{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T16:03:07Z","timestamp":1790352187056,"version":"4.1.0"},"reference-count":50,"publisher":"Scientific Scholar","content-domain":{"domain":["surgicalneurologyint.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec id=\"st1\">\n<jats:title>Background: <\/jats:title>\n<jats:p>Lhermitte\u2013Duclos disease (LDD) is a rare tumor, with only about 300 reported cases. It often shows comorbidity with Cowden syndrome (CS); however, it can occur by itself. Radiologically, the \u201ctiger-stripe\u201d appearance is considered pathognomonic. Surgical resection remains the mainstay of treatment. This report aims to describe the clinical and radiological characteristics of LDD and its relationship with CS according to age group.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st2\">\n<jats:title>Methods: <\/jats:title>\n<jats:p>PubMed electronic databases were searched in August 2022. The search terms included \u201cLhermitte\u2013 Duclos disease\u201d and \u201cdysplastic gangliocytoma,\u201d which yielded 297 and 103 research articles, respectively. The articles were collected and reviewed by three researchers.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st3\">\n<jats:title>Results: <\/jats:title>\n<jats:p>Out of 400 identified articles, we analyzed 302 reported cases. The mean age at presentation was 33.6 \u00b1 16 years; 171 patients (56.6%) were female, and 123 (40.7%) were male. The most commonly reported symptom was headache (174 patients, 57.6%), followed by ataxia (109, 36.1%). In addition, 99 cases (32.8%) were associated with CS, and 60 (19.9%) had a confirmed phosphatase and tensin homolog (<jats:italic>PTEN<\/jats:italic>) mutation. A tiger-stripe appearance was observed in 208 cases (58.7%); surgical resection was performed in 64.2% of the cases. Mortality and recurrence rates were 4.3% and 8.6%, respectively. No statistically significant difference was found between adult- and pediatric-onset LDD for the association with CS (<jats:italic>P<\/jats:italic> = 0.128).<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"st4\">\n<jats:title>Conclusion: <\/jats:title>\n<jats:p>Our findings suggest that adult and pediatric LDD have major commonalities; however, further prospective studies are warranted.<\/jats:p>\n<\/jats:sec>","DOI":"10.25259\/sni_555_2023","type":"journal-article","created":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T05:37:06Z","timestamp":1695965826000},"page":"351","update-policy":"https:\/\/doi.org\/10.25259\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Lhermitte\u2013Duclos disease: A systematic review"],"prefix":"10.25259","volume":"14","author":[{"given":"Aued Iaed","family":"Alanazi","sequence":"first","affiliation":[{"name":"Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tariq","family":"Alanezi","sequence":"additional","affiliation":[{"name":"Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ziyad Fahad","family":"Aljofan","sequence":"additional","affiliation":[{"name":"Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alwaleed","family":"Alarabi","sequence":"additional","affiliation":[{"name":"Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sherif","family":"Elwatidy","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, King Saud University, Riyadh, Saudi Arabia."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10894","published-online":{"date-parts":[[2023,9,29]]},"reference":[{"key":"10.25259\/SNI_555_2023\/ref-1","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1093\/jnen\/64.4.341","article-title":"Lhermitte-Duclos disease: A report of 31 cases with immunohistochemical analysis of the PTEN\/AKT\/mTOR pathway","volume":"64","author":"Abel","year":"2005","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.25259\/SNI_555_2023\/ref-2","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1055\/s-0038-1670636","article-title":"LhermitteDuclos disease with cervical arteriovenous fistula","volume":"80","author":"Almubarak","year":"2019","journal-title":"J Neurol Surg A Cent Eur Neurosurg"},{"key":"10.25259\/SNI_555_2023\/ref-3","doi-asserted-by":"crossref","first-page":"118","DOI":"10.11604\/pamj.2019.33.118.16809","article-title":"The Lhermitte-Duclos disease: A rare bilateral cerebellar location of a rare pathology","volume":"33","author":"Borni","year":"2019","journal-title":"Pan Afr Med J"},{"key":"10.25259\/SNI_555_2023\/ref-4","doi-asserted-by":"crossref","first-page":"309","DOI":"10.4103\/0028-3886.63799","article-title":"Bilateral LhermitteDuclos disease","volume":"58","author":"Bozbuga","year":"2010","journal-title":"Neurol India"},{"key":"10.25259\/SNI_555_2023\/ref-5","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1007\/BF01961328","article-title":"About ganglion cell tumors in the brain","volume":"300","author":"Christensen","year":"1937","journal-title":"Virchows Arch Pathol Anat Physiol Klin Med"},{"key":"10.25259\/SNI_555_2023\/ref-6","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1097\/00007611-199612000-00016","article-title":"Lhermitte-Duclos disease (cerebellar gangliocytoma)","volume":"89","author":"da Silva","year":"1996","journal-title":"South Med J"},{"key":"10.25259\/SNI_555_2023\/ref-7","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1007\/BF02011314","article-title":"Dysplastic gangliocytoma of cerebellum in a newborn. 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