Sadece biyopsi uygulanan uzun kemik ve pelvis yerleşimli eozinofilik granülomların uzun dönem takipleri

Abstract

 

Amaç: Uzun kemik ve pelvis yerleşimli eozinofilik granülomu olan ve biyopsi sonrası ilave tedavi uygulanmayan hastaların uzun dönem klinik ve radyolojik sonuçları değerlendirildi.

 

Çalışma planı: Uzun kemik ve pelvis yerleşimli eozinofilik granülomu olan 17 çocuk (11 erkek, 6 kız; ortalama yaş 8.5 [dağılım, 3.5–14 yaş]) biyopsi sonrası takip edildi. Femur (5), tibia (3), humerus (2), ulna (1), fibula (1), pelvis (3), skapula (1) ve klavikula (1) tutulan kemikleri oluşturdu. Biyopsiyle tanının teyit edilmesini takiben hastalara hiçbir ilave cerrahi girişim uygulanmadı. Preoperatif ve postoperatif MSTS ekstremite fonksiyon skorlaması ve VAS ölçümleriyle klinik takip yapıldı. Lezyonun iyileşmesi periyodik radyografilerle izlendi. Tutulan kemiklerde lezyona bağlı uzunluk farkı veya deformite gelişimi ile lezyonun progresyonu veya nüksü kaydedildi. Ortalama takip süresi 65 ay (dağılım, 28–115 ay) idi.

 

Bulgular: Hastaların preoperatif ortalama MSTS skoru %45 (dağılım, %30–56.6) iken, postoperatif 6., 12. ve 24. ay takiplerdeki ortalama değerler sırasıyla %76 (dağılım, %70–83.3), %88 (dağılım, %73.3–93.3) ve %94 (dağılım, %86.6–100) ölçüldü. Ortalama preoperatif VAS skoru değerlendirmesi 8.4 (dağılım, 6–10) hesaplanırken, bu değer 3., 6. ve 12. aylarda sırası ile 3.5 (dağılım, 2–5), 2.2 (dağılım, 1–3.5) ve 1.1 (dağılım, 0–2) olarak belirlendi. Radyografik takip lezyonların çoğunluğunda 12. ayda, yassı kemiklerde ise 24. ayda tam iyileşmeyi gösterdi. Hiçbir hastada ekstremite uzunluk farkı veya deformite gelişmedi. Lezyonların tamamı biyopsi sonrası gerileme gösterdi, progresyon veya nüks gözlenmedi.

 

Çıkarımlar: Eozinofilik granülom spontan remisyon potansiyeli olan ve biyopsiyle tanının teyit edilmesi sonrası ilave girişim yapılmadan iyi fonksiyonel ve radyolojik sonuçların elde edilebileceği bir lezyondur.

 

Keywords

• Eozinofilik granülom; langerhans hücreli histiositoz.

Long-term follow-up of long and flat bone eosinophilic granulomas managed only with biopsy

Abstract

Objective: We evaluated long-term clinical and radiological follow-up results of patients with long and flat bone eosinophilic granulomas managed only with biopsy.
Methods: Seventeen patients [11 male, 6 female; average age 8.5 years (range: 3.5 to 14 years)] with long and flat bone eosinophilic granulomas were followed after biopsy. Involved bones were femur (5), tibia (3), humerus (2), ulna (1), pelvis (3), scapula (1) and clavicle (1). After confirmation of diagnosis by biopsy, no further surgical intervention was performed. Clinical follow-up was done with preoperative and postoperative MSTS and VAS scores. The healing process was followed with periodic radiographs. Limb-length discrepancy, deformity, and lesion progression or recurrence were recorded. Average follow-up was 65 months (range: 28 to 115 months).
Results: Average preoperative MSTS score was 45% (range: 30 to 56.6%), while postoperative 6, 12 and 24 months scores were measured as 76% (range: 70 to 83.3%), 88% (range: 73.3 to 93.3%) and 94% (range: 86.6 to 100%) respectively. Average VAS score, which was 8.4 (range: 6 to 10) preoperatively, had a tendency to decrease postoperatively, and was measured as 3.5 (range: 2 to 5), 2.2 (range: 1 to 3.5) and 1.1 (range: 0 to 2) at 3, 6, and 12 months. Even though the majority of lesions demonstrated complete radiographic healing at 12 months, the healing process extended to 24 months for flat bones. No patients experienced limb-length discrepancy or deformity. All lesions regressed following biopsy and no recurrence was seen.
Conclusion: Eosinophilic granuloma has a spontaneous healing potential, and confirming the diagnosis by biopsy is sufficient to obtain good functional and radiological results without any further
intervention.

Keywords

• Eosinophilic granuloma
• langerhans cell
• histiocytosis

References

1. Glotzbecker MP, Carpentieri DF, Dormans JP. Langerhans cell histiocytosis: a primary viral infection of bone? Human herpes virus 6 latent protein detected in lymphocytes from tissue of children. J Pediatr Orthop 2004;24:123–9.
2. Erol B, States L, Pawel BR, Tamai J, Dormans JP. Muscu- loskeletal tumors in children. In: Dormans JP, editor. Pedi- atric Orthopaedics and Sports Medicine: The Requisites in Pediatrics. Philadelphia: Mosby; 2004. p. 299–336.
3. Greenspan A, Remagen W. Round Cell Lesions. In: Greens- pan A, Remagen W, editors. Differential Diagnosis of Tu- mors and Tumor-Like Lesions of Bones and Joints. Phila- delphia: Lippincott-Raven Publishers; 1998. p. 247–88.
4. Lieberman PH, Jones CR, Steinman RM, Erlandson RA, Smith J, Gee T, et al. Langerhans cell (eosinophilic) granu- lomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol 1996;20:519–52.
5. Enneking WF. A system of staging musculoskeletal neo- plasms. Clin Orthop Relat Res 1986;204:9–24.
6. Enneking WF, Dunham W, Gebhardt MC, Malawar M, Pritchard DJ. A system for the functional evaluation of re- constructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop Relat Res 1993;286:241–6.
7. Velez-Yanguas MC, Warrier RP. Langerhans’ cell histiocy- tosis. Orthop Clin North Am 1996;27:615–23.
8. Lahey ME. Prognostic factors in histiocytosis X. Am J Pe- diatr Hematol Oncol 1981;3:57–60.

9. Bollini G, Jouve JL, Gentet JC, Jacquemier M, Bouyala JM. Bone lesions in histiocytosis X. J Pediatr Orthop 1991;11:469–77.
10. Sessa S, Sommelet D, Lascombes P, Prévot J. Treatment of Langerhans-cell histiocytosis in children. Experience at the Children’s Hospital of Nancy. J Bone Joint Surg Am 1994;76:1513–25.
11. Meyer JS, De Camargo B. The role of radiology in the di- agnosis and follow-up of Langerhans cell histiocytosis. He- matol Oncol Clin North Am 1998;12:307–26.
12. Erol B, Segal L, Huff D, States L, Dormans JP. Low back pain in a 13-year-old girl. Clin Orthop Relat Res 2003;413:323–31.
13. Broadbent V, Gadner H. Current therapy for Langer- hans cell histiocytosis. Hematol Oncol Clin North Am 1998;12:327–38.
14. Womer RB, Raney RB Jr, D’Angio GJ. Healing rates of treated and untreated bone lesions in histiocytosis X. Pedi- atrics 1985;76:286–8.
15. Egeler RM, Thompson RC Jr, Voûte PA, Nesbit ME Jr. In- tralesional infiltration of corticosteroids in localized Lang- erhans’ cell histiocytosis. J Pediatr Orthop 1992;12:811–4.
16. Capanna R, Springfield DS, Ruggieri P, Biagini R, Picci P, Bacci G, et al. Direct cortisone injection in eosinophilic granuloma of bone: a preliminary report on 11 patients. J Pediatr Orthop 1985;5:339–42.
17. Mavrogenis AF, Abati CN, Bosco G, Ruggieri P. Intrale- sional methylprednisolone for painful solitary eosinophilic granuloma of the appendicular skeleton in children. J Pedi- atr Orthop 2012;32:416–22.
18. Komp DM, Born LP. Langerhans cell histiocytosis. In: Moosa AR, Schimpff SC, Robson MC, editors. Compre- hensive textbook of oncology. 2nd ed. Baltimore: Williams & Wilkins; 1991. p. 1582–6.
19. Starling KA. Chemotherapy of histiocytosis-X. Hematol Oncol Clin North Am 1987;1:119–22.
20. Sumeet GBA, Dormans JP. Primary Tumors of the Spine in Children: A Review from the Pediatric Musculoskel- etal Tumor Program at the Children’s Hospital of Phila- delphia. The University of Pennsylvania Orthopaedic J 2003;16:19–29.