SPR Unknown # 92 -- FINAL

Peter Marcovici, MD

UCSF Benioff Children's Hospital


Publication Date: 2013-07-25

History

Newborn male patient with a prenatal ultrasound diagnosis of a chest wall mass. After birth, the baby became unstable, and developed a severely decreased platelet count.

Findings

Chest/abdomen radiograph demonstrates a large soft tissue mass in the left lateral chest wall with superior displacement of the scapula and left upper extremity. No internal calcifications or phleboliths are present.

MRI demonstrates a large, complex predominantly T2 hyperintense mass centered in the lateral left chest wall extending superiorly to the left axilla and inferiorly to the level of the upper abdomen. No intra-thoracic or intra-abdominal extension is present. Multiple flow voids are present within the lesion, with a dominant arterial supply from a hypertrophied branch of the left axillary artery. There is additional arterial blood supply from multiple left-sided intercostal arteries. Dominant venous drainage is into the left axillary vein. Additional venous drainage into the intercostal veins is present. There is overlying skin thickening and subcutaneous edema.

Discussion

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that usually affects soft tissues of newborns and infants. Although histologically benign, KHE is locally aggressive because of its tendency to inīŦltrate surrounding tissues. KHE may be associated with Kasabach-Merritt syndrome (KMS), a severe consumptive coagulopathy caused by a platelet trapping syndrome, as in our case.

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