Abstract
13 cases of intracranial lipomatous hamartomas are presented, 12 of which were incidentally found at autopsy. Only one case, verified by biopsy, showed progressive focal symptoms and lipomatous infiltration of the acoustic nerve. 5 lipomatous hamartomas were located in the cisterna ambiens region, 3 in the cerebellopontine angle, 2 in the hypothalamic and 1 in the callosal regions. 2 cases had multiple intracranial adipose tissue masses. Extracranial malformations were present in 4 cases, whereas 8 observations showed (micro)dysgenesias of brain and meningeal tissues surrounding the lipomatous hamartoma (mixed neuroglial-mesenchymal tissue, glial dystopias, aberrant nerve fibres, “hypertrophic” nerve bundles, peripheral myelin, micropolygyria, cerebellar microdysgenesias). 5 cases are described in detail: 1. Pontine lipomatous hamartoma combined with body malformations, frontobasal epidermoid cyst, tentorial osteoma and cerebellar microdysgenesias. 2. Cerebellar medulloblastoma associated with fibrolipomatous meningeal hamartoma and cerebellar micropolygyria. 3. Large lipomatous hamartoma of the callosal region encircling a colloid cyst. 4. Multiple meningeal fatty nodules associated with facial malformations, cerebellar dysgenesias and tentorial osteoma. 5. Intracranial hamartomatosis with lipo-fibro-angiomatosis of meninges, megadolichobasilaris and multiple “subependymomas”. The origin of intracranial fatty tissue is discussed; intracranial lipomatous hamartoma should be regarded as a complex malformation, affecting both the cerebral ectomesenchyma and brain parenchyma, and classified as true hamartoma (tumour-like, but primarily non-neoplastic malformation). The term “lipoma”, implicating a neoplastic character, should be abandoned. Links to phakomatoses are suggested.
Published Version
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