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Imaging Diagnosis

Tumoral calcinosis has a distinctive radiographic pattern (Figs. 29.2-29.11). It may be first recognized as a small discrete calcified nodule or nodules but the size is variable; some will be large and more definitely lobulated tumors (e.g., Fig. 29.6). The lobulated appearance, and the conglomeration of multiple small opacities separated from each other by thin linear radiolucent lines, results from the characteristics of the tumor. The radiolucencies are the fibrovascular septa which divide the tumor masses into loculi. The calcification may have a lacy appearance or may be linear or beaded. The density of the calcification is variable within each tumor and between different tumors. Some tumors will be extremely dense, whereas others are faint and less well defined. (The pattern of some tumors has been likened to that of the fragmented and sclerotic epiphysis seen in osteochondrosis of the femoral head.)

Tumoral calcinosis virtually never affects an adjacent joint. One patient had calcification extending into a knee joint. An African patient had the carpal tunnel syndrome and was found to have a thin "fleck" of calcification in the wrist preoperatively.

Patchy calcifications have occurred in the medullary cavity in the mid-third of the tibia and femur in some patients, associated with a concentric periosteal reaction which could easily be mistaken for infection or neoplasm. Such bone lesions are uncommon and are associated with activity on bone scintigraphy. Dural and diploic calcification has been seen in the skull, again with increased radionuclide uptake. This also is rare in the literature, and might be coincidental.

If the patient is radiographed in the erect position or with a horizontal beam transversely, fluid levels may be seen in the calcified masses, particularly those around the scapula or in the gluteal region where the tumors are usually larger (Fig. 29.5). There is no gas within the cystic spaces; the "fluid levels" result from the sedimentation of calcium which is in suspension within the fluid (the sedimentation sign). This finding is so characteristic that an erect view should be done when tumoral calcinosis is suspected. Once seen, a definitive diagnosis can be made: no other type of soft tissue calcification shows a fluid level in a calcified mass (in the absence of a contrast examination or infection). Unfortunately, a fluid level is not found in every case.

At the elbow, there is another characteristic finding and an exception to the rule that the bone near a tumor is not affected (Figs. 29.2, 29.3). Many radiographs in published series show flattening of the lower end of the humerus posteriorly. There is no visible bone reaction, but there is change in the normal shape of the bone. Tumoral calcinosis around the elbow is frequently bilateral and the flattening of the humerus will be seen on both sides, although not necessarily symmetrically. This is so characteristic that in one patient the flattening of the humerus was demonstrated without any visible calcification in the soft tissues. There was a history of a previous "operation" and a small scar was clinically visible: the hospital where this procedure had been performed reported that a "calcified bursa" had been removed. A search for and review of the histopathology showed that this was not a calcified bursa, but tumoral calcinosis (Fig. 29.2 E).

Radiography usually provides the correct diagnosis when there is one or more of the characteristic tumors or patches of nodular calcification. If there is a fluid level, then there is no doubt and further imaging is not necessary, except to exclude other tumors if any suspicious areas are found clinically.

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Fig. 29.2A-F. Six different cases of tumoral calcinosis at the elbow. In each one the lower end of the humerus is flattened posteriorly. The extent of the flattening does not seem to be related to the size or position of the calcified tumor. This change in shape is not seen in any other bone, even when inclose apposition to large tumors. It is so typical that in E the tumor had been removed surgically about a year or more before, but review of the histology confirmed the diagnosis of tumoral calcinosis, not previously recognized.

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Fig. 29.3A-E. The appearance of tumoral calcinosis around, the elbow in five further patients.

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Fig. 29.4A-C. Tumoral calcinosis in siblings. An African girl (A,B) and her younger brother (C). Both show molding of the lower end of the humerus. Neither had any other masses at this time. The young boy has a well-marked periosteal reaction around the lower end of the humerus. This is very uncommon in African patients with tumoral calcinosis. In the authors' combined series of over 70 patients with tumoral calcinosis, these are the only siblings (as far as is known).

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Fig. 29.5 A-F. Five different cases of tumoral calcinosis around the chest wall. A A young African male with bilateral swellings over the scapulae; they caused him no pain or discomfort, but had been steadily growing over the last year or more. B The chest radiograph of the same patient. Large bilateral masses of tumoral calcinosis; there are additional masses in the left shoulder region. C A large mass posterolaterally curving around the chest wall with additional tumors close to the shoulder. D A different patient with more extensive shoulder deposits and a similar mass on the chest wall. E A more solid tumor on the chest wall. F A less heavily calcified mass which extended further and produced a larger clinical swelling than B and C. In many such cases when films are taken in the erect position fluid levels will be visible as the calcium in suspension settles within the tumor mass. This does not indicate infection, but allows the definitive diagnosis of tumoral calcinosis.

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