Hypervascularity in Lhermitte-Duclos Disease —Case Report—

Ishizaki, Kenichi; Daita, G; Yonemasu, Yukichi; Kunimoto, M; Miyokawa, Naoyuki

doi:10.2176/nmc.37.403

Cited by 15 publications

(5 citation statements)

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“…Most cases documented have occurred in adults in the third and fourth decades of life, with no sex predilection (2,8). LDD in the elderly is quite rare, with only 5 other cases reported in patients over 60 years old (2,(5)(6)(7)10). Table I summarizes the clinical features of LDD in the elderly, including the present case.…”

Section: Discussionmentioning

confidence: 79%

“…On the other hand, subtle enhancement along the folia on contrast-enhanced T1-weighted imaging and slight tumor blush on angiography were detected. Such findings may indicate that part of the tumor has a rich blood flow (2,5). In fact, histological examination showed increased numbers of vessels compared to normal cerebellar tissue.…”

Section: Resultsmentioning

confidence: 97%

“…Lack of enhancement is the rule for this tumor, except in rare cases (2,5). Angiography shows LDD as an avascular mass (5).…”

Section: Resultsmentioning

confidence: 99%

“…CT usually reveals a low-density mass in the lesion and calcification is sometimes present (2,6,8). MRI shows the lesion better than CT, with a characteristic striated (2,5,7,10). In addition, extensive resection risks include neurological deficits such as cerebellar mutism (1).…”

Section: Discussionmentioning

confidence: 99%

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Lhermitte-duclos disease treated surgically in an elderly patient: case report and literature review

Matsumoto¹,

Minami²,

Yoshida³

2014

Turkish Neurosurgery

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A 75-year-old man with Lhermitte-Duclos Disease (LDD) manifesting as progressive headache is presented. Magnetic resonance imaging demonstrated a right cerebellar mass lesion with the characteristic "tiger-striped appearance". A mild mass effect was evident at the medulla oblongata, accompanied by inferior displacement of the right cerebellar tonsil. Thus, tonsillar herniation was considered the cause of his headache and he underwent partial resection of the tumor to solve tonsillar herniation. His headache gradually improved. LDD in the elderly is quite rare. Several differences in the nature of the lesion are seen in the elderly, with tendencies toward a male predominance, hypervascularity, and low rates of association with Cowden disease. Moreover, partial resection to reduce mass effect can improve clinical symptoms without recurrence. In symptomatic LDD in the elderly, to avoid surgical complication, we recommend partial resection to reduce mass effect. KeywOrds: Dysplastic gangliocytoma, Elderly patient, Headache, Lhermitte-Duclos Disease ÖZYetmiş beş yaşında bir erkek hastada progresif baş ağrısı ile ortaya çıkan Lhermitte-Duclos Hastalığı (LDH) sunulmaktadır. Manyetik rezonans görüntüleme "Kaplan çizgileri" görünümü olan bir sağ serebellar kitle lezyonunu gösterdi. Medulla oblongata'da hafif bir kitle etkisi vardı ve sağ serebellar tonsil inferiora doğru yer değiştirmişti. Bundan dolayı tonsiller heniasyonun baş ağrısına neden olduğu düşünüldü ve hastaya tonsiller herniasyonun tedavisi için tümörün parsiyel rezeksiyonu uygulandı. Hastanın baş ağrısı tedrici olarak düzeldi. LDH yaşlılarda oldukça nadirdir. Bu hastalığın yaşlılarda görülen formunda tümörün natüründe bazı farklılıklar vardır. Bunlar, erkek predominansı, hipervaskülarite ve Cowden hastalığı ile daha düşük oranda birlikteliktir. Ayrıca parsiyel rezeksiyon kitle etkisini azaltarak nüks olmadan klinik semptomların iyileşmesini sağlamıştır. Semptomatik LDH olan yaşlı hastalarda cerrahi komplikasyondan kaçınmak için kitle etkisini azaltmak amacıyla parsiyel rezeksiyon yapılmasını öneriyoruz.

show abstract

Section: Discussionmentioning

confidence: 79%

Section: Resultsmentioning

confidence: 97%

“…Lack of enhancement is the rule for this tumor, except in rare cases (2,5). Angiography shows LDD as an avascular mass (5).…”

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Lhermitte-duclos disease treated surgically in an elderly patient: case report and literature review

Matsumoto¹,

Minami²,

Yoshida³

2014

Turkish Neurosurgery

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“…2,17,36,41 The T 2 -weighted images demonstrate a well-circumscribed, high-intensity lesion with a "tiger-striped" pattern. Modern neuroimaging, particularly the MR imaging modality, has greatly facilitated the diagnosis and care of patients with LDD.…”

Section: Lhermitte-duclos Diseasementioning

confidence: 99%

Cowden disease and Lhermitte–Duclos disease: an update

Robinson

Cohen²

2006

FOC

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Object Cowden disease is a rare autosomal-dominant phacomatosis and cancer syndrome that is associated with Lhermitte–Duclos disease (LDD), also called dysplastic cerebellar gangliocytoma. Methods In this review the authors summarize the additions to the literature during the past 5 years, with emphasis on new case reports and advances in imaging and molecular biology. Adult-onset LDD is now considered pathognomonic for Cowden disease. Approximately 220 cases of LDD have been reported. Magnetic resonance imaging in patients with LDD is often diagnostic, and imaging studies have facilitated accurate diagnosis and contributed to the improved outcome in affected patients. Cowden disease and other rare, related disorders, such as Bannayan-Riley-Ruvalcaba, Proteus, and Proteus-like syndromes, are often caused by mutations of the PTEN gene. Conclusions Because of the high incidence of systemic cancer in patients with Cowden disease, it is important for neurosurgeons to recognize the association between this disease and LDD and to refer affected patients for appropriate cancer screenings and interventions.

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Contrast enhancement in Lhermitte–Duclos disease of the cerebellum: correlation of imaging with neuropathology in two cases

et al. 2003

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Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma, is a rare cerebellar lesion. It has long been regarded as avascular. We report two patients with surgically proven LDD in whom contrast enhancement was observed on MRI. Neuropathological examination revealed proliferation of veins. We suggest that peripheral enhancement of LDD probably reflects vascular proliferation of the cerebellar venous draining system, and should be considered part of the imaging features of LDD.

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Hypervascularity in Lhermitte-Duclos Disease —Case Report—

Cited by 15 publications

References 0 publications

Lhermitte-duclos disease treated surgically in an elderly patient: case report and literature review

Lhermitte-duclos disease treated surgically in an elderly patient: case report and literature review

Cowden disease and Lhermitte–Duclos disease: an update

Contrast enhancement in Lhermitte–Duclos disease of the cerebellum: correlation of imaging with neuropathology in two cases

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