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Year : 2011  |  Volume : 54  |  Issue : 2  |  Page : 410-411
Papillary meningioma: A rare malignant variant

Institute of Pathology, ICMR, New Delhi, India

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Date of Web Publication27-May-2011

How to cite this article:
Jairajpuri Z, Jain I, Singh A. Papillary meningioma: A rare malignant variant. Indian J Pathol Microbiol 2011;54:410-1

How to cite this URL:
Jairajpuri Z, Jain I, Singh A. Papillary meningioma: A rare malignant variant. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Jun 6];54:410-1. Available from: http://www.ijpmonline.org/text.asp?2011/54/2/410/81595


The histopathological spectrum of meningiomas is broad with several variants. Papillary meningioma (PM) is a rare, distinctive malignant histological variant of meningioma with a highly aggressive clinical course and grim prognosis. It is important to recognize this variant in view of its implications for management as well as prognostication in order to reduce the morbidity and mortality. We report a case of papillary meningioma in a female patient presenting with a history of intermittent headache and loss of vision.

A 45-year-old woman presented with complaints of intermittent headache for twenty days and loss of vision for five days. Ophthalmic examination revealed papilloedema while on CT scan, a contrast enhancing hypodense mass in the left frontoparietal area was seen.

Microscopy of sections from the lesion showed tumor tissue composed of sheets of cells arranged in a predominantly papillary pattern with perivascular pseudorosettes. Tumor cells displayed moderate eosinophilic cytoplasm and vesicular nuclei and few mitoses [Figure 1]. Microscopic foci of necrosis were also seen. Immunohistochemical staining revealed positivity for epithelial membrane antigen (EMA) [Figure 2]b and vimentin [Figure 2]a, while it was non-reactive for glial fibrillary acidic protein (GFAP). All these findings led to a diagnosis of PM.
Figure 1: Photomicrograph H and E ×100 showing tumor tissue composed of sheets of cells arranged in a predominantly papillary pattern; cells show moderate eosinophilic cytoplasm and vesicular nuclei

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Figure 2: (a) Immunohistochemistry showing vimentin positivity; (b) Immunohistochemistry showing epithelial membrane antigen ×400 positivity

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Meningiomas are among the most common brain neoplasms accounting for approximately 13-19% of all primary intracranial tumors, they represent wide biological and histological spectrum. [1] PM are a malignant variant and are designated grade 3 by classification of WHO. [2] They are histologically characterized exclusively or in part by papillary structures and are often intermixed with more classic meningothelial morphology. Knowledge of this variant is of clinical significance, as a variety of tumors can exhibit papillary features and are very pertinent to the differential diagnosis of an intracranial mass such as metastatic adenocarcinoma EMA and cytokeratin (CK) positive and vimentin negative, chemodectoma, papillary ependymoma and astroblastoma (GFAP positive). [3],[4]

Recognition of the lesion, as a distinct subtype, has been slow. Cushing and Eisenhardt were the first to describe papillary meningiomas as malignant variant with intracerebral recurrence. [5] The age at presentation has been variably reported by different authors; however, an age range of 3 to 69 years with no gender predominance has been seen. They are frequently seen in the supratentorial compartment and rarely reported in the posterior fossa, jugular foramen [3] and oculomotor nerve. [4]

Papillary meningioma are known to metastasize, with both local as well as distant metastasis. [3],[6] Local recurrences are more common with the tumor seen in cerebrospinal pathways also. [3] Distant metastasis is seen to occur in the lungs and liver. Tumor is known to recapitulate the original lesion at the metastatic site also. Lesions with papillary patterns are known to have an aggressive character with typical and atypical mitoses, necrosis, pleomorphism and recurrences associated with metastasis. The aggressive behavior of papillary meningioma associated with morbidity and mortality warrants a timely recognition of the diagnosis.

The standard treatment of PM is surgical resection, ranging from debulking to radical removal. Median survival has been noted to be eight years, illustrating the malignant propensity of these tumors. [7] Aggressive surgery followed by adjuvant radiotherapy is advocated in cases of malignant meningiomas. However, effectiveness of radiotherapy is disputed.

To conclude, PM is a rare variant of meningioma and its early diagnosis and management is important because of its propensity for recurrence and poor clinical outcome.

   References Top

1.Avninder S, Vermani S, Sharma S, Chand K. Papillary meningioma: A rare but distinct variant of malignant meningioma. Diagn Pathol 2007;2:3.  Back to cited text no. 1
2.Perry A. Meningiomas. In: McLendon RE, Rosenblum MK, Bigner DD, editors. Russell and Rubinstein's Pathology of Tumors of the Nervous System. 7 th ed. 2006. p. 427-74.  Back to cited text no. 2
3.Ludwin SK, Rubinstein LJ, Russell DS. Papillary meningioma: A malignant variant of meningioma. Cancer 1975;36:1363-73.  Back to cited text no. 3
4.Piatt JH Jr, Campbell GA, Oakes WJ. Papillary meningioma involving the occulomotor nerve in an infant. Case report. J Neurosurg 1986;64:808-12.  Back to cited text no. 4
5.Cushing H, Eisenhardt L. Meningiomas: Their classification, regional behavior, life history and surgical end results. monograph on Meningiomas after In: Thomas CC, editor. Ill, Baltimore, Springfield; 1938. p. 692-717.  Back to cited text no. 5
6.Kepes JJ, MacGee EE, Vergara G, Sil R. A case report: Malignant meningioma with extensive pulmonary metastases. J Kans Med Soc 1971;72:312-6.  Back to cited text no. 6
7.Kross JM, Cella F, Bakker SL, Paz Y Geuze D, Egeler RM. Papillary meningioma with pleural metastasis: Case report and literature review. Acta Neurol Scand 2000;102:200-2.  Back to cited text no. 7

Correspondence Address:
Ila Jain
SFS Flats B-3, Sheikh Sarai-1, New Delhi - 110 017
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.81595

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  [Figure 1], [Figure 2]

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