Tumoral calcinosis is characterized by tumor-like periarticular
deposits of calcium, and it commonly presents as a growing calcific mass in
the soft tissue surrounding major
joints1-6.
It may be associated with bone erosion and destruction and may have a mass
effect on neurovascular
structures7-9.
Tumoral calcinosis can be a component of CREST syndrome, an acronym for
calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and
telangiectasia.
Spinal and paraspinal tumoral calcinosis has been reported to involve both
the vertebral bodies and the posterior elements of the cervical, thoracic, and
lumbar
spine6,10-18.
To our knowledge, tumoral calcinosis of the cervical spine as a component of
CREST syndrome has been reported in only three
patients7,8,10.
The diagnosis of tumoral calcinosis in the spine is difficult because of its
rarity and its ability to mimic a neoplasm or infectious
cause6,14,16,19.
Neurologic deficits by mass effect can occur and may manifest as radiculopathy
and/or
myelopathy7-10,18.
Moreover, it has been shown that tumoral calcinosis of the spine has presented
diagnostic dilemmas to clinicians, radiologists, and pathologists because of a
lack of familiarity with the
entity16.
We report the clinical presentation, radiographic findings, histopathologic
results, and the surgical management of tumoral calcinosis of the cervical
spine in a patient with CREST syndrome. Our patient is unique in that there
was radiographic evidence of rapid progression of bone erosion and crystal
deposition involving the posterior elements and paraspinal tissues within a
one-year period. In addition, an anomalous, single vertebral artery on the
affected side supplied the basilar artery system. The patient was informed
that data concerning the case would be submitted for publication.
A fifty-nine-year-old woman presented to the Orthopaedic Spine
Clinic with a history of chronic pain in the posterior aspect of the neck,
occasional mild right-sided radiculopathy, and mild bilateral weakness that
had worsened over a two-week period. She had a history of Raynaud disease,
esophageal dysmotility, sclerodactyly, and telangiectasias and had normal
findings on a pulmonary function test. Physical examination revealed a
well-developed woman with a normal gait. The range of motion of the cervical
spine revealed only minimal rotational limitation. There was no motor weakness
or sensory loss to pin-prick testing. She had symmetric deep-tendon reflexes,
no pathologic reflexes, and a negative Lhermitte sign. The erythrocyte
sedimentation rate, white blood-cell count, and serum C-reactive protein,
chemistry, and calcium levels were all within normal limits. She had a
positive test for antinuclear antibodies.
Cervical spine radiographs from the prior year demonstrated normal
findings. The new radiographs revealed normal cervical alignment with erosive
changes involving the spinous processes of C3 to C5 as well as the pedicles,
laminae, and facet joints on the right. Additionally, there was mineralization
and a posterior soft-tissue mass (Fig.
1). Magnetic resonance imaging demonstrated expansion and
sclerosis of the right pedicles and spinous processes at C4 and C5 with a
posterior heterogenous mass (Fig.
2). There were hypointense masses on T1-weighted images and
heterogenous signal intensity within areas of low signal intensity on
T2-weighted images. Multidetector computerized tomographic scanning with
reconstructions showed a destructive erosive process involving the spinous
processes and right facet joints with evidence of surrounding soft-tissue
calcifications involving levels C3 to C6 posteriorly
(Fig. 3). These findings were
consistent with tumoral calcinosis. An aggressive neoplasm or an atypical
chronic infectious process was possible but considered less likely because of
the overall radiographic appearance and pattern of mineralization. Needle
aspiration and a computed tomography-guided biopsy were negative for neoplasm
and infection.
The patient was first seen at the orthopaedic clinic eight days after the
biopsy, with white fluid draining from the biopsy site and increasing neck
pain. There was a concern that she had a possible atypical infection and/or a
neoplastic process such as chondrosarcoma. A cerebral angiogram revealed the
right vertebral artery to be widely patent, but the left vertebral artery
terminated into a left posterior inferior cerebellar artery without
opacification of the basilar artery. The patient underwent surgery.
Intraoperative findings included many pockets of liquefied calcium in the
bone, soft tissue, and muscle. The tumorous mass had destroyed the facets of
C4 and C5 and had extended cephalad to C2 on the right side. The operative
procedure included wide resection of the lesions, laminectomy of C4 and C5,
and facetectomy as well as partial resection of C3 and C6 with spinal cord
monitoring. The right C5 and C6 nerve roots were decompressed, and calcified
tissue was resected from the C2 to C6 levels. An arthrodesis of the spine was
then performed with lateral mass screws and rod fixation from C3 to C7 on the
left and autogenous iliac crest strut-grafting and wiring from C2 to C7
directly posteriorly.
Our surgical pathologist described the gross appearance of the core
specimen as milky-white fluid (milk of calcium). Macroscopically, the tumor
specimen consisted of skeletal muscle with fibroconnective tissue and
fragments of bone (Fig. 4). On
cross section, multiple scattered 0.1 to 0.2-cm white to yellow nodules
composed of chalky, calcific material were seen throughout the soft tissue and
bone. Histologically, there were islands of amorphous, calcified material
bordered by histiocytes and multinucleated giant cells with a surrounding
foreign-body-type reaction. In the osseous specimen, the calcifications were
admixed with bone in some areas. Each of the nodules represented an island
separated by dense fibrocollagenous septae with foci of hemorrhage and
multiple acute inflammatory cells. These findings were consistent with the
diagnosis of tumoral calcinosis.
The patient was evaluated at six weeks, twelve weeks, four months, and one
year after surgery and was found to be doing well. At one year, the neck pain
had resolved and the neurovascular status remained intact with no sequelae
from the vertebral artery mobilization. The cervical spine instrumentation
remained intact and a solid fusion was evident
(Fig. 5).