From Oculoplasty and Paediatric Ophthalmology Services (NP, DS, SK, BC), Institute Rotary Cancer Hospital (SB), and the Ocular Pathology Division (SS), All India Institute of Medical Sciences, New Delhi, India.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Saurbhi Khurana, MD, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. E-mail: firstname.lastname@example.org
Hemangiopericytoma is a soft tissue tumor derived from mesenchymal cells with pericytic differentiation.1 It usually occurs during the fifth decade of life and is rare in children.2,3 Orbital hemangiopericytomas are uncommon. There are only a few reported cases in children and surgical excision is the usual management.4,5 We report the first case of an infant who presented with an orbital hemangiopericytoma that was successfully treated with primary chemotherapy.
A 7-month-old female infant presented with protrusion of the left eye that had been noticed since 2 months of age. On examination, the child was following light with both eyes. She had proptosis in the left eye with associated conjunctival chemosis, restricted ocular movements, lagophthalmos, and a relative afferent pupillary defect (Fig. 1A). Fundus examination was normal.
Figure 1. (A) Proptosis of the left eye associated with conjunctival chemosis. (B) Magnetic resonance imaging (T1-weighted post-contrast axial scan) showing an intraconal mass with intense post-contrast enhancement.
Ultrasonography showed an intraconal mass with heterogenous echogenicity and multiple cystic spaces. Magnetic resonance imaging revealed an enhancing intraconal mass with extraconal extension, encasing the optic nerve and inseparable from the lateral rectus muscle (Fig. 1B).
With a differential diagnosis of an orbital hemangioma, the patient received oral prednisone at a dose of 1 mg/kg body weight. There was no clinical improvement and so an incisional biopsy was performed through a transconjunctival approach. Intraoperatively, the lesion was ill defined, pulsatile, and bluish in color and there was excessive bleeding that was controlled with cautery. A temporary para-median tarsorrhaphy was done to prevent exposure keratopathy.
Histopathology of the specimen revealed a vascular tumor with proliferation of spindle-shaped cells around thin-walled endothelium-lined vascular channels with two to three mitosis per high-power field. Immunohistochemistry showed positivity for CD34, smooth muscle antigen, and vimentin and negativity for desmin, S100, and CD99. This was consistent with a diagnosis of hemangiopericytoma (Fig. 2).
Figure 2. (A) Photomicrograph showing proliferation of spindle-shaped cells around vascular channels (hematoxylin–eosin stain, original magnification ×400). (B) Immunohistochemistry staining positive for CD34.
Chest radiograph and bone scan revealed no evidence of metastatic disease. In view of a diffuse retrobulbar mass encasing the optic nerve, the child received four cycles of chemotherapy consisting of ifosfamide (5 g/m2 over 3 days) and doxorubicin (60 mg/m2 over 2 days) every 3 weeks. The proptosis resolved and a computed tomography scan done following treatment showed a significant decrease in the mass. Three subsequent computed tomography scans done 6 months apart revealed a small stable residual mass. At 20 months following treatment, there was no evidence of increase in the size of the persistent tumor mass (Fig. 3).
Figure 3. (A) Post-chemotherapy with resolved proptosis. (B) Computed tomography scan (axial cut) 1 year after treatment with residual mass.
The child is receiving regular long-term follow-up. Her visual acuity is 6/15 in the right eye and 6/24 in the left eye at 50 cm on Cardiff visual acuity assessment. Ocular movements are normal in both eyes.
Hemangiopericytoma commonly occurs in the musculoskeletal system (lower extremities), pelvis, and retroperitoneum. Approximately 15% of these tumors arise in the extracranial part of the head and neck region, usually in the nasal cavity or paranasal sinuses.1 Its occurrence in the orbit is rare.4,5
Hemangiopericytoma is a tumor of adult life; the cases reported in children account for less than 10% of all hemangiopericytoma cases.2 Two distinct clinical entities are described in childhood: the adult-type in children older than 1 year, with a clinical behavior similar to hemangiopericytoma in adults, and the infantile-type occurring in the first year of life.
Infantile hemangiopericytomas represent approximately one-third of pediatric cases and differ in many aspects from their adult counterparts.3 Although histopathologically similar to the adult type, they are considered to be closely related to infantile myofibroblastic lesions. They typically arise in the subcutis and oral cavity and multifocal and meta-static lesions have been reported. Histologic findings that indicate a poor prognosis in adults such as increased mitosis and necrosis are often found but the clinical behavior is usually favorable, with an overall survival of 80%.3
Surgical resection remains the mainstay of treatment for all resectable hemangiopericytomas including the orbit.5 Infantile hemangiopericytomas are known to have high chemosensitivity. A good response has been reported and chemotherapy should be considered for unresectable or residual lesions and aggressive tumors. There is a role of radiotherapy after incomplete tumor resection in older children with adult-type hemangiopericytoma.3 A case of recurrent hemangiopericytoma in an adult was given preoperative radiotherapy that reduced the tumor volume, allowing a complete surgical excision of the mass.6
Croxatto and Font reported two cases of infantile orbital hemangiopericytoma in their series of 30 patients. Tumor recurrence after surgical excision was found in both cases. After surgical excision (combined with radiotherapy and chemotherapy in one case), no further recurrence was noted. In their literature review, they found only three other cases of orbital hemangiopericytoma reported in children (whose ages were 3 years or younger).5 A single case of malignant orbital hemangiopericytoma in an infant who developed distant metastasis even after repeated surgical excision and radiotherapy and succumbed to the disease has been reported by Arshad and Normala.4
In our case, in view of an ill-defined intraconal orbital mass that was not amenable to complete resection, the child underwent chemotherapy. A dramatic response was observed after the first cycle and hence the treatment was continued for a total of four cycles. The child responded favorably and no further surgical resection was required. There has been no recurrence at 20 months after completion of chemotherapy.
To the best of our knowledge, this is the first case of an orbital infantile hemangiopericytoma treated successfully with primary chemotherapy without any surgical resection. Hence, in cases of infantile hemangiopericytoma not amenable to complete surgical excision, the role of primary chemotherapy can be considered and evaluated further.
- Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor featuring Zimmermann’s pericytes. Ann Surg. 1942;116:26–33. doi:10.1097/00000658-194207000-00004 [CrossRef]
- Enzinger FM, Smith BH. Hemangiopericytoma: an analysis of 106 cases. Hum Pathol. 1976;7:61–82. doi:10.1016/S0046-8177(76)80006-8 [CrossRef]
- Ferrari A, Casanova M, Bisogno G, et al. Haemangiopericytoma in pediatric ages: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer. 2001;92:2692–2698. doi:10.1002/1097-0142(20011115)92:10<2692::AID-CNCR1623>3.0.CO;2-Y [CrossRef]
- Arshad AR, Normala B. Infantile malignant hemangiopericytoma of the orbit. Ophthal Plast Reconstr Surg. 2008;24:147–148. doi:10.1097/IOP.0b013e31816746b4 [CrossRef]
- Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinic pathologic study of 30 cases. Hum Pathol. 1982;13:210–218. doi:10.1016/S0046-8177(82)80179-2 [CrossRef]
- Shinder R, Jackson TL, Araujo D, Prieto VG, Guadagnolo BA, Esmaeli B. Preoperative radiation therapy in the management of recurrent orbital hemangiopericytoma. Ophthal Plast Reconstr Surg. 2011;27:e126–e128. doi:10.1097/IOP.0b013e318201cfe0 [CrossRef]