Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence
An 11-month-old female infant was admitted to our hospital with a chief complaint of intermittent fever that had persisted for the prior month. The patient was born at term during an uneventful delivery and had no medical problems. Physical examination revealed splenomegaly and multiple petechiae in both inguinal areas. On blood testing there were findings of anemia and thrombocytopenia. No specific findings were observed during a bone marrow examination. Contrast-enhanced abdominal CT and chest CT showed hepatosplenomegaly (spleen length: 14 cm), and irregular, multifocal low-density lesions in the spleen (). Mild enlargements of the lymph nodes were found in the cervical, mediastinal, and pulmonary hilar regions, and the in the porta hepatis. Small amounts of bilateral pleural effusions and ascites were also observed ().\nSince the patient's fever did not subside after treatment with antibiotics and steroids, a splenectomy and multiple mesenteric lymph node biopsies were performed. LCS was confirmed by immunohistochemical study, as tumor cells were positive for CD1a and S-100 with malignant cytological features (). The patient was started on chemotherapy with etoposide and dexamethasone based on the hemophagocytic lymphohistiocytosis (HLH) 2004 protocol. On follow-up, a whole-body MRI obtained 10 months after chemotherapy, the only remaining findings were mild hepatomegaly; and the multiple lymph node enlargements had disappeared.\nA contrast-enhanced orbit CT and MRI conducted 6 months later as a result of a right orbital swelling, found a well-enhancing soft tissue mass invading the anterior skull base and both orbital areas, accompanied by bony destruction with an irregular, but clear boundary. Neither periosteal reaction nor calcification was observed inside the mass (). Biopsy confirmed the recurrence of LCS with positive CD1a and S-100. Treatment for recurrent LCS was given with 3 cycles of ifosfamide, carboplatin, and etoposide.\nOn a follow-up brain MRI obtained after 2 months, the skull lesion showed complete response. We are now following the patient after performing bone marrow transplantation.