Cases
and
cafe-au-lait
nance
(MR)
imaging
spots. of her
to the hospain in her cutaneous
Magnetic cervical
U
Computed
tomography
performed
(CT)
to search
tho-
le-
FINDINGS
CT scans
of the abdomen
trast material retroperitoneal values
ranging
from
Index
terms:
Abdomen,
neoplasms,
I
From
1992;
the
May
Jewish
General
C RSNA,
18 and
30
to 35 87. 18
initially
discovered
in
numerous
surgical
procedures,
con-
exhibited
a myxoid
#{149} Retroperitoneai
space,
gelatinous
appearance.
multiple attenua-
HU
(Fig
ib).
#{149} Neurofibromatosis,
87. 18
87.18
12:1033-1034
Department
quested
with
revealed la) with
(Fig
tion
RadloGraphics
September
obtained
enhancement masses
was
including a mastectomy for removal of a large plexiform neurofibroma of the right breast. At surgery performed at this admission, large nodular masses were excised from the right paraspinal region and from the second, third, and fourth intercostal spaces. The cut section
sions.
U
DISCUSSION
undergone
of the abdomen
for additional
neurofibromatosis.
our patient at 13 years of age. She has marked thoracic scoliosis, numerous cutaneous neurofibromas, and cafe-au-lait spots. She has
resoand
Plexiform
Neurofibromatosis
racic spine revealed large right-sided paravertebral and posterior mediastinal masses without evidence of intraspinal extension. was
Day
DIAGNOSIS:
U HISTORY A 28-year-old woman was admitted pital for evaluation of intractable right shoulder. She had numerous masses
ofthe
of Radiology, receivedjune
Hospital,
3755
McGill
University,
16; acceptedJune Cote
St Catherine
Montreal.
From
17. Address Rd.
Montreal.
the
reprint Que,
Canada
1991 requests H3T
RSNA
scientific
to M.J.P.,
assembly. Department
Received ofRadiology,
May
4, 1992; Sir Mortimer
revision
re
B. Davis-
1E2.
1992
1992
Galley
et a!
U
RadioGrapbics
U
1033
Histologic analysis revealed a loose pattern of interlacing bands of delicate spindle cells, with occasional giant cells and nerve fibers seen scattered throughout the tumor (Fig 2).
The pathologic
diagnosis
was plexiform
neu-
rofibromatosis.
‘.
.
V
,
.e’.’
Recklinghausen
disease
congenital cell layers
be manifested
in any organ
is an autoso-
dysplasia and can
5,’
“_.-, ..
,_
#{149}
\.
: .
;‘
.,.
V
.
:-,-
.
‘;.#{149}
Vft/
;
0
#{149}
V
Whether
,#b.:V!::. -,
.
.
.,
,.
.
#{149}#{149}V:
#‘
..
2.
Figure
..,
.
Photomicrograph
V.: .
V
..‘
(hematoxylin-eosin
stain) shows irregularly shaped spindle cells scattered within loose connective tissue, consistent with neurofibroma.
diagnosis
for plexiform
matosis.
neurofibromatosis
includes
lymphoma,
Typical sites of plexiform neurofibromas include the neck, pelvis, and extremities (1,2). Thoracic involvement, most commonly of the sympathetic nerve chains, has been described (1). Abdominal plexiform neurofibromas typically arise in the retroperitoneum and paraspinal regions (3). Nonenhanced CT scans typically reveal lowattenuation masses with ill-defined margins; attenuation values range from 15 to 20 HU (1), to averages of3O HU (3). Several factors
static
and
have
the low atten-
U
the high water the lipid na-
1.
Bourguoin
2.
McLoud TC. Plexiform neurofibromatosis of the mediastinum: CT appearance. AJR 1988; 151:461-463. Paling MR. Plexiform neurofibroma of the pelvis in neurofibromatosis: CT findings. J Comput Assist Tomogr 1984; 8:476-478. Mirich DR, Gray BR, Grosman H. Abdominal
uation content
ture
been
proposed
after
of contrast
have
tional
cells,
and incorporation tissue into the
enhancement tion
to explain
of the lesions, including of the mucinous matrix,
of Schwann
ation pose
been
and
the cystic
intravenous
findings
intensity
is seen
on
T2-weighted
adenopathy,
cafe-au-lait
history;
peared
similar
on CT scans
nodules,
thus,
to pseudomyxoma
4.
peritonei
(3).
PM, ShepardJO,
Moore
neurofibromatosis
pseudomyxoma
J Comput
709-711. KumarAJ,
Kahujda
on
Assist FP,
computed
Tomogr
Tomogr
1983;
et a!
13:
CR, Fishman
Martinez
DV, Seigelman 55. Computed mography of extracranial nerve sheath with pathologic correlation. J Comput 5.
to.
1989;
Jezic
to-
tumors Assist
7:857-865.
HarkinJC, Reed RJ. Tumors ofthe peripheral nervous system. In: Atlas of tumor pathology, ser 2, fasc 3. Washington, DC: Armed Forces Institute of Pathology, 1969; 67-96. Aughenbough GL. Thoracic manifestations neurocutaneous diseases. Radiol Clin North Am 1984; 22:741-756. Burk DL, BrunbergJA, Kanal E, Latchaw RE, Wolf GL. Spinal and paraspinal neurofibro-
matosis: surface-coil MR imaging ology 1987; 162:797-801.
Galley
EH,
simulating
peritonei
mography.
EK,
(7).
7.
U
and
adenopathy
REFERENCES
plexiform
6.
RadioGraphics
of
secondary to lymphoma or metastatic disease can be excluded. Mirich et al (3) reported a case of plexiform neurofibromatosis that ap-
3.
Occasional lesions show central areas of decreased signal intensity on T2-weighted images, a finding that corresponds to dense collagenous tissue. This may prove to be a useful feature in distinguishing neurofibromas from other soft-tissue tumors (7).
perito-
identification locations,
subcutaneous
family
meta-
pseudomyxoma
is aided by the in characteristic spots,
a positive
(1,4,6).
images
differential
nei. Diagnosis neurofibromas
administra-
and calcifications but these are excep-
nonspecific
The
degener-
of surrounding adimasses (4,5). Peripheral
material
observed,
and
Ti-weighted spin-echo MR images reveal masses with a signal intensity slightly greater than that of muscle. Markedly increased signal
U
#{149} #{149}
.
.
.
..‘
._.ni,.
the neural tumors are neoplastic or hamartomatous in nature is controversial. Histologically, the tumors consist of a proliferation of spindle cells from either the neuroectodermal Schwann cell sheath or mesodermal fibrous connective tissue. When involvement is diffuse, producing fusiform tumorlike masses along the course of a nerve and its branches, the condition is called plexiform neurofibro-
1034
:
. ‘
#{149}
#{149}
#{149} #{149}
V.,., .
#{149}
)_...#{149}
V#{149}’
affecting potentially
system.
‘S
b
,
.
.
V
....
‘‘ #{149}#{149}‘
V
V..
-
Von
V
#{149},
-
:,
:
-.
‘
,
mal dominant all three germ
.
...
of
at 1.5 T. Radi-
Volume
12
Number
5
and
cafe-au-lait
nance
(MR)
imaging
spots. of her
to the hospain in her cutaneous
Magnetic cervical
U
Computed
tomography
performed
(CT)
to search
tho-
le-
FINDINGS
CT scans
of the abdomen
trast material retroperitoneal values
ranging
from
Index
terms:
Abdomen,
neoplasms,
I
From
1992;
the
May
Jewish
General
C RSNA,
18 and
30
to 35 87. 18
initially
discovered
in
numerous
surgical
procedures,
con-
exhibited
a myxoid
#{149} Retroperitoneai
space,
gelatinous
appearance.
multiple attenua-
HU
(Fig
ib).
#{149} Neurofibromatosis,
87. 18
87.18
12:1033-1034
Department
quested
with
revealed la) with
(Fig
tion
RadloGraphics
September
obtained
enhancement masses
was
including a mastectomy for removal of a large plexiform neurofibroma of the right breast. At surgery performed at this admission, large nodular masses were excised from the right paraspinal region and from the second, third, and fourth intercostal spaces. The cut section
sions.
U
DISCUSSION
undergone
of the abdomen
for additional
neurofibromatosis.
our patient at 13 years of age. She has marked thoracic scoliosis, numerous cutaneous neurofibromas, and cafe-au-lait spots. She has
resoand
Plexiform
Neurofibromatosis
racic spine revealed large right-sided paravertebral and posterior mediastinal masses without evidence of intraspinal extension. was
Day
DIAGNOSIS:
U HISTORY A 28-year-old woman was admitted pital for evaluation of intractable right shoulder. She had numerous masses
ofthe
of Radiology, receivedjune
Hospital,
3755
McGill
University,
16; acceptedJune Cote
St Catherine
Montreal.
From
17. Address Rd.
Montreal.
the
reprint Que,
Canada
1991 requests H3T
RSNA
scientific
to M.J.P.,
assembly. Department
Received ofRadiology,
May
4, 1992; Sir Mortimer
revision
re
B. Davis-
1E2.
1992
1992
Galley
et a!
U
RadioGrapbics
U
1033
Histologic analysis revealed a loose pattern of interlacing bands of delicate spindle cells, with occasional giant cells and nerve fibers seen scattered throughout the tumor (Fig 2).
The pathologic
diagnosis
was plexiform
neu-
rofibromatosis.
‘.
.
V
,
.e’.’
Recklinghausen
disease
congenital cell layers
be manifested
in any organ
is an autoso-
dysplasia and can
5,’
“_.-, ..
,_
#{149}
\.
: .
;‘
.,.
V
.
:-,-
.
‘;.#{149}
Vft/
;
0
#{149}
V
Whether
,#b.:V!::. -,
.
.
.,
,.
.
#{149}#{149}V:
#‘
..
2.
Figure
..,
.
Photomicrograph
V.: .
V
..‘
(hematoxylin-eosin
stain) shows irregularly shaped spindle cells scattered within loose connective tissue, consistent with neurofibroma.
diagnosis
for plexiform
matosis.
neurofibromatosis
includes
lymphoma,
Typical sites of plexiform neurofibromas include the neck, pelvis, and extremities (1,2). Thoracic involvement, most commonly of the sympathetic nerve chains, has been described (1). Abdominal plexiform neurofibromas typically arise in the retroperitoneum and paraspinal regions (3). Nonenhanced CT scans typically reveal lowattenuation masses with ill-defined margins; attenuation values range from 15 to 20 HU (1), to averages of3O HU (3). Several factors
static
and
have
the low atten-
U
the high water the lipid na-
1.
Bourguoin
2.
McLoud TC. Plexiform neurofibromatosis of the mediastinum: CT appearance. AJR 1988; 151:461-463. Paling MR. Plexiform neurofibroma of the pelvis in neurofibromatosis: CT findings. J Comput Assist Tomogr 1984; 8:476-478. Mirich DR, Gray BR, Grosman H. Abdominal
uation content
ture
been
proposed
after
of contrast
have
tional
cells,
and incorporation tissue into the
enhancement tion
to explain
of the lesions, including of the mucinous matrix,
of Schwann
ation pose
been
and
the cystic
intravenous
findings
intensity
is seen
on
T2-weighted
adenopathy,
cafe-au-lait
history;
peared
similar
on CT scans
nodules,
thus,
to pseudomyxoma
4.
peritonei
(3).
PM, ShepardJO,
Moore
neurofibromatosis
pseudomyxoma
J Comput
709-711. KumarAJ,
Kahujda
on
Assist FP,
computed
Tomogr
Tomogr
1983;
et a!
13:
CR, Fishman
Martinez
DV, Seigelman 55. Computed mography of extracranial nerve sheath with pathologic correlation. J Comput 5.
to.
1989;
Jezic
to-
tumors Assist
7:857-865.
HarkinJC, Reed RJ. Tumors ofthe peripheral nervous system. In: Atlas of tumor pathology, ser 2, fasc 3. Washington, DC: Armed Forces Institute of Pathology, 1969; 67-96. Aughenbough GL. Thoracic manifestations neurocutaneous diseases. Radiol Clin North Am 1984; 22:741-756. Burk DL, BrunbergJA, Kanal E, Latchaw RE, Wolf GL. Spinal and paraspinal neurofibro-
matosis: surface-coil MR imaging ology 1987; 162:797-801.
Galley
EH,
simulating
peritonei
mography.
EK,
(7).
7.
U
and
adenopathy
REFERENCES
plexiform
6.
RadioGraphics
of
secondary to lymphoma or metastatic disease can be excluded. Mirich et al (3) reported a case of plexiform neurofibromatosis that ap-
3.
Occasional lesions show central areas of decreased signal intensity on T2-weighted images, a finding that corresponds to dense collagenous tissue. This may prove to be a useful feature in distinguishing neurofibromas from other soft-tissue tumors (7).
perito-
identification locations,
subcutaneous
family
meta-
pseudomyxoma
is aided by the in characteristic spots,
a positive
(1,4,6).
images
differential
nei. Diagnosis neurofibromas
administra-
and calcifications but these are excep-
nonspecific
The
degener-
of surrounding adimasses (4,5). Peripheral
material
observed,
and
Ti-weighted spin-echo MR images reveal masses with a signal intensity slightly greater than that of muscle. Markedly increased signal
U
#{149} #{149}
.
.
.
..‘
._.ni,.
the neural tumors are neoplastic or hamartomatous in nature is controversial. Histologically, the tumors consist of a proliferation of spindle cells from either the neuroectodermal Schwann cell sheath or mesodermal fibrous connective tissue. When involvement is diffuse, producing fusiform tumorlike masses along the course of a nerve and its branches, the condition is called plexiform neurofibro-
1034
:
. ‘
#{149}
#{149}
#{149} #{149}
V.,., .
#{149}
)_...#{149}
V#{149}’
affecting potentially
system.
‘S
b
,
.
.
V
....
‘‘ #{149}#{149}‘
V
V..
-
Von
V
#{149},
-
:,
:
-.
‘
,
mal dominant all three germ
.
...
of
at 1.5 T. Radi-
Volume
12
Number
5
