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Chapter 29

Tumoral Calcinosis

There are many so-called tropical diseases which in practice occur throughout the world: tumoral calcinosis is one. A few of the illustrations in this chapter come from California; others from Washington, D.C., but the majority come from the tropics because tumoral calcinosis is undoubtedly much more common in tropical Africa and New Guinea than anywhere else. Currently almost all journal descriptions describe it as "rare" or "uncommon;' yet there are over 2000 case reports in the literature and as we know from our own experience, there are many more which are not recorded. There are series of 13, 17, 21, 50, and 56 cases; there are also other series of two, three, or five cases in which individual patients have been redescribed on several occasions in different publications, focusing on a particular aspect of the disease, such as biochemistry or genetics. In spite of this confused reporting, or perhaps because of it, the etiology of tumoral calcinosis is still hotly debated. Although the cause may be obscure, the histopathological findings are characteristic and definitive; the radiological appearances are almost equally diagnostic and clinically the findings are similar wherever the disease is seen. There is some doubt about the best way to cure it, and no matter what is done, a small number of patients will die from this "benign" disease.

Synonyms

Tumoral (tumoural) calcinosis. Massive calcinosis. Lipocalcinogranulomatosis. Lipocalcinogranulomatous bursitis. Granulomatous calcinosis. Calcifying collagenolysis. Exudative calcifying fasciitis. Calcium gout. Teutschlander's disease. Sp: Tumor calcinosis. Lipogranulomatosis calcificante. Fr: La calcinose tumorale. Lipocalcinogranulomatose. Ger: Lipokalzinogranulomatose.

Definition

Tumoral calcinosis (the name first used by Inclan in 1943) describes a dense, nodular, calcareous mass in the subcutaneous or interstitial tissues, most often around the hips, elbows, shoulders, and feet but occasionally elsewhere. There is no evidence of malignancy. Many other criteria have been suggested to elaborate upon this definition, but, as more cases are described, not all remain valid. Tumoral calcinosis should at least fulfill most of the following:

1. The patient should be otherwise healthy and free from renal, metabolic, or collagen diseases which can cause soft tissue calcification.
2. The serum chemistry should be normal, except that some (but not all) cases show hyperphosphatemia and elevated serum 1,25-dihydroxyvitamin D.
3. The masses (tumors) should be painless. As they increase in size, symptoms may be caused by pressure on nerves, skin, etc., but there should be no intrinsic tumoral sensitivity. When there is calcific myelitis, there may be bone pain.
4. The tumors are likely to be multiple, although not necessarily occurring simultaneously. Not all patients will demonstrate multiple tumors when first seen, and the interval between removal of the original mass and the appearance of another tumor elsewhere in the body may be months or 5-10 years.
5. Some patients may have siblings with tumoral calcinosis.
6. There may be calcified dental pulp stones and changes in the bone marrow (calcific myelitis). In some series there is a periosteal reaction.

Not every case of tumoral calcinosis (or even the majority) has all the characteristics described in some reports, and this is not because the more sophisticated biochemical and imaging results are unavailable. Calcium metabolism is not disturbed in many tropical patients. Normal laboratory tests certainly do not invalidate the diagnosis, because the disease is best recognized by its histological and radiological characteristics, especially the fact that the "fluid" or "pus" from the tumors has a high calcium content and is radiopaque; such a finding is most unusual for tissue fluids, particularly those from tumors.

Geographic Distribution

The majority of patients are in Africa and Papua-New Guinea, but the disease is known in North and South America, the Caribbean, England, throughout Europe, Turkey, Iran, India, Saudi Arabia, Australia, China, and other parts of Asia. In Africa, where the majority of cases have been noted, there is no tribal predilection. It is most common south of the Sahara, especially within the tropics, but no part of the continent is spared, and many patients have been recorded in Ethiopia, Somalia, Algeria, and Tunisia; patients have also been seen in Egypt, although not all are recorded.

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Copyright: Palmer and Reeder