Adrenocortical tumors in children: a case report

H.SAKLY1, MA. JELLALI1, A. ZRIG1, W.MNARI, M.MAATOUK1,

W.HARZALLAH1, R. SALEM1, I.KRICHENE, A.NOURI , M. GOLLI1.

5th ARAB RADIOLOGY CONGRESS25th - 28th April 2012

1 Radiology Department, CHU F.B Monastir.2 Pediatric surgery Department, CHU F.B Monastir

PEDIATRICS : PD 8

INTRODUCTIONPrimary 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.

Mass occuping the entire abdomen

Physical examination:

Cushing’s syndrome manifestations mixed with virilization manifestations including deepening of the voice, acne, hirsutism, and increasing of muscle mass.

Contrast-enhanced CT scan of the abdomen reveals a bulky, circumscribed, lobulated, heterogeneous intra-abdominal 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.

Computed tomography

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.

DISCUSSIONAdrenocortical 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

Children

Typically guided by clinical

presentation

Adults

Incidentally discovered in asymptomatic

adults

Diagnosis imaging of adrenocortical neoplasms

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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