Extraconal orbital tumors in children—a spectrum

Abstract

Orbital masses in children are uncommon but extremely challenging problems for clinicians and pathologists due to their critical location and availability of limited diagnostic material. We analyzed 47 specimens comprising biopsies, excision specimens, and FNAC of extraconal pediatric orbital masses (excluding retinoblastoma) accessioned in the pathology department over 5 years in a tertiary referral cancer center. Immunohistochemistry (IHC-74%) and molecular methods (one case) were done where necessary. The chief presenting symptom was proptosis in 55.3% patients and radiologically 53.8% malignant tumors showed extraorbital extension. A diagnostic algorithm was formulated to assess which cases needed pathology evaluation. Malignant round cell tumors (76.6%), chiefly embryonal rhabdomyosarcoma (51%), benign spindle cell neoplasms, and infectious lesions (tuberculosis, fungal infections), were seen. Of the malignant tumors, those confined to the orbit achieved good treatment response and had an event-free follow-up while those with extraorbital spread had poor outcome. Pediatric orbital masses range from completely treatable infectious lesions, surgically resectable benign neoplasms to aggressive malignancies requiring chemotherapy and radiotherapy. Pathologists play a key role in distinguishing these on small biopsy material and expediating accurate treatment thus saving the vision or life of a patient.

Visual function alterations in orbital tumors and factors predicting visual outcome after surgery

“…2,5,9 The frequency and distribution of tumor diagnosis and their association with age is in accordance with past studies that too have demonstrated a relatively higher frequency of vascular and lacrimal gland tumors among operable orbital neoplasms. 3,5,[9][10][11][12][13] Proptosis is the commonest presentation reported in orbital tumors, and in our series the mean proptosis is quite similar to previous data. 3,14,15 In effect, our series is a universally representative sample of orbital tumors.…”

Quiste dermoide en la infancia bajo el diagnóstico de ptosis

“…Por su localización orbitaria, el diagnóstico de inicio es clínico. Son necesarias las técnicas de imagen (TAC o RMN) para la confirmación de la presencia de una masa orbitaria, y para valorar localización y extensión 5 .…”

Role of Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in Various Orbital Malignancies

“…Currently, there is not enough evidence to suggest routine use of PET/CT in orbital RMS at present. [ 10 11 ]…”