PET Imaging in Langerhans Cell Histiocytosis Christiane Franzius Bremen, Germany
Histiocytosis Histiocytosis Idiopathic proliferation of histiocytes Two types of histiocytes macrophages: antigen processing dendritic cells: antigen presentation
Classification: Histiocyte Society and WHO Disorders of varied biological behavior dendritic cell related Langerhans cell histiocytosis juvenile xanthogranuloma secondary dendritic cell processes, macrophage related hemophagocytic syndromes Rosai-Dorfman disease Erdheim-Chester disease, Malignant disorders monocyte related leukemias malignant histiocytosis,
Langerhans cell histiocytosis (LCH) Proliferation of a type of dendritic cells: Langerhans cells histiocytosis x (Lichtenstein) Letterer-Siwe Hand-Schüller-Christian eosinophilic granuloma incidence: 0,2-1,0/100 000 children/year male:female: 2:1
Langerhans cell histiocytosis (LCH) Letterer-Siwe (infants, young children) hepatosplenomegaly lymphadenopathy cutaneous lesions otitis media pancytopenia pulmonary involvement fulminant, often fatal course
Langerhans cell histiocytosis (LCH) Hand-Schüller-Christian (older children) diabetes incipitus exophthalmos osteolytic lesions more indolent disease
Langerhans cell histiocytosis (LCH) Eosinophilic granuloma (older children, young adults) osteolytic lesions lung involvement more indolent disease
Langerhans cell histiocytosis (LCH) Monoclonal proliferation of Langerhans cells, no proof of malignancy LCH localized form: good prognosis disseminated multisystem disease: variable prognosis, course unpredictable
Langerhans cell histiocytosis (LCH) Organ involvement bones (90%, 30% single bone) skin (30-40%) lung ears eyes bowel liver spleen lymph nodes blood central nervous system mouth
Langerhans cell histiocytosis (LCH) Diagnosis histology (bone, skin, liver, lung) immunohistochemistry CD1a and/or CD207 Staging blood tests imaging: x-ray bones, thorax, bone scan (?) CT thorax MRI brain, depending on signs and symptoms
limitations LCH: imaging x-ray high sensitivity remains abnormal in lesions no longer metabolically active bone scan (not recommended) low sensitivity showes healing changes prior to x-ray remains positive in fully treated lesions limited usefulnes for therapeutic decisions
Schulte M, JNM 00; Aoki J, Radiology 01 some cases: high FDG-uptake in bone lesions with high concentration of histiocytes FDG PET Staging active osseous lesions? Monitoring therapy response? Showing soft-tissue involvement?
Daldrup-Link HE, Franzius C, et al, AJR 2001: 39 children, 9 children with LCH, osseous lesions: FDG PET vs. MRI vs. bone scan No. of lesions detected MRI 11 bone scan 8 FDG PET 13 total 14 FDG PET false negative finding: skull
Daldrup-Link HE, Franzius C, et al, AJR 2001: 39 children, 9 children with LCH, osseous lesions: FDG PET vs. MRI vs. bone scan True positive skull lesion University Hospital Muenster
Binkovitz, Pediatr Radiol 2003: 3 cases, coincidence FDG PET
Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 7-y-old boy, left hip pain baseline: bone scan
Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 7-y-old boy baseline: bone scan 4 weeks later: bone scan, PET
Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 7-y-old boy after chemotherapy bone scan, PET PET: correlation with response
Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 21-y-old patient: history of multifocal LCH, ecaluation prior to facial reconstructive surgery bone scan: multiple lesions PET: no active lesions no evidence of active disease during 1 year follow-up
Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 11-y-old boy bone scan: glenoid PET: glenoid histology: LCH, no evidence of multifocal disease on follow-up
Binkovitz, Pediatr Radiol 2003: coincidence FDG PET FDG PET identifies all sites of active disease (histologic confirmation) showes treatment response prior to bone scan excludes active disease in persistent abnormal sites on bone scan
Kaste S, Pediatric Radiology 2007: FDG PET-CT 5 cases
Kaste S, Pediatric Radiology 2007: FDG PET-CT 18-month-old boy, proptosis, limp left and right orbital lesion soft-tissue lesion in right orbit, destroying the sphenoid bone PET CT MRI
Kaste S, Pediatric Radiology 2007: FDG PET-CT 18-month-old boy, proptosis, limp bone scan PET LCH, multifocal disease
Kaste S, Pediatric Radiology 2007: FDG PET-CT 18-month-old boy after chemotherapy PET: resolution of all lesions bone scan: little change
Kaste S, Pediatric Radiology 2007: FDG PET-CT 7-month-old girl: anemia, irritability FDG PET-CT multifocal disease, bone and soft-tissue, more lesions than x-ray histology: LCH
Kaste S, Pediatric Radiology 2007: FDG PET-CT 7-month-old girl: anemia, irritability mild changes baseline after 6 weeks induction therapy
Kaste S, Pediatric Radiology 2007: FDG PET-CT 8-year-old girl: history of LCH, pain right femur x-ray PET CT
Kaste S, Pediatric Radiology 2007: FDG PET-CT 8-year-old girl: history of LCH, pain right femur 6 weeks later later: FDG PET/CT new lesion in the skull, x-ray negative, palpable mass chemotherapy
Kaste S, Pediatric Radiology 2007: FDG PET-CT 8-year-old girl: history of LCH, pain right femur PET CT after chemotherapy response
Kaste S, Pediatric Radiology 2007: FDG PET-CT 5 cases FDG PET-CT is able to delineate sites of metabolically active LCH influence therapy monitor therapy response estimate prognosis
Calming U, J Pediatr 2002: CNS LCH 7 cases metabolic response to chemotherapy
Calming U, J Pediatr 2002: CNS LCH 7 cases FDG PET increased metabolism: active ongoing disease decreased uptake: burnt-out lesions assessment of therapeutic effects additional information to MRI: alterations in CNS metabolic activity
Stauss J, Brigham and Women's Hospital and Children's Hospital Boston, Harvard Medical School, Boston, USA, 2008 27-year-old patient, headaches MRI CT
Stauss J, Boston LCH: FDG PET histology: eosinophilic granuloma bone scan after skull operation
Stauss J, Boston LCH: FDG PET bone scan after skull operation
Stauss J, Boston LCH: FDG PET
Stauss J, Boston LCH: FDG PET
Phillips M, Annual Meeting of the Histiocyte Society 2007 102 FDG PET scans 44 patients with biopsy proven LCH 83 scans with other imaging modalities 39% PET superior (new lesions, recurrence, response) 57% PET confirmed locations 5% PET false positive or false negative
Phillips M, Annual Meeting of the Histiocyte Society 2007 (abstract) 95% superior or confirmed 93% pelvic 100% rib 96% skull 71% vertebra bones 97% other bones 86% other sites (brain, lung, liver, lymph nodes,...)
Limitations LCH: low incidence multi-case reports standard of reference (follow-up?) no prospective, systematic evaluation no comparison with MR diffusion imaging and MR spectroscopy
LCH: Conclusion FDG PET and PET/CT detection of active lesions therapy response influence on therapy superior to conventional imaging