Spontaneous Regression Of An Orbital Langerhans Cell Histiocytosis After Biopsy: A Case Report. Spontaneous Regression Of An Orbital Langerhans Cell Histiocytosis
Yahiaoui, S., Ghorbel, A., Ben Zid, K., Zarraa, S., Kchaou, L., FidaNoubigh, Kaabia, W., Bouguerra, F., Mousli, A., Abidi, R., Yousfi, A., Nadia, B., & Nasr, C. (2022). Spontaneous regression of an orbital Langerhans cell histiocytosis after biopsy: A case report. Spontaneous regression of an orbital Langerhans cell histiocytosis. Rare tumors, 14, 20363613221135987. https://doi.org/10.1177/20363613221135987
View Original Source →Abstract
Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.
Case Details
Disease Location
Orbita and dura mater
Personal Characteristics
18 year-old male
Clinical Characteristics
Referred after discovering a left orbital mass. Physical examination found a tumor of the left superolateral orbit involving bone and dura associated to left exophthalmia. CT and MRI showed a superolateral extraconal tissular mass extending through the superior orbital fissure and along the dura mater, measuring 36.6 mm x 32.7 mm. Histopathological examination disclosed clusters of langerhans cells admixed with numerous eosinophils and osteoclast-like giant cells. Langerhans cells have grooved, indented and lobed nuclei with inconspicuous nucleoli. The cytoplasm is moderately abundant and slightly eosinophilic. On immunohisto- chemistry, langerhans cells express s100 and cd1a
Remission Characteristics
CT was taken to plan radiation therapy, and had shown no infiltrative lesion or abnormalities of the orbital bone to delineate. Orbital MRI revealed no abnormalities. The lesion had completely disappeared in later orbital MRI at 9 months
Treatment & Mechanisms
Proposed Remission Mechanisms
Biopsy related
Clinical Treatment
Biopsy
Non-Clinical Treatment
None reported