McCune-Albright-Syndrom in Assoziation mit Meningeom sowie mentaler und psychischer Retardierung

Abstract

Report of a boy aged 17 years and review of the literature on the neoplasias of CAS. The cause of examination was a psychic maldevelopment. Cranial arteriography exhibited extreme wide Carotis communis and CT temporal meningioma right and septum pellucidum cyste. A psychic frontal lobe syndrome and cosmetic impairment by skull changes of the CAS are discussed being partial causes of the psychic maldevelopment. Atypical there were retardation of calcification and dentition, bilateral coarctation of the auditory passage with resulting hearing loss. Till now only J. Frankel and coworkers have published the case of a 50 year old Pakistani with the combination of CAS and Meningioma as a complication of fibrous dysplasia of the skull. We suppose relations between CAS and neurofibromatosis and its blastomatous tendency to meningioma.

Meningioma associated with McCune-Albright syndrome

“…This case and the two reports mentioned above [3,4] suggest that meningiomas may be another feature of MAS. MAS must be differentiated from neurofibromatosis (NF) type 1, a well-known neurocutaneous disorder also presenting with hyperpigmentations, the café-au-lait spots.…”

“…In a review by Ringel et al [8] summarizing the clinical manifestations in 158 individuals the presence of various benign and malignant tumours is described including adenomas of the pituitary gland and in other endocrine organs, myxomas, and osteosarcomas. There is one case report of meningioma in association with MAS [3] and a further case associated with fibrous dysplasia but not the full syndrome [4].…”

Onyx embolization of an intraosseous pseudoaneurysm of the middle meningeal artery in a patient with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome

“…33 There are only 2 case reports of meningiomas arising in association with MAS. 4,8 A report of an orbital and palpebral arteriovenous malformation was described in a patient with MAS; 32 however, vascular anomalies are not associated features of MAS. There are several reports of aneurysmal bone cysts forming in the setting of fibrous dysplasia; 10,21 however, the imaging and angiographic appearance in this case was not that of an aneurysmal bone cyst.…”

Co-existing fibrous dysplasia and meningothelial meningioma

“…Monostotic form involves the skull and facial bones in 10-25% and polyostotic in 50% cases. Fibrous dysplasia can be present as one of the components of McCune-Albright syndrome 1,2,3,7 . Approximately one-third of patients with dysplasia fibrous has involvement of cranial and face bones.…”

“…A search at "PubMed" (U.S. National Library of Medicine) with terms "fibrous", "dysplasia" and "meningioma" demonstrated a total of 7 cases reported. Three cases in association with McCune-Albright syndrome 2,3 , two cases in the Chinese literature 4 , one case with multiple globoid meningiomas with craniomandibular fibrous dysplasia 5 , and one reported case of atypical lymphoplasmacyte-rich meningioma 1 . To the best of our knowledge, this is the first reported case of co-existing fibrous dysplasia and meningothelial meningioma with histopathological diagnosis 1,6,7 .…”