AI Summary & Key Takeaways
AI InsightConclusion : The disease classified as single focus or multi focus eosinophilic granuloma in the recent years should come to mind in the cases of periorbital edema. ptosis. and proptosis during childhood with the orbital tomography showing lytic lesions in the bone tissue and/or soft tissue masses.
📌 3 Key Takeaways
Abstract
Purpose : We aimed to present two cases of Langerhans´ cell histiocytosis (histiocytosis X) showing orbital involvement. Methods : Case 1: On the CT and MRI of the 3 year old patient having proptosis on the left eye. there was destruction on the left temporal and sphenoid bones and on the lateral wall of the orbital cavity. and there was a mass lesion on the orbital roof. After the curettage of the orbital mass. the lesion was evaluated cytopathologically. Case 2: On the CT of the 4 year old patient having proptosis on the left eye. there was a mass lesion extending to the orbital roof. whose margins could not be differentiated from the lateral rectus. and there were widespread lytic lesions all over the cranium. The biopsy taken from the hairy skin with diffused squames was evaluated cytopathologically. Results : After the cytopathological evaluation. both cases were diagnosed as Langerhans´ cell histiocytosis. Case 1 was referred to the oncology hospital with the diagnosis of single focus langerhans´ cell (eosinophilic) granuloma and radiotherapy was performed. After 1 year of remission there was a relapse seen as a partial diabetes insipidus and a mass lesion at the infundibulum detected in MRI. Case 2 was referred to the oncology hospital with the diagnosis of multi focus langerhans´ cell granuloma and died during the chemotherapy sessions. Conclusion : The disease classified as single focus or multi focus eosinophilic granuloma in the recent years should come to mind in the cases of periorbital edema. ptosis. and proptosis during childhood with the orbital tomography showing lytic lesions in the bone tissue and/or soft tissue masses.
Özet
Amaç: Orbita tutulumu gösteren Langerhans hücreli histiositozis (histiositozis X) saptanan iki olguyu takdim etmek. Hastalar ve Yöntem: Olgu 1: 3 yaşında sol gözde proptozis bulunan olgunun beyin ve orbita bilgisayarlı tomografi ve magnetik resonans görüntülemesinde sol temporal ve sfenoid kemik ile orbita dış duvarında doku yıkımı ve orbita tavanında kitle saptandı. Orbita kitlesi kü-rete edilerek histopatolojik olarak değerlendirildi. Olgu 2: 4 yaşında, sol gözde proptozis mev¬cut olan olgunun bilgisayarlı tomografisinde dış rektusdan sınırları tam ayırt edilemeyen orbita tavanına uzanan kitle, kafatasında yaygın litik lezyonlar tespit edildi. Yaygın skuamlar bulunan saçlı deriden alınan biyopsi histopatolojik olarak değerlendirildi. Bulgular: Her iki olgunun histopatolojik değerlendirme sonucu Langerhans hücreli histio¬sitozis olarak belirlendi. Olgu 1, tek odaklı Langerhans hücreli (eozinofilik) granulom tanısı ile onkoloji hastanesine sevk edilip radyoterapi uygulandı. Olguda 1 yıl süren remisyon sonrası gelişen parsiyel diabetes insipitus ve magnetik rezonans görüntülemede infindubulumda kitle lezyonu ile nüks gelişti. Olgu 2 ise, çok odaklı langerhans hücreli granulom tanısı ile onkoloji hastanesinde kemoterapi uygulaması esnasında sepsis nedeniyle kaybedildi. Sonuç: Son yıllarda tek odaklı ve çok odaklı eozinofilik granulom olarak da sınıflanan bu hastalık, çocukluk çağında periorbital ödem, ptozis, proptozis saptanan ve orbital tomografide kemik dokuda litik lezyonlar ve / veya yumuşak doku kitlesi saptanan olgularda hatırlanmalıdır.