Abstract
Introduction
Langerhans’ cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells, which is seen extremely rarely in adults. Conventional imaging modalities, such as skeletal surveys and bone scans, were accepted to be standard methods for diagnosis; however, 18-fluorodeoxyglucose positron emission tomography (PET-CT) has been increasingly used.
Methods
We report on a 33-year-old female patient with disseminated LCH treated with radiotherapy and systemic chemotherapy where PET-CT has been used for defining the extent of the disease, RT planning and assessment of treatment response during follow-up.
Results
The patient was treated with 24 Gy 3-dimensional conformal radiotherapy (RT), given as 2 Gy a day, 5 days a week. The patient was also treated with systemic prednisolone 20 mg/m2, concurrently. The chemotherapeutic regimen switched to cytosine-arabinoside with a dose of 100 mg/m2 subcutaneously daily for 4 days, vincristine 1.5 mg/m2 given on the 1st day and prednisolone 20 mg/m2 for 4 cycles. After local RT with adjuvant chemotherapy, the patient was alive for 54 months and remained disease-free at last visit.
Conclusion
RT is a treatment choice in multi-system LCH as well as solitary lesions. Low-dose RT is adequate to control large masses of LCH including soft tissue and lymph nodes.
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References
Howarth DM, Gilchrist GS, Mullan BP, et al. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 1999;85:2278–90.
Willman CL, Busque L, Griffith BB, et al. Langerhans’-cell histiocytosis (histiocytosis X)—a clonal proliferative disease. N Engl J Med. 1994;331:154–60.
Olschewski T, Seegenschmiedt MH. Radiotherapy for bony manifestations of Langerhans cell histiocytosis. Review and proposal for an international registry. Strahlenther Onkol. 2006;182:72–9.
Azouz EM, Saigal G, Rodriguez MM, et al. Langerhans’ cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol. 2005;35:103–15.
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 (discussion 292–294).
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.
Baumgartner I, von Hochstetter A, Baumert B, et al. Langerhans’-cell histiocytosis in adults. Med Pediatr Oncol. 1997;28:9–14.
Edelweiss M, Medeiros LJ, Suster S, et al. Lymph node involvement by Langerhans cell histiocytosis: a clinicopathologic and immunohistochemical study of 20 cases. Hum Pathol. 2007;38:1463–9.
Olschewski T, Seegenschmiedt MH. Radiotherapy of Langerhans’ cell histiocytosis: results and implications of a national patterns-of-care study. Strahlenther Onkol. 2006;182:629–34.
Kaste SC, Rodriguez-Galindo C, McCarville ME, et al. PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 2007;37:615–22.
Lee HJ, Ahn BC, Lee SW, et al. The usefulness of F-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Langerhans cell histiocytosis. Ann Nucl Med. 2012;26:730–7.
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Onal, C., Oymak, E., Reyhan, M. et al. Multifocal soft tissue Langerhans’ cell histiocytosis treated with PET-CT based conformal radiotherapy. Jpn J Radiol 33, 603–606 (2015). https://doi.org/10.1007/s11604-015-0466-6
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DOI: https://doi.org/10.1007/s11604-015-0466-6