King Khalid National Guard Hospital, Jeddah, Saudi Arabia
Correspondence to Hamed Hassan Al-Bargi, Department of Oral and Maxillofacial Surgery, P.O. Box 9515, Jeddah 21423, Saudi Arabia Tel: +966 2 624 0000, x212423; e-mail: firstname.lastname@example.org
Received January 15, 2011
Accepted January 30, 2012
Purpose: Pediatric orbital fibrosarcoma is an uncommon tumor that occurs occasionally in the periorbital and orbital regions. This article reports one case of orbital fibrosarcoma and describes the associated clinical, histopathological, and radiological findings.
Design: One interventional case report with clinicopathologic correlation.
Intervention: Treatment consisted of preoperative chemotherapy and excision of tumors.
Main outcome measures: Histological and clinical evaluation of the tumor.
Results: The patient was a 5-month-old boy with a large tumor extending from his left orbit. Histological and radiological nuclear medicine was used to evaluate the lesion and the possibility of bone marrow involvement. He presented with continuous tearing, white discharge, and proptosis in his left eye. Incisional biopsy revealed a tumor consistent with congenital orbital fibrosarcoma. He completed six cycles of chemotherapy, followed by gross surgical resection and remains tumor free one and half years later.
Conclusion: Congenital orbital fibrosarcoma is a rare tumor that occurs in the orbit. Differential diagnosis based on histological features can be difficult. As soon as the diagnosis is made, brain, orbits, chest, and abdominal imaging is used to evaluate the presence of distant metastasis, and a bone scan is conducted to detect any bone involvement. Surgical treatment and chemotherapy remain the treatment of choice and lead to a favorable prognosis.
Primary fibrosarcoma of the orbit is very rare 1. Only five cases were reported in 1948 among a total of 986 orbital tumors in adults 1. Rhabdomyosarcoma was the most common malignancy in children, and lymphoma was the most common malignancy in older patients 2. Fibrosarcomas are known malignant tumors arising from fibroblasts. Study of orbital fibrosarcomas and generally all related orbital pathologies requires a multidisciplinary approach 3. We encountered a case of primary orbital fibrosarcoma above the left globe in a 5-month-old boy, causing significant exophthalmos without optic nerve involvement.
A 1-year-old boy was referred for investigation at the age of 5 months because of a left ocular mass that presented shortly after birth. His parents complained that the child suffered from continuous tearing, white discharge, and a slowly progressive left eye mass, which increased in size.
The lesion improved spontaneously within the first two months of life; by the age of 4 months, at a vaccination clinic, the patient was observed to have a proptoic left eye. He was referred to an ophthalmologist, and an MRI was performed, which showed a supraorbital lesion in the left eye but not involving the globe (Fig. 1). The patient was then referred to an oncology clinic.
His medical history revealed a full-term spontaneous vaginal delivery, a normal antenatal period, birth weight of 3.4 kg, an uneventful neonatal and early infancy period, normal development history, full course of immunization, and no history of hospitalization. The family history was unremarkable. His parents are first-degree cousins and his four siblings are alive and well developed.
His physical examinations showed a generally active and playful child, having a pink complexion that was well perfused. He weighed 6.4 kg and was 64 cm in length; his systemic review was unremarkable. Local examinations showed restricted left eye movement with a mass in the retrobulbar area and a nonpulsating proptoic left eye (Fig. 2). The eyes were firm on palpation, with a prominent left supraorbital ridge and left orbital walls (Fig. 3). There was no squint. The patient could follow light without restriction in eye movements. His pupils were 3-mm wide in both eyes and were reactive to light. Fundoscopy was difficult to perform because of the patient’s movements.
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.
1. Ohtsuka K, Saito KForrester JV, Dick AD, McMenamin PG. Primary orbital fibrosarcoma developing in the scleral stroma. The eye: basic sciences in practice. London W.B. Saunders Co; 1995; pp. 1–422
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
5. Weiner JM, Hidayat AA. Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases. Arch Ophthalmol. 1983;101:253–259