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Schwachman Syndrome
Joseph Junewick, MD FACR
over 7 years ago
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Highly primitive malignant neoplasm of the Pancreas

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: US CT
Activities:
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History

4 year old male with elevated estrogen levels and gynecomastia.


Case Images


Diagnosis

Highly primitive malignant neoplasm of the pancreas with features of juvenile granulosa cell tumor.

Clinical Notes

Immunoperoxidase positivity for inhibin, CD10, calretin, estrogen receptors and progesterone receptors.

Findings

US – Hyperemic solid and cystic left upper quandrant mass.

CT – Well-circumscribed mass arising from the tail of the pancreas.

Discussion

Granulosa/theca cell tumors generally involve the gonads. These tumors are large at the time of diagnosis and usually solid but occasionally contain cystic components. Most granulosa/theca cell tumors produce estrogen, resulting in precocious development in females and gynecomastia in males.



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