Tumoral calcinosis

Tumoral calcinosis is an unusual clinical disorder in which large masses of calcium are deposited in the periarticular tissues of the body. The characteristic clinical, radiological and histopathological features of this disorder occurring in three middle aged female patients are reported.     

Tumoral calcinosis in scleroderma

A 36-year-old female patient with scleroderma/Sjögren's syndrome developed multiple cystic tumors on the dorsal aspect of her left hand, right elbow, and left shoulder joint two years after the onset of scleroderma. Histologically, amorphous eosinophilic substances located in subcutaneous tissue showed a strong positive reaction to PTAH and rosindole stain, and focal positive reaction to Von Kossa stain. Rheumatoid rice body like substances with chalky fluid were discharged from tumoral lesions. From these results, this case was diagnosed as tumoral calcinosis secondary to connective tissue degeneration due to the pathogenetic mechanism underlying scleroderma/Sjögren's syndrome.     

肿瘤样钙质沉着症

报告1例肿瘤样钙质沉着症.患儿男,8岁.全身结节、肿块1年余.皮肤科检杳:颈部、躯干、四肢、臀部及手足可见约百个大小不一的结节和肿块,部分融合,边界清晰,少数包块顶端破溃,流出石灰状物质.皮损组织病理检查示真皮及皮下组织大量颗粒状灶性钙沉积.诊断:肿瘤样钙质沉着症.     

Tumoral calcinosis: a pathological study of fifty-six cases

Fifty-six patients with tumoral calcinosis are presented. The lesions were located predominantly about the hip, elbows and scapula. The pathological features are described and the aetiology is discussed.     

Tumoral calcinosis: an unusual cause of cutaneous calcification

Tumoral calcinosis is an uncommon ectopic calcification syndrome characterized clinically by the presence of irregular, painless, periarticular soft tissue calcifying masses, and pathologically by fibrous-walled cystic spaces containing structureless calcific debris and associated with a variable inflammatory reaction. The pathogenesis remains obscure but the condition probably represents a disordered tissue reparative process. Of the previous literature reports, almost all have been in patients of African origin. We report a case in a white English woman.     

Tumoral calcinosis: a case report with an electron microscopic study

A 68-year-old woman developed large subcutaneous masses on her abdomen and thighs after a bruise sustained in a traffic accident. She had severe pain when sitting up straight. Histological examination revealed calcified tissues in the entire dermis of the injured areas. On electron microscopy, crystalline materials were observed in the dermis, which seemed to be formed by the deposition of hydroxyapatite on unusual proteoglycan. In a vessel wall, a thick, layered basement membrane was observed. This suggests that vascular injury and subsequent hypoxia play a role in the process of calcinosis. We performed a partial resection with good results in alleviating the patient's pain.     

Tumoral mycetoma of the buttock

BACKGROUND: Mycetomas are actinomycosic or fungal infections where the infectious agent produces grains. We report an atypical case of fungal mycetoma presenting as a tumoral formation on the buttock. CASE REPORT: A 56-year-old unemployed man from the Diourbel region of central Senegal consulted in February 1997 for a fistulalized tumor of the right buttock which had developed spontaneously and progressed for 5 years. The patient's general health remained satisfactory. Physical examination showed a voluminous 25 cm tumefaction extending from the right buttock to the perineum. The tumor showed a few areas of fistualization which discharged black grains under pressure. There was a 1.5 cm right inguinal node which did not appear to be inflammatory. The remainder of the physical examination was normal. Pathology reported inflammatory connective tissue surrounding a brownish polycyclic grain composed of spores and mycele filaments. The diagnosis of fungal mycetoma was retained and surgical excision under general anesthesia was performed. DISCUSSION: This is an atypical case of fungal mycetoma because of its tumoral form and gluteal localization. The differential diagnosis was cutaneous neurofibroma, myoma, or Darier-Ferrand dermatofibrosarcoma. The frequency of extrapodal red grain mycetomas has been pointed out by several authors from Senegal, but extrapodal black grain forms as seen in our case are more exceptional.     

家族性肿瘤样钙沉着症

家族性肿瘤样钙沉着症是一种常染色体隐性遗传性疾病,其发病与基因突变有关。家族性肿瘤样钙沉着症分为两型,高磷酸血症型和正常磷酸水平型。临床主要表现为皮肤及皮下组织、近关节软组织和（或）内脏组织的钙盐沉着,正常磷酸水平型患者具有高磷酸血症型所没有的炎症表现。特殊的临床表现结合病理活检和影像学检查可确立诊断。此病治疗药物和手术切除均可选择,但疗效欠佳。