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Joseph Junewick, MD FACR
over 7 years ago
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Mesenteric Angiomyolipoma (Fat Poor) - Tuberous Sclerosis

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: US CR CT
Activities:
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History

11 year old male with tuberous sclerosis.


Case Images


Diagnosis

Mesenteric Angiomyolipoma (Fat Poor) – Tuberous Sclerosis

Clinical Notes

Not pathologically proven.

Findings

CR – Supine AP radiograph shows multiple calcifications (the largest calcification has a central lucency) and paucity of bowel gas in the midabdomen.

US – Hyperechoic mass with multiple cystic areas incidentally noted on a retroperitoneal/renal examination.

CT – Mixed density infiltrative mesenteric mass with nodular features and scattered calcifications.

Discussion

Tuberous sclerosis, the second most common phakomatosis after neurofibromatosis, is related to mutation in either the TSC1 or TSC2 gene (both tumor suppressor genes which encode for hamartin and tuberin proteins). Angiomyolipomas are benign hamartomatous neoplasms composed of abnormal blood vessels, smooth muscle and fat. Angiomyolipomas are most commonly found in the kidneys but can be found in other abdominal viscera and retroperitoneum. Angiomyolipomas enlarge with time especially under the influence of estrogen and progesterone. Extrapulmonary lymphangioleimyomatosis is a rare manifestation of TSC and usually found only in women with pulmonary lymphangioleiomyomatosis and consequently an unlikely etiology of this lesion. Immunohistochemical findings suggest that angiomyolipomas and lymphangioleiomyomas have a neuroectodermal origin.

Reference

Baskin HJ. The pathogenesis and imaging of the tuberous sclerosis complex. Pediatr Radiol (2008); 38:936-952.



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