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Tumoral Calcinosis of the Cervical Spine Related to Chronic Renal Failure 2007

General Spine

Divyesh G Patel, MD
Joseph G Craig, MB ChB, Non ASSR Member

Excerpta Extraordinaire

Excerpta

Tumoral calcinosis of the spine is a rare manifestation of an uncommon condition. To date the authors are only aware of 10 reported cases involving the cervical and cervicothoracic spine.

Purpose

We present a case of tumoral calcinosis of the cervical spine causing myelopathy that was thought to represent a tumor or infection.

Methods & Materials

A 29-year old African American female presented to the Emergency Department with progressively worsening debilitating back pain that was rated as 10 out of 10. The patient denied any radiation or weakness, but did have upper extremity paresthesias. There was no history of trauma or twisting injury.

The patient's past medical history was significant for end-stage renal disease requiring hemodialysis.

The physical examination demonstrated the patient to be in mild distress, with midline tenderness at the cervicothoracic junction. Mild weakness of both upper extremities was also noted. Otherwise, the physical examination was noncontributory.

Radiographs of the cervical and thoracic spine showed a calcified/ossified mass involving the posterior elements of C6 through T1 with some encroachment on the neural foramina at these levels. CT of the cervical spine and thoracic spine showed a calcified mass about the spinous processes from C6-T2. The lamina, pedicles, and spinous process of C7 were expanded and had a mixed sclerotic/lytic appearance. There were some small lytic regions within the T1 laminae, as well as incorporation of the T1 spinous process by the mass. MRI of the cervical spine demonstrated the mass to be heterogeneous with large areas of diminished signal on T2, T2*, and T1 images. There was narrowing of the central spinal canal from C6 to T1, with complete effacement of the cerebrospinal fluid space at C7. Both neural foramina at C7-T1 were narrowed. No abnormal signal was seen with the spinal cord.

Results

The orthopedic spine service was consulted. The patient was taken for decompression for spinal cord compression that was presumed to be related to a tumor. The mass was composed of calcific and inflammatory tissue which seemed pus-like to the surgical team. Intraoperatively there was concern that this was caseous type necrosis related to tuberculosis infection. The intraoperative gram stain and AFB stains were negative. Intraoperative frozen section was inconclusive for tumor versus inflammatory condition versus infection. The surgical team did note there was significant destruction of the posterior elements by the infiltrative mass. Complete excision of this tissue was performed with decompression at C7 and T1 via laminectomy, partial facetectomies, foraminotomies, and partial vertebral resection of C7.

The final histology showed bands of fibrocollagenous tissue in a trabecular pattern encircling extensive deposits of hydroxyapatite. The deposits were surrounded by a cellular infiltrate of lymphocytes and numerous histiocytes with frequent multinucleated giant cells. The epithelial markers failed to demonstrate any cells. There was no bone in the tissues examined.

Conclusion

Tumoral calcinosis is a rare disorder characterized by soft tissue calcification deposition, typically in a periarticular distribution about the hip, shoulder, and elbow. First described by Inclan et al in the American literature in 1943, the disorder was differentiated from dystrophic and metabolic calcifications in that patients had normal calcium levels and elevated phosphate levels. In the mid-1960s a familial predisposition and a higher incidence in the African American population was found to be associated with the disorder.

Over time a laxity developed in defining the disorder based on the metabolic criteria originally proposed by Inclan et al. As a result three different clinical presentations have been described as representing tumoral calcinosis. The familial variant usually manifests before the third decade with painless lesions about the hip and elbow. It can also occur as a result of chronic renal failure as in our patient. Finally, a sporadic form is thought to occur in patients with tissue degeneration or degenerative joint disease. Durant et al have proposed that calcific tendonitis is a common manifestation of this sporadic form. Recently, however, Olsen et al have suggested that the radiology community reexamine the liberal use of the term tumoral calcinosis, and only apply it to those cases of periarticular calcifications that are associated with a hereditary metabolic dysfunction of phosphate regulation.

Histologically, these lesions demonstrate masses of calcium hydroxyapatite crytals associated with neovascularity, histiocytes, and foreign body giant cells. There usually is a fibrous capsule related to a foreign body type reaction. There have been reports of calcium hydroxyapatite and calcium pyrophosphate hydroxyhydrate (CPPD) crystals coexisting within a lesion, as well as cases of isolated CPPD crystals within lesions classified as tumoral calcinosis. There is controversy related to this as some believe that CPPD crystal deposition seen within spinal ligaments is a distinctly different entity from tumoral calcinosis. However, there have been documented cases of calcium hydroxyapatite and CPPD found together within articular chondrocalcinosis and in a subcutaneous tophaceous mass from pseudogout. Kawano and colleagues even showed that linear CPPD crystal deposits within the ligamentum flavum later changed into calcium hydroxyapatite cystals, the more stable final form of calcium phosphate.

The rarity of this entity and its ability to mimic infection and neoplasm makes diagnosis difficult. In the 21 cases of spinal involvement presented by Durant et al, tumoral calcinosis was not considered as a pre-operative diagnosis in any of the patients. Whether we define tumoral calcinosis as a periarticular calcified mass associated with a hereditary defect in phosphate metabolism or as periarticular calcifications associated with an underlying disorder, it is a diagnostic consideration we should keep in mind.

References

1. Inclan A, Leon P, Camejo MG. Tumoral calcinosis. J Am Med Assoc 1943;121:490-95.
2. Shaffrey CI, Munoz EL, Sutton CL, et al. Tumural Calcium Phyrophosphate Dihydrate Deposition Disease Mimicking a Cervical Spine Neoplasm: Case Report. Neurosurgery 1995;37:335-39.
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4. Ohashi K, Yamada T, Ishikawa T, et al. Idiopathic Tumoral Calcinosis Involving the Cervical Spine. Skeletal Radiol 1996;25:388-90.
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6. Durant DM, Riley LH 3rd, Burger PC, et al. Tumoral Calcinosis of the Spine: a Study of 21 Cases. Spine 2001;26:1673-79.
7. Matsukado K, Amano T, Itou O, et al. Tumoral Calcinosis in the Upper Cervical Spine Causing Progressive Radiculomyelopathy - Case Report. Neurol Med Chir (Tokyo) 2001;41:411-14.
8. Teng Al, Robbin MR, Furey CG, et al. Tumoral Calcinosis in the Cervical Spine in a Patient with CREST Syndrome. A Case Report. J Bone Joint Surg Am 2006; 88:193-97.
9. Olsen KM, Chew FS. Tumoral Calcinosis: Pearls, Polemics, and Alternative Possibilities. Radiographics 2006;26:871-85.

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