Progressive systemic sclerosis with massive paraspinal soft-tissue calcinosis.

Abstract

but normal calcium levels. Inclan et al. specified that this condition occurred in the absence of renal, metabolic, or collagen vascular disease. However, the term “tumoral calcinosis” has gradually and imprecisely encompassed all tumorlike multilobulated calcific deposits in a paraarticular distribution in soft tissues. Because of the expanding definition in the literature, lesions now have a pathologically based classification: primary normophosphatemic tumoral calcinosis, primary hyperphosphatemic tumoral calcinosis, and secondary tumoral calcinosis [2]. Primary forms of tumoral calcinosis are less common than the secondary forms, which may be associated with renal failure, hemodialysis, collagen vascular disease, sarcoidosis, pseudoxanthoma elasticum, massive osteolysis, and other conditions. Patients present with a mass, regional compressive symptoms, or both, but pain and radiculopathy have also been described. Most of these lesions are related to the extensor surfaces of the hip, shoulder, elbows, wrists, and feet and likely originate in adjacent bursae. Rare locations also include dental and paraspinal regions [3]. Lesions of tumoral calcinosis consist of macroscopic masses of amorphous calcified material, sometimes in aqueous suspension, surrounded by chronic fibrotic and inflammatory reaction. A paraarticular distribution is common. Conventional radiographs show a characteristic lobulated mass of several centimeters with amorphous calcific deposits. Although a cystic component may be present, evident as a fluid‐ sedimentation level, this feature is usually absent when tumoral calcinosis is associated with connective tissue disease [4]. Bone scintigraphy shows increased radiotracer activity in the corresponding paraarticular distribution. CT shows soft-tissue calcinosis without direct communication with adjacent bones or joints. Lesions with a cystic component may have high-attenuation septations as compared with the low-attenuation centers. Calcium layering, known as the “sedimentation” sign, may be seen. MRI may show fluid‐fluid levels with high signal layered over low signal on fluid-sensitive sequences [5]. If symptomatic, lesions of tumoral calcinosis may be excised. Although recurrent lesions are common, their growth in secondary tumoral calcinosis may be retarded with treatment of the underlying condition.