Slavin R E, Wen J, Kumar D, Evans E B
Department of Pathology, University of Texas Medical Branch, Galveston.
Am J Surg Pathol. 1993 Aug;17(8):788-802.
We describe histopathologic and ultrastructural changes in tumoral calcinosis (TC) occurring in seven siblings from a single family. Tumoral calcinosis appears to be triggered by bleeding followed by aggregation of foamy histiocytes. These in turn are transformed, with participation of collagenolysis, into cystic cavities lined by osteoclast-like giant cells and histiocytes--the lesion resembling adventitious bursae. Movement and friction, forces generated from the periarticular location of the TC lesions, putatively are key to this transformation. Concomitantly, two calcifying events develop, possibly driven by concurrent hyperphosphatemia or endogenous hypervitaminosis D. One occurs on membranous fragments in antiprotease-containing large cytoplasmic vesicles within osteoclast-like giant cells and mononuclear cells lining the TC cavities; the second, in the TC locules on membranous and cellular debris derived from cavity-lining cells and erythrocytes. The TC cavities ultimately fill with calcified material, losing their synovial-like lining, become encapsulated by fibrous tissue, and ossify. Hydroxyapatite may gain entrance to capillary lumens and embolize to the lung. We conclude that TC represents a disordered reparative process that often is exaggerated because episodes of bleeding, caused by TC-induced vascular injury, provoke development of new lesions. The capricious response of TC to treatment is correlated with its morphologic features.
我们描述了一个家族中七名兄弟姐妹所患肿瘤性钙化症（TC）的组织病理学和超微结构变化。肿瘤性钙化症似乎由出血引发，随后出现泡沫状组织细胞聚集。这些组织细胞在胶原溶解的参与下，进而转变为内衬破骨细胞样巨细胞和组织细胞的囊腔——该病变类似于滑膜囊。TC病变位于关节周围，由此产生的运动和摩擦可能是这种转变的关键因素。与此同时，可能由并发的高磷血症或内源性维生素D过多症驱动，发生了两种钙化事件。一种发生在破骨细胞样巨细胞和内衬TC囊腔的单核细胞内含有抗蛋白酶的大细胞质囊泡中的膜碎片上；另一种发生在源自囊腔衬里细胞和红细胞的膜性及细胞碎片上的TC小体中。TC囊腔最终充满钙化物质，失去其滑膜样内衬，被纤维组织包裹并发生骨化。羟基磷灰石可能进入毛细血管腔并栓塞至肺部。我们得出结论，TC代表一种紊乱性修复过程，由于TC诱导的血管损伤引起的出血发作会引发新病变的发展，该过程常常被夸大。TC对治疗的反复无常的反应与其形态学特征相关。
