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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 3  |  Page : 329-331

Orbital infantile fibrosarcoma presenting as intraconal mass in a 2-year-old child: A case report

Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission29-Jan-2014
Date of Decision04-Mar-2014
Date of Acceptance29-May-2014
Date of Web Publication18-Jul-2014

Correspondence Address:
Ram Nawal Rao
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.137018

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How to cite this article:
Rao RN, Bansal M, Raghuvanshi S, Mishra D. Orbital infantile fibrosarcoma presenting as intraconal mass in a 2-year-old child: A case report. Neurol India 2014;62:329-31

How to cite this URL:
Rao RN, Bansal M, Raghuvanshi S, Mishra D. Orbital infantile fibrosarcoma presenting as intraconal mass in a 2-year-old child: A case report. Neurol India [serial online] 2014 [cited 2021 Jan 16];62:329-31. Available from:


Infantile/congenital fibrosarcoma is a malignant soft tissue tumor arising from fibroblasts, and its orbital location in infancy is extremely rare presentation. [1] It accounts for 5-10% of all sarcomas in children younger than 1 year. [2] This tumor has also been diagnosed during pregnancy and in a fetus. [3] Orbital infantile fibrosarcoma is attached to the posterior pole of the globe and is uncapsulated at its posterior wall with fibrous tissue similar to the sclera. The tumor may infiltrate the posterior pole of the globe and the optic nerve sheath. Based on these observations, it is thought to have developed in the scleral stroma. [4]

A 2-year-old male child presented with gradually progressive proptosis of right eye since 6 months. On examination, there was forward and downward protrusion of the eyeball and resistance on retropulsion present. Magnetic resonance imaging (MRI) showed an enhancing mass predominantly in intraconal compartment of right orbit with non-visualization of superior rectus. Ultrasonography (USG)-guided fine needle aspiration (FNA) cytology showed clusters and singly scattered spindle to oblong cells with high N:C ratio, hyperchromatic irregular elongated nuclei, inconspicuous nucleoli and scant to moderate cytoplasm on a background of myxoid material, and hemorrhage. Cellblock of the USG-guided FNA material showed sheets of spindle-shaped cells with typical herring bone pattern [Figure 1]. The patient underwent surgery, and operative findings showed a friable, globular and well-encapsulated intraconal mass (3 × 3 × 2 cm). Grossly, mass was globular and grayish white on cut surface. Histopathologic examination showed a highly cellular mesenchymal tumor with spindle-shaped nuclei, condensed chromatin, inconspicuous nucleoli, and scant to moderate amount of cytoplasm. Tumor cells were arranged in fascicles and had a characteristic herring bone pattern [Figure 2]. The mitotic count was 4/10 hpf (high power fields). Immunohistochemistry was strongly positive for vimentin and negative for desmin, myogenin, Smooth muscle actin (SMA), S100, Bcl2, CD117, Anaplastic lymphoma kinase 1 (ALK1), CD34, HMB 45, Epithelial membrane antigen (EMA), CD99 and Cytokeratin (CK), confirming the diagnosis of infantile fibrosarcoma [Figure 3], indicating that the tumor had originated from mesodermal cells, high Ki 67 index (12-15%) [Figure 4] and herringbone patterns. After 6 month of follow up, our patient is well and active. No recurrence has been noted after surgical resection of orbital mass.
Figure 1: Cellblock showed sheets of spindle-shaped cells with typical herring bone pattern. (hematoxylin and eosin, H and E; ×20 magnification)

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Figure 2: Histopathology section shows sheets of atypical spindleshaped cells with elongated hyperchromatic nuclei, condensed chromatin, inconspicuous nucleoli, and moderate cytoplasm with characteristic herring bone pattern (H and E, ×40 magnification)

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Figure 3: Immunohistochemistry showed tumor cells with strongly positive for vimentin (Vimentin ×40 magnification)

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Figure 4: Ki-67 showed 12-15% of nuclear proliferating index (Ki-67, ×40 magnification)

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Only a few cases of histologically verified infantile fibrosarcoma of the orbit on have been described. [5],[6],[7],[8] Some fibrosarcomas in previous reports were described as non-encapsulated or poorly circumscribed. The cytomorphological differential diagnosis [Table 1] includes soft tissue sarcomas like spindle cell rhabdomyosarcoma (positive for desmin or myogenin), solitary fibrous tumor (positive for S100 and Bcl2), leiomyosarcoma (positive for SMA), schwannoma/malignant peripheral nerve sheath tumor (S100), fibrohistiocytic tumor (CD34), extra-gastrointestinal stromal tumor (CD117), inflammatory myofibroblastic tumor (ALK-1), sarcomatoid carcinoma (CK), synovial sarcoma (EMA, CD99), infantile myofibroma (vimentin and S100) and infantile fibromatosis (B-catenin). Other study showed infantile fibrosarcoma tumor cells revealed diffusely positive for vimentin and focal positivity for actin but are negative for S100, desmin, and myoglobin. Although histologically similar to fibrosarcomas occurring in adults, the congenital lesions differ in their clinical behavior. Metastasis is rare but less than 10% and local recurrence is common. Compared with adult fibrosarcoma, congenital fibrosarcoma has slower growth rate and better prognosis. [9] A study revealed a high degree of cellularity, rapid growth, and extensive local invasion; metastases are rare and occur mostly in the lungs, bones, and sometimes in the lymph nodes. Local recurrence is noted and occurs in 20-40% of cases. [10]
Table 1: Immunohistochemical findings in differential diagnosis of infantile fibrosarcoma in orbit

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  References Top

1.Tarik E, Lamiae R, Abdelouahed A, Tarik M, Hassan G, Anouar DM. Unusual case of congenital/infantile fibrosarcoma in a new born. Afr J Paediatr Surg 2013;10:185-7.  Back to cited text no. 1
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2.Akyaaz C, Sari N, Vargel I, Gedikoglu G, Haliloglu M, Büyükpamukçu M. A newborn with infantile fibrosarcoma of foot: Treatment with chemotherapy and extremity-sparing surgery. J Perinatol 2010;30:63-5.  Back to cited text no. 2
3.Kodet R, Stejskal J, Pilat D, Kocourková M, Smelhaus V, Eckschlager T. Congenital-infantile fibrosarcoma: A clinicopathogical study of five patients entered on the prague children′s tumour registry. Pathol Res Pract. 1996;192:845-50.  Back to cited text no. 3
4.Ohtsuka K, Saito K. Primary orbital fibrosarcoma developing in the scleral stroma. British Journal of Ophthalmology. 1996;80:932-3.  Back to cited text no. 4
5.Yanoff M, Scheie HG. Fibrosarcoma of orbit. Report of two patients. Cancer 1966;19:1711-6.  Back to cited text no. 5
6.Jakobiec FA, Tannenbaum M. The ultrastructure of orbital fibrosarcoma. Am J Ophthalmol 1974;77:899-917.  Back to cited text no. 6
7.Scott SM, Reiman HM, Pritchard DJ, IIstrup DM. Soft tissue fibrosarcoma. A clinicopathologic study of 132 cases. Cancer 1989;64:925-31.  Back to cited text no. 7
8.Tsang HH, Dolman PJ, Courtemanche DJ, Rassekh SR, Senger C, Lyons CJ. Prenatal presentation of fronto-orbital congenital infantile fibrosarcoma: A clinicopathologic report. JAMA Ophthalmol 2013;131:965-7.  Back to cited text no. 8
9.Weiss SW. Congenital and Infantile fibrosarcoma. In: Enzinger FM, Weiss SW, editors. Soft Tissue Tumours. 4 th ed. Missouri: Mosby Elsevier; 2001. p. 377-9.  Back to cited text no. 9
10.Rosai J, Ackerman LV. Surgical Pathology. Newyork: Mosby Elsevier; 2004. p. 2253.  Back to cited text no. 10


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

  [Table 1]


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