|Year : 2017 | Volume
| Issue : 1 | Page : 145-147
Anaplastic hemangiopericytoma of eyelid: An unusual location
Pradeep Ventrapati1, Sushmita Pathy2, Ajeet Kumar Gandhi1, Seema Kashyap3
1 Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiation Oncology, Dr. B.R. Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
3 Department of Ocular Pathology, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||16-May-2017|
Department of Radiation Oncology, Dr. B.R. Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Hemangiopericytomas (HPCs) are rare soft tissue tumors. The eyelid is a very uncommon site for these tumors, and an anaplastic variant of HPC in the eyelid has not been reported before. A 44-year-old male presented with complaints of slowly progressive, painless swelling on the inner aspect of the left upper eyelid for 9 months. He underwent local excision of the swelling and histopathology revealed a WHO Grade III anaplastic HPC. Whole body 18 F-fluorodeoxyglucose positron emission tomography-computed tomography done postoperatively did not show any evidence of local or distant disease. The patient was planned for adjuvant radiotherapy of 60 Gy in 30 fractions over 6 weeks in view of high grade of histopathology and doubtful margins. He is disease free at the time of the last follow-up. To the best of our knowledge, this is the first case of anaplastic HPC of eyelid being reported in English literature.
Keywords: Eyelid, hemangiopericytoma, radiotherapy
|How to cite this article:|
Ventrapati P, Pathy S, Gandhi AK, Kashyap S. Anaplastic hemangiopericytoma of eyelid: An unusual location. J Can Res Ther 2017;13:145-7
| > Introduction|| |
Hemangiopericytomas (HPCs) are rare soft tissue tumors. They arise from the pericytes of Zimmerman, which are extravascular cells found around the capillaries or postcapillary venules. They belong to the group of solitary fibrous tumors. HPC carries an indolent course but is locally aggressive and potentially malignant. The common sites are lower extremities, retroperitoneum, and head and neck region. In the head and neck region, HPCs usually arise in the nasal cavity, paranasal sinuses, and less frequently, the orbital region. While eyelid is a very uncommon site for these tumors and only three cases have been reported in literature so far ,, anaplastic variant of HPC in the eyelid has not been reported before. We hereby report a case of primary anaplastic HPC of the eyelid.
| > Case Report|| |
A 44-year-old nonhypertensive, nondiabetic male presented to radiation oncology clinic in January 2015 with a 9 months history of a slowly progressive, painless swelling on the left upper eyelid. There was no associated vision loss. Two months ago, he underwent en bloc excision of the mass in the left upper eyelid. On physical examination, performance status was Eastern Cooperative Oncology Group 1. Local examination revealed well-healed scar in the left upper eyelid, no palpable mass and not associated with diplopia. Best-corrected visual acuity was 20/20 and 20/30 (Snellen equivalent in feet) in the right and left eye, respectively. Ocular movements were within normal limits. There was no palpable cervical lymph node. Systemic examination did not reveal any signs of metastasis and was within normal limits. Contrast-enhanced computed tomography (CT) of orbit, face, and neck at radiotherapy planning did not reveal any residual mass or cervical lymphadenopathy. Whole body 18 F-fluorodeoxyglucose positron emission tomography-CT was done which ruled out the presence of any distant metastases.
The patient underwent en bloc excision of the left upper eyelid swelling, and histopathology revealed a highly vascular tumor with high cellularity. The tumor showed moderate to severe pleomorphism and mitotic figures ranging from 6 to 7/10 high power field (HPF) and large areas of hemorrhage [Figure 1]a and [Figure 1]b. Immunohistochemical stains for vimentin, Bcl-2, CD99, and CD34 were positive, while S100 was negative in the tumor cells [Figure 2]a and [Figure 2]b. Based on the histopathological features, a diagnosis of anaplastic HPC (WHO Grade III) was made. Adjuvant radiotherapy was planned in view of high-grade histopathology and doubtful margins. A dose of 60 Gy in 30 fractions was delivered with 9 MeV electrons by a direct anterior field on a linear accelerator (Varian Medical System, Palo Alto, California, United States). The corneal shield was used to reduce the doses to underlying ocular structures, and a customized lead cutout was made for shielding of other areas of the face. Radiation dose to the surrounding critical structures was within acceptable limits.
|Figure 1: (a) Microphotograph showing highly cellular tumor with branching vasculature (H and E, ×200). (b) High power view shows marked atypia in the tumor cells along with mitotic figures (arrow) (H and E, ×400)|
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|Figure 2: (a) Immunohistochemical stain CD34 is focally positive in tumor cells. (b) Diffuse positivity of immunohistochemical stain vimentin in tumor cells|
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The patient tolerated the treatment well with no Grade III or higher toxicity as per Radiation Therapy Oncology Group acute morbidity criteria. At 4 months after completion of the treatment, the patient is under follow-up in our clinic and has no evidence of disease.
| > Discussion|| |
Of all the malignant tumors of the eyelid, basal cell carcinoma is the most common and accounts for 90% of all cases. It is followed by squamous cell carcinoma, sebaceous cell carcinoma, and melanoma.
HPCs are very rare soft tissue neoplasms. HPCs mostly occur in the lower extremities, head and neck, retroperitoneum, and brain. They constitute only 1–2% of all orbital tumors. The lacrimal sac and bulbar conjunctiva are other rare sites. Patients usually present with a painless or painful orbital mass, proptosis, or mass over the medial canthus. The eyelid is a very rare site of presentation of HPCs and has been reported infrequently ,, of interest is the controversy regarding the biological behavior and histological differentiation between benign and malignant tumor. The diagnosis of HPC is made on the basis of histological architecture and immunohistochemical staining. It shows very close resemblance with other soft tissue tumors like solitary fibrous tumor, fibrous histiocytoma, and fibrous meningioma, which makes it more difficult to diagnose.
As per the WHO classification (2007), HPC is divided into two separate categories: Grades II and III (anaplastic HPC). Anaplastic HPC is characterized by >5 mitoses and/or necrosis and any two of the following features; hemorrhage, moderate to high nuclear atypia, and moderate to high cellularity. In the case reported histological examination showed moderate to severe pleomorphism and mitotic figures ranging from 6 to 7/10 HPF and large areas of hemorrhage.
Given the rarity and unpredictable tumor biology, the management of these tumors remains a therapeutic challenge. Surgical resection is the accepted primary treatment and was followed in the current report. In the case of incomplete surgical resection, postoperative radiotherapy is recommended to a dose of 60–65 Gy over 6–7 weeks. In a retrospective study by Carew et al., patients who received postoperative radiation therapy for high-risk features such as positive surgical margins, high-grade histology, or recurrent disease were disease-free at a median follow-up of 48 months. In this case report, an excision of the mass was done initially, and the margins on histopathology were inconclusive. Anaplastic HPC is likely to have increased propensity for local recurrence and distant metastasis as other malignant tumor. The high tumor grade (Grade III) and doubtful margins warranted the use of adjuvant radiotherapy.
Postoperative radiotherapy to a dose of 60–65 Gy over 6–7 weeks provides local tumor control and has also been practiced in the current report. HPC regresses slowly after radiotherapy which is characteristic of soft tissue tumor. The role of chemotherapy is not well-established.
This report highlights the unique location and the high-grade histology in HPC and the need to include HPC in the differential diagnoses of eyelid swellings.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]