|Year : 2016 | Volume
| Issue : 2 | Page : 172-174
A case series of extremely rare and unusual cases of intracranial hemangiopericytoma: An institutional experience
Abhishek Purkayastha, Neelam Sharma, Virender Suhag, Tejas Pandya
Department of Radiation Oncology, Army Hospital Research and Referral, New Delhi, India
|Date of Web Publication||3-Mar-2016|
Department of Radiation Oncology, Army Hospital Research and Referral, Dhaula Kuan, Delhi Cantonment - 110 010, New Delhi
Source of Support: None, Conflict of Interest: None
We report a case series of three extremely rare and unusual cases of intracranial hemangiopericytoma diagnosed and treated in our institute, scarcely reported in world literature until date. These are highly aggressive and uncommon tumors accounting for <1% of all intracranial neoplasms, arising from Zimmerman's pericytes around capillaries and postcapillary venules. First two patients presented in 2011 and 2012 and were diagnosed as hemangiopericytoma Grade II with vimentin and CD34 positivity on immunohistochemistry. In 2014, a third patient was diagnosed as an anaplastic Grade III tumor, positive for vimentin and negative for epithelial membrane antigen. All three patients were treated with adjuvant radiotherapy to the brain and were kept on follow-up. However, the first patient recurred after 5 years for which he underwent redo-surgery. Other two patients are on follow-up with no evidence of recurrence or distant metastasis.
Keywords: Anaplastic, hemangiopericytoma, intracranial, radiotherapy
|How to cite this article:|
Purkayastha A, Sharma N, Suhag V, Pandya T. A case series of extremely rare and unusual cases of intracranial hemangiopericytoma: An institutional experience. Clin Cancer Investig J 2016;5:172-4
|How to cite this URL:|
Purkayastha A, Sharma N, Suhag V, Pandya T. A case series of extremely rare and unusual cases of intracranial hemangiopericytoma: An institutional experience. Clin Cancer Investig J [serial online] 2016 [cited 2020 Apr 2];5:172-4. Available from: http://www.ccij-online.org/text.asp?2016/5/2/172/177128
| Introduction|| |
Hemangiopericytoma is an extremely rare and unusual tumor with an aggressive natural history, accounting for <1% of all intracranial tumors.,, These are highly vascular dural-based neoplasms  with a propensity for local recurrence and distant metastasis. They arise predominantly in soft tissues and very rarely in central nervous system (CNS). With radiographic resemblance to meningioma and histological similarity to soft tissue tumors, it poses a diagnostic dilemma and a therapeutic challenge. Radiotherapy (RT) remains the cornerstone of adjuvant therapy due to high probability of local recurrence after surgery  while chemotherapy as salvage therapy has shown inconsequential results.
| Case Reports|| |
A 28-year-old male presented with headache and vomiting in March 2011. Magnetic resonance imaging (MRI) showed 8 cm × 5.6 cm × 5.5 cm well-circumscribed mass lesion left parieto-occipital region.
A 35-year-old male presented with headache in July 2012. MRI brain revealed an extra-axial globular mass lesion 7.0 cm × 6.0 cm × 5.8 cm right temporoparietal region [Figure 1]. Post gross total resection (GTR) histopathological report (HPR) of both patients revealed hemangiopericytoma WHO Grade II, vimentin and CD34 positivity [Figure 2], Ki-67 <10% and <8%, respectively, on immunohistochemistry (IHC). Both patients were treated with adjuvant conformal RT to brain to a dose of 54 Gy in 27 fractions.
|Figure 1: Magnetic resonance imaging brain showing an extra-axial globular mass lesion 7.0 cm ×6.0 cm ×5.8 cm right temporoparietal region in case 2|
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A 33-year-old male presented with headache in November 2014. MRI brain showed a well-defined extra-axial mass lesion measuring 7.2 cm × 5.2 cm × 5.1 cm in midline anterior frontal region, compressing and displacing both frontal lobes causing erosion of overlying inner skull table. Magnetic resonance venography showed highly vascularized mass infiltrating anterior one-third of superior sagittal sinus. Metastatic work-up with positron emission tomography scan was negative. He underwent GTR with excision of the tumor. Postsurgery HPR revealed anaplastic hemangiopericytoma WHO Grade III with staghorn pattern of cells [Figure 3]. IHC was immunopositive for vimentin [Figure 4], negative for epithelial membrane antigen (EMA) with Ki-67 > 18%. He received adjuvant conformal RT to brain to a dose of 60 Gy in 30 fractions.
|Figure 3: Histopathology report of case 3 showing staghorn pattern in highly cellular tumor cells with scant cytoplasm interspersed with numerous vascular channels and mild nuclear atypia (H and E, ×400)|
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|Figure 4: Immunohistochemistry showing tumor cells positive for vimentin (×40)|
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All patients tolerated treatment well and were kept on regular follow-up. The first patient presented with headache and progressive weakness of right upper limb in August 2015. MRI brain revealed 5.7 cm × 5.3 cm × 4.8 cm lesion at the primary site suggestive of recurrence for which he underwent redo-surgery. Postoperative period was uneventful. The other two patients are also on follow-up without any recurrence or distant metastasis.
| Discussion|| |
HPC is an extremely rare and unusual neoplasm that accounts for <1% of CNS tumors ,, and 0.8% of all CNS tumors diagnosed and treated in our institute from 2008 to 2015. HPCs represent 3–4% of meningeal tumors, 3–5% of all soft tissue sarcomas, and 1% of vascular tumors. About 15–30% of all HPCs occur in head and neck region, and 5% occur in the sinonasal area. Historically, the term hemangiopericytoma was first described by Schmidt in 1937 and named by Stout in 1942. Guthrie et al. gave the term angioblastic meningioma; however, WHO in 1993 changed the term to hemangiopericytoma and classified it as a distinct pathologic entity mesenchymal nonmeningothelial tumor in 2000. HPC occurs in both sexes with equal frequency and is found mainly in adults  at a median age of 42 years.
Most common location of intracranial HPCs is frontal, middle, and posterior fossa. They are highly vascular, occur at an earlier age than other meningeal tumors, recur locoregionally with higher frequency at a mean time of 75 months, and metastasize to bone, lung, liver, kidney, pancreas, and adrenals. HPCs have an indolent course but are notable for high rates of recurrence and distant metastasis many years after treatment, like our first case recurred after 4 years. Studies suggest that high mitotic rates and proliferative indices may signify malignant behavior.
Neoplasms mimicking intracranial HPC are meningiomas and dural-based lymphomas. Clinically and radiographically intracranial HPCs resemble meningiomas  which frequently show hyperostosis of bone and calcifications within the tumor on MRI or computed tomography while HPCs show bone erosion and lack of both calcification and hyperostosis., HPCs are composed of tightly packed pericytes around thin-walled endothelial lined vascular channels  and show a classical staghorn vascular pattern of spindle cells. Since histopathologic features of HPC and meningioma can mimic each other, IHC plays an important role in differentiating these two entities. HPC shows positivity for CD34 while meningioma is EMA positive.
There is a role of RT as definitive modality in case of surgically unresectable and inaccessible tumor or as adjuvant therapy, since this tumor tends to recur even after complete excision., Postoperative RT has been associated with decreased local recurrence and increased overall survival (OS) with effect of radiation seen as long as 5 years after treatment. Regarding the role of chemotherapy in the treatment of HPCs, no impact on locoregional disease control, disease-free survival, or OS has been seen. Chamberlain and Glantz  reported a case series of 15 patients using chemotherapeutic agents vincristine, adriamycin, and cyclophosphamide followed by alfa-interferon and subsequently by ifosfamide, carboplatin, and etoposide regimen. However, it failed to demonstrate any significant benefit as a salvage therapy in recurrent and refractory cases. Though GTR is the established definite treatment, conformal RT remains the mainstay of adjuvant management as it is reduces local recurrence, increases OS, improves the quality of life and overall prognosis.
| Conclusion|| |
Intracranial HPC is an extremely rare CNS neoplasm. We reported this case series to highlight its extreme rarity, its clinicopathological findings and define the role of RT as the primary adjuvant treatment affecting the final prognosis. We recommend that the diagnosis of HPC should always be considered in a young adult presenting with focal motor deficit and dural based well-circumscribed vascular mass lesion in view of its higher rates of local recurrence and systemic metastasis as compared to other CNS tumors.
We thank the patient for allowing us to publish the case report and use the images taken during his stay in the hospital.
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Conflicts of interest
There are no conflicts of interest.
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