Acta Oncol Tur.. 2019; 52(2): 282-288 | DOI: 10.5505/aot.2019.75046

Calvarial Langerhans cell histiocytosis: single center experience of 29 cases

Sahin Hanalioglu¹, Ilkay Isikay^2
1Health Sciences University, Dýþkapý Yýldýrým Beyazýt Trainin and Research Hospital, Neurosurgery Clinic, Ankara, Turkey
²Hacettepe University Medical School, Neurosurgery Department, Ankara, Turkey

INTRODUCTION: In this study, our aim was to analyse and discuss epidemiology, clinical and radiologic features, differential diagnosis, histopathologic characteristics, key elements of treatment strategies and follow-up results of Langerhans cell histiocytosis (LCH) in a 29-patient clinical series.
METHODS: All the patients who had been operated for multiple or solitary calvarial LCH between 2001-2018 in our clinic were included in our study. Patient discharge notes, radiologic workup, operation records, patholoy reports and follow-up data were retrospectively evaluated. Age, sex, location and size of the lesion; radiologic features, extent of resection, histopathologic features; adjuvant treatment, recurrence, reoperation and follow-up data were collected.
RESULTS: In total 29 patients (15 male, 14 female) were evaluated. Except one, all patients (97%) were under 18 years of age. Patients’ mean age at the time of diagnosis was 8.4±7.7 years (median 8; range 1-38). Most of the patients admitted with subdermal/palpable mass. In 5 patients there were multiple calvarial and/or other organ involvement. The most common location was parietal bone. In 3 patients with multiple lesions, only biopsy was performed; other patients undergone total extirpation of the primary/solitary lesion. Patients with multiple lesions and other organ involvement had chemotherapy. Most of the patients had a remission whereas two patients developed progression of residue in the follow-up period.
DISCUSSION AND CONCLUSION: Langerhans cell histiocytosis is a benign and rare calvarial lesion, in which total surgical excision is an effective and succesful treatmet. A thorough evaluation of a patient with suspected LCH is needed for differential diagnosis and in order to identify extracalvarial lesions. In cases where total excision is not possible and for patients with multiple and/or skull base involvement chemotherapeutics may be used.

Keywords: Eosinophilic granuloma, Langerhans cell histiocytosis, skull, calvarium, pediatrics

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Kafatasý yerleþimli Langerhans hücreli histiyositoz: 29 olguluk tek merkez deneyimi

Sahin Hanalioglu¹, Ilkay Isikay^2
1Saðlýk Bilimleri Üniversitesi, Dýþkapý Yýldýrým Beyazýt Eðitim ve Araþtýrma Hastanesi, Nöroþirürji Kliniði, Ankara, Türkiye
²Hacettepe Üniversitesi Týp Fakültesi, Nöroþirürji Ana Bilim Dalý, Ankara, Türkiye

GÝRÝÞ ve AMAÇ: Bu çalýþmanýn amacý kalvaryum yerleþimli Langerhans hücreli histiyositozlarýn (LHH) epidemiyolojisi, klinik-radyolojik özellikleri, ayýrýcý tanýsý, histopatolojik karakteristikleri, tedavi stratejilerindeki kilit noktalarý ve takip sonuçlarýný 29 hastalýk bir klinik seriden elde edilen deneyimler ýþýðýnda analiz etmek ve tartýþmaktýr.
YÖNTEM ve GEREÇLER: Çalýþmaya 2001-2018 yýllarý arasýnda, kafatasý yerleþimli soliter veya multiple LHH nedeniyle kliniðimizde ameliyat edilen tüm hastalar dahil edildi. Hasta epikrizleri, radyolojik görüntüleri, ameliyat notlarý, patoloji sonuçlarý ve takip verileri retrospektif olarak incelendi. Yaþ, cinsiyet, lezyonun yeri ve büyüklüðü, görüntüleme özellikleri, cerrahi rezeksiyonun derecesi, histopatolojik özellikler, adjuvan tedavi, nüks, reoperasyon ve takip sonuçlarýný içeren veriler toplandý.
BULGULAR: Toplam 29 hasta (15 erkek, 14 kýz) tespit edildi. Hastalarýn biri dýþýnda tamamý (%97) çocukluk yaþ grubundaydý. Hastalarýn taný anýndaki yaþ ortalamasý 8,4±7,7 yýldý (ortanca yaþ 8 yýl; aralýk 1-38 yýl). Hastalarýn büyük çoðunluðu cilt altý þiþlik / ele gelen kitle þikayeti ile baþvurdu. Toplam 5 hastada multipl kalvaryal ve/veya diðer organ tutulumlarý tespit edildi. En sýk yerleþim yeri parietal kemik idi. Multiple lezyonlarý olan 3 hastada sadece biyopsi yapýlýrken diðer tüm hastalarda tek veya primer lezyonun total eksizyonu saðlandý. Multipl lezyonlarý ve ek organ tutulumlarý olan olgulara kemoterapi uygulandý. Hastalarýn çoðunda olguda remisyon saðlanýrken, takipte 2 hastada rezidü progresyonu tespit edildi.
TARTIÞMA ve SONUÇ: Benign ve nadir bir kafatasý lezyonu olan LHH olgularýnda cerrahi total eksizyon tek baþýna en etkili ve baþarýlý tedavi yöntemidir. LHH þüphesi olan tüm hastalarda ayýrýcý taný iyi yapýlmalý ve ekstrakalvarial lezyon bakýmýndan tüm vücut taranmalýdýr. Cerrahi olarak total eksizyonun mümkün olmadýðý multipl ve/veya kafa kaidesi yerleþimli olgular ile nüks izlenen olgularda kemoterapötik ajanlar kullanýlabilir.

Anahtar Kelimeler: eozinofilik granüloma, langerhans hücreli histiyositoz, kafatasý, kalvaryum, pediatrik

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