Langerhans cell histiocytosis - skull

Case contributed by Samantha Scott

Presentation

Patient presents from the pediatrician with an enlarging scalp mass.

Patient Data

Age: 2 years
Gender: Male

Lateral and frontal views of the skull demonstrate a cluster of lytic lesions centered along the right coronal suture with characteristic "beveled edges". There is overlying soft tissue prominence.

Axial CT with bone window illustrates coalescent sharply marginated lytic lesions with characteristic "beveled edges" (unequal lysis of the inner and outer tables of the skull) centered along the right coronal suture. Contrast-enhanced CT with brain window displays a large adjacent soft tissue mass with heterogeneous enhancement and cystic components. Similar lesions are seen in the left temporal bone as well as region of the tuberculum sella above the right sphenoid sinus.

Case Discussion

This is a case of the typical appearance of the skull manifestations of Langerhans cell histiocytosis which was pathologically proven upon surgical resection. The sharply marginated "beveled edge" (unequal lysis of the inner and outer tables of the skull) is particularly characteristic. Langerhans cell histiocytosis may have an associated soft tissue component as seen in this case.

Co Author: Daniel P. Gewolb, MD

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