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BRIEF COMMUNICATION
Year : 2014  |  Volume : 2  |  Issue : 3  |  Page : 155-157

Osteosarcoma metastasis to the orbit presenting as severe proptosis following trivial trauma


Department of Neurosurgery, Narayana Medical College Hospital, Chinthareddypalem, Nellore, Andhra Pradesh, India

Date of Submission07-Oct-2013
Date of Acceptance24-Feb-2014
Date of Web Publication16-Aug-2014

Correspondence Address:
Dr. Amit Agrawal
Department of Neurosurgery, Narayana Medical College Hospital, Chinthareddypalem, Nellore - 524 003, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-3897.138862

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  Abstract 

Orbital metastasis of osteosarcoma is rare with only few cases reported in literature. We report an unusual case of an eight-year-old female child who sustained injury to the left eye. Following injury, parents noticed swelling of the left eye. Computed tomographic (CT) scans showed a soft-tissue mass lesion involving the left frontal region and a biconvex extraconal high-density mass-lesion located in upper part of the left orbit pushing the eyeball downward. Also, a biconvex hyperdense intracranial lesion was noticed in left frontal region. Based on clinical and imaging findings, a left frontal extradural and orbital hematoma was suspected. At surgery, there was a highly vascular, firm tumor mass adherent to frontal bone extending into the orbit. There was erosion of and destruction of fronto-zygomatic bone. Histopathological examination of the tumor confirmed the diagnosis of osteosarcoma. In spite of radiotherapy, chemotherapy, and surgery, adequate local or regional control is not possible and the prognosis of osteosarcoma involving skull is poor and the treatment can only partially relieve the symptoms, but cannot eradicate the tumor.

Keywords: Metastatic osteosarcoma, orbit, proptosis


How to cite this article:
Agrawal A. Osteosarcoma metastasis to the orbit presenting as severe proptosis following trivial trauma. J Clin Ophthalmol Res 2014;2:155-7

How to cite this URL:
Agrawal A. Osteosarcoma metastasis to the orbit presenting as severe proptosis following trivial trauma. J Clin Ophthalmol Res [serial online] 2014 [cited 2022 Jul 7];2:155-7. Available from: https://www.jcor.in/text.asp?2014/2/3/155/138862

Osteosarcoma is the most common primary malignant tumor involving metaphysis of the long bones. [1],[2],[3] The tumor composed of pleomorphic, spindle-shaped cells that produce osteoid in the pediatric population, with usual metastasis to the lungs. [1],[2],[3] Metastasis of osteosarcoma to the orbit is extremely rare with only few cases reported. [1],[4],[5],[6],[7] We report an unusual case of osteosarcoma metastasized to the left eye and it was recognized following an injury to the eye.


  Case Report Top


An eight-year-old female child presented with left leg pain of one and half month duration. The parents suspected that she would have had sprain, and the child received treatment at a local hospital with analgesics and relief in pain. Gradually, they noticed that the swelling around the left knee was increasing, and an X-ray was performed, which was apparently normal. The swelling continued to progress, and follow up lower extremity radiography showed a metaphyseal lesion in the proximal femur with bone destruction and new bone formation. A clinical and radiologi­cal diagnosis of osteosarcoma was made [Figure 1] and [Figure 2]. A plain chest radiograph did not reveal any abnormality. The child got hit in school while she was playing and progressive proptosis of left eye with worsening in vision [Figure 1]. On examination, the child was dull and mildly febrile (99o F). There was severe proptosis, abduction, and peri-orbital ecchymosis of left eye [Figure 1]. Because of altered sensorium detail, examination was not possible. On Doll's maneuver, extraocular movements were restricted in left eye; pupil was dilated in left eye. Direct and consensual light reflexes were sluggish in left eye. No bruit or pulsation over the eyeball could be detected. Laboratory findings, including the coagulation profile, were within normal range. Computed tomographic (CT) scans showed an extracranial soft-tissue density area in the left frontal region and a biconvex extraconal high-density mass in the upper part of the left orbit. The mass was compressing the left eyeball pushing it downward. In addition, there was biconvex hyperdense lesion in left frontal region [Figure 3]. Based on these findings, and in view of history of trauma, a left frontal extradural hematoma with orbital hematoma was suspected [Figure 3]. The child was planned for a left frontal craniotomy and lateral orbitotomy. A left frontal hairline incision was made, and frontal bone was exposed. There was highly vascular, firm tumor adherent to frontal bone identified. It was extending into the orbit with destruction of fronto-zygomatic bone area [Figure 4]. The tumor was removed in pieces; however, there was massive bleeding from the tumor bed. The child became hemo-dynamically unstable, a gel foam pack was applied, and hemostasis was achieved. Intracranial component was not touched, and procedure was abandoned. Histopathological examination of the tumor confirmed the diagnosis of osteosarcoma.
Figure 1: Photograph of the patient showing marked proptosis, sub-conjunctival chemosis, please note injury scar over the eye (b) and swelling over the lower end of left femur

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Figure 2: AP and lateral X-rays of the left femur showing the details of the mass, lesion is poorly marginated, associated with destruction of the cancellous and cortical elements of the bone, showing ossification within the soft tissue component and the bone formation within this mass

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Figure 3: Axial computed tomographic (CT) scans showing a soft-tissue density area in the left orbit with marked proptosis and bony erosion and a biconvex hyperdense mass in the left frontal region. Tumor is showing sun ray appearance (lower left image) and cloud-like pattern of calcification (lower right image)

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Figure 4: Intra-operative photographs showing purplish highly vascular mass lesion with hemorrhage

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  Discussion Top


Osteosarcomas primarily metastasize hematogenously to the lungs, [2],[8] bone, lymph node, or brain; metastases are mostly seen following or concomitantly with pulmonary metastatic disease [2],[8] and in absence of pulmonary lesions, the involvement of the central nervous system in osteosarcoma is uncommon. [2] As may be in present case, in cases without clinically evident pulmonary lesions, the spread of tumor to the orbit may have possi­bly been through the Batson's paravertebral system. [5],[7] The most common clinical presentation is pain in the involved bone, with or with­out a tissue mass. [2] The most common symptom of orbital metastatic osteosarcoma is rapidly progressive, painful proptosis with vi­sual acuity ranging widely from normal to no light perception. [5],[6],[7],[9] As in present case, local trauma [9] lead to the hemorrhage into the tumor tissue causing the development of rapidly progressive proptosis and making the diagnoses of metastases apparent. In comparison to primary orbital tumors, metastatic orbital osteosarcoma causes rapid proptosis, often associated with pain, diplopia, and disturbances of ocular motility, vision loss, ptosis, conjunctival chemosis/injection, eyelid edema, and epiphora. [5],[6],[7],[9] CT scan with bone window provides excellent detection of tumor calcification, cortical involvement, and, in most instances, soft tissue as well as extension into the adjacent structures with typical sun ray appearance of the lesion. [8] As was evident in the present case, axial CT osteosarcoma that extends into the soft tissues may produce cloud-like pattern of mineralization. [10],[11] Combined radiotherapy, chemotherapy, and surgery have been shown to be effective in the palliative treatment of orbital metastatic osteosarcoma although can partially relieve symptoms, but cannot completely eradicate the tumors. [7] Although the role of surgery for metastatic disease in osteosarcoma is controversial, in cases of orbital metastases, surgery is indicated to decompress the optic nerve, to salvage the vision, and to confirm the histological diagnoses. [12]

To summarize, orbital metastasis of osteosarcoma is rare. In spite of combined radiotherapy, chemotherapy, and surgery, adequate loco-regional control is difficult. The prognosis of osteosarcoma involving skull is poor as the treatment can partially relieve symptoms, but cannot completely eradicate the tumors; [5],[6],[7],[9],[13],[14] there is a need for new thera­peutic approaches to improve the prognosis for these patients with the most severe disease. [7],[14]

 
  References Top

1.Attili SV, Jain A, Saini KV, Batra U, Govind Babu K, Sajeevan KV, et al. Orbital metastasis: A rare presentation of osteosarcoma. Int Ophthalmol 2008;28:433-6.  Back to cited text no. 1
    
2.Meyers PA, Gorlick R. Osteosarcoma. Pediatr Clin North Am 1997;44:973-89.  Back to cited text no. 2
    
3.Giuliano AE, Feig S, Eilber FR. Changing metastatic patterns of osteosarcoma. Cancer 1984;54:2160-4.  Back to cited text no. 3
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4.Peyster RG, Shapiro MD, Haik BG. Orbital metastasis: Role of magnetic resonance imaging and computed tomography. Radiol Clin North Am 1987;25:647-62.  Back to cited text no. 4
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5.Misra A, Misra S, Chaturvedi A, Srivastava PK. Osteosarcoma with metastasis to orbit. Br J Ophthalmol 2001;85:1387-8.  Back to cited text no. 5
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6.Mohadjer Y, Wilson MW, Fuller CE, Haik BG. Primary Pelvic Telangiectatic Osteosarcoma Metastatic to Both Orbits. Ophthal Plast Reconstr Surg 2004;20:77-9.  Back to cited text no. 6
    
7.Lin PY, Chen WM, Hsieh YL, Chen WY, Chen TH. Orbital metastatic osteosarcoma. J Chin Med Assoc 2005;68:286-9.  Back to cited text no. 7
    
8.Hettmer S, Fleischhack G, Hasan C, Kral T, Meyer B, Bode U. Intracranial manifestation of osteosarcoma. Pediatr Hematol Oncol 2002;19:347-54.  Back to cited text no. 8
    
9.Weil RJ, Lonser RR, Quezado MM. CNS manifestations of malignancies: Case 2. Skull and brain metastasis from tibial osteosarcoma. J Clin Oncol 2005;23:4226-9.  Back to cited text no. 9
    
10.Garg LN, Agrawal A, Goyal R, Trehan SS, Mittal A, Dureja N. Cloud-like pattern of mineralization in skull base osteosarcoma. J Neurosci Rural Pract 2011;2:103-4.  Back to cited text no. 10
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11.Nielsen GP, Rosenberg AE. Update on bone forming tumors of the head and neck. Head Neck Pathol 2007;1:87-93.  Back to cited text no. 11
    
12.Kelkar G, Moiyadi AV, Kane SV. Extraskeletal osteosarcoma of the chest-wall with delayed metastasis to the sphenoid. Indian J Cancer 2010;47:82-4.  Back to cited text no. 12
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13.Haddad H, Benchakroun N, Sahraoui S, Benider A. Osteosarcoma of the skull base: A case report. Neurochirurgie 2007;53:483-5.  Back to cited text no. 13
    
14.Mialou V, Philip T, Kalifa C, Perol D, Gentet JC, Marec-Berard P, et al. Metastatic osteosarcoma at diagnosis. Cancer 2005;104:1100-9.  Back to cited text no. 14
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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