A Ahmed

A Al Kaissi

A McLaughlin

CE Dumitrescu

Dan Wang

DJ McCune

E Diah

F Albright

JS Lee

M Riminucci

MC Papadopoulos

N Bulakbaşı

Ping Li

S Lumbroso

Xian-Feng Li

Xu-Dong Xia

Ying Jiang

Zai-Ren Zhang

English

PubMed

Crossref

MR and CT findings of cyst degeneration of sphenoid bone in McCune-Albright syndrome: a case report

Springer - Publisher Connector

BioMed CentralCases Journal
ssOpen AcceCase Report
MR and CT findings of cyst degeneration of sphenoid bone in 
McCune-Albright syndrome: a case report
Ping Li*1, Zai-Ren Zhang1, Ying Jiang2, Xu-Dong Xia1, Dan Wang1 and Xian-
Feng Li3
Address: 1Department of Radiology, the Second Affiliated Hospital, Harbin Medical University, 246 Xue Fu Road, Harbin, Heilongjiang, 150086, 
China, 2Department of Pathology, the Second Affiliated Hospital, Harbin Medical University, 246 Xue Fu Road, Harbin, Heilongjiang, 150086, 
China and 3Department of Neurosurgery, the Second Affiliated Hospital, Harbin Medical University, 246 Xue Fu Road, Harbin, Heilongjiang, 
150086, China
Email: Ping Li* - pinglee_2000@yahoo.com; Zai-Ren Zhang - zrzhang1603@sina.com.cn; Ying Jiang - jying1972@163.com; Xu-
Dong Xia - xiaxd888@163.com; Dan Wang - joshuastone@163.com; Xian-Feng Li - wzhit@163.com
* Corresponding author    
Abstract
Introduction: McCune-Albright syndrome (MAS) is a rare disorder characterized by the classic
triad of precocious puberty, polyostotic fibrous dysplasia and café-au-lait pigmented skin lesions.
Cystic change is rare in fibrous dysplasia (FD), especially in McCune-Albright syndrome. There
were no reports about cyst degeneration in MAS which resulted in abnormal visual acuity and visual
fields. Herein, we report a female patient with MAS associated with sphenoid bone cysts which
resulted in visual deterioration to describe the computed tomography (CT) and magnetic
resonance (MR) imaging findings of cyst degeneration in McCune-Albright syndrome.
Case presentation: A 20-year-old female presented with right temporal hemianopsia and visual
loss in the right eye suddenly. A café-au-lait spot was found on her neck and left shoulder.
Endocrinologic examination revealed elevated basal level of serum PRL, FT3 and FT4 with decreased
serum TSH. Fibrous dysplasia (FD) generally manifest as round-glass appearance with well defined
borders and cystic areas within involved bone were seen as hypointensity on CT. They were
showed as hypointense in T1-weighted sequences and as hyperintense in T2-weighted sequences
of MRI. After surgery the right temporal hemianopsia improved.
Conclusion: CT combined with MRI is the most effective method to evaluate the extent and
complications of fibrous dysplasia in patients with MAS. The treatment of surgery can not cure MAS
but relieve the symptom.
Introduction
Fibrous dysplasia (FD) is primarily a developmental
abnormality of the bone-forming mesenchyme in which
fibrous tissue gradually expands and replaces the bone. It
is a common benign bone disease existing in monostotic
and polyostotic forms. Polyostotic fibrous dysplasia is a
component of McCune-Albright syndromes (MAS). MAS
is a rare disorder characterized by the classic triad of pre-
cocious puberty, polyostotic fibrous dysplasia and café-
au-lait pigmented skin lesions. Additional endocrine
Published: 22 December 2009
Cases Journal 2009, 2:9376 doi:10.1186/1757-1626-2-9376
Received: 23 September 2009
Accepted: 22 December 2009
This article is available from: http://www.casesjournal.com/content/2/1/9376
© 2009 Li et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:9376 http://www.casesjournal.com/content/2/1/9376abnormalities may also be present, including hyperthy-
roidism, growth hormone excess and hyperprolactinemia.
Cystic change is rare in fibrous dysplasia, especially in
MAS [1]. There were no reports about cyst degeneration of
FD in MAS which resulted in abnormal visual acuity and
visual fields. Herein, we report a female patient with MAS
associated with sphenoid bone cysts which resulted in vis-
ual deterioration.
Case presentation
A 20-year-old female presented with right temporal hemi-
anopsia and visual loss in the right eye. She presented
café-au-lait spots first noted at 2 months old. Her vision
deteriorated suddenly when she was in hospital for ankle
joint sprain 10 days ago. On admission, she had no
acromegalic features, was 165 cm tall, weighing 66 kg. The
blood pressure was 100/80 mmHg. The right side of his
face was slightly deformed and proptosis of her right eyes
was obvious. A café-au-lait spot was found on her neck
and left shoulder (Fig. 1). The skin lesions were enlarged
gradually during past 20 years. Endocrinologic examina-
tion revealed elevated basal level of serum PRL (687.29
nIU/ml, normal < 513 nIU/ml), FT3 (9.76 pmol/L, normal
< 5.7 pmol/L) and FT4 (27.69 pmol/L, normal < 19.5
pmol/L) with decreased serum TSH (0.0011 μIU/ml, nor-
mal 0.35 μIU/ml ~4.94 μIU/ml). Other endocrine func-
tions were normal.
CT and MRI scans of the head showed extensive abnormal
thickening of the skull involving the right frontal, occipi-
tal, sphenoid and maxillary bones. Asymmetrical widen-
ing of facial bones resulted in lion face (leontiasis ossea)
that is best showed with three-dimensional CT images
(Fig. 2). Fibrous dysplasia caused narrowing of the optic
canal and compression of the right optic nerve. Admission
CT and MR scans revealed a cystic mass of sella turcica
containing a fluid-fluid level (Fig. 3, 4, 5, 6). There was
associated narrowing of the right optic canal and com-
pression of the optic chiasma. On sagittal images of MR,
normal pituitary gland can be found (Fig. 7, 8). There
were several other cysts manifested as hypertensity on T2
weighted images in frontal bone (Fig. 9). Her visual acuity
and visual fields were normal 2 months ago. There were
no cyst changes in sphenoid bone at that time (Fig. 10).
In view of the clinical presentation, cystic pituitary ade-
noma complicated with hemorrhage was suspected at
first, but radiological appearances of the lesion showed
that cyst degeneration of fibrous dysplasia can not be
excluded because the normal pituitary gland can be seen.
Surgery was performed by subfrontal approach. Right
sphenoid bone was removed partially and the fluid in the
cyst was drained. The fluid was lucid and there was no sign
A café-au-lait spot was found on her neck and left shoulderFigure 1
A café-au-lait spot was found on her neck and left 
shoulder.
Reconstructed three-dimensional CT image shows lion face appearance due to asymmetrical involvement and enlarge-ment of the bones of the face and the skullFigure 2
Reconstructed three-dimensional CT image shows 
lion face appearance due to asymmetrical involve-
ment and enlargement of the bones of the face and 
the skull.Page 2 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:9376 http://www.casesjournal.com/content/2/1/9376of hemorrhage. The walls of the cyst did not collapse. Tis-
sues obtained at surgery was immediately fixed in 10%
formalin and embedded in paraffin. Histological exami-
nation of the biopsied bone confirmed the presence of
fibrous dysplasia. The histology of fibrous dysplasia
reveals fibroblasts, collagen, and scattered islands of carti-
lage, occasional giant cells and woven bone with excess
blood supply (Fig. 11). After surgery the right temporal
hemianopsia improved.
Discussion
FD is a developmental disease of bone in which there is
replacement of normal spongiosa and filling of the med-
ullary cavity of affected bones by an abnormal fibrous tis-
sue that contains trabeculae of poorly calcified primitive
bone formed by osseous metaplasia. It is believed to be a
benign lesion of bone of unknown origin. Fibrous dyspla-
sia might be monostotic or polyostotic, involve a large
area of the skull or involve single bones. The most severe
form of FD is McCune-Albright syndrome, which is more
commonly found in females[2].
The McCune-Albright Syndrome is a sporadic rare disor-
der first described in 1936 by McCune and separately by
Albright [3,4]. It is a rare disease with estimated preva-
lence between 1/100,000 and 1/1,000,000. McCune-
Albright syndrome (MAS) is characterized by the associa-
tion of precocious puberty (mostly in girls), polyostotic
fibrous dysplasia and café-au-lait pigmented skin lesions.
In addition to this classical triad, several endocrine disor-
ders, all due to autonomous hormonal hyperproduction,
can also be associated, such as pituitary adenomas secret-
ing growth hormone, hyperthyroid goiters, or adrenal
hyperplasia. The most commonly encountered endocrine
dysfunction is gonadal hyperfunction. MAS is caused by a
postzygotic activating mutation of the Gsalpha gene,
which impair the guanosine triphosphatase (GTPase)
activity of Gsalpha thus resulting in excess intracellular
cAMP and leading to a mosaic distribution of cells bearing
constitutively active adenyl cyclase activity[5].
Craniofacial structures are affected in 25%~30% of
patients with fibrous dysplasia, most commonly involv-
ing the frontal bone, followed by the sphenoid and eth-
moid bones. Facial bone involvement may cause visual
disturbances, proptosis, nasalobstruction, sinusitis, facial
asymmetry or hearing abnormalities. When the sphenoid
ridge is involved, the commonest presenting sign is prop-
tosis. Lee et al. concluded that encasement of the optic
canal in fibrous dysplasia causes narrowing of the canal
but this in itself does not result in visual loss [2,6]. Acute
Axial CT image FD presents as "ground-glass" appearance on CT and there is a cystic and solid mass of sella turcicFigure 3
Axial CT image FD presents as "ground-glass" 
appearance on CT and there is a cystic and solid 
mass of sella turcica.
Axial T1-weighted MR images show abnormal thickening of the bone involving the frontal bone, sphenoid bone, lateral and medial orbital walls, wi h right c nal is shall w and optic nerve is compressedFigur  4
Axial T1-weighted MR images show abnormal thick-
ening of the bone involving the frontal bone, sphe-
noid bone, lateral and medial orbital walls, with right 
canal is shallow and optic nerve is compressed.Page 3 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:9376 http://www.casesjournal.com/content/2/1/9376visual loss due to fibrous dysplasia is uncommon and in
such chroinic cases optic nerve decompression does not
lead to visual impairment. Cyst degeneration occurred
most commonly in the sphenoid and frontal bones. Pres-
sure on the optic nerves by a cyst is one of the causes of
acute optic nerve compression in fibrous dysplasia [7].
The cysts tend to have a more aggressive course. It may be
that the development of cyst degeneration does not
depend on a single pathogenetic event, but that there are
several potential mechanisms. Some cysts are thought to
develop from disruption of venous diploic channels. It
has also been proposed that bone cysts arise from an
intraosseous vascular defect that results in intramedullary
hemorrhage. The cysts can expand rapidly and produce
symptoms which vary, depending on the location [8,9].
The development of cyst degeneration may cause rapid
expansion which resulted in sudden visual deterioration.
This 20-year-old female presented with right temporal
hemianopsia and visual loss in the right eye quickly after
the cyst developed.
Diagnosis is usually easy in polyostotic FD and MAS
according to the imaging [10]. There were three patterns
of the radiographic features of fibrous dysplasia of the
skull and facial bones. The pagetoid or ground-glass pat-
tern is most common and consists of a mixture of dense
and radiolucent areas of fibrosis. This is due to the rela-
tively greater degree of mineralization of FD tissue in the
craniofacial bones [11]. Sclerotic lesions are homogene-
ously dense, whereas cyst degeneration is the least com-
mon finding is characterized by a spherical or ovoid
lucency surrounded by a dense bony shell. Histologic cor-
relates have shown cystic lesions result from an abun-
dance of fibrous elements [8].
Computed tomography (CT) scanning is the "gold-stand-
ard" imaging technique for FD, allowing characterization
of the three main imaging patterns of involved bone. On
CT, FD generally presents as "ground-glass" appearance
with well defined borders, medullary widening and corti-
cal thinning. Cyst degeneration has occurred in about 5%
of the patients with FD in the NIH cohort (unpublished
data) [8]. Cystic areas within involved bone were seen as
hypointensity on CT. CT should be repeated periodically,
annually before stability has been demonstrated. MR
imaging are useful adjuncts. Particularly in cases of cystic
fibrous dysplasia, MR imaging may be useful to assess the
soft tissue and fibrous components and to evaluate the
effect of these primarily bony lesions on adjacent soft tis-
sue structures of the skull base, especially sellar turcica.
MRI can provide the exact delimitation of the lesions.
Normal pituitary gland can be found on sagittal MR
images in this case, so cystic pituitary adenoma can be
excluded. FD manifest as intermediate or low signal inten-
Axial T2-weighted MR images show abnormal thickening of the bone involving the frontal bone, sphenoid bone, lateral and medial orbital walls, wi h right c nal is shall w and optic nerve is compressedFigur  5
Axial T2-weighted MR images show abnormal thick-
ening of the bone involving the frontal bone, sphe-
noid bone, lateral and medial orbital walls, with right 
canal is shallow and optic nerve is compressed.
Fluid-fluid level can be showed clearly on FLAIR T1-weighted images (arrow)igure 6
Fluid-fluid level can be showed clearly on FLAIR T1-
weighted images (arrow).Page 4 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:9376 http://www.casesjournal.com/content/2/1/9376sity in T1-weighted MRI sequences, and intermediate or
high signal intensity in T2-weighted MRI sequences. If
fibrous tissue predominates, both T1- and T2-weighted
signal intensity may be low [12]. Cystic fibrous dysplasia
was showed as hypointense in T1-weighted images and as
hyperintense in T2-weighted images of MRI. There was no
heterogeneous contrast enhancement. A useful role for
routine MR imaging of the sella turcica in all cases of clin-
ically suspected McCune-Albright syndrome is suggested.
While bone marrow involvement was shown more clearly
with MRI, compression of cranial and spinal nerves was
determined most effectively by evaluation of CT and MRI
together. CT and MRI should be employed together in
order to demonstrate the extent of disease, and complica-
tions of craniospinal involvement of FD in patients with
MAS.
The treatment of MAS is complex and can vary from obser-
vation in asymptomatic cases to medical treatment or
extensive surgery. The goal of the surgical treatment of
MAS of the skull is to remove neural decompression of FD
or mass. Total excision of the abnormal bone is not possi-
ble owing to its extent or involvement of the skull base,
and treatment is limited to symptomatic relief.
T2-weighted MR image show cysts of frontal bone manifest as hypertensity w th well-definedFigur  9
T2-weighted MR image show cysts of frontal bone 
manifest as hypertensity with well-defined. These 
lesions are more frequent in the craniofacial bones.
T1-weighted coronal MRI scans of the headFigure 8
T1-weighted coronal MRI scans of the head. Normal 
pituitary gland can be found (arrow). The fluid-filled nature of 
the lesion is demonstrated by the fluid-fluid level.
T1-weighted sagittal MRI scans of the headFigure 7
T1-weighted sagittal MRI scans of the head. Optic chi-
asma is compressed.Page 5 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:9376 http://www.casesjournal.com/content/2/1/9376Conclusions
In conclusion, FD of skull generally manifest as round-
glass appearance with well defined borders and cystic
areas within involved bone were seen as hypointensity on
CT. They were showed as hypointense in T1-weighted
sequences and as hyperintense in T2-weighted sequences
of MRI. CT combined with MRI is the most important
method to evaluate the extent and complications of
fibrous dysplasia in patients with MAS. The treatment of
surgery can not cure MAS but relieve the symptom.
Abbreviations
CT: Computed tomography; MR: magnetic resonance;
MAS: McCune-Albright syndrome; FD: Fibrous dysplasia.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
All of the authors were involved in the clinical and radio-
graphic assessment and writing the manuscript. All
authors have read and approved the final manuscript.
References
1. Ahmed A, Sgouros S, Scotcher S: Cystic fibrous dysplasia of the
skull with intracystic haemorrhage.  Br J Neurosurg 2004,
18:401-402.
2. Al Kaissi A, Klaushofer K, Grill F: Outward bulging of the right
parietal bone in connection with fibrous dysplasia in an
infant: a case report.  Cases J 2008, 1:347.
3. McCune DJ: Osteitis fibrosa cystica; the case of a nine year old
girl who also exhibits precocious puberty, multiple pigmen-
tation of the skin and hyperthyroidism.  Am J Dis Child 1936,
52:743-744.
4. Albright F, Butler AM, Hampton AO, Smith P: Syndrome charac-
terized by osteitis fibrosa disseminata, areas of pigmentation
and endocrine dysfunction, with precocious puberty in
females: report of five cases.  N Engl J Med 1937, 216:727-746.
5. Lumbroso S, Paris F, Sultan C: Activating Gsα mutations: analy-
sis of 113 patients with signs of McCune-Albright syndrome-
a European collaborative study.  J Clin Endocrinol Metab 2004,
89:2107-2113.
6. Lee JS, FitzGibbon E, Butman JA, Dufresne CR, Kushner H, Wien-
troub S: Normal vision despite narrowing of the optic canal in
fibrous dysplasia.  N Engl J Med 2002, 347:1670-1676.
7. Papadopoulos MC, Casey AT, Powell M: Craniofacial fibrous dys-
plasia complicated by acute, reversible visual loss: report of
two cases.  Br J Neurosurg 1998, 12:159-161.
8. Diah E, Morris DE, Lo LJ, Chen YR: Cyst degeneration in cranio-
facial fibrous dysplasia: clinical presentation and manage-
ment.  J Neurosurg 2007, 107:504-508.
9. Dumitrescu CE, Collins MT: McCune-Albright syndrome.
Orphanet J Rare Dis 2008, 3:12.
10. Riminucci M, Liu B, Corsi A, Shenker A, Spiegel AM, Robey PG: The
histopathology of fibrous dysplasia of bone in patients with
activating mutations of the Gs alpha gene: site-specific pat-
terns and recurrent histological hallmarks.  J Pathol 1999,
187:249-258.
11. Bulakbas¸ı N, Bozlar U, Karademir I, Kocaoğlu M, Somuncu I: CT and
MRI in the evaluation of craniospinal involvement with poly-
ostotic fibrous dysplasia in McCune-Albright syndrome.
Diagn Interv Radiol 2008, 14:177-181.
12. McLaughlin A, Stalley P, Magee M, Soper J, Wall H Van der: Correl-
ative imaging in an atypical case of Mazabraud syndrome.
AJR Am J Roentgenol 2007, 189:W353-W356.
The histology of fibrous dysplasia reveals fibroblasts, colla-gen, and scattered islands of c rtilage, occasi nal giant cells and woven bone w th exce s blood supplyFigure 11
The histology of fibrous dysplasia reveals fibroblasts, 
collagen, and scattered islands of cartilage, occa-
sional giant cells and woven bone with excess blood 
supply. HE ×400.
Axial CT imagesFigure 10
Axial CT images. There were no cyst changes in sphenoid 
bone 2 months ago. The density of sellar turcica is uniform.Page 6 of 6
(page number not for citation purposes)


Activating Gsα mutations: analysis of 113 patients with signs of McCune-Albright syndromea European collaborative study.

Collins MT: McCune-Albright syndrome.

Craniofacial fibrous dysplasia complicated by acute, reversible visual loss: report of two cases.

Cystic fibrous dysplasia of the skull with intracystic haemorrhage.

F: Outward bulging of the right parietal bone in connection with fibrous dysplasia in an infant: a case report. Cases J

Osteitis fibrosa cystica; the case of a nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism.

PG: The histopathology of fibrous dysplasia of bone in patients with activating mutations of the Gs alpha gene: site-specific patterns and recurrent histological hallmarks.

Somuncu I: CT and MRI in the evaluation of craniospinal involvement with polyostotic fibrous dysplasia in McCune-Albright syndrome. Diagn Interv Radiol

Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases.

Wientroub S: Normal vision despite narrowing of the optic canal in fibrous dysplasia.

YR: Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and management.

file:///data/core-remote/dit/data/Springer-OA/pdf/a03/aHR0cDovL2xpbmsuc3ByaW5nZXIuY29tLzEwLjExODYvMTc1Ny0xNjI2LTItOTM3Ni5wZGY=.pdf

MR and CT findings of cyst degeneration of sphenoid bone in McCune-Albright syndrome: a case report

Abstract

Similar works

Full text

Available Versions

Crossref

Springer - Publisher Connector

Springer - Publisher Connector