PET Imaging in Langerhans Cell Histiocytosis

Transcription

1 PET Imaging in Langerhans Cell Histiocytosis Christiane Franzius Bremen, Germany

2 Histiocytosis Histiocytosis Idiopathic proliferation of histiocytes Two types of histiocytes macrophages: antigen processing dendritic cells: antigen presentation

3 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,

4 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/ children/year male:female: 2:1

5 Langerhans cell histiocytosis (LCH) Letterer-Siwe (infants, young children) hepatosplenomegaly lymphadenopathy cutaneous lesions otitis media pancytopenia pulmonary involvement fulminant, often fatal course

6 Langerhans cell histiocytosis (LCH) Hand-Schüller-Christian (older children) diabetes incipitus exophthalmos osteolytic lesions more indolent disease

7 Langerhans cell histiocytosis (LCH) Eosinophilic granuloma (older children, young adults) osteolytic lesions lung involvement more indolent disease

8 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

9 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

10 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

11 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

12 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?

13 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

14 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

15 Binkovitz, Pediatr Radiol 2003: 3 cases, coincidence FDG PET

16 Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 7-y-old boy, left hip pain baseline: bone scan

17 Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 7-y-old boy baseline: bone scan 4 weeks later: bone scan, PET

18 Binkovitz, Pediatr Radiol 2003: coincidence FDG PET 7-y-old boy after chemotherapy bone scan, PET PET: correlation with response

19 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

20 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

21 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

22 Kaste S, Pediatric Radiology 2007: FDG PET-CT 5 cases

23 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

24 Kaste S, Pediatric Radiology 2007: FDG PET-CT 18-month-old boy, proptosis, limp bone scan PET LCH, multifocal disease

25 Kaste S, Pediatric Radiology 2007: FDG PET-CT 18-month-old boy after chemotherapy PET: resolution of all lesions bone scan: little change

26 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

27 Kaste S, Pediatric Radiology 2007: FDG PET-CT 7-month-old girl: anemia, irritability mild changes baseline after 6 weeks induction therapy

28 Kaste S, Pediatric Radiology 2007: FDG PET-CT 8-year-old girl: history of LCH, pain right femur x-ray PET CT

29 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

30 Kaste S, Pediatric Radiology 2007: FDG PET-CT 8-year-old girl: history of LCH, pain right femur PET CT after chemotherapy response

31 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

32 Calming U, J Pediatr 2002: CNS LCH 7 cases metabolic response to chemotherapy

33 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

34 Stauss J, Brigham and Women's Hospital and Children's Hospital Boston, Harvard Medical School, Boston, USA, year-old patient, headaches MRI CT

35 Stauss J, Boston LCH: FDG PET histology: eosinophilic granuloma bone scan after skull operation

36 Stauss J, Boston LCH: FDG PET bone scan after skull operation

37 Stauss J, Boston LCH: FDG PET

38 Stauss J, Boston LCH: FDG PET

39 Phillips M, Annual Meeting of the Histiocyte Society 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

40 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,...)

41 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

42 LCH: Conclusion FDG PET and PET/CT detection of active lesions therapy response influence on therapy superior to conventional imaging