Clin Orthop Surg.  2009 Jun;1(2):63-67. 10.4055/cios.2009.1.2.63.

Management of Eosinophilic Granuloma Occurring in the Appendicular Skeleton in Children

• Han I
• Suh ES
• Lee SH
• Cho HS
• Oh JH
• Kim HS

• KMID: 1122634
• DOI: http://doi.org/10.4055/cios.2009.1.2.63

Abstract

BACKGROUND: We compared indomethacin therapy with the more aggressive approaches of anti-cancer chemotherapy and surgery in the treatment of isolated Langerhans cell histiocytosis (LCH) of bone in children.
METHODS
Comparisons were made with respect to healing of the lesion without recurrence, time to radiological healing of the lesion, time to functional recovery, and complications related to treatment.
RESULTS
Complete radiologic healing of the lesion (mean, 15.3 months) and functional recovery (mean, 5.6 months) were observed in all patients treated with either approach. No significant differences were noted in the time to complete radiologic healing or the time to functional recovery between the two groups. There were no recurrences with either approach until the last follow-up (mean, 56 months). Complications were common with anti-cancer chemotherapy, but indomethacin was well-tolerated.
CONCLUSIONS
Indomethacin seems to be effective for treating isolated LCH of bone in children. Hence, morbidities associated with aggressive treatment approaches such as anti-cancer chemotherapy or surgery can be avoided.

Keyword

Langerhans cell histiocytosis; Indomethacin; Children

MeSH Terms

Adolescent
Anti-Inflammatory Agents, Non-Steroidal/*therapeutic use
Antineoplastic Combined Chemotherapy Protocols/adverse effects/therapeutic use
Bone Diseases/*drug therapy/radiography/radionuclide imaging
Child
Child, Preschool
Cyclooxygenase Inhibitors/*therapeutic use
Eosinophilic Granuloma/*drug therapy/radiography/radionuclide imaging
Female
Humans
Indomethacin/*therapeutic use
Infant
Male
Recurrence

Figure

• 

  Fig. 1 An illustrative case of isolated LCH of radius in a 5-year-old boy treated with indomethacin. (A) A destructive lesion of the radius is seen. (B) Radiographs made 3 months after treatment shows progressive healing of the lesion. (C) Radiographs made 15 months after treatment demonstrates the complete disappearance of the lesion and remodeling of the bone.

Reference

1. Willman CL, Busque L, Griffith BB, et al. Langerhans'-cell histiocytosis (histiocytosis X): a clonal proliferative disease. N Engl J Med. 1994. 331(3):154–160.

2. Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK. Langerhans' cell histiocytosis (histiocytosis X) of bone: a clinicopathologic analysis of 263 pediatric and adult cases. Cancer. 1995. 76(12):2471–2484.

3. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 1999. 85(10):2278–2290.

4. Komp DM. Langerhans cell histiocytosis. N Engl J Med. 1987. 316(12):747–748.

5. Plasschaert F, Craig C, Bell R, Cole WG, Wunder JS, Alman BA. Eosinophilic granuloma: a different behaviour in children than in adults. J Bone Joint Surg Br. 2002. 84(6):870–872.

6. Womer RB, Raney RB Jr, D'Angio GJ. Healing rates of treated and untreated bone lesions in histiocytosis X. Pediatrics. 1985. 76(2):286–288.

7. Sessa S, Sommelet D, Lascombes P, Prevot J. Treatment of Langerhans-cell histiocytosis in children: experience at the Children's Hospital of Nancy. J Bone Joint Surg Am. 1994. 76(10):1513–1525.

8. Capanna R, Springfield DS, Ruggieri P, et al. Direct cortisone injection in eosinophilic granuloma of bone: a preliminary report on 11 patients. J Pediatr Orthop. 1985. 5(3):339–342.

9. Ladisch S, Gadner H. Treatment of Langerhans cell histiocytosis: evolution and current approaches. Br J Cancer Suppl. 1994. 23:S41–S46.

10. Greis PE, Hankin FM. Eosinophilic granuloma: the management of solitary lesions of bone. Clin Orthop Relat Res. 1990. (257):204–211.

11. Arenzana-Seisdedos F, Barbey S, Virelizier JL, Kornprobst M, Nezelof C. Histiocytosis X Purified (T6+) cells from bone granuloma produce interleukin 1 and prostaglandin E2 in culture. J Clin Invest. 1986. 77(1):326–329.

12. Gonzalez-Crussi F, Hsueh W, Wiederhold MD. Prostaglandins in histiocytosis-X: PG synthesis by histiocytosis-X cells. Am J Clin Pathol. 1981. 75(2):243–253.

13. Munn SE, Olliver L, Broadbent V, Pritchard J. Use of indomethacin in Langerhans cell histiocytosis. Med Pediatr Oncol. 1999. 32(4):247–249.

14. McLean TW, Pritchard J. Langerhans cell histiocytosis and hypercalcemia: clinical response to indomethacin. J Pediatr Hematol Oncol. 1996. 18(3):318–320.

15. Komp DM, Herson J, Starling KA, Vietti TJ, Hvizdala E. A staging system for histiocytosis X: a Southwest Oncology Group Study. Cancer. 1981. 47(4):798–800.

16. Raab P, Hohmann F, Kuhl J, Krauspe R. Vertebral remodeling in eosinophilic granuloma of the spine: a long-term follow-up. Spine. 1998. 23(12):1351–1354.

17. Yeom JS, Lee CK, Shin HY, Lee CS, Han CS, Chang H. Langerhans' cell histiocytosis of the spine: analysis of twenty-three cases. Spine. 1999. 24(16):1740–1749.

18. Egeler RM, Thompson RC Jr, Voute PA, Nesbit ME Jr. Intralesional infiltration of corticosteroids in localized Langerhans' cell histiocytosis. J Pediatr Orthop. 1992. 12(6):811–814.

19. Scaglietti O, Marchetti PG, Bartolozzi P. Final resu lts obtained in the treatment of bone cysts with methylprednisolone acetate (depo-medrol) and a discussion of results achieved in other bone lesions. Clin Orthop Relat Res. 1982. (165):33–42.

20. Yasko AW, Fanning CV, Ayala AG, Carrasco CH, Murray JA. Percutaneous techniques for the diagnosis and treatment of localized Langerhans-cell histiocytosis (eosinophilic granuloma of bone). J Bone Joint Surg Am. 1998. 80(2):219–228.