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Whorling-sclerosing meningioma. A review on the histological features of a rare tumor including an illustrative case. | LitMetric

Whorling-sclerosing meningioma. A review on the histological features of a rare tumor including an illustrative case.

Clin Neurol Neurosurg

Neuroacademy Research Group, Memorial Hospital, Department of Neurosurgery, Kaptanpasa Mh, Okmeydani/Sisli, Istanbul, 34384, Turkey.

Published: November 2017

AI Article Synopsis

  • The Whorling-Sclerosing variant of meningioma (WSM) is an extremely rare type of brain tumor, with only 28 documented cases in English literature, characterized by unique morphological features like collagen whorls and psammoma bodies.
  • Diagnosis of WSM relies heavily on examining its shape and cellular composition rather than advanced immunohistochemical tests, with tumors displaying low levels of cell growth (Ki-67 indices between 0-4%) and typically lacking aggressive characteristics.
  • A new case of WSM is reported that includes unusual uncalcified collagen structures and showed no signs of malignancy, underscoring the need to consider this variant in diagnosis to prevent unnecessary aggressive treatments.

Article Abstract

Whorling-Sclerosing variant of meningioma (WSM) is a very rare variant of meningioma and only 28 cases were previously reported in the English medical literature. The term "whorling" describes different morphological features including psammoma bodies formed by precipitated calcium or by layered whorling sheets of tumor cells in meningothelial meningiomas. In WSM, the sclerosing structures are formed by typical paucicellular or acellular collagen whorls which form the majority of tumor volume. Hence, diagnosis of these tumors is based more often on morphological and histochemical features rather than on immunohistochemical findings. WSMs often contain cells with both fibroblast-like spindle cell morphology predominantly immunopositive for vimentin and meningoepithelial cells immnunopositive for EMA. The range of their Ki-67 indices differs between 0 and 4 percent. These tumors show no focal pleomorphism, necrosis and high mitotic activity. In some WSMs, entrapped GFAP immunopositive astrocytes may be seen during invasion of the adjacent neural parenchyma. Brain invasion by WSMs may give rise to erroneous diagnoses, i.e. malignant (Grade-3) meningioma, meningiosarcoma, ganglioglioma and even astrocytoma, leading detrimental overtreatment. However; hitherto, no WSM was reported exerting any aggressive behavior. Besides reviewing the literature, we also report a new WSM with abundant uncalcified paucicellular collagen whorls forming aggregates of nodules. Unlike to previous reports, this tumor was costained with p53 and progesterone receptor. The tumor showed no morphological malignancy characteristics, e.g. cellular atypia, prominent nucleoli, hypercellularity, micronecrosis/geographical necrosis, sheeting and small cells. This rare meningioma variant should be kept in mind among differential diagnoses to avoid overtreatment that might endanger patients' outcome.

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Source
http://dx.doi.org/10.1016/j.clineuro.2017.09.009DOI Listing

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