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Spontaneous Reduction of Tumoral Calcinosis of the Cervical Spine After CT Guided Biopsy and Aspiration. 2012

General Spine

Patrick, H, Luetmer, MD
Felix, E, Diehn, M.D., Non ASSR Member
Daryl, C, Bergen, M.D., Non ASSR Member
John, I, Lane, M.D., Non ASSR Member
Karen, Fritchie, M.D., Non ASSR Member
A. Noelle, Larson, M.D., Non ASSR Member

Excerpta

We describe an unusual case of tumoral calcinosis (TC) in an infant involving the upper cervical spine and associated with torticollis, which mimicked a malignant chondroid or osseous neoplasm and nearly completely resolved after CT guided aspiration and biopsy.

A five month old white male was referred with a history of torticollis and a calcified cervical mass. Pregnancy and delivery were unremarkable. There was no evidence of birth trauma or torticollis at birth. At two months, the patient developed limited neck range of motion, and his parents noted flattening of his right posterior skull. Head control was poor with apparent pain on hyperextension, and neck rotation was full to the right but restricted to midline to the left. No tightness was appreciated in the sternocleidomastoid muscle and the deformity was rotational only, with no associated head tilt as is typical of congenital muscular torticollis. His physical exam was otherwise normal. He was treated with a cranial orthosis and passive stretching, which were poorly tolerated, prompting cervical radiographs that demonstrated a calcific mass in the precervical tissues.

CT and MR imaging obtained at 3 months showed a peripherally calcified mass centered at the right C2 anterior articular pillar and involving the right C1-C2 and C2-C3 facet joints. CT and MRI at 5 months showed progression of the mass with increase in permeative erosion of the right pedicle and anterior articular pillars of C2 and C3, extension into the right C2 and C3 foramen transversarium and significant progression of a complex multilobulated right prevertebral soft tissue component which partially effaced the right posterior oropharynx. (Fig. 1a-e) There was interval development of milky central calcifications. MRI demonstrated fluid-sediment levels (fig.1a), predominantly low T1 and mixed low and high T2 signal and peripheral contrast enhancement. There was mild ventral epidural extension without spinal cord compromise. MRA showed marked narrowing of the right vertebral artery with preservation of flow.

Radiographic differential diagnosis included TC, tumoral calcium pyrophosphate dihydrate crystal deposition disease (tCPPDcdd), fibrodysplasia ossificans progressiva (FOB), as well as chondrosarcoma and osteosarcoma.

ACVR1 genetic sequencing for FOB, skeletal survey, FGF23 genetic sequencing for autosomal recessive hyperphosphatemic familial tumoral calcinosis (HFTC), and calcium metabolism were all normal. Family history was significant for a maternal aunt and cousin with a history of juvenile rheumatoid arthritis.

Using CT guidance, a 15 gauge needle was placed into the lateral aspect of the mass and 1.5 cc of milky fluid was aspirated. Using a coaxial 16 gauge cutting needle, three core samples were then taken. Compared to the prior CT and MRI, shifting fluid-sediment levels were noted at CT biopsy, which was performed with the patient in the lateral decubitus position. (White arrows, fig. 1a-c)

Pathology demonstrated deposits of amorphous calcified material with absence of polarizable crystals consistent with tumoral calcinosis. This is in distinction to the rhomboid and rod shaped crystals with weakly positive birefringence seen with tCPPDcdd. There was no evidence of malignancy.

The patient was managed expectantly. Cervical range of motion improved significantly after aspiration/biopsy, with now 70 degrees of right rotation and 60 degrees of left and no apparent discomfort. At 7 months the child could sit independently with excellent head control and resolving plagiocephaly. CT performed at age 7 months, 2 months after biopsy, showed marked interval reduction in the mass, with resolution of the permeative erosion at C2 and C3 and some residual sclerosis. (Fig. 1f)

TC is a rare non-neoplastic condition that can occur at any age, but is most common in the first two decades of life.1 The hip is most commonly involved, but there are multiple reports of cervical spine involvement, including in a 4 month old boy.2 TC typically displays larger lobulated masses with milky calcifications, while tCPPDcdd is characterized by smaller round masses typically within ligamentous structures. Shifting sediment levels can be seen with change in patient positioning.3 Three subgroups of TC have been described: (1) related to minor trauma,4 (2) genetic or (3) as a manifestation of metabolic disease.5 Both TC and tCPPDcdd should be considered in the differential diagnosis of aggressive appearing calcified cervical masses.

Conclusion: Spontaneous reduction of TC can occur after aspiration and biopsy, at least at limited follow-up. Knowledge of the radiographic appearance of tumoral calcinosis can prevent unnecessarily aggressive treatment.

References/Financial Disclosures

1. Carlson AP, Yonas HM, Turner PT. Disorders of tumoral calcification of the spine: illustrative case study and review of the literature. J Spinal Disord Tech 2007;20:97-103 2. Issa El Khoury F, Kreichati G, Kharrat K, et al. Tumoral calcinosis of the cervical spine and its association with Caffey disease in a 4-month-old boy: case report and review of the literature. J Pediatr Orthop B 2011 3. Feldman ES, Dalinka MK, Schumacher HR. Diffuse soft tissue calcification in tumoral calcinosis. Skeletal Radiol 1981;7:33-35 4. Smack D, Norton SA, Fitzpatrick JE. Proposal for a pathogenesis-based classification of tumoral calcinosis. Int J Dermatol 1996;35:265-271 5. Slavin RE, Wen J, Kumar D, et al. Familial tumoral calcinosis. A clinical, histopathologic, and ultrastructural study with an analysis of its calcifying process and pathogenesis. Am J Surg Pathol 1993;17:788-802 No financial disclosures

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