Imaging of Ancient Schwannoma

Kenichi Isobe1, Tominaga Shimizu, Tsutomu Akahane and Hiroyuki Kato

1

All authors: Department of Orthopedic Surgery, Shinshu University, School of Medicine, 3-1-1
Asahi, Matsumoto, Nagano 390-8621, Japan.
Received November 3, 2003; accepted after revision December 9, 2003.

Address correspondence to K. Isobe (bechikun@hsp.md.shinshu-u.ac.jp

Abstract
OBJECTIVE. We surveyed the clinical symptoms and
radiologic features of ancient schwannoma, a rare variant of
schwannoma characterized by degenerative changes.

).

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Abstract
Introduction
Materials and Methods
Results
Discussion
References

MATERIALS AND METHODS. We present the clinical,

radiologic, and pathologic features in seven patients with ancient schwannoma (mean age,
62 years; range, 4580 years) treated at our department between 1998 and 2003.
RESULTS. The most characteristic clinical features were a sign like Tinel's sign and a
long interval between the onset of symptoms and surgery (mean interval, 8.3 years).
Ancient schwannomas can grow large; the biggest tumor seen in our study was 14 cm
long. The highly accurate radiologic assessment made possible with contrast-enhanced
MRI and CT scanning showed enhancement at a peridegenerative area and sometimes at a
capsule. These findings differ from those of the typical schwannoma and neurofibroma
patterns reported to date. Furthermore, bone scintigraphy showed uptake in the tumor, but
no accumulation was seen on gallium-67 citrate scintigraphy.
CONCLUSION. The characteristic clinical and radiologic findings of ancient
schwannoma should make it possible to differentiate it from malignant tumors.

Introduction
Schwannoma, one of the most common soft-tissue tumors, is a
benign tumor that arises from the Schwann cells of the nerve
sheath and presents with symptoms of pain or paresthesia.
Ancient schwannoma, a degenerative neurilemmoma, is a
schwannoma subtype characterized by degeneration and

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Abstract
Introduction
Materials and Methods
Results
Discussion
References

diffuse hypocellular areas. These changes are believed to occur because it takes a long
time for schwannomas to develop [1]. Schwannomas with these degenerative changes can
be misdiagnosed as sarcomas or as other forms of soft-tissue neoplasms. In this report, we
describe the clinical symptoms and radiologic features of ancient schwannoma as an aid
for preoperative differential diagnosis.

Materials and Methods

The seven patients entered into this study were diagnosed at
pathology as having 24 schwannomas that were treated
surgically in our department between 1998 and 2003. None of
the patients had a history of Recklinghausen's disease. The
tumors originated from the tibial nerve in three cases and in
one case each from the lateral cutaneous femoral nerve, sciatic
nerve, sural nerve, and plantar digital nerve.

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Abstract
Introduction
Materials and Methods
Results
Discussion
References

Sonography, radiography, CT, MRI, bone scintigraphy, and gallium citrate scintigraphy
were all used for the examination. A 1.5-T superconductive MRI unit (Signa, GE
Healthcare) was used to produce spin-echo images. T1-weighted MRI with TR/TE
(ranges, 500716/1217) and T2-weighted imaging (ranges, 3,0004,000/81.3100) using
a body coil and field of view of 3040 cm depending on body size were performed for all
patients. Gadolinium-enhanced scans at a dosage rate of 0.2 mL/kg were obtained for all
patients, and all underwent sonography, radiography, CT, and MRI. Six patients
underwent bone scintigraphy, five underwent 67Ga citrate scintigraphy, and four
underwent contrast-enhanced CT. Images from all patients with ancient schwannoma who
underwent radiologic examinations were interpreted independently by three authors who
reached agreement by consensus. After the radiologic examinations, the tumors were
surgically treated and evaluated for pathology. The radiologic features of the tumors were
then assessed and compared with the MRI findings for the pathologic specimens.

Results
Clinical Features of Ancient Schwannoma
The clinical records of the seven patients are summarized in
Table 1. Tenderness and numbness were the most frequent
symptoms, but five patients reported radiating pain produced
by tapping on the tumor (Tinel's sign). The mean interval
between onset of symptoms and surgery was 8.3 years (range,
120 years). All ancient schwannomas were located in the
lower limbs except for one in a retroperitoneal space.

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Abstract
Introduction
Materials and Methods
Results
Discussion
References

View this table: TABLE 1 Patient Data for Ancient Schwannoma

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Radiologic Features of Ancient Schwannoma

Radiologic features of the ancient schwannomas are summarized in Table 2.

View this table: TABLE 2 Features of Ancient Schwannomas in Seven

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Sonography.Sonograms showed a well-defined mass containing internal highly

reflective areas or hypoechoic areas. An adjacent nerve was visualized in four cases.
CT.CT scans showed a well-defined mass, and enhancement was observed in capsules
or pericystic areas after IV contrast medium infusion (Figs. 1A and 1B). Enhancement of
areas surrounding degeneration was seen in all patients who underwent enhanced CT.

Fig. 1A. CT evaluation in 80-year-old woman with

ancient schwannoma. Axial CT scan of lower leg
shows large, well-circumscribed low-density mass with
partial central high-density area in posterior
compartment.

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Fig. 1B. CT evaluation in 80-year-old woman with

ancient schwannoma. Contrast-enhanced CT scan
shows enhancement in circumference of degenerative
area and peripheral rim.

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MRI findings compared with pathologic specimen results.The gross appearance of the
tumor was well circumscribed with a thin fibrous capsule, and the internal matrix was
yellowish with some scattered myxoid areas and hemorrhagic, cystic, and necrotic areas
ranging from red to brown (Figs. 2D and 3D).

Fig. 2D. MRI and histologic findings in 60-yearold man with ancient schwannoma. Photograph
shows gross appearance of tumor. Squares show
circumference of degenerative areas (E), area of
degenerative change (F), and substance (G).

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Fig. 3D. MRI and histologic findings in 56-yearold woman with ancient schwannoma. Photograph
of gross appearance of tumor shows marked
degeneration.

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Microscopic examination showed that the tumors consisted mostly of Antoni type B areas
that show low signal intensity on T1-weighted MR images and high signal intensity on
T2-weighted images. Perivascular hyalinization and gapped vessels with organizing
thrombus and hematoma or cystlike spaces mimicking vascular structures were clearly
visible. These degenerative areas of hematoma or cystic formations varied in size.
Contrast-enhanced MRI depicted these lesions as of various signal intensities without any
enhancement. Both the Antoni type A and Antoni type B areas were enhanced by IV
contrast medium. The nonenhancing areas of the tumor appeared in all cases to be areas
of hemorrhage or degeneration. The circumference of these lesions was clearly enhanced,
and the fibrous capsule of the tumor was sometimes enhanced. In all patients, enhanced
areas surrounded the degenerative lesions, and enhancement in the capsule was seen on
MRI in five patients. The Antoni type A area was reduced and seen mostly around the
degenerative lesions (Figs. 2A, 2B, 2C, 2D, 2E, 2F, 2G and 3A, 3B, 3C, 3D).
Calcification was histologically confirmed in two tumors, and ossification in one patient.

Fig. 2A. MRI and histologic findings in 60-yearold man with ancient schwannoma. Axial T1weighted MR image shows large wellcircumscribed mass with isointense signal. Note
round high-signal-intensity area (arrow) in mass.

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Fig. 2B. MRI and histologic findings in 60-yearold man with ancient schwannoma. T2-weighted
image shows most of tumor as hyperintense signal
and partially heterogeneous signal in internal oval
area. Note areas of Antoni A (arrow) and Antoni B
(arrowhead) tumor.

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Fig. 2C. MRI and histologic findings in 60-yearold man with ancient schwannoma. Gadolinium
contrast-enhanced image shows enhancement at
circumference of degenerative lesion (arrow),
peripheral rim (arrowhead), and substantial part.

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Fig. 2E. MRI and histologic findings in 60-yearold man with ancient schwannoma.
Photomicrograph shows Antoni A area. (H and E,
x100)

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Fig. 2F. MRI and histologic findings in 60-yearold man with ancient schwannoma.
Photomicrograph clearly shows hematoma and
degenerative collagen tissue. (H and E, x100)

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Fig. 2G. MRI and histologic findings in 60-yearold man with ancient schwannoma.
Photomicrograph clearly shows Antoni B area. (H
and E, x100)

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Fig. 3A. MRI and histologic findings in 56-yearold woman with ancient schwannoma. Axial T1weighted MR image of adductor magnus shows
large tumor with isointense signal.

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Fig. 3B. MRI and histologic findings in 56-yearold woman with ancient schwannoma. T2-weighted
image shows heterogeneous tumor with high signal
intensity in internal oval area (arrow).

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Fig. 3C. MRI and histologic findings in 56-yearold woman with ancient schwannoma. Gadolinium
contrast-enhanced image shows enhancement in
circumference of degenerative area (arrowhead)
and peripheral rim of tumor (star). Oval
degenerative area (arrow) is not enhanced.

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Bone scintigraphy and 67Ga citrate scintigraphy.Moderate to high uptake in the tumor
was seen in five cases on the bone scintigram, but 67Ga citrate scintigraphy showed no
such accumulation in six cases.

Discussion
Schwannomas are benign slow-growing encapsulated tumors of nerve sheath origin that
are found most commonly in the extremities. Their histology
Top
generally displays a biphasic pattern with areas of highly
Abstract
ordered cellularity (Antoni type A) and less cellular areas
Introduction
where a highly myxoid matrix predominates (Antoni type B)
Materials and Methods
[2]. As for the radiologic findings, sonography of ordinary
Results
schwannomas shows a well-defined hypoechoic mass located
Discussion
References
along a nerve and eccentric to the nerve axis [3]. The CT
appearance has been described as a well-circumscribed,
inhomogeneous mass of low density, which can be explained
by the following microscopic pattern: hypocellular areas (Antoni type B) adjacent to more
cellular regions (Antoni type A) and cystic degeneration [4]. The radiologic findings for
schwannoma are similar to those for ancient schwannoma, which is a rare variant of
schwannoma first described by Ackerman and Taylor in 1951 [5].
Ancient schwannoma is usually located deep in the head and neck [6, 7], thorax [8],
retroperitoneum [9, 10], pelvis [11], and extremities [12, 13] of elderly patients. It is
characterized by degenerative changes typified by perivascular hyalinization,
calcification, cystic necrosis, relative loss of Antoni type A tissue, and degenerative nuclei
that may be misinterpreted as sarcomatous pleomorphisms [14]. Calcification is the usual
degenerative change, but ossification, which was observed in our patient 7, is a rare

degenerative variant [14, 15]. In our study, however, calcification and ossification could
not be detected on radiologic examinations.
These degenerative changes are thought to be the result of the long-term progression of
this tumor. Retroperitoneal ancient schwannoma is difficult to recognize if it has no
clinical symptoms, or if it is located in the extremities with no other symptoms than local
swelling, and patients tend to ignore the mass until it has finally reached an excessive
size. For example, patient 1 had a tumor of the calf measuring 13.8 x 8.0 x 6.5 cm, which,
to the best of our knowledge, is the largest reported ancient schwannoma in this location.
However, the only complaint by the patient was of partial numbness on the side of the
dorsum pedis, which he did not have medically examined until 10 years after first noticing
the tumor.
Because it contains cystic areas, ancient schwannoma has been radiologically
misdiagnosed as other tumor types, such as malignant fibrous histiocytoma, malignant
peripheral nerve sheath tumor, liposarcoma, synovial sarcoma, or hemangiopericytoma.
However, only a few reports have dealt with the radiologic features of ancient
schwannoma because this tumor is so rarely encountered. Although Shultz et al.
suggested that a diagnosis of ancient schwannoma should be considered when a patient
presents with a hypervascular soft-tissue mass containing amorphous calcification on
radiographs and cystic areas on MRI [13], calcification is not always visible on films [9,
12].
Our study suggests that MRI is the most useful technique for further evaluation of ancient
schwannoma. Ordinary schwannomas show their peripheral region as a lowsignalintensity area on T1-weighted images and a high-signal-intensity area on T2weighted imageswhich correspond to the Antoni B areaand as low-to-intermediatesignal-intensity areas on T1- and T2-weighted images. Ordinary schwannomas are
strongly enhanced by gadolinium contrast medium, which is also characteristic of the
Antoni A area. On T1-weighted images, the tumors are isointense or slightly hyperintense
relative to muscle, and a target pattern with a peripherally hyperintense rim and central
low intensity on T2-weighted images has been reported for 52% of benign nerve sheath
tumors (neurofibromas and schwannomas) [17]. This target pattern corresponds
histologically to peripheral myxomatous tissue and central fibrocollagenous tissue; is
absent in lesions with cystic, hemorrhagic, or necrotic degeneration; and is not visible in
malignant peripheral nerve sheath tumors.
The ancient schwannoma in our series, however, showed a well-circumscribed complex
cystic mass and different enhancement patterns. The circumference of the degenerative
hematoma and cyst and the fibrous capsule of the tumor were enhanced. The Antoni type
B area occupied most of the tumors, and the Antoni type A area had become smaller and
was seen in the areas around the degenerative lesions. We therefore suspect that the
Antoni type A areas had degenerated to hematomas or cysts.
Scintigraphy is also a helpful radiologic technique. A previous study found that
schwannomas with the widest diameter ( 3 cm) were positive for technetium-99m

dimercaptosuccinic acid and negative for 67Ga citrate [18]. Our patients with ancient
schwannoma showed the same expression pattern, which indicates that a scintigram
should be used to examine a large neurogenic tumor.
The preoperative differential diagnosis of a comparatively large soft-tissue tumor in an
extremity that appears as a well-enhanced inhomogeneous tumor on MRI tends to identify
it as malignant. Differentiation from a malignant tumor can be achieved by taking into
account the characteristics of the clinical findings and radiologic features of ancient
schwannoma. In this study, we made a tentative preoperative diagnosis of the tumors as
schwannomas, except for patient 7. Although all the tumors had been diagnosed as
ancient schwannoma, patient 7 was problematic before this study was performed because
the tumor was large and had an indistinct margin, and so it was thought to be malignant.
In conclusion, the following points are important for differentiating ancient schwannoma
and other tumors: The first is a mass with a long-term clinical course presenting with the
symptoms of a neurogenic tumor. The second is a palpable mass with local pain and
characteristics similar to Tinel's sign and distinct radiologic features that include an
adjacent nerve visualized on sonography, a circumference of a degenerative area and
fibrous tumor capsule enhanced on MRI, and positive accumulation seen on a bone scan
but not on a gallium scan.

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Abstract
Introduction
Materials and Methods
Results
Discussion
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