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Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases
Abstract:
Five patients had juvenile fibrosarcoma of the orbit and eyelid. At initial surgical intervention, the patients ranged in age from newborn to 8 years (mean, 3 years 9 months). Three patients were male and two were female. Proptosis and/or painless swelling of the eyelid were the most common signs and symptoms. Four tumors grew within a few weeks or months, and all five were poorly circumscribed. They were composed of immature, spindle-shaped fibroblastic cells in a classic herringbone pattern or in interlacing fascicles. Hypercellularity and mitotic activity were present in all lesions. The differential diagnosis included rhabdomyosarcoma, fibromatosis, and fibrous histiocytoma. Electron microscopy confirmed the fibroblastic nature of the tumor cells in three cases. Follow-up ranged from one to 32 years (median, seven years). Two of five tumors recurred locally but none metastasized. Apparently surgical excision is the treatment of choice and the tumor has a favorable prognosis.
Insights
Juvenile fibrosarcoma of the orbit and eyelid is a rare tumor in children. Surgical excision is the preferred treatment, offering a favorable prognosis with no observed metastasis in this study.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Surgical Pathology
Background:
- Juvenile fibrosarcoma is a rare soft tissue tumor affecting children.
- Orbital and eyelid involvement presents unique diagnostic and management challenges.
Purpose of the Study:
- To describe the clinical presentation, histopathology, and outcomes of juvenile fibrosarcoma in the orbit and eyelid.
- To evaluate the efficacy of surgical intervention for this rare pediatric tumor.
Main Methods:
- Retrospective case series of five pediatric patients with juvenile fibrosarcoma of the orbit and eyelid.
- Clinical data including age, sex, signs, symptoms, tumor characteristics, and follow-up were analyzed.
- Histopathological evaluation and electron microscopy were utilized for diagnosis.
Main Results:
- Patients ranged from newborn to 8 years old, presenting with proptosis or eyelid swelling.
- Tumors were poorly circumscribed, hypercellular, with mitotic activity, and spindle-shaped fibroblastic cells.
- Two of five tumors recurred locally; no distant metastasis was observed during a median follow-up of seven years.
Conclusions:
- Juvenile fibrosarcoma of the orbit and eyelid requires careful histopathological assessment.
- Complete surgical excision appears to be the primary treatment modality.
- The tumor demonstrates a favorable prognosis with a low risk of metastasis.