AJR 2002; 178:208-210
© American Roentgen Ray Society
Thoracic Epidural Castleman's Disease
Recha S. Eisenstat1,
Donald B. Price1,
Alan D. Rosenthal2,
Allan L. Schuss3 and
Douglas S. Katz1
1
Department of Radiology, Winthrop University Hospital, 259 First St., Mineola,
NY 11501.
2
Department of Neurosurgery, Winthrop University Hospital, Mineola, NY
11501.
3
Department of Pathology, Winthrop University Hospital, Mineola, NY
11501.
Received March 12, 2001;
accepted after revision May 25, 2001.
Address correspondence to D. S. Katz.
Introduction
Castleman's disease is a rare lymphoproliferative disorder of unknown
origin that usually affects lymph nodes and, less commonly, nonnodal tissue
[1]. Involvement of the central
nervous system by either the hyaline-vascular or plasma cell type of
Castleman's disease is rare. To our knowledge, previous reports of cases in
which Castleman's disease presented as an epidural or subdural mass in the
central nervous system are few
[2,3,4].
We report the history as well as the MR imaging and pathologic findings for a
patient who proved to have the hyaline-vascular type of Castleman's disease
and who presented with back pain and a thoracic epidural mass.
Case Report
A 54-year-old man presented to the emergency department of our institution
with history of 1 week of progressive leg weakness and 1 month of lower back
pain. He had no history of trauma, incontinence, or weight loss. Ten years
earlier, the patient had been treated by surgical evacuation for an
intracranial hemorrhage, the origin of which had never been established. A
seizure disorder subsequently developed.
At admission, the patient's physical examination revealed midline
tenderness at vertebral body levels L3—L5. His gait was spastic, and
hyperreflexia affected his right arm and both legs. Sensation below the T8
level was decreased. Findings of routine admission laboratory studies were
within normal limits, and chest, thoracic spine, and lumbar spine radiographs
were unremarkable.
MR imaging performed on the day of admission showed an epidural soft-tissue
mass causing spinal cord compression from T8 to T11 (Figs.
1A and
1B). The lesion was
predominantly dorsal and wrapped around the spinal cord bilaterally. The
lesion was isointense to the spinal cord on T1-weighted images, hyperintense
to the cord on T2-weighted images, and enhanced homogeneously with IV
gadolinium (Figs. 1C and
1D). Abnormally high signal
intensity was present in the spinal cord on the T2-weighted images at the
levels of compression. The most likely diagnosis was believed to be lymphoma;
among the other diagnoses considered were meningioma and infection. A CT scan
of the chest, abdomen, and pelvis performed with oral and IV contrast material
revealed no other mass lesions or lymphadenopathy.
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Fig. 1A. —54-year-old man with progressive leg weakness and back pain.
Sagittal T1-weighted MR image shows dorsal epidural mass (solid
arrows) that is isointense to spinal cord and interrupts epidural fat
superiorly (open arrow).
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Fig. 1B. —54-year-old man with progressive leg weakness and back pain.
Sagittal T2-weighted MR image shows high signal intensity of mass (black
arrows). Spinal cord has abnormally increased signal intensity (white
arrows) at levels of compression by mass.
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Fig. 1C. —54-year-old man with progressive leg weakness and back pain.
Sagittal T1-weighted gadolinium-enhanced MR image with fat suppression reveals
marked homogeneous enhancement of mass (arrows). Full extent of mass
is from T8 to T11 vertebral body levels.
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Fig. 1D. —54-year-old man with progressive leg weakness and back pain.
Axial T1-weighted gadolinium-enhanced MR image with fat suppression reveals U
shape of enhancing mass (arrows) at T9 level.
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The next day, a T7-T12 laminectomy was performed, and the mass was resected
in its entirety. In the operating room, a frozen section was interpreted as
having features consistent with lymphoma.
Histopathologic examination of the resected specimen revealed a
lymphoproliferative process with follicles of varying size that had abnormal
germinal structures at their centers. Many of these structures had a
hyaline-vascular or "burnt-out" appearance. The mantle zone was
expanded with concentric layering of small lymphocytes producing an
"onionskin" appearance. The highly vascularized interfollicular
zone was confirmed immunohistochemically with factor VIII. Immunohistochemical
stains revealed a normal distribution of B and T cells and intact germinal
centers with a normal distribution of dendritic reticulum cells. Flow
cytometry confirmed the nonclonal nature of the lymphocytes. The final
pathologic diagnosis was Castleman's disease, hyaline-vascular type.
Discussion
Castleman's disease, also known as angiofollicular lymph node hyperplasia,
is a rare lymphoproliferative disorder that usually affects lymph nodes and,
less commonly, nonnodal tissue
[1,
5]. Histologically, the disease
has been categorized into two types: the hyaline-vascular type, representing
approximately 80-90% of cases, and the plasma cell type
[3,
5]. Intermediate forms with
both hyaline-vascular and plasma cell type features have also been described.
The hyaline-vascular type is a benign localized form, whereas the plasma cell
type is a more aggressive multifocal form that may present with systemic and
laboratory abnormalities [1,
5]. An increased risk of
non-Hodgkin's lymphoma and Kaposi's sarcoma has been associated with the
multicentric form [1,
2]. The plasma cell variant is
associated with systemic findings in approximately 50% of patients, including
fever, splenomegaly, lymphadenopathy, chronic anemia, leukocytosis,
thrombocytosis, elevated sedimentation rate, and hypergammaglobulinemia
[1,
4]. Those patients with the
localized form may be treated with local resection or radiation therapy
[2,
4], but those with the
multifocal variant generally require treatment with surgery or radiation
therapy, as well as systemic chemotherapy
[1].
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Fig. 1E. —54-year-old man with progressive leg weakness and back pain.
Magnified photograph of representative section of mass shows
characteristically layered "onionskin" pattern of small
lymphocytes (long arrows) surrounding central folliclelike structure
(short arrows) containing blood vessels. Findings are representative
of hyaline-vascular type of Castleman's disease. (H and E, x200)
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The cause of Castleman's disease is unknown
[5]; the disorder may represent
an infectious, inflammatory, or hamartomatous process
[6]. A reactive
lymphoproliferative response to an unknown stimulus, with defective
immunoregulation has been also suggested
[2].
Castleman's disease most frequently involves the mediastinum, abdomen,
neck, and axillae, and, less commonly, the retroperitoneum, mesentery, and
pelvis [3]. Extranodal disease
has also been described in the larynx, thymus, lung, pericardium, and vulva
[4].
Most patients are asymptomatic at diagnosis; the most likely reason for
discovery of Castleman's disease is the detection of a mediastinal mass on
chest radiographs obtained for other reasons
[5].
Patients with the plasma cell form of Castleman's disease may occasionally
have central nervous system symptoms, but CT and MR imaging examinations are
usually unremarkable [4].
However, very rarely, Castleman's disease of the central nervous system may
present as a focal leptomeningeal mass
[2,3,4].
Castleman's lesions in the central nervous system have been reported as
well-circumscribed, homogeneously enhancing masses. On MR images, these
lesions are generally hypointense on T1-weighted images and hyperintense on
T2-weighted images [2]. On CT
or MR images, the lesions may enhance moderately or markedly, as is typical of
Castleman's lesions occurring elsewhere in the body
[5,6,7].
A specific preoperative diagnosis of Castleman's disease of the central
nervous system, however, cannot be made on the basis of imaging findings
alone. The few patients with intracranial Castleman's disease who have
undergone CT or MR imaging have all had the preoperative diagnosis of
meningioma [1,
4,
5].
Subdural Castleman's disease of the spine has rarely been described
[2], but, to our knowledge, the
only prior case of spinal epidural Castleman's disease was reported by Alper
et al. [2] in a 10-year-old
boy, who presented with a lobulated extradural mass on MR images that extended
from C6 to T2 and compressed the spinal cord. Their patient had the less
common plasma cell type of the disease and presented because of the
intraspinal mass as well as systemic symptoms and multiple laboratory
abnormalities [2]. Our patient
had the more common hyaline-vascular form of Castleman's disease; patients
with this form of the disease do not present with systemic complaints or
laboratory abnormalities.
The imaging features of our patient's lesions also differed from those in
the previously reported patient. In our patient, the lesion was thoracic and
dorsal within the spinal canal, whereas in the previously described patient,
the lesion was cervical and ventral in position
[2]. The mass in our patient
was hyperintense on T2-weighted images, whereas the lesion in the other
reported patient was hypointense on T2-weighted images
[2]. We are not exactly certain
of the reason that the imaging findings for our patient differed from the
patient reported by Alper et al.
[2], but we believe the
principal reason may be the difference in the form of the disease: their
patient had the plasma cell type as opposed to the hyaline-vascular type seen
in our patient. Also, the MR signal intensity and enhancement characteristics
of our patient's mass conform to those reported for other Castleman's lesions
found elsewhere in the body
[5,6,7].
Spinal epidural masses may be benign or malignant. Epidural metastases are
common in cancer patients, but only 5% of spinal metastases are purely
epidural; most of these lesions are round cell tumors, particularly lymphoma,
leukemia, and extramedullary plasmacytoma
[8]. Lymphoma would be a much
more likely explanation for the findings in our patient—a homogeneously
enhancing epidural mass without bony involvement
[8]—and we believe that
the correct diagnosis could not have been made preoperatively in our patient.
Other diagnoses considered for our patient were based on his clinical history
and MR findings and included meningioma and infection in the phlegmonous
stage. Meningiomas are typically subdural lesions, but approximately 5-15% of
spinal meningiomas are epidural. An epidural infection may present as a
homogeneously or nearly homogeneously enhancing epidural mass
[8]. Epidural hematomas do
occur, but this diagnosis was considered unlikely in our patient because the
imaging characteristics of epidural hematomas are dominated by the metabolism
of hemoglobin, and they do not enhance with IV contrast material.
In summary, although Castleman's disease is quite rare in the central
nervous system, it is another entity to be considered in the differential
diagnosis of an enhancing epidural spinal mass.
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Introduction
Case Report
