Langerhans cell histiocytosis - humerus

Case contributed by Domenico Nicoletti
Diagnosis almost certain

Presentation

Pain and swelling about the left elbow for three months unresponsive to acetaminophen.

Patient Data

Age: 11 years
Gender: Female

There is an osteolytic lesion about 2 cm at the distal humerus metadiaphysis with wide zone of transition, centered in medullary cavity and demonstrates no chondroid or osteoid matrix. There is a lamellar periosteal reaction. Joint effusion also noted. No pathologic fracture is evident.

The left elbow lesion is redemonstrated with low T1 and high T2 signal intensity and avid contrast enhancement. There is associated bone marrow and surrounding soft tissue edema along with moderate joint effusion. The lesion was biopsied and the diagnosis of Langerhans Cell Histiocytosis was confirmed.

PET/CT study did not find any other lesions.

Case Discussion

Langerhans cell histiocytosis(LCH) is a rare disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multisystem disease. LCH is part of a group of clinical syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages). These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukaemias and lymphomas.

The disease has gone by several names, including Hand–Schüller–Christian disease, Abt-Letterer-Siwe disease, and histiocytosis X, until it was renamed in 1985 by the Histiocyte Society.

Acknowledgement: Dott.ssa Sandra Pennacchini

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.