�ber die Hamartome (Ganglioneurome) des Kleinhirns

Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm?

“…From the very early case reports until the end of the 1970s, preoperative neuroimaging modalities to evaluate indirectly the mass effect of dysplastic cerebellar gangliocytoma mainly consisted of plain skull radiograms, vertebral angiography and ventriculography (2, 4, 5, 7–11, 13). Later, computed tomography (CT) became a sufficient radiographic tool to demonstrate the tumour mass, its distribution within the posterior fossa and its interference with the cerebellar parenchyma, nerval structures and CSF compartments more directly (3, 15, 34, 37, 41).…”

“…Surgery is definitely the therapeutic procedure commonly performed for the treatment of dysplastic cerebellar gangliocytoma (3, 5–7, 11–15, 17, 18, 30, 31, 33, 35, 37–39). Outcome in not surgically treated patients was uniformly poor in early cases of Lhermitte–Duclos disease, obviously as a result of the progressive mass nature of the growing tumour process (2, 5, 15).…”

“…In most cases, the affected areas were remarked to be paler in colour than the surrounding normal cerebellar cortex (5, 15, 16, 21, 34, 45, 46). Cross‐section shows a fairly enlargement of the cerebellar cortex, sometimes extending into the vermis (5), with gross reduction or even absence of the central white matter (1, 2, 5, 10, 15). The principal microscopic abnormality is massive replacement and expansion of the internal granular cell layer by large neurones with vesicular nuclei and prominent nucleoli (5, 34, 46).…”

“…The authors originally described the lesion under the name of “ganglioneurome myélinique diffus de l'écore cérébelleuse”. Subsequently, this entity was referred to by a variety of synonymous descriptions, such as diffuse ganglioneuroma of the cerebellar cortex (2, 3) (benign) hypertrophy of the cerebellum (4–6), Purkinjoma (7), hamartoma of the cerebellum (2, 8), gangliocytoma myelinicum diffusum of the cerebellar cortex (9), dysplastic gangliocytoma of the cerebellum (10–14), granule cell hypertrophy of the cerebellum (15) or simply Lhermitte–Duclos disease (16–20).…”

“…Regarding the nature of the disease, the original opinion expressed by Lhermitte and Duclos was that the tumour was a combination of a congenital malformation and a neoplasm arising from ganglion cells (1). Other early observers of this entity suggested the lesion to be a “neurocystic blastoma” (8), “hamartoma” (2, 8) or “hyperplasia” (4, 5, 15, 21). In 1930, Bielschowsky and Simons used contradictory terms to define the tumour's nature, such as “hamartoma” and “hamartomablastoma”, but finally concluded that it is neoplastic (8).…”