Clinical, microscopic and imaging findings associated to Mccune-Albright syndrome: report of two cases

Abstract

McCune-Albright syndrome is characterized by the triad café-au-lait cutaneous spots, polyostotic fibrous dysplasia and endocrinopathies. This article presents two cases of McCune-Albright syndrome in a middle-aged woman and a young girl. Both patients presented café-au-lait spots on the face and other parts of the body and expansion of the mandible with radiopaque-radiolucent areas with ground-glass radiographic appearance, and were diagnosed as having fibrous dysplasia and endocrine disorders. The patient of Case 1 had fibrous dysplasia on the upper and lower limbs, thorax, face and cranium, early puberty, hyperglycemia, hyperthyroidism and high serum alkaline phosphatase levels. The patient of Case 2 presented lesions on the upper limbs and evident endocrine disorders. In both cases presented in this article, the initial exam was made because of the mandibular lesion. However, a diagnosis of fibrous dysplasia must lead to investigation of the involvement of other bones, characterizing polyostotic fibrous dysplasia, which is manifested in a number of diseases. An accurate differential diagnosis is mandatory to determine the best treatment approach for each case.

Uncommon Cone-Beam Computerized Tomography Findings in McCune-Albright Syndrome in an Implant Candidate Patient: A Case Report

“…The analysis of the histopathology view confirmed fibrous dysplasia, consisting of irregular immature trabecular bone tissue in hyper cellular fibrous connective tissue. There was no capsule surrounding the bonny lesion mixing with the normal bone tissue (2). These findings were also present in cases reported by in Xavier et al (2).…”

“…There was no capsule surrounding the bonny lesion mixing with the normal bone tissue (2). These findings were also present in cases reported by in Xavier et al (2). Some data suggest that the cancer incidence in adulthood increases in patients with FD (10).…”

“…The cafe-au-lait pigmentation is the presence of large irregular macules with hypermelanosis, occurring mainly on the anterior and posterior area of the neck, buttocks, thorax, back, shoulder and pelvis and rarely on the face, lips and buccal mucosa (2). It occurs in 10-20% of normal individuals and about 35% of patients with McCune-Albright syndrome (1).…”

Oral manifestations of McCune-Albright syndrome

“…Polyostotic fibrous dysplasia may affect 75% of the skeleton, exhibiting slow, progressive bone growth, that may result in pathological fractures and deformities of multiple bones. [3] Café – au – lait skin pigmentation is commonly seen as large hypermelanotic macules of irregular and serpiginous borders on buttocks, thorax, back, shoulder and posterior area of the neck. [3] The cutaneous and bone lesions tend to respect the midline, occurring on one side of the body.…”

Gigantism Treated by Pure Endoscopic Endonasal Approach in a Case of McCune-Albright Syndrome with Sphenoid Fibrous Dysplasia: A Case Report

“…different hormone secreting (prolactin [PRL]-, growth hormone [GH]-, PRL-GH-, thyroid-stimulating hormone [TSH]-, and adrenocorticotropic hormone [ACTH]) pituitary adenomas , precocious puberty, adrenal primary hyperplasia, hypophosphatemic rickets/osteomalacia, and even ovarian cysts. 2,6,11,[14][15][16] Secreting üituitary adenomas are reported in association with more than 50% of the MAS cases. 14 As well as endocrinologic disorders, fibrous dysplastic bone changes in MAS occur in various forms, such as expansions, cystic lesions, and fibrous thickening that, quite unlike the sclerotic tissues, have an active vascular network in the interior.…”