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Presented by Ralph Hruban, M.D. and prepared by Jeffrey T. Schowinsky, M.D.
Case 5: This infant with a history of a previous neoplasm developed precocious puberty and abdominal distention.
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1. Question
Week 269: Case 5
This infant with a history of a previous neoplasm developed precocious puberty and abdominal distention. A 20 cm abdominal mass was identified on imaging.images/41706RH5a.jpg
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images/41706RH5e.jpgCorrect
Answer: Adrenocortical carcinoma
Histology: This large neoplasm is composed atypical eosinophilic cells. The mitotic rate is high and necrosis is apparent. Immunhistochemical labeling revealed that the neoplastic cells express alpha-inhibin and, focally, melan A (using the A103 antibody).
Discussion: This unfortunate girl has a history of Li-Fraumeni syndrome and a choroid plexus carcinoma. This adrenocortical carcinoma represents her second neoplasm. The large size, the high mitotic rate, and the extensive necrosis help establish that this adrenal cortical neoplasm is malignant.
There are a number of familial forms of adrenocortical carcinoma including the Li-Fraumeni syndrome and the Beckwith-Wiedeman syndrome.
Reference(s):
– Libe R, Bertherat J. Molecular genetics of adrenocortical tumours, from familial to sporadic diseases. Eur J Endocrinol. 153:477-87, 2005.Incorrect
Answer: Adrenocortical carcinoma
Histology: This large neoplasm is composed atypical eosinophilic cells. The mitotic rate is high and necrosis is apparent. Immunhistochemical labeling revealed that the neoplastic cells express alpha-inhibin and, focally, melan A (using the A103 antibody).
Discussion: This unfortunate girl has a history of Li-Fraumeni syndrome and a choroid plexus carcinoma. This adrenocortical carcinoma represents her second neoplasm. The large size, the high mitotic rate, and the extensive necrosis help establish that this adrenal cortical neoplasm is malignant.
There are a number of familial forms of adrenocortical carcinoma including the Li-Fraumeni syndrome and the Beckwith-Wiedeman syndrome.
Reference(s):
– Libe R, Bertherat J. Molecular genetics of adrenocortical tumours, from familial to sporadic diseases. Eur J Endocrinol. 153:477-87, 2005.
