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Year : 2014  |  Volume : 57  |  Issue : 4  |  Page : 603-605
Recurrent extradural hemangiopericytoma of thoracic spine: A case report

1 Department of Pathology, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India
2 Department of Neurosurgery, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India

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Date of Web Publication11-Oct-2014


Hemangiopericytoma (HPC) is a rare tumor that arises from pericapillary cells or pericytes of Zimmerman. In the central nervous system, it accounts for less than 1% of tumors, and spinal involvement is very rare. Meningeal hemangiopericytomas show morphological similarities with meningiomas particularly with angiomatous meningioma, where one needs to take the help of immunohistochemistry (IHC) to delineate HPC from meningioma. Here, we report a case of recurrent extradural HPC in a 16 year-old girl, who 5 years back had a pathological diagnosis of angiomatous meningioma, for D5-D6 lesion. On evaluation, magnetic resonance imaging (MRI) showed a large extradural tumor with a significant cord compression involving D5-D6 body, pedicle and ribs. Excision of the lesion and spinal stabilization was performed. The histopathological examination and immunohistochemistry performed on tumor sections revealed features favoring HPC. To conclude, detailed IHC is helpful in avoiding misdiagnosis and in further management of the patient.

Keywords: Angiomatous meningioma, extradural spinal tumor, hemangiopericytoma, recurrence, thoracic spine

How to cite this article:
Jayashankar E, Prabhala S, Raju S, Tanikella R. Recurrent extradural hemangiopericytoma of thoracic spine: A case report . Indian J Pathol Microbiol 2014;57:603-5

How to cite this URL:
Jayashankar E, Prabhala S, Raju S, Tanikella R. Recurrent extradural hemangiopericytoma of thoracic spine: A case report . Indian J Pathol Microbiol [serial online] 2014 [cited 2023 Nov 30];57:603-5. Available from:

   Introduction Top

Hemangiopericytoma (HPC) was first described by Stout and Murray in 1942. [1] It is a rare soft tissue tumor arising from pericapillary cells of Zimmerman. [1],[2] Meningeal HPC is rare; it accounts for less than 1% of all central nervous system tumors. It mainly occurs in adults, and is locally aggressive and potentially malignant tumor and can recur ranging from 50 to 80%, or may present with metastases (14 to 30%). [3],[4],[5] HPC shows limited response to irradiation and is best treated with complete resection. [6] The tumor is very common in the cranium; spinal involvement is rare, and only few cases have been reported in the literature. [5] Both meningioma and HPC show dural attachment, and morphological resemblance, particularly angiomatous meningioma type. To differentiate, we should perform immunohistochemical staining on paraffin embedded sections of the tumor. Here in, we present a case of recurrent extradural hemangiopericytoma of thoracic spine which was earlier labeled as angiomatous meningioma. We did IHC with vimentin, EMA, CD34 and Ki67 and confirmed the diagnosis of meningeal HPC.

   Case report Top

A 16 year-old girl was admitted to our hospital due to low backache, gradually increasing in its severity, relieved with back support, rest and medication. Five years ago, she had presented with severe low backache, weakness of lower limbs for which she was operated and had a histopathological diagnosis of angiomatous meningioma. Her postoperative status was nil remarkable. Since 20 days she again had similar complaints, for which she was evaluated with MRI dorsal spine, which revealed large extradural tumor at D5-D6 level with significant cord compression involving body and left pedicle of D6 vertebra, and left sixth rib, with intense enhancement on contrast [Figure 1]. Her complete blood profile was normal. Vitals were stable. Re-exploration and excision of the tumor were done with support of two units of blood transfusion, and the specimen was sent for histopathological examination. Prior to this, we had reviewed the patient's earlier slides, and considered the possibility of HPC in view of its histology rather than angiomatous meningioma. Hence, we decided to perform simultaneously routine hematoxylin and eosin (H&E) staining and immunohistochemical (IHC) staining on the current specimen. For IHC study, we used vimentin, epithelial membrane antigen (EMA), CD 34 and Ki-67 antigen. On H&E stain, the tumor was highly cellular and characterized by closely packed, randomly oriented tumor cells separated by slit-like or staghorn type of vascular spaces and small foci of necrosis. The tumor cells showed round to oval nuclei with mild to moderate nuclear pleomorphism, hyperchromatic or vesicular nuclei with prominent nucleoli, and indistinct cytoplasm [Figure 2]. Mitotic activity is seen (6 per10 high power fields). IHC revealed diffuse positivity with vimentin, Ki-67 in 10% tumor cells, indicating high proliferative activity, and EMA negativity [Figure 3]. CD 34 was positive in endothelial cells. Based on the histomorphological and IHC study, a diagnosis of anaplastic hemangiopericytoma, WHO grade III was made.
Figure 1: (a and b) (T1- and T2- weighted sagittal MRI images without contrast), (c and d) (T1 and T2 weighted saggital MRI images with contrast) showing extradural tumor involving D5-D6 vertebral body, pedicle, and cord compression

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Figure 2: (a) Tumor with characteristic staghorn-like vascular spaces. (hematoxylin and eosin, x100) (b-d) Cellular lesion with areas of mitotic activity, perithelial arrangement, and necrosis (hematoxylin and eosin, ×400)

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Figure 3: Immunohistochemistry revealed a positive reaction with vimentin, ki-67(10%), and negative reaction with EMA and CD34. (IHC ×400)

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   Discussion Top

Hemangiopericytoma (HPC) is a mesenchymal tumor, first described by Stout and Murray in 1942, thought to be arising from pericytes of Zimmerman. [1],[2] HPC mainly occurs in adults, locally aggressive and potentially malignant. [3] It tends to recur and metastasize to different organs. [4],[5] HPC can occur anywhere in the body where capillaries are seen, but they show more predilection for lower extremities and deep retro-peritoneum. In the central nervous system (CNS), the tumor accounts for less than 1% of tumors, and is more common in cranium. Spinal involvement is very rare. Very few cases of spinal HPC reported in the literature. [7] Both HPC and meningioma are dura-based, and show morphologic similarities particularly with angiomatous meningioma. In a review of 44 cases, Guthrie et al. [8] noted the recurrence rate of 15, 65 and 76% respectively at 1, 5 and 10 years.

Although CT or MRI gives no clear distinction between meningioma and HPC, presence of lytic lesions of bone are seen in HPC compared to meningioma, where hyperostosis is noted. [8],[9] In our case, destructive lesion of D5, with involvement of the pedicle and the rib radiologically also supported the diagnosis of HPC. IHC, genetic and molecular studies are very useful in distinguishing anaplastic meningioma from hemangiopericytoma. [10] IHC clearly brings out HPC, showing positive vimentin and negative EMA, whereas meningioma is positive for both vimentin and EMA, which suggest that meningioma is of mesenchymal/epithelial origin where as HPC is purely a mesenchymal tumor. [11],[12],[13] These findings were noted in our case also. CD34, a transmembrane glycoprotein, has been proposed as a sensitive marker for solitary fibrous tumor (SFT), [14] and can be used to differentiate SFT from HPC. CD34 will be diffuse in SFT but not in HPC, where it appears patchy. Ki-67 labeling index is helpful in assessing the proliferative activity of the tumor. Mena et al. [15] reported that tumors with higher mitotic rate tend to recur. Hara et al. [16] found that recurrent meningeal HPC appeared to show a different rate of growth as suggested by increased Ki-67 mitotic index. In our case, the Ki-67 was 10% and hence we categorized as anaplastic HPC, WHO grade III tumor. Karyotypic abnormalities of chromosomes 12 and 3 were seen in meningeal HPC, with the absence of NF2 mutations, whereas the latter was observed in meningiomas. [17] We could not carry out genetic and molecular studies in our case.

In view of high chances of recurrence in meningeal HPC as seen with our patient, a long-term follow-up is mandatory in all cases. In addition to routine histopathological examination, immunohistochemical study as well as genetic and molecular studies is essential to differentiate from angiomatous/anaplastic meningiomas and meningeal hemangiopericytomas so that appropriate treatment like radiotherapy and/or chemotherapy can be planned and recurrences can be avoided or reduced.

   References Top

Stout AP, Murray MR. Hemangiopericytoma: A vascular tumor featuring Zimmerman's pericytes. Ann Surg 1942;116:26-33.  Back to cited text no. 1
Betchen S, Schwartz A, Black C, Post K. Intradural hemangiopericytoma of the lumbar spine: Case report. Neurosurgery 2002;50:654-7.  Back to cited text no. 2
Mohammadianpanah M, Torabinejad S, Bagheri MH, Omidvari S, Mosalaei A, Ahmadloo N. Primary epidural malignant hemangiopericytoma of thoracic spinal column causing cord compression: Case report. Sao Paulo Med J 2004;122:220-2.  Back to cited text no. 3
Chang CC, Chang YY, Lui CC, Huang CC, Liu JS. Meningeal hemangiopericytoma with delayed multiple distant metastases. J Chin Med Assoc 2004;67:527-32.  Back to cited text no. 4
Jaiswal S, Vij M, Srivastava A, Jaiswal AK, Lal H, Behari S. Primary epidural hemangiopericytoma of thoracic spine: A case report. J Spinal Surg 2010;2:327-30.  Back to cited text no. 5
Ramakrishna R, Rostomily R, Sekhar L, Rockhill J, Ferreira M. Hemangiopericytoma: Radical resection remains the cornerstone of therapy. J Clin Neurosci 2014;21:612-5.  Back to cited text no. 6
Kumar R, Vaid VK, Kumar V, Kalra SK. Hemangiopericytoma of thoracic spine: A rare bony tumor. Child Nerv Syst 2007;23:1215-9.  Back to cited text no. 7
Guthrie BL, Ebersold MJ, Scheithauer BW, Shaw EG. Meningeal hemangiopericytoma: Histopathological features, treatment, and long-term follow-up of 44 cases. Neurosurgery 1989;25:514-22.  Back to cited text no. 8
Jääskeläinen J, Servo A, Haltia M, Wahlström T, Valtonen S. Intracranial hemangiopericytoma: Radiology, surgery, radiotherapy, and outcome in 21 patients. Surg Neurol 1985;23:227-36.  Back to cited text no. 9
Rajaram V, Brat DJ, Perry A. Anplastic meningioma versus meningeal hemangiopericytoma: Immunohistochemical and genetic markers. Hum Pathol 2004;35:1413-8.  Back to cited text no. 10
Iwaki T, Fukui M, Takeshita I, Tsuneyoshi M, Tateishi J. Hemangiopericytoma of the meninges: A clinicopathologic and immunohistochemical study. Clin Neuropathol 1988;7:93-9.  Back to cited text no. 11
Theunissen PH, Debets-Te Baerts M, Blaauw G. Histogenesis of intracranial hemangiopericytoma and hemangioblastoma. An immunohistochemical study. Acta Neuropathol 1990;80:68-71.  Back to cited text no. 12
Winek RR, Scheithauer BW, Wick MR. Meningioma, meningeal hemangiopericytoma (angioblastic meningioma), peripheral hemangiopericytoma, and acoustic schwannoma. A comparative immunohistochemical study. Am J Surg Pathol 1989;13:251-61.  Back to cited text no. 13
Alston SR, Francel PC, Jane JA Jr. Solitary fibrous tumor of the spinal cord. Am J Surg Pathol 1997;21:477-83.  Back to cited text no. 14
Mena H, Ribas JL, Pezeshkpour GH, Cowan DN, Parisi JE. Hemangiopericytoma of the central nervous system: A review of 94 cases. Hum Pathol 1991;22:84-91.  Back to cited text no. 15
Hara M, Aoyagi M, Nagashima G, Wakimoto H, Mikami T, Yamamoto S, et al. Recurrence in meningeal hemangiopericytomas. Surg Neurol 1998;50:586-91.  Back to cited text no. 16
Gianni C, Reushing EJ. Hainfellaer in hemangiopericytoma. In: Louis DN, Ihgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumors of Central Nervous System. Lyon: IARC; 2007. p. 178-80.  Back to cited text no. 17

Correspondence Address:
Erukkambattu Jayashankar
Quarter No 7, Kamineni Hospitals, LB Nagar, Hyderabad - 500 068, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.142686

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