{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,3,9]],"date-time":"2024-03-09T10:18:49Z","timestamp":1709979529792},"reference-count":67,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2009,2]]},"abstract":"<jats:p>Malignancies are a common feature of Maffucci syndrome, with chondrosarcomas being the most common tumor type. The authors present the first case of Maffucci syndrome associated with a pituitary adenoma and a probable brainstem glioma and review the literature concerning intracranial tumors related to this disease.<\/jats:p>\n<jats:p>They report the case of a 35-year-old woman with Marfucci syndrome (diagnosed when she was 22 years old) who presented with complaints of decreased visual acuity and visual field defect. Neuroimaging revealed a pituitary macroadenoma and a suspected brainstem tumor. The macroadenoma was partially removed. There were no postoperative complications and the patient experienced rapid improvement in visual acuity. On follow-up examination 2 years later, the lesion in the pons showed the same dimensions. No sarcomatous changes of enchondromas or hemangiomas occurred.<\/jats:p>\n<jats:p>To the authors' knowledge, including the present case, only 7 cases of Maffucci syndrome associated with glioma and 7 cases associated with pituitary adenoma have been reported in the literature. This report emphasizes that patients with this disease are at a higher risk for primary intracranial tumors and reinforces the concept of the multiplicity of tumors that may arise in this syndrome. It also underscores the importance of early diagnosis, regular clinical surveillance, and follow-up studies of these patients.<\/jats:p>","DOI":"10.3171\/2008.8.jns08150","type":"journal-article","created":{"date-parts":[[2008,10,31]],"date-time":"2008-10-31T15:15:47Z","timestamp":1225466147000},"page":"363-368","source":"Crossref","is-referenced-by-count":7,"title":["Maffucci syndrome associated with a pituitary adenoma and a probable brainstem tumor"],"prefix":"10.3171","volume":"110","author":[{"given":"Joana","family":"Ruivo","sequence":"first","affiliation":[{"name":"Departments of Neuroradiology and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jo\u00e3o Lobo","family":"Antunes","sequence":"additional","affiliation":[{"name":"Neurosurgery, Hospital de Santa Maria, Lisbon, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-1100363","series-title":"Am J Clin Dermatol","doi-asserted-by":"publisher","first-page":"61","DOI":"10.2165\/00128071-200809010-00008","article-title":"Recurrent chondrosarcoma of the right skull base in a patient with Maffucci syndrome","volume":"9","author":"Abdelmalek","year":"2008"},{"key":"b2-1100363","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"479","DOI":"10.3171\/jns.1981.55.3.0479","article-title":"Maffucci's syndrome complicated by intracranial chondrosarcoma and a carotid body tumor","volume":"55","author":"Armstrong","year":"1981"},{"key":"b3-1100363","series-title":"Arch Neurol","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1001\/archneur.1960.03840110066008","article-title":"Dyschondroplasia with hemangioma (Maffucci's syndrome). 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