Oeggerli P, Maurer R, Kistler H
Institut für Pathologie, Stadtspital Triemli, Zürich.
Schweiz Med Wochenschr. 1991 Sep 21;121(38):1387-92.
Tumoral calcinosis (TC) is a rare syndrome involving ectopic calcifications in the vicinity of the large joints. In about one third of patients the disorder is familial and is associated with hyperphosphatemia, elevation of 1,25-dihydroxy-vitamin-D levels and peculiar dental lesion. TC is inherited in an autosomal-recessive manner. In a normophosphatemic male patient with alcoholic cirrhosis of the liver, TC occurred first in the thoracic wall. Seven years after excision of the first lesion, a large tumor mass around the right hip developed. Infection of the calcified masses with Staph. aureus led to extensive abscess formation, septicemia and death at the age of 64. Clinical, dental and biochemical examination of the 7 descendants of the patient revealed no constitutional signs of the disease, thus identifying our patient as a sporadic case. Clinical and pathological findings in the patient are discussed and the literature is reviewed.
肿瘤性钙化症（TC）是一种罕见的综合征，涉及大关节附近的异位钙化。约三分之一的患者病情具有家族性，与高磷血症、1,25-二羟基维生素D水平升高及特殊的牙齿病变有关。TC以常染色体隐性方式遗传。在一名患有酒精性肝硬化的正常血磷男性患者中，TC首先出现在胸壁。首次病变切除7年后，右髋周围出现一个大的肿瘤块。钙化块被金黄色葡萄球菌感染导致广泛脓肿形成、败血症，并于64岁时死亡。对该患者的7名后代进行临床、牙齿和生化检查，未发现该病的体质性体征，因此将我们的患者确定为散发病例。本文讨论了该患者的临床和病理发现并回顾了相关文献。
