PET-CT in the diagnostics and monitoring of pulmonary Langerhans cell histiocytosis

Authors: Z. Adam 1;  Z. Řehák 2;  R. Koukalová 2;  P. Szturz 1;  L. Pour 1;  M. Krejčí 1;  P. Krupa 3;  T. Nebeský 4;  Z. Adamová 5;  J. Mayer 1
Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc. 1;  Oddělení nukleární medicíny a pozitronové emisní tomografie Masarykova onkologického ústavu Brno, přednosta prim. MUDr. Karol Bolčák 2;  Klinika zobrazovacích metod Lékařské fakulty MU a FN u sv. Anny Brno, přednosta doc. MUDr. Petr Krupa, CSc. 3;  Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil A. Válek, CSc. 4;  Chirurgické oddělení Nemocnice Vsetín, přednosta prim. MUDr. Jaroslav Sankot 5
Published in: Vnitř Lék 2010; 56(Supplementum 2): 123-130
Category: Langerhans cell histiocytosis and some other Hematology rare diseases


In a sample of 7 patients with pulmonary LCH, we monitored disease activity through repeated PET-CT examinations. The small size of LCH infiltrates in bronchioles (pulmonary nodules) precludes direct measurement of their activity. Therefore, SUVmax (maximum standardized uptake value) in the right lung and SUVmax in the liver were measured, as this was achievable with the available PET-CT software. The maximum activity measured in the right lung to maximum activity measured in the liver ratio was used to express the pulmonary activity of the Langerhans cell histiocytosis, i.e. the SUVmax pulmo/SUVmax liver index. In total, 25 measurements were performed in 7 patients with pulmonary LCH. This index values correlated with the development of the disease activity evaluated through overall patient status, HRCT imaging of the lungs and according to functional examination of the lungs. So far, this is a pilot project and the usefulness of this index in evaluating activity of the pulmonary LCH will require further verification.

Key words:
PET-CT – Langerhans cell histiocytosis – diabetes insipidus


1. Calming U, Jacobsson H, Henter JI. Detection of Langerhans cell histiocytosis lesions with somatostatin analogue scintigraphy – a preliminary report. Med Pediatr Oncol 2000; 35: 462–467.

2. Weinmann P, Crestani B, Tazi A et al. 111In-pentetreotide scintigraphy in patients with Langerhans’ cell histiocytosis. J Nucl Med 2000; 41: 1808–1812.

3. Lastoria S, Montella L, Catalano L et al. Functional imaging of Langerhans cell histiocytosis by 111In-DTPA-D-Phe(1)-octreotide scintigraphy. Cancer 2002; 94: 633–640.

4. Schmidt GP, Reiser MF, Baur-Melnyk A. Whole-body imaging of bone marrow. Semin Musculoskelet Radiol 2009; 13: 120–133.

5. Steiner M, Prayer D, Asenbaum S et al. Modern imaging methods for the assessment of Langerhans’ cell histiocytosis-associated neurodegenerative syndrome: case report. J Child Neurol 2005; 20: 253–257.

6. Grois N, Fahrner B, Arceci RJ et al. Histiocyte Society CNS LCH Study Group. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr 2010; 156: 873–881.

7. Ertan G, Huisman TA. Susceptibility weighted imaging in neurodeneneration in Langerhans cell histiocytosis. J Pediatr 2010; 156: 1032.

8. Grois N, Prayer D, Prosch H et al. CNS LCH Co-operative Group. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005; 128: 829–838.

9. Carpinteri R, Patelli I, Casanueva FF et al. Pituitary tumours: inflammatory and granulomatous expansive lesions of the pituitary. Best Pract Res Clin Endocrinol Metab 2009; 23: 639–650.

10. Daldrup-Link HE, Franzius C, Link TM et al. Whole-body MR imaging for detection of bone metastases in children and young adults: comparison with skeletal scintigraphy and FDG PET. AJR Am J Roentgenol 2001; 177: 229–236.

11. Naumann R, Beuthien-Baumann B, Fischer R et al. Simultaneous occurrence of Hodgkin’s lymphoma and eosinophilic granuloma: a potential pitfall in positron emission tomography imaging. Clin Lymphoma 2002; 3: 121–124.

12. Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J 2006; 27: 1272–1285.

13. Prasse A, Kayser G. Seltene zystische Lungenerkrankungen: Lymphangioleimyomatose, pulmonale Langerhanszell-Histiocytose, lymphocytäre interstitielle Pneumonie. Dtsch Med Wochenschr 2010; 135: 461–465.

14. Nagarjun Rao R, Moran CA, Suster S. Histiocytic disorders of the lung. Adv Anat Pathol 2010; 17: 12–22.

15. Krajicek BJ, Ryu JH, Hartman TE et al. Abnormal fluorodeoxyglucose PET in pulmonary Langerhans cell histiocytosis. Chest 2009; 135: 1542–1549.

16. Aoki J, Watanabe H, Shinozaki T et al. FDG PET of primary benign and malignant bone tumors: standardized uptake value in 52 lesions. Radiology 2001; 219: 774–777.

17. Calming U, Bemstrand C, Mosskin M et al. Brain 18FDG PET scan in central nervous system langerhans cell histiocytosis. J Pediatr 2002; 141: 435–440.

18. Blum R, Seymour JF, Hicks RJ. Role of 18FDG-positron emission tomography scanning in the management of histiocytosis. Leuk Lymphoma 2002; 43: 2155–2157.

19. Binkovitz LA, Olshefski RS, Adler BH. Coincidence FDG-PET in the evaluation of Langerhans’ cell histiocytosis: preliminary findings. Pediatr Radiol 2003; 33: 598–602.

20. Weiss SE, O’Connor L, Welsh JS. Refinement of radiation therapy based on PET data in an adult with Langerhans cell histiocytosis of soft tissues. Clin Adv Hematol Oncol 2006; 4: 290–292.

21. Ribeiro MJ, Idbaih A, Thomas C et al. 18F-FDG PET in neurodegenerative Langerhans cell histiocytosis: results and potential interest for an early diagnosis of the disease. J Neurol 2008; 255: 575–580.

22. Büchler T, Cervinek L, Belohlavek O et al. Langerhans cell histiocytosis with central nervous system involvement: follow-up by FDG-PET during treatment with cladribine. Pediatr Blood Cancer 2005; 44: 286–288.

23. Giovanella L, Ceriani L, Crippa S et al. Imaging in endocrinology: Langherans cell histiocytosis of the thyroid gland detected by 18FDG-PET/CT. J Clin Endocrinol Metab 2007; 92: 2866–2867.

24. Takahashi T, Yoshimoto M, Kondoh N. Spontaneously regressed Langerhans cell histiocytosis of lymph nodes in an elderly patient. Intern Med 2007; 46: 1757–1760.

25. Kaste SC, Rodriguez-Galindo C, McCarville ME et al. PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol 2007; 37: 615–622.

26. Derenzini E, Fina MP, Stefoni V et al. MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis: experience on seven patients. Ann Oncol 2010; 21: 1173–1178.

27. Phillips M, Allen C, Gerson P et al. Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer 2009; 52: 97–101.

28. Arnaud L, Malek Z, Archambaud F et al. 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in follow up than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum 2009; 60: 3128–3138.

29. Tan IB, Padhy AK, Thng CH et al. Intensely hypermetabolic extra-axial brainstem tumor in Erdheim-Chester disease. Clin Nucl Med 2009; 34: 604–607.

30. Fichter J, Doberauer C, Seegenschmiedt H. Langerhans cell histiocytosis in adults: An interdisciplinary challenge. Dtsch Arztebl 2007; 104: A2347–A2353.

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