SOLITARY BONE LESIONS IN CHILDREN WITH LANGERHANS CELL HISTIOCYTOSIS: A SURGICAL DISEASE OR NOT?


Ocak S., Kalyoncu Uçar A., Kuruğoğlu S., Çomunoğlu N., Özşahin M. K., Küçükyürük B., ...Daha Fazla

international society of pediatric oncology, Athens, Yunanistan, 22 - 24 Ekim 2023, cilt.70, ss.32, (Özet Bildiri)

  • Yayın Türü: Bildiri / Özet Bildiri
  • Cilt numarası: 70
  • Basıldığı Şehir: Athens
  • Basıldığı Ülke: Yunanistan
  • Sayfa Sayıları: ss.32
  • İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet

Özet

The aim of the study is to present clinical and prognostic features children with single bone lesion LCH treated in our institution. Between 2000- 2023, 64 children (20 girls, 44 boys) with monostotic LCH were admitted to our department. Median age on diagnosis was 7,4 years (range 1,7-15,9 yrs.). All but 2 children had either pain or swelling on the lesion site. Standard baseline laboratory evaluation with CT or MRI of the primary lesion were performed. All but 3 patients had histologic confirmation of LCH. The lesions were in calvarium (n=32), craniofacial bones (n=13), extremities (n=10), vertebra (n=4), pelvic bones and thoracic cage (n=5). The surgical resection/curettage was the most common first-line treatment (43/64 cases). Overall, 27/32 children had non-cns risk calvarial lesions and 8 small lesions located on the long bones had surgical intervention. After biopsy or surgical resection, 15 children received chemotherapy (vinblastine and prednisolone) for 6-12 months. Wait and watch strategy was resulted in spontaneous regression of the lesion in 5 children (without biopsy in 3) in 9-12 months. In 6 children, local or distant bony recurrence was observed and treated with chemotherapy. The median follow-up duration was 5,3 years (3 months-18,3 years). At last control 56 patient were alive with no active bone lesion. Scoliosis, diabetes insipidus and neurodegenerative LCH were developed in 3, 2 and 1 children, respectively. The growing data supports the treatment of monostotic bone lesion in LCH by conservative methods. In Turkey, the primary treatment of bone lesions in LCH are mostly at the surgeons' discretion. Our results showed that awareness for the new treatment approaches for monostotic LCH should be increased in our country.