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Orbital (desmoid type) fibromatosis
A Fornelli1, F Salvi, M Mascalchi
1Department of Oncology, Section of Anatomic Pathology "M. Malpighi", Bologna, Italy
Orbit (Amsterdam, Netherlands)
|June 5, 2002
Summary
Extra-abdominal fibromatosis (EF) is a rare orbital tumor. This case study details a 35-year-old man with EF, highlighting its MRI characteristics and successful surgical removal without recurrence.
Area of Science:
- Ophthalmology
- Oncology
- Radiology
Background:
- Extra-abdominal fibromatosis (EF) is a rare benign mesenchymal tumor.
- Orbital involvement of EF is exceptionally uncommon, presenting diagnostic challenges.
Purpose of the Study:
- To describe a rare case of orbital extra-abdominal fibromatosis (EF).
- To highlight the magnetic resonance imaging (MRI) features of orbital EF.
- To report the outcome of surgical management for this condition.
Main Methods:
- Case report of a 35-year-old male with orbital mass.
- Magnetic resonance imaging (MRI) evaluation including T1-weighted, T2-weighted, and contrast-enhanced sequences.
- Histopathological examination for definitive diagnosis.
- Surgical excision and clinical follow-up.
Main Results:
- The patient presented with progressive exophthalmos, ptosis, and diplopia.
- MRI revealed an orbital mass with specific signal characteristics (intermediate T1WI, low T2WI, non-homogeneous enhancement).
- Histopathology confirmed the diagnosis of extra-abdominal fibromatosis.
- Complete surgical removal was achieved, with no recurrence over 40 months.
Conclusions:
- Orbital extra-abdominal fibromatosis is a rare entity that can mimic other orbital pathologies.
- Characteristic MRI findings can aid in the diagnosis of orbital EF.
- Surgical excision is an effective treatment modality with a favorable long-term prognosis.