Adrenocortical tumors in children: a case report

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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 Radiology Department, CHU F.B Monastir. 2
Pediatric surgery Department, CHU F.B Monastir
PEDIATRICS PD 8
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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.

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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.

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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.

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• Mass occuping the entire abdomen

• Physical examination

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• Cushings syndrome manifestations mixed with
  virilization manifestations including deepening
  of the voice, acne, hirsutism, and increasing of
  muscle mass.

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• Computed tomography

• Contrast-enhanced CT scan of the abdomen
  reveals a bulky, circumscribed, lobulated,
  heterogeneous intra-abdominal mass measuring
  181317cm. Curvilinear foci of high attenuation,
  consistent with calcification, delimit tumor
  lobules. The origin of this mass were impossible
  to determine.

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• Biology plasma cortisol , testosterone
• aFP, ßHCG, CA125 normal
• Biopsy histological examination
• adrenocortical carcinoma

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Metastasis

• Pulmonary metastasis

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• - Cerebral metastasis

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• Treatment
• chemotherapy was proposed to the parents however
  they refused all treatment.

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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, Cushings Syndrome,
  aldosteronism, or feminization.

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Clinical Features
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• Occur more frequently between the age of 3 and 5.
• Its 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, Cushings Syndrome,
  aldosteronism, or feminization

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• 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

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Diagnosis imaging of adrenocortical neoplasms

• Children
• Typically guided by clinical presentation

• Adults
• Incidentally discovered in asymptomatic adults

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• 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.

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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
• 
  

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Radiologic Features
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• 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.

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• 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.

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Treatment and Prognosis
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• 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

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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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BIBLIOGRAPHY

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