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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 449-451

Calcifying pseudoneoplasm of the cerebellomedullary cistern: A case report and review of the literature


1 Department of Neurosurgery, Faculty of Medicine, Adana Practice and Research Center, Baskent University, Adana, Turkey
2 Department of Pathology, Faculty of Medicine, Adana Practice and Research Center, Baskent University, Adana, Turkey

Date of Web Publication19-Sep-2014

Correspondence Address:
Aydemir Fatih
Department of Neurosurgery, Faculty of Medicine, Adana Practice and Research Center, Baskent University, Adana
Turkey
Aydemir Fatih
Department of Neurosurgery, Faculty of Medicine, Adana Practice and Research Center, Baskent University, Adana
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.141255

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How to cite this article:
Fatih A, Melih C, Ozgur K, Feyzi SB, Kadir T, Fazilet K, Fatih A, Melih C, Ozgur K, Feyzi SB, Kadir T, Fazilet K. Calcifying pseudoneoplasm of the cerebellomedullary cistern: A case report and review of the literature. Neurol India 2014;62:449-51

How to cite this URL:
Fatih A, Melih C, Ozgur K, Feyzi SB, Kadir T, Fazilet K, Fatih A, Melih C, Ozgur K, Feyzi SB, Kadir T, Fazilet K. Calcifying pseudoneoplasm of the cerebellomedullary cistern: A case report and review of the literature. Neurol India [serial online] 2014 [cited 2020 Oct 20];62:449-51. Available from: https://www.neurologyindia.com/text.asp?2014/62/4/449/141255


Sir,

Calcifying pseudoneoplasms (CPNs) are rare, calcified, non-neoplastic lesions and can occur in any part of the central nervous system (CNS). They were first described by Rhodes and Davis. [1] In the pathophysiology, although there is not a complete consensus among authors, they are thought to occur after a reactive or metaplastic process in the neuraxis. Our patient is the first reported case in this anatomic location and second time reported case of CPN with its imaging features in the literature.

Medical history of 59-year-old female patient with a long-lasting headache was unremarkable. Neurological examination was intact. Brain computed tomography (CT) revealed that a hyperdense [Figure 1], brain magnetic resonance imaging (MRI) demonstrated an extra-axial mass lesion located in cerebellomedullary cistern. Mass lesion was showing linear band-style extension with intense enhancement toward choroid plexus of the fourth ventricle [Figure 2]. As meningiomas and ependymomas were the differentials and patient was symptomatic, surgical treatment was considered. The mass was totally excised with a midline suboccipital craniectomy. The tumor was extremely tough, solid, white, and with a good cleavage from the normal tissue. On histopathological examination of tumor tissue, tissues showed calcified chondroid and osseous metaplasia. In focal areas, epithelial structures in papilla-like patterns were observed without surrounding atypia. Epithelial cells were widespread positively stained with GFAP, keratin, and S-100, and negative staining were detected with epithelial membrane antigen (EMA) mucin and PAS-A blue [Figure 3]. Staining pattern-observed cells were evaluated as choroid plexus cells, and the histopathologic diagnosis of tumor was reported to be compatible with CPN.
Figure 1: At the cerebellomedullary cistern, computed tomography showed hyperdens lesion bouth on brain (a) and bone (b) windows

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Figure 2: Brain MRI showed a lesion hypointense on T1-weighted images (a) and heterogeneous in appearance T2-weighted images (b). The lesion was densyl enhanced after Gd. injection (c,d)

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Figure 3: Focal chondroid metaplasia with amorphous calcified material and chondromyxoid matrix (H and E, original magnification 200)

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Including our patient, 39 cases of intracranial CPN have been published in the literature [Table 1]. Although clinical findings vary according to location and size of the lesion, they mostly present with headache and seizures. Of 33 cases described in the literature that underwent operation, 22 had gross total resection and 11 had subtotal resection. In the 22 patients, average follow-up was of 57.9 months, no recurrence was reported in patients who underwent gross total resection, and a recurrence had been reported after 3 years from the first operation in one patient who underwent subtotal resection. [2] In two patients, growth of the lesions were observed 5 and 8 years after initial diagnosis of the lesion, and these patients had been operated. [3],[4]
Table 1: Summary of 39 reported cases of intracranial CPN

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Other calcified intracranial lesions should be considered in differential diagnosis. Calcifying pseudoneoplasms are observed radiographically as a hyperdense lesion on CT. On MRI, they are usually hypointense on T1- and T2-weighted sequences in appearance. They show different contrast enhancement after the injection of Gd. In most cases, interlinear or rim-type contrast enhancement is observed, whereas besides our case, intense contrast enhancement has been reported only in one case in the literature. [5] In addition, in two of the reported cases, there has been no contrast enhancement. [6]

Typical histopathological findings of CPN are typical chondromyxoid matrix in nodular pattern, palisading spindle to epithelioid cells, varying amounts of fibrous stroma, calcification, osseous metaplasia, scattered psammoma body and foreign body reaction with giant cells. [6] These findings may not be all together. Mitosis and necrosis have not been reported. Although a wide variety of immunohistochemical stains have been used to characterize CPN, they show highly positive staining with EMA and vimentin and highly negative staining with GFAP and S-100. [4]

Most authors, on the basis of findings of granulomatous inflammation surrounding these, believe that these calcified lesions occur after a reactive process in neuraxis. [2],[6],[7] Rodriguez et al. [8] presented a CPN case coexisting with ependymomas. They reported that CPN might have developed due to reactive process around ependymomas. Salim et al. [9] reported a CPN in touch with an intra-axial lipoma and reported that CPN might most likely occur as a result of reactive process. It has been suggested, but not proven, that CPN may develop as a healing response to an array of inciting factors, which can account for the variations in histopathologic features. The causal factors are not yet understood, but response to possible trauma, infection, or inflammation has been proposed. [10]

It is impossible to give some diagnostic and treatment algorithms because of rarity of the condition. Imaging methods are insufficient for a definitive diagnosis in growing number of these reported lesions, and they should be considered in the differential diagnosis of calcified lesions. Moreover, these lesions may grow and have recurrence risk. Thus, surgical resection seems to be the rationalistic treatment for now. Surgical removal of lesions is important both in elimination of the patient's symptoms and obtaining histopathological diagnosis, and besides prevention of aggressive adjuvant treatments. To have enough opinion for the course of the disease, we think that there is a need for a greater number of patients and longer follow-up period.

 
  References Top

1.Rhodes RH, Davis RL. An unusual fibro-osseous componentn intracranial lesions. Hum Pathol 1978;9:309-19.  Back to cited text no. 1
    
2.Bertoni F, Unni KK, Dahlin DC, Beabout JW, Onofrio BM. Calcifying pseudoneoplasms of the neural axis. J Neurosurg 1990;72:42-8.  Back to cited text no. 2
    
3.Jun C, Burdick B. An unusual fibro-osseous lesion of the brain. Case report. J Neurosurg 1984;60:1308-11.  Back to cited text no. 3
    
4.Kerr EE, Borys E, Bobinski M, Shahlaie K. Posterior fossa calcifyingpseudoneoplasm of the central nervous system. J Neurosurg 2013;118:896-902.  Back to cited text no. 4
    
5.Montibeller GR, Stan AC, Krauss JK, Nakamura M. Calcifying pseudoneoplasm of the inferior colliculus: An unusual location for a rare tumor: Case report. Neurosurgery 2009;65:E1005-6.  Back to cited text no. 5
    
6.Qian J, Rubio A, Powers JM, Rosenblum MK, Pilcher WH, Shrier DA, et al. Fibro-osseous lesions of the central nervous system: Report of four cases and literature review. Am J Surg Pathol 1999;23:1270-5.  Back to cited text no. 6
    
7.Tatke M, Singh AK, Gupta V. Calcifying pseudoneoplasm of the CNS. Br J Neurosurg 2001;15:521-3.  Back to cited text no. 7
    
8.Rodriguez FJ, Scheithauer BW, Fourney DR, Robinson CA. Ependymoma and intraparenchymal calcifying pseudoneoplasm of the neural axis: Ýncidental collision or unique reactive phenomenon? Acta Neuropathol 2008;115:363-6.  Back to cited text no. 8
    
9.Salim AA, Wilson PJ, Cherukuri RK, McKenzie S, Buckland ME. An unusual association of calcifying pseudoneoplasm of the neuraxis with interhemispheric lipoma and agenesis of corpus callosum. Pathology 2012;44:657-9.  Back to cited text no. 9
    
10.Aiken AH, Akgun H, Tihan T, Barbaro N, Glastonbury C. Calcifying pseudoneoplasms of the neuraxis: CT, MR imaging, and histologic features. AJNR Am J Neuroradiol 2009;30:1256-60.  Back to cited text no. 10
    


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