25 YEARS OF LANGERHANS CELL HISTIOCYTOSIS EXPERIENCE OF A SINGLE CENTER

İnce D., Kızmazoğlu D., Çeçen R. E., Güleryüz Uçar H., Özer E., Olgun H. N.

The 54th Annual Congress of the International Society of Paediatric Oncology, Barcelona, İspanya, 28 Eylül - 01 Ekim 2022, ss.1859

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: Barcelona
  • Basıldığı Ülke: İspanya
  • Sayfa Sayıları: ss.1859
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

Background and Aims

Evaluation of patients diagnosed with Langerhans cell histiocytosis (LCH) and treated in our center.

Methods

The medical records of patients with LCH were rewieved. The clinical characteristics, treatment details and responses were analyzed retrospectively.

Results

There were 60 patients with LCH between 1987-2022. The median age of diagnosis was 55 months (2.5mos –18yrs), M/F=1.3. Single system involved LCH (SSIG-LCH) (68%, n:41); involvement sites were bone (88%), skin (7%), lymph node (2.5%), lung (2.5%). Nine patients had vertebral involvement. Surgery was performed in 66%, chemotherapy consisted vinblastin, prednisolone+methotrexate, mercaptopurine was given in 63%. Radiotherapy (RT) was given in 12% of cases (3 of them vertebral LCH). Relapse occured in 5 cases. The median follow-up time was 6.5yrs (1month -18yrs), 15-years OS rate was 100% and 5-year and 15-years EFS rates were 84%. Multisystem involved LCH (MSIG-LCH) (32%, n:19); involvement sites were bone (95%), skin (53%), lung (42%), pituitary gland (21%), Liver (16%), Gastrointestinal system (16%), spleen (5%). Eight of them had risky organ (RO) involvement, 4 of 8 patients had lung involvement as RO. Surgery was performed in 58% of cases, chemotherapy consisted vinblastin, prednisolone+methotrexate, mercaptopurine was given in all 19, and RT was given in two patients. Refractory disease (n:2) and relapse (n:6) occured in 8 patients. The median follow-up time was 5years (5months-17yrs), the 2-and 15- years OS rates were 88% and EFS rates were 39%. One infant had skin+pulmonary LCH (risky organ), and died with refractory LCH despite anticancer treatment at 21months. Other infant died from meningococcemia. Remission achieved with vemurafenib in two cases.

Conclusions

In MSI-LCH group age tends to be younger than SSI-LCH, these patients particularly had RO involvement. Although lung involvement is not considered as risky organ, pulmonary LCH can be fatal especially in the infancy. In relapsed/ refractory cases, remission can be achieved by anti-BRAF drugs.