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Tumoral Calcinosis: Clinical Features, Diagnosis, and Treatment

This article explores the clinical features, diagnosis, and treatment of tumoral calcinosis, a large periarticular deposition of calcium resembling a neoplasm. It predominantly affects adolescents and young adults and is often familial. The article discusses the pathology, microscopic features, and treatment options for this condition.

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Tumoral Calcinosis: Clinical Features, Diagnosis, and Treatment

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  1. U05-15217

  2. Clinical History • Dialysis patient • Large right thigh soft tissue calcified mass. • ? malignancy

  3. Diagnosis: • Tumoral Calcinosis

  4. Tumoral Calcinosis • A large periarticular deposition of calcium resembling a neoplasm • Usually occurs as multiple lesions of hip, shoulder & elbow • Predominantly adolescents, young adults • Often familial • Majority of cases in African americans • No abnormalities in calcium metabolism

  5. “Tumoral Calcinosis-like lesion” • Morphologically identical to TC • Pts usually older & have additional calcifications in visceral organs • Hypocalcemia /hyperphosphatemia associated with Chronic renal Dz or hypercalcemia due to other causes • Very similar to TC – support use of term “tumoral calcinosis” with qualification of subtype (ie/secondary)

  6. Pathology • Unencapsulated, Firm rubbery mass • Extends into adjacent muscles/tendons • 5-15 cm • On sectioning, dense fibrous tissue containing spaces filled with yellow gray, pasty or chalky milky liquid • Easily washed out leaving cystic spaces

  7. Microscopic • Active and inactive phases, often together in same lesion • Activephase – central mass of amorphous granular calcified material bordered by a florid proliferation of macrophages, giant cells & fibroblasts • Inactive phase – calcified material surrounded by dense fibrous material extending into adjacent tissues or cystic space surrounded by calcium deposits

  8. Treatment • Initially conservative – phosphate binders/antacids, optimization of dialysis, diet • Subtotal parathyroidectomy • Surgical resection – last resort

  9. References • 1. Enzinger FM, Wiess SW: Soft tissue tumors(ed 3). St Louis, MO, Mosby, • 2. Mockel G, Buttgereit F et al (2003) Tumoral Calcinosis revisited: pathophysiology and treatment. Rheumatol Int 25:55-59 • 3. McGregor D, Mowry M et al (1995) Nonfamilial Tumoral Calcinosis Associated with Chronic renal failure and Secondary Hyperparathyroidism: Report of two cases with clincopathological, Immunohistochemical, and electron microscopic findings. Human Pathology 26:607-613

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