DOI:10.2214/AJR.07.7072
AJR 2008; 191:S37-S39
© American Roentgen Ray Society
AJR Teaching File: Facial Mass in a Child
Annette C. Douglas-Akinwande1,
Chewarat Wirojtananugoon1 and
Eyas M. Hattab2
1 Department of Radiology, Indiana University School of Medicine, 550 N
University Blvd., Rm. 0279, Indianapolis, IN 46202.
2 Department of Pathology and Laboratory Medicine, Indiana University School of
Medicine, Indianapolis, IN.
Received January 22, 2008;
accepted after revision April 21, 2008.
Presented at the 2005 annual meeting of American Roentgen Ray Society, New
Orleans, LA.
Address correspondence to A. C. Douglas-Akinwande
(andougla{at}iupui.edu).
Keywords: hemangioma • juvenile fibromatosis • rhabdomyosarcoma • schwannoma
Clinical History
A 3-year-old girl with a medical history of shunted hydrocephalus,
seizures, and cranial reconstruction for cranial synostosis at 7 months of
age, presents to the emergency department after a fall. Physical examination
reveals a palpable mass. Previous MRI at birth revealed no mass lesions. CT
and MRI were performed.
Radiologic Description
An axial unenhanced CT scan obtained using the soft-tissue window setting
shows a well-circumscribed, isodense mass in the right masticator space
(Fig. 1A). Axial unenhanced CT
scan obtained using the bone window setting shows erosion of the right
mandibular ramus and the right temporal bone
(Fig. 1B). Axial T1-weighted
image shows a mixed-signal (isointense to hyperintense) lesion in the right
masticator space (Fig. 1C).
Coronal T2-weighted image shows a mixed-signal (isointense to hyperintense)
lesion in the right masticator space (Fig.
1D). A coronal contrast-enhanced fat-saturated T1-weighted image
shows homogeneous enhancement and extension of the mass into the right
infratemporal fossa (Fig.
1E).
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Fig. 1A —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall.
Soft-tissue window setting of head CT scan shows well-circumscribed mass that
is isodense to muscle.
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Fig. 1B —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall. Axial
unenhanced CT image obtained with bone window setting shows erosion of right
mandibular ramus and right temporal bone.
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Fig. 1C —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall. Axial
unenhanced T1-weighted image shows hypo- to isointense, well-circumscribed
mass in right masticator space.
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Fig. 1D —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall. Coronal
T2-weighted image shows mixed-signal (isointense to hyperintense) lesion in
right masticator space.
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Fig. 1E —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall. Coronal
contrast-enhanced fat-saturated T1-weighted image shows homogeneous
enhancement and extension of mass into right infratemporal fossa.
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|
Differential Diagnosis
The differential diagnosis for a mass in the masticator space of a child
includes rhabdomyosarcoma, juvenile fibromatosis, infantile hemangioma,
metastatic neuroblastoma, and schwannoma.
Diagnosis
The diagnosis is juvenile fibromatosis.
Histopathology
H and E–stained sections (Figs.
1F and
1G) show a low cellular lesion
characterized by intersecting broad bands of spindle cells in a variably
collagenous stroma. The tumor is composed of bland spindle-shaped cells
(proliferating fibroblasts). Mitotic activity and necrosis are absent.
Abundant collagen (not shown) is present on trichrome preparation. Reticulum
stain shows minimal amount of reticulin. Immunostaining for Ki-67
(MIB-1) marker shows low proliferative activity (< 1%
nuclear staining).
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Fig. 1F —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall. H and
E–stained sections show low cellular lesion characterized by
intersecting broad bands of spindle cells in variably collagenous stroma.
Tumor consists of bland spindle-shaped cells (proliferating fibroblasts).
Mitotic activity and necrosis are absent. Abundant collagen was present on
trichrome preparation. Reticulum stain showed minimal amount of reticulin.
Immuno staining for Ki-67 showed low proliferative activity (< 1% nuclear
staining).
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Fig. 1G —3-year-old girl with medical history of shunted
hydrocephalus, seizures, and cranial reconstruction for cranial synostosis at
7 months of age who presented to emergency department after a fall. H and
E–stained sections show low cellular lesion characterized by
intersecting broad bands of spindle cells in variably collagenous stroma.
Tumor consists of bland spindle-shaped cells (proliferating fibroblasts).
Mitotic activity and necrosis are absent. Abundant collagen was present on
trichrome preparation. Reticulum stain showed minimal amount of reticulin.
Immuno staining for Ki-67 showed low proliferative activity (< 1% nuclear
staining).
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Commentary
Fibromatosis is a rare tumor of unknown cause that may originate from
different soft-tissue structures throughout the entire body. The most commonly
affected sites are muscles of the lower extremities followed by muscles of the
head and neck [1]. Two broad
categories of fibromatosis have been described, adult and juvenile. Each
category contains several subtypes of fibromatosis.
The adult fibromatoses include the Dupuytren-type fibromatoses and desmoid
fibromatoses. The several types of juvenile fibromatoses include congenital
fibrosarcoma-like fibromatosis, congenital generalized fibromatosis,
congenital localized fibromatosis, fibromatosis colli, diffuse infantile
fibromatosis, juvenile aponeurotic fibroma, fibrous hamartoma of infancy,
recurring digital fibrous tumor of childhood, juvenile nasopharyngeal
angiofibroma, hereditary gingival fibromatosis, and juvenile hyaline
fibromatosis [2,
3].
Children are usually affected during the first or second year of life.
Prevalence is slightly higher in boys than in girls
[1]. Fibromatosis generally
presents as a painless swelling that has been observed for several months. On
clinical examination, fibromatosis is often fixed to the underlying muscle or
bone. Rarely, fibromatosis may be fatal when it encroaches on vital structures
such as the trachea, carotid arteries, or base of skull with erosion into the
cranial fossa [4,
5]. On contrast-enhanced CT,
the tumors are characterized by generally high attenuation relative to muscle,
and they have either ill-defined or well-defined margins.
On MRI, fibromatosis has low signal intensity relative to muscle on
T1-weighted imaging and variable signal intensity on T2-weighted. No specific
imaging features distinguish them from other soft-tissue neoplasms such as
fibrosarcoma, rhabdomyosarcoma, or schwannoma, and biopsy is always necessary
[6,
7]. Even their histologic
appearance can be confused by an inexperienced pathologist with other
soft-tissue tumors [1,
2,
4,
5].
Although there are a few reports of spontaneous regression of fibromatoses
[3,
8], most cases should be
evaluated and treated as low-grade malignancy. Therapy typically includes wide
en bloc resections of tumor and surrounding normal tissues. Adjuvant
irradiation and/or chemotherapy have been used
[8].
Rhabdomyosarcoma, the most common sarcoma of the head and neck, arises from
primitive mesenchymal cells that are committed to skeletal cell
differentiation. Rhabdomyosarcoma has an invasive appearance with local bone
destruction. On MRI, rhabdomyosarcoma is isointense on T1-weighted images, is
hyperintense on T2, and enhances heterogeneously. Because of the overlapping
imaging features with other tumors, biopsy is required for diagnosis
[3,
6,
7].
Infantile hemangioma is a common benign endothelial cell neoplasm, 60% of
which occur in the head and neck; most commonly in the parotid or buccal
space. Infantile hemangiomas may be associated with osseous deformity or
hypertrophy, but intraosseous invasion and bone erosion are uncommon
[3].
Schwannoma is a benign nerve sheath tumor derived from schwann cells.
Approximately 40% occur in the head and neck. Schwannoma typically presents as
a painless, expansile, slowly growing mass. On MRI it has intermediate T1- and
variable T2-weighted signal. Enhancement with contrast material is typical on
CT and MRI. Remodeling, expansion, and thinning of the bone may be seen on CT.
Any sign of aggressive bone destruction should raise the possibility of a
malignant neoplasm [3,
9].
Objective
The educational objectives of this teaching article are to review the
various diseases that may be present as a mass in the masticator space in a
child and to discuss the radiologic and pathologic features and management of
juvenile fibromatosis.
Conclusion
The evaluation of a mass in the masticator space in the pediatric
population can be challenging. The differential diagnoses of these lesions may
have overlapping imaging features. This case illustrates the overlapping
features of juvenile fibromatosis and several other disorders, some of which
have a worse prognosis. This case underscores the importance of obtaining
pathologic diagnosis.
References
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Am J Surg 1966;112
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- Quinn SF, Erikson SJ, Dee PM, et al. MR imaging in fibromatosis:
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AJR 1991; 156:539
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Clinical History
Radiologic Description
