PMC4531958_01
(A) Intraoral view of the left side of the mandible. Note buccal expansion of the tumor witn pinkish yellow surface. (B) Computerized tomography finding of the tumor. Note the soft tissue mass protruding from the middle portion of the left mandible.
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At the beginning of the physical examination, a walnut-sized, pinkish, hard, round, protruded mass was found on the lateral side of the left lower molar teeth (Fig. 1A). The computerized tomography of the oral cavity tumor revealed an ovoid heterogenous mass protruding from the ramus of the left mandible (Fig. 1B).
Histologic sections of the iliac bone biopsy specimen. (A) Light microscopic examination (Goldner's modified Masson's trichrome stain, x100) demonstrating a small amount of mineralized bone surrounded by a large amount of unmineralized osteoid. (B) Fluorescent microscopic examination showing little tetracycline labelling throughout the whole mineralization fronts.
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The section of the undecalcified bone demonstrated a small amount of mineralized bone surrounded, by a large amount of unmineralized osteoid (Fig. 2A). Tetracycline labelling showed little labelling throughout the whole mineralization fronts, suggesting very severe mineralization defect (Fig. 2B).
Changes of the biochemical and hormonal changes during the preoperative period of medical treatment and the postoperative period.
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D3, in the form of either 1alpha-hydroxyvitamin D3 or 1,25-dihydroxyvitamin D3, was tried for 5 weeks before removal of the tumor. 1alpha-hydroxyvitamin D3 (Onealfa, Teijin Ltd., Japan) was administered orally at a daily dose of 20mug for 3 weeks with 3.0g of daily oral sodium phosphate for a week, and then she was off all medications for a week. 1,25-dihydroxyvitamin D3 (Rocaltrol, Roche, Switzerland) was given orally again at a daily dose of 10mug for two weeks without any symptomatic improvement. During the treatment, serum phosphorus concentration and TRP remained below the normal range, whereas the concentration of serum calcium increased (Fig. 3A, B). While serum alkaline phosphatase activity did not change significantly, serum osteocalcin concentration increased (Fig. 3C, D). The concentration of plasma 25-hydroxyvitamin D3 remained in the normal range and the level of plasma 1,25-dihydroxyvitamin D3 increased transiently only after administration of 1,25-dihydroxyvitamin D3 (Fig. 3E, F). The plasma PTH concentration decreased to the upper limit of the normal range (Fig. 3G), but the levels of serum calcitonin (Fig. 3H) remained in the normal range without any significant changes. B). Both of the serum alkaline phosphatase activity and serum osteocalcin level dropped sharply (Fig. 3C, D), but the plasma 1,25-dihydroxyvitamin D3 level slightly increased (Fig. 3F). The plasma 1,25-dihydroxyvitamin D3 concentration steadily increased until the third postoperative week, whereas the plasma 25-hydroxyvitamin D3 level remained in the normal range (Fig. 3E, F). The plasma PTH concentration transiently increased for four weeks after removal of the tumor (Fig. 3G).
Histologic sections of the mixed connective tissue tumor (H & E, x100). Note the woven bone surrounded by the primitive spindle cells.
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In some areas, there were prominent vascularities and some foci of fibrous lesion composed of spindle cells disposed in whorled storiform patterns (Fig. 4).