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CASE REPORT
Year : 2012  |  Volume : 7  |  Issue : 1  |  Page : 29-31

Anaplastic hemangiopericytoma manifesting as a rapidly enlarging extracranial mass lesion


Department of Neurosurgery, National Defense Medical College, Saitama, Japan

Date of Web Publication4-May-2012

Correspondence Address:
Naoki Otani
Department of Neurosurgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1793-5482.95694

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  Abstract 

We, herein, present a patient with a recurrent anaplastic hemanigiopericytoma manifesting as a rapidly enlarging extracranial mass lesion, which was revealed by pathological and intraoperative findings. In practice, this case highlights the mandatory need for a careful long-term follow-up for patients with hemangiopericytoma, since recurrence with a greater degree of malignancy can develop following an extended disease-free interval, as such knowledge will be helpful for planning the optimal surgical procedures.

Keywords: Anaplastic hemanigiopericytoma, brain tumor, gamma knife radiosurgery, meningeal neoplasm


How to cite this article:
Otani N, Nawashiro H, Wada K, Minamimura K, Takeuchi S, Shima K. Anaplastic hemangiopericytoma manifesting as a rapidly enlarging extracranial mass lesion. Asian J Neurosurg 2012;7:29-31

How to cite this URL:
Otani N, Nawashiro H, Wada K, Minamimura K, Takeuchi S, Shima K. Anaplastic hemangiopericytoma manifesting as a rapidly enlarging extracranial mass lesion. Asian J Neurosurg [serial online] 2012 [cited 2021 Nov 28];7:29-31. Available from: https://www.asianjns.org/text.asp?2012/7/1/29/95694


  Introduction Top


Hemangiopericytoma (HPC) arising from Zimmerman pericytes around capillaries and postcapillary venules is an extremely rare neoplasm, accounting for <1% of all central nervous system tumors. [1] The tumors are characteristically highly cellular and richly vascular, and are aggressive tending to recur even after macroscopic total resection. The 2007 World Health Organization classification of brain tumors was a revision of the former classification, which updated the concept of grading and added several new entities and variants; among the newly-codified entities was anaplastic HPC. The histological criteria for anaplastic HPC include at least more than five mitoses and/or necrosis, and at least two of the following items after hemorrhage, moderate to high nuclear atypia, and moderate to high cellularity. [2] Few accounts have been published of such a highly anaplastic and malignant change in a recurrent HPC. We, herein, described a patient with a recurrent anaplastic HPC after an initial complete resection and radiosurgery.


  Case Report Top


A 57-year-old male presented with a two-month history of right hand grip difficulty. On admission, magnetic resonance imaging revealed a well-demarked extra-axial mass lesion in the left frontal lobe [Figure 1]a-c. Partial invasion in the superior sagittal sinus (SSS) was observed on the gadolinium-enhanced T1-weighted sequences [Figure 1]a-c. After the surgical removal of the mass, a histological examination revealed HPC [Figure 1]d-f. Immunohistological findings showed that the CD34, EMA, and α-actin to be negative, while Bcl-2, S-100, vimentin, and CD99 were positive, and the MIB-1 index showed a percentage of 10-15%. Additional gamma-knife irradiation was performed for the remnant tumor in the SSS. After two years postoperatively, the remnant tumor underwent rapid regrowth [Figure 1]g-i; thus, necessitating a second surgical intervention [Figure 1]j-l. Histological examination revealed an anaplastic HPC with increasing mitotic cells [Figure 1]m and n. In addition, the recurrent tumor displayed a tumor doubling time of one week [Figure 2]a-f. A third operation was done [Figure 2]g-i. A histological examination conducted after the third procedure revealed further increasing of mitotic cells and an enlarged necrotic component [Figure 2]j. Immunohistological findings showed that CD34 was positive, and the MIB-1 index was almost 100% [Figure 2]k and l. The postoperative course was uneventful with no recurrence of the tumor evident three months following the third operation.
Figure 1: The initial enhanced T1-weighted MR image. (a-c) Show an extra-axial mass lesion in the left frontal lobe. (d, e) Postoperative MR images revealing the remnant tumor beside the SSS. (f) Histopathological findings showed the diagnosis of HPC. (g-l) Additional gamma-knife irradiation was performed. Two-years after the operation, the remnant tumor showed a rapidly regrowth (g-i), and the patients underwent a second surgical intervention (j-l). An immunohistochemical analysis using CD34 was positive (m) and a histological examination revealed an anaplastic HPC with increasing mitotic cells (n)

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Figure 2: Synospsis of the recurrence of the tumor. (a-f) The tumor recurred, with a tumor doubling time of 1-week. (g-i) The tumor recurrence necessitated a third operation. (j) A histological examination revealed further increased mitotic cells and an enlarged the necrotic component. (k) An immunohistological examination revealed CD34 positivity. (l) The MIB-1 index was almost 100%

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  Discussion Top


HPC arising from Zimmerman pericytes around capillaries and postcapillary venules is an extremely rare neoplasm, accounting for <1% of all central nervous system tumors. [1] The tumors are characteristically highly cellular and richly vascular, and are aggressive tending to recur even after macroscopic total resection. A microscopic diagnosis is based on the recognition of an architecture characterized by a pericytomatous pattern. However, the same pattern occurs in a variety of neoplasms, such as fibrous histiocytoma, solitary fibrous tumor, and fibrous meningioma. Therefore, distinguishing HPC from other tumors can be difficult, especially when the characteristic features of other neoplasms are inconspicuous. However, because of differences in the prognosis and patient management, a proper diagnosis is critical. For example, HPC has a much higher propensity to recur and metastasize than fibrous meningioma. In addition, an immunohistochemical analysis is helpful for differentiating among meningeal tumors, since there is a considerable overlap in their immunoprofiles.

The 2007 World Health Organization classification of brain tumors was a revision of the former classification, which updated the concept of grading and added several new entities and variants; among the newly-codified entities was anaplastic HPC. The histological criteria for anaplastic HPC include at least more than five mitoses and/or necrosis, and at least two of the following items after hemorrhage, moderate to high nuclear atypia, and moderate to high cellularity. [2] A malignant clinical course is associated with a large tumor, an increased mitotic rate, high cellularity, immature and pleomorphic tumor cells, and foci of hemorrhage and necrosis. [3] The best treatment to manage the HPCs has not yet established. However, several authors suggested that a significant prolongation in recurrence-free interval as well as long-term survival was observed in patients who underwent a complete macroscopic resection and adjuvant radiation therapy. [4],[5] Thus, HPCs are difficult to cure surgically; postoperative radiotherapy has been proposed, even after a gross total tumor resection. [6],[7]

In the present case, the residual tumor cells, after the initial excision, grew, and accumulated genetic and phenotypic changes to become more highly malignant at the time of clinical recurrence. Few accounts have been published of such a highly anaplastic and malignant change in a recurrent HPC. Therefore, the present case serves as an important warning of this risk. In summary, we, herein, described a patient with a recurrent anaplastic HPC after an initial complete resection and radiosurgery. This case highlights the mandatory need for a careful long-term follow-up for patients with HPC, since recurrence with a greater degree of malignancy can develop following an extended disease-free interval.

 
  References Top

1.Guthrie BL, Ebersold MJ, Scheithauer BW, Shaw EG. Meningial hemangiopericytoma: Histopathological features, treatment, and long-term follow-up of 44 cases. Neurosurgery 1989;25:514-22.  Back to cited text no. 1
[PUBMED]    
2.Gianni C. Hemangiopericytoma. In: WHO classification of tumors of the central nervous system. 4 th ed, In: Louis DN (editors), IARC, Lyon: WHO Press; 2007. p.178-80.  Back to cited text no. 2
    
3.Enzinger FM, Smith BH. Hemangiopericytoma. An analysis of 106 cases. Hum Pathol 1976;7:61-82.  Back to cited text no. 3
[PUBMED]    
4.Rutkowski MJ, Bloch O, Jian BJ, Chen C, Sughrue ME, Tihan T, et al. Management of recurrent intracranial hemangiopericytoma. J Clin Neurosci 2011;18:1500-4.  Back to cited text no. 4
    
5.Schiariti M, Goetz P, El-Maghraby H, Tailor J, Kitchen N. Hemangiopericytoma: long-term outcome revisited. Clinical article. J Neurosurg 2011;114:747-55.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Galanis E, Buckner JC, Scheithauer BW, Kimmel DW, Schomberg PJ, Piepgras DG. Management of recurrent meningeal hemangiopericytoma. Cancer 1998;82:1915-20.  Back to cited text no. 6
[PUBMED]    
7.Olson C, Yen CP, Schlesinger D. Radiosurgery for intracranial hemangiopericytomas: outcomes after initial and repeat Gamma Knife surgery. J Neurosurg 2010;1:133-9.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]


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