Case Report
Meningeal Hemangiopericytoma of Brain: Role of Radiation Therapy
Authors
Paramjeet Kaur, Department of Radiotherapy, Regional Cancer Centre,
Anil Khurana, Department of Radiotherapy, Regional Cancer Centre,
Ashok K Chauhan, Department of Radiotherapy, Regional Cancer Centre,
Singh Gajender, Department of Pathology,
Sunita Singh, Department of Pathology,
Pt. BD Sharma PGIMS, Rohtak, Haryana, India.
Address for Correspondence
Dr. Paramjeet Kaur,
Associate Professor,
Department of Radiotherapy,
Pt. BDS PGIMS, Rohtak,
Haryana, India.
E-mail:
drparamjitkaur@rediffmail.com
Citation
Kaur P, Khurana A, Chauhan AK, Singh G, Singh S. Meningeal Hemangiopericytoma of Brain: Role of Radiation Therapy. Online J Health Allied Scs.
2013;12(3):12. Available at URL:
http://www.ojhas.org/issue47/2013-3-12.html
Open Access Archives
http://cogprints.org/view/subjects/OJHAS.html
http://openmed.nic.in/view/subjects/ojhas.html
Submitted: May 20,
2013; Suggested Revision: Jul 4, 213; Revised: Aug 7, 2013; Accepted: Oct 25, 2013. Published: Nov 15, 2013. |
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Introduction:
Hemangiopericytomas are an uncommon intracranial tumor of mesenchymal origin (MHPC) and constitute less than 1%
of all central nervous system tumors.[1] They have been observed at the falx, occipital, spinal dura, tentorium and cerebellopontine angle.[2]
These tumors are usually present as dura-based masses and clinically indistinguishable from meningiomas.
MHPCs are characterized by locally aggressive biological behavior with tendency to locally recur and metastasize extra cranially.[3] MHPC occurs
most frequently during the fifth decade.
Management of these tumors includes aggressive total excision. However, late local recurrences and distant
extraneural metastases ranging from 12% to 57% are reported in literature after complete removal. Adjuvant radiotherapy is indicated in
completely excised tumor with an aim to maximizing local tumor control.[4] The aim of this study was to evaluate the effectiveness of adjuvant
radiotherapy and determine the outcome.
Case Report:
A 60 years old female patient present with weakness in limbs, associated with inability to walk, loss of sensation,
disorientation and loss of memory of four months duration. There were also complaints of loss of bowel and bladder control of two months duration.
There was no history of seizers and trauma. General and physical examination were normal. Computed tomography of brain showed a large hypodense
area in left frontal, temporo -parietal and occipital regions. Right lateral ventricle was dilated with periventricular edema and left lateral
ventricle and third ventricle were compressed.[Fig 1,2] CECT of chest and abdomen were normal and there was no evidence of bone metastases on
radioisotope bone scan examination.
Patient underwent left parieto-occipital craniotomy and excision of tumor.(Fig 3) The histopathological
examination of growth revealed Meningeal Hemangiopericytoma. Reticulin stain showed staghorn vessels with increased perivascular reticulin.
On immunohistochemistry EMA and PR were negative, CD 34 and VWF were positive in vascular channels.(Fig 4-7) Patient was treated with adjuvant
external beam radiotherapy with dose of 56 Gy in 30 fractions over 6 weeks after six weeks of surgery. Patient is disease free three years after
treatment.
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Fig 1, 2: Pre operative CECT scan showing intracranial mass lesion |
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Fig 3: Post operative CT scan shwoing tumor clearance |
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Fig 4: Photomicrograph showing oval to spindle shaped tumor cells along with staghorn vessels. |
Fig 5: Photomicrograph showing staghorn vessels with increased perivascular reticulin |
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Fig 6,7: Positivity for CD 34 in vascular channels. |
Discussion:
Hemangiopericytoma is a rare soft tissue tumor. Extracranial hemangiopericytoma was first reported by Stout
and Murray in 1942.[5] Clinically these lesions are indistinguishable from meningiomas. The World Health Organization (WHO) classification of
brain tumors has considered them as ‘mesenchymal, non-meningothelial tumors.[6] Aggressive surgical resection remains cornerstone of treatment.
However, complete excision is sometimes difficult to achieve due to high vascularity and tendency of these tumours to grow along sinus.[2]
Traditionally radiotherapy had been used for unresectable tumor or recurrent tumors. Now, Post operative radiotherapy
has a well established role in management of these tumours and associated with increased survival. Administration of post operative radiotherapy
is most strongly related to the overall prognosis.[7] Radiation responses are dose dependent, with > 50 Gray (50 Gy–60Gy) providing superior
long-term disease-free survival.[8]
In addition, it has been reported that adjuvant post operative radiotherapy can be beneficial in reducing
local recurrence rate that also be resulted in prolongation of disease free and overall survival. Other modalities such as helical tomography
and stereotactic radiosurgery are indicated in recurrent and metastatic disease.
However, aggressive biological behaviour and a tendency of local recurrence after complete excision of tumor are
characteristic features of hemangiopericytoma. Moreover these tumors also have the unique characteristic of metastases extracranially,
mainly to bone, liver and lung. Guthrie et al. demonstrated increasing metastatic frequency, reporting 5-, 10- and 15-year metastasis rates of
13, 33 and 64%, respectively.[9] There is limited role of chemotherapy in metastatic setting as reported in clinical series.
To evaluate the outcome the long term extensive follow up is mandatory that included routine investigation
such as liver function test, kidney function test, and Chest X ray, Ultrasound examination of abdomen and pelvis and radionuclide bone scan.
Conclusion:
The patient was treated with adjuvant external beam radiotherapy with dose 56 Gy in 30 fractions.
Postoperative radiotherapy has resulted in a significantly better local control which has a major impact on the survival of primary meningeal
hemangiopericytoma. Radiation therapy is a recommended modality as adjuvant treatment after complete surgical removal of this tumor so as to achieve
high probability of an increased recurrence – free interval and overall survival. Extensive long term follow up also recommended ruling out
local recurrences and late extracranial metastases.
References:
- Tsou H, Wang Y, Yang D, et al .Intra extracranial hemangiopericytoma: clinical manifestations, histopathological features, diagnosis, treatment and outcomes. Chin Med J 2002 (Taipei).65:314-319.
- Penel N, Amela YE, Decanter G,et al. Solitary Fibrous Tumors and So-Called Hemangiopericytoma. Solitary Fibrous Tumors and So-Called Hemangiopericytoma. Sarcoma 2012. doi:10.1155/2012/690251.
- Soyuer S, Chang EL, Selek U, McCutcheon IE, Maor MH. Intracranial meningeal hemangiopericytoma: the role of radiotherapy: report of 29 cases and review of the literature. Cancer. 2004;100:491-497.
- Ecker RD, Marsh WR, Pollock BE, Kurtkaya-Yapicier O, McClelland R, Scheithauer BW, Buckner JC. Hemangiopericytoma in the central nervous system: treatment, pathologic features, and long-term follow-up in 38 patients. J Neurosurg 2003;98:1182-1187.
- Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor featuring Zimmerman’s pericyte. Ann Surg 1942;116:26-33.
- Giannini C, Rushing EJ, Hainfellner JA. Hemangiopericytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavanee WK, editors. WHO classification of tumours of the central nervous system. 4th ed. Lyon: International Agency for Research on Cancer (IARC), 2007; p.178-180.
- Kaye AH, Laws ER (eds) 1995 Brain Tumors. Livingstone, Tokyo pp 705-711.
- Bastin KT, Mehta MP. Meningeal hemangiopericytoma: defining the role for radiation therapy. Neurooncol 1992;14(3):277-287.
- Guthrie BL, Ebersold MJ, Scheithauer BW, et al. Meningeal hemangiopericytomas: histopathological features, treatment and long term follow –up of 44 cases. Neurosurgery 25:514-522.
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