Primitive embryonal tumours of the meninges are an exceedingly rare entity and has been predominantly reported in the paediatric population. It poses a unique challenge in establishing the diagnosis as it closely resembles meningioma. This makes the incorporation of sophisticated techniques such as immunohistochemistry, molecular studies, and genetic analysis mandatory to arrive at a confirmative diagnosis. We hereby report a case of a 13-year boy who was diagnosed with primitive embryonal tumour that was initially sought to be a meningioma. Alongside, we have also included a review of literature on this entity focussing on paediatric population, where only a handful of case reports have been published till date. Although these tumours are biologically aggressive, but they tend to have good response rate with treatment. Due to rarity of this entity, consensus on the management guidelines is lacking. However, an acquaintance to this entity is important amongst neuro-oncologists to decrease the possibility of misdiagnosis and improve the cure rates and survival.

One hundred and twenty‐two fine needle aspirates (FNA) from female patients were studied to determine whether CA‐125 assay contributed to cytologic diagnosis and CEA assay. Cytologic examination was done on Papanicolaou‐stained smears and cell blocks, CEA by EIA (Abbott Laboratory, > 5 ng/ml cutoff) and CA‐125 by RIA (Abbott Laboratory, North Chicago, IL, > 66 μ/ml cutoff). Final diagnosis were correlated with histologic diagnosis when available, clinical, radiologic studies, and follow‐up. Results: 29 benign, 93 malignant. Sensitivities and specificities: cytology, 91%, 100%; CEA: 59%, 86%; CA‐125, 50%, 55%. CEA plus cytology sensitivity, 97%. CA‐125 content was highest in endometrial/ovarian carcinoma (39,899 μ/ml) and < 5,000 μ/ml in other tumors and benign FNA in contrast to CEA which showed highest levels in carcinomas of colon, pancreas, and lung (> 280 ng/ml). While elevated CEA enhances the sensitivity of cytologic diagnosis of carcinomas of the colon, pancreas, and lung, low CEA and high CA‐125 content supports an ovarian/endometrial primary. Diagn Cytopathol 1996;14:121–125. © 1996 Wiley‐Liss, Inc.

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