT
CONCLUSION
This observation underscore:
 Imaging importance in diagnosing and evaluating
the extension of these tumors.
 The importance of considering genetic testing and
counselling for families of young children with
ACC.
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
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