Orbital histiocytosis and fibrohistiocytosis: the clinicopathological characteristics of 117 patients, over a decade of experience

• Published in: International ophthalmology 2023-12, Vol.43 (12), p.4997-5009
• Main Authors: Rajabi, Mohammad Taher, Amoli, Fahimeh Asadi, Koochakzadeh, Leili, Rafizadeh, Seyed Mohsen, Rajabi, Mohammad Bagher, Hosseini, Seyedeh Simindokht, Aghajani, Amir Hossein, Pezeshgi, Saharnaz, Aghdam, Masoud Abdolahzadeh, Farrokhpour, Hossein, Abla, Oussama, Sadeghi, Reza
• Format: Article
• Language: English
• Subjects: Acuity; Age; Autoimmune diseases; Biopsy; Bone imaging; Computed tomography; Demographics; Diagnosis; Disease; Disorders; Eye; Females; Hematology; Histiocytosis; Hospitals; Immunohistochemistry; Kinases; Light microscopy; Medical imaging; Medical records; Medicine; Medicine & Public Health; Microscopy; Oncology; Ophthalmology; Optical microscopy; Original Paper; Pathology; Patients; Research centers; Statistical analysis; Tumors; Variables; Visual acuity
• ISSN: 1573-2630; 0165-5701; 1573-2630
• DOI: 10.1007/s10792-023-02903-y

Purpose To describe the clinicopathological features of a large cohort of patients with orbital histiocytoses and fibrohistiocytosis, such as Langerhans cell histiocytosis (LCH) and non-LCH disorders, and correlate patients’ clinical characteristics with their pathological diagnosis. Methods In this retrospective study, medical records of patients presenting to Farabi Eye Hospital, a tertiary eye care center in Tehran, Iran, from 2010 until 2022, were reviewed. Patients’ demographics, chief complaint, location and laterality of the tumor, best-corrected visual acuity, presence of bone erosion on imaging, and their pathological diagnosis were retrieved. Excisional biopsy was performed and evaluated through light microscopy and immunohistochemistry study for their respective markers, including CD1a, CD68, CD207, and S100. Results A total of 117 patients with 11 pathological subtypes of histiocytoses and fibrohistiocyosis were identified, with 56.4% male and 43.6% female patients. The mean age at presentation was 23.4 years (range 1.5 months–73 years). Swelling and palpable mass were the most common chief complaints. LCH was the most common pathology (32.5%), followed by juvenile xanthogranuloma (26.5%) and adult xanthogranuloma (21.4%). Age, lesion location, and bone erosion had a statistically significant difference among the various diagnosed subtypes. Conclusions Histiocytoses and fibrohistiocytosis are diverse and rare disorders potentially involving multiple organ systems. Ophthalmic manifestations of these diseases are even more uncommon. We reviewed their orbital presentation along with their respective histopathological findings. Our results also suggested that an orbital CT scan can be of diagnostic value to discriminate LCH from other histiocytic pathologies.