Multidisciplinary teams were involved in the management, including an oral and maxillofacial surgeon, an ophthalmologist, a pediatric oncologist, a radiologist, and a histopathologist. The initial investigation was designed as a basic laboratory investigation and included the following: complete blood count; urea and electrolyte analysis; liver function tests; analysis of the coagulation profile; tissue biopsy; bone marrow biopsy; fluorescence in-situ hybridization; a preoperative imaging study including a bone scan to rule out bone marrow involvement; computed tomography scan of the brain, orbit, chest, abdomen, and pelvis; and MRI of the orbit.
Differential diagnosis included rhabdomyosarcoma, metastatic tumors, benign tumors, and hemangioma. Results of laboratory examinations were within normal limits. Tissue biopsy showed a fibrosarcoma (Fig. 4). The fluorescence in-situ hybridization test showed that none of the cells had a split ETV6 signal, and it was unlikely that there was a t(12;15) (p13;q25) present. Bone marrow biopsy showed no metastasis. Preoperative and prechemotherapy brain, orbit, chest, abdomen, and pelvis CT scans showed a left extra-ocular mass with no evidence of metastasis. Preoperative and prechemotherapy orbital MRI suggested a relatively slow-growing, well-defined, left extra-ocular mass compatible with an inflammatory pseudotumor or rhabdomyoma that was unlikely to be a vascular mass or sarcoma.
Nuclear medicine bone scan showed left eye proptosis and a hypervascular left orbit soft tissue pathology with no scintigraphic evidence of direct or distant bone involvement and mildly increased left maxillary sinus activity, which may have been related to patient positioning.
Further management was designed as preoperative chemotherapy including six cycles of vincristine (0.05 mg/kg), actinomycin (0.025 mg/kg), and cyclophosphamide (40 mg/kg), followed by postchemotherapy imaging studies, surgical intervention through excision of the orbital mass, postoperative brain, orbital, chest, and abdominal CT scans, and orbital MRI studies (Fig. 5).
Surgical intervention included craniotomy. This involved indexing the upper orbit rim with screws and plates, removing the screws and plates, and retracting the brain; the entire orbital roof was then resected up to the anterior cranial fossa, exposing the tumor, which was removed. Thereafter, the orbital roof with the orbital rim was placed back in the original position and fixed in place with plates and screws. The cranium was placed back and final closures made (Fig. 6).
The whole specimen was sent for histopathological examination and showed poorly circumscribed spindle cell proliferation that was myxoid with the focal area showing increased cellularity (Fig. 7). The spindle cells were medium sized but displayed hyperchromasia and pleomorphism and irregular nuclear contours. Almost the entire lesion was embedded in the myxoid matrix, which was similar to the focal myxoid area seen in the previously excised specimen.
The prominent hypercellular herringbone pattern displayed in the previously excised specimen was not seen here. Mitoses were sparse approximating 2–3/10 HPF and were less than the mitoses seen in the hypercellular area in the previous specimen but parallel to the positive mitoses seen in the myxoid areas of the previous specimen. The spindle cell proliferation infiltrated into the surrounding soft tissue and was seen at several inked margins. It also infiltrated into a ganglionic nucleus. From the above-mentioned features a conclusion was drawn of a 3.5 cm congenital fibrosarcoma exhibiting predominantly myxoid features.
Frequent follow-up after one and half years showed no growth, no proptosis, and better eye movements and vision (Fig. 8).
A child with orbital tumors differs substantially from an adult patient with a high incidence of congenital lesions, a higher frequency of infection, and a unique benign and malignant tumor involving the orbit. The most common orbital tumors presenting in children are cystic lesions of the orbit, mainly dermoids, followed by vasculogenic lesions, with the second most common being capillary hemangiomas, lymphangiomas, or cavernous hemangiomas.
The rest of the orbital tumors are small, and most of these tumors are benign. A differential diagnosis should include inflammatory lesions, adipose-containing lesions, lacrimal gland masses, lymphoid tumors and leukemia, optic nerve and meningeal tumors, osseous and fibro-osseous masses, and metastatic lesions, with the most common malignant processes including rhabdomyosarcoma, metastatic disease, lymphomas, and leukemia 4.
Five cases were reported in 1948 among a total of 986 orbital tumors in adults 1. An additional five patients with juvenile fibrosarcoma of the orbit and eyelids were reported in 1983. The patients ranged in age from newborn to 8 years (mean, 3 years and 9 months).
Three patients were male and two were female 5.
Study of orbital fibrosarcoma and generally all related orbital pathologies requires a multidisciplinary team approach that includes an oral and maxillofacial surgeon, an ophthalmologist, a pediatric oncologist, a radiologist, and a histopathologist, with a long-term follow-up for the possibility of recurrences. Surgical excision and chemotherapy remain the treatment of choice and have a favorable prognosis.
The authors thank Dr H. Kutub, Consultant Neurosurgeon; Dr M. Abualrimish, Consultant Neurosurgeon; and Dr Y. Mazroaei, Consultant Ophthalmologist, for their help with the surgery.
Conflicts of interest
There are no conflicts of interest.
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2. Talan Hraniloviæ J, Tomas DTalan Hraniloviæ J, Jurak L. Orbital tumors and pseudotumors. 15th Ljudevit Jurak International Symposium on Comparative Pathology Conference Papers; April 19. Croatia University Department of Pathology, Sestre Milosrdnice University Hospital, 2004; 13–21
3. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery lecture, part 1. Ophthalmology. 2004;111:997–1008
4. Underbrink M, Quinn FB, Ryan MW Orbital tumors grand rounds presentation. UTMB, Department of Otolaryngology, 2001
© 2012 Egyptian Associations of Oral and Maxillofacial Surgery
5. Weiner JM, Hidayat AA. Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases. Arch Ophthalmol. 1983;101:253–259