Fibrous dysplasia of the skull with acromegaly and sarcomatous transformation

Mortensen, Alan; Bojsen‐Møller, Marie; Rasmussen, Peter Vestergaard

doi:10.1007/bf00149375

Cited by 36 publications

(14 citation statements)

References 13 publications

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“…Pituitary surgery in this syndrome is beset with problems mainly related to fibrous dysplasia and the resultant thickening of bones [3,4,19,28]. Occasional instances of sarcomatous transformation of fibrous dysplastic bones have been reported with radiation therapy [18,23]. Bromocryptine, cabergoline and long acting somatostatin analogue have been used with some success in acromegaly with MAS [3,4,28,31,33].…”

Section: Discussionmentioning

confidence: 99%

Acromegaly with Fibrous Dysplasia: McCune-Albright Syndrome-Clinical Studies in 3 Cases and Brief Review of Literature-

et al. 2003

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Abstract. The McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies including growth hormone (GH) excess. Polyostotic bone lesions and café-au-lait macules are common while monostotic bone lesions are rare. Similarly, acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon and in most of the instances somatotropinoma has not been documented. We report 3 patients, two of them had monostotic lesion, none had café-au-lait macules and all had GH secreting pituitary macroadenoma. All of them underwent transfrontal pituitary adenomectomy and had histopathological confirmation of GH secreting pituitary adenoma. A brief review of literature is also presented.

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Section: Discussionmentioning

confidence: 99%

Acromegaly with Fibrous Dysplasia: McCune-Albright Syndrome-Clinical Studies in 3 Cases and Brief Review of Literature-

et al. 2003

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“…Radiation therapy and chemotherapy have no role in the treatment of this disease and radiation therapy may increase the risk of malignant transformation [21, 40, 41, 42]. The treatment of fibrous dysplasia of the skull is, therefore, entirely surgical.…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

et al. 2002

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Introduction: We evaluate the role of surgery in the treatment of fibrous dysplasia of the skull in children. Methods: We identified 48 consecutive cases of fibrous dysplasia of the skull that were surgically treated at a single institution over a 23-year interval. The 28 patients that initially presented during the first two decades of life were selected for further analysis. Presenting symptoms, signs, surgical treatment, surgical outcome and the state of the disease at the extended follow-up interval were recorded. Results: Fibrous dysplasia of the skull in children most often involved the frontal, sphenoid and ethmoid bones. Most patients presented with facial asymmetry or proptosis. A gross total resection was achieved in 7 patients, subtotal resection in 17 patients, and 4 patients underwent biopsy alone. Over the follow-up interval, 7 patients had symptomatic progression of subtotally resected fibrous dysplasia and 3 patients had asymptomatic progression. The extent of resection was correlated with recurrence risk. Conclusions: In order to prevent progression of disease, an attempt at gross total resection is indicated in cases of fibrous dysplasia of the skull in childhood where the risk of neurologic morbidity is low and cosmetic results will be acceptable.

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“…3 Although an incidence (25%) of recurrence associated with partial removal of the lesion has been reported, 59 radiotherapy has not been recommended because of the risk of malignant transformation. 89 Even without radiotherapy, however, there have been reports of spontaneous malignant transformation.…”

Section: Fibrous Dysplasiamentioning

confidence: 99%

Tumors of the skull base in children: review of tumor types and management strategies

Tsai¹,

Santoreneos²,

Rutka³

2002

FOC

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Although many treatment strategies for skull base tumors in adults have been reported, relatively little has been reported regarding such therapies in the pediatric population. Skull base tumors in children present a therapeutic challenge because of their unique pathological composition, the constraints of the maturing skull and brain, and the small size of the patients. In this review, the authors examine the pediatric skull base lesions that occur in the anterior, middle, and posterior cranial base, focusing on unique pediatric tumors such as encepahalocele, fibrous dysplasia, esthesioneuroblastoma, craniopharyngioma, juvenile nasopharyngeal angiofibroma, cholesteatoma, chordoma, chondrosarcoma, and Ewing sarcoma. They review management strategies that include radio- and chemotherapy, as well as surgical approaches with emphasis on the modifications and complications associated with the procedures as they apply in children. Evidence for the advantages and limitations of radiotherapy, chemotherapy, and surgery as it pertains to the pediatric population will be examined. With a working knowledge of skull base anatomy and special considerations of the developing craniofacial skeleton, neurosurgeons can treat skull base lesions in children with acceptable morbidity and mortality rates. Outcomes in this population may be better than those in adults, in part because of the benign histopathology that frequently affects the pediatric skull base, as well as the plasticity of the maturing nervous system.

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Fibrous dysplasia of the skull with acromegaly and sarcomatous transformation

Cited by 36 publications

References 13 publications

Acromegaly with Fibrous Dysplasia: McCune-Albright Syndrome-Clinical Studies in 3 Cases and Brief Review of Literature-

Acromegaly with Fibrous Dysplasia: McCune-Albright Syndrome-Clinical Studies in 3 Cases and Brief Review of Literature-

Surgical Treatment of Fibrous Dysplasia of the Skull in Children

Tumors of the skull base in children: review of tumor types and management strategies

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