Korean J Radiol.  2013 Jun;14(3):520-524. 10.3348/kjr.2013.14.3.520.

Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence

• Chung WD
• Im SA
• Chung NG
• Park GS

• KMID: 1705469
• DOI: http://doi.org/10.3348/kjr.2013.14.3.520

Abstract

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.

Keyword

Langerhans cell sarcoma; Langerhans cell histiocytosis; CT; MR

MeSH Terms

Female
Hepatomegaly/diagnosis
Humans
Infant
Langerhans Cell Sarcoma/*diagnosis
Magnetic Resonance Imaging
Mediastinal Neoplasms/*diagnosis
Neoplasm Recurrence, Local
Skull Neoplasms/*diagnosis
Splenomegaly/diagnosis
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Case 1. 11-month-old female infant presented with intermittent fever. A. Contrast-enhanced CT of abdomen shows splenomegaly (about 14 cm) with multifocal low density lesions in spleen (arrows). Soft tissue density lesions are seen in porta hepatis (arrowheads). B. Contrast-enhanced CT of chest shows nodular enlargement of thymus with bilateral pleural effusions. C. Typical histology of Langerhans cell sarcoma with malignant cytological features, such as atypia, hyperchromatic nuclei (H&E, × 400), and prominent nucleoli. D, E. Cells are immunopositive for CD1a (× 400) and S-100 (× 400). F. At 16 months after chemotherapy, follow-up MRI of orbit (fat-suppressed T2-weighted image) shows well-enhancing soft tissue mass extending from anterior skull base (including sphenoid wing and infratemporal fossa) to lateral aspect of right orbit and frontal area (arrow). G. Follow-up CT of orbit shows associated adjacent bony destruction with irregular sharp margin (arrow). Osteolytic lesions involving anterior skull base to lateral aspect of left orbit are also noted (arrowheads).

• 

  Fig. 2 Case 2. 17-month-old girl presented with intermittent fever. A. Contrast-enhanced CT of abdomen shows huge soft tissue density lesion with heterogeneous enhancement in anterior mediastinum. B. In upper abdomen, soft tissue density lesions encase main portal vein, hepatic artery and splenic artery, without evidence of luminal narrowing (arrows). C, D. At 17 months after chemotherapy, follow-up CT of skull shows multifocal osteolytic lesions including greater wing of left sphenoid bone, and right temporal bone (arrows). E, F. One year after salvage chemotherapy, follow-up contrast-enhanced CT of abdomen shows nodular calcifications along biliary trees.

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