The sonographic appearance of lesions of the spleen and pancreas in an infant with Langerhans' cell histiocytosis

Abstract

Langerhans' cell histiocytosis (LCH), the term now used to describe the group of diseases known as histiocytosis X, is a rare disorder of the bone marrow-derived histiocytes that may involve the skin, bone, bone marrow, liver, spleen, lungs, lymph nodes, and rarely the pancreas. Sonographically demonstrable lesions of the spleen and pancreas have not been reported. We present a case of disseminated LCH in a 4-week-old infant to demonstrate the sonographic appearance of splenic and pancreatic lesions that occur with this disease.

Langerhans' cell histiocytosis as a cause of periportal abnormal signal intensity on MRI

“…Some hypoechoic nodules looked like targets. We suspect that periportal hypoechogenicity may be attributed to the infiltration of histiocytes and inflammatory cells based on our biopsy result, which accorded with the reports of Hara et al [4] and Muwakkit et al [5]. MRI findings of the liver in LCH have been noted in two case reports [6,8].…”

“…US finding of the liver in LCH has been described as hypoechoic nodule or periportal hyperechogenicity that may be attributed to inflammatory cell infiltration or fat content [4,5,7,8]. US in the present cases showed periportal hypoechogenicity that was band-like or nodular.…”

“…A review of the literature shows that the findings may depend on the difference of duration of the disease and the main pathological features in each case. Ultrasonographic (US) or cholangiographic findings of the liver have been discussed in a few cases of young children with disseminated LCH [2,4,5]. In adult cases, magnetic resonance imaging (MRI) has demonstrated periportal fat infiltration or fibrosis mimicking multiple hepatic tumors [6,8].…”

Langerhans cell histiocytosis infiltration into pancreas and kidney

“…Keeling et al 9 described the autopsy findings in 12 cases of LCH with gastrointestinal involvement—the pancreas was found to be normal in all cases. Direct involvement of pancreas has been reported only on two occasions 5,10. In both of these case reports, pancreatic involvement occurred as a part of multisystem disease.…”

A Pattern-oriented Approach to Splenic Imaging in Infants and Children

“…Langerhans cell histiocytosis is characterized by a proliferation of bone marrow-derived histiocytes and predominantly involves the skin, bone, bone marrow, reticuloendothelial system, and lungs. It may cause splenic enlargement or (less often) multiple hypoechoic nodules within the spleen that do not necessarily disappear after therapy (51,52).…”