La calcinose tumorale chez un patient hémodialysé chronique : intérêt de l’imagerie hybride tomographie d’émission monophotonique/tomodensitométrie

IntroductionTumoral calcinosis is a rare benign disease, defined by the presence of calcified deposits in periarticular tissues. It can be hereditary or secondary at chronic renal failure at the stage of dialysis. This work illustrates the contribution of single-photon emission computed tomography (SPECT/CT) in the diagnosis and management of tumoral calcinoses in a chronic hemodialysis patient, based on a clinical case.Case reportA 62-year-old patient, chronic hemodialysis since 24 years, presented a mechanical pain shoulders, knees and hips with limitation of joint mobility. The clinical exam found a mass of soft tissue in the buttocks. The radiological exam showed the presence of periarticular calcifications with no bone involvement. The SPECT/CT revealed a multifocal tumoral calcinosis affecting shoulders, elbows, wrists, hips and knees, associated with alveolar and abdominal calcinosis.Discussion and conclusionTumoral calcinosis is a distinct clinicopathological entity characterised by periarticular soft tissue calcium deposits. The SPECT/CT is important in the diagnosis, the assessment of extension and monitoring of tumoral calcinosis after treatment.     

Calcinosis in rheumatic diseases

BACKGROUND: Calcinosis, or dystrophic soft-tissue calcification, occurs in damaged or devitalized tissues in the presence of normal calcium/phosphorus metabolism. It is often noted in the subcutaneous tissues of connective tissues diseases--primarily systemic lupus erythematosus, scleroderma, or dermatomyositis--and may involve a relatively localized area or be widespread. The calcinotic accumulations may lead secondarily to muscle atrophy, joint contractures, and skin ulceration complicated by recurrent episodes of local inflammation and infection. OBJECTIVES: To review the classification, pathogenesis, clinical features, and treatment of calcinosis in rheumatic diseases. METHOD: A MEDLINE search of articles from 1972 to 2004 was conducted utilizing the index word "calcinosis" with the coindexing terms "scleroderma," "lupus," "dermatomyositis," and "dystrophic calcification." RESULTS: Calcinosis may be the source of both pain and disability in connective tissue disease patients. Illustrative cases of patients with severe calcinosis are described. The literature available was critically reviewed. While warfarin, colchicine, probenecid, bisphosphonates, diltiazem, minocycline, aluminum hydroxide, salicylate, surgical extirpation, and carbon dioxide laser therapies have been used, no treatment has convincingly prevented or reduced calcinosis. CONCLUSIONS: Calcinosis is common in the conditions reviewed and a number of agents have been used for treatment. However, the approach to calcinosis management is disorganized, beginning with the lack of a generally accepted classification and continuing with a lack of systematic study and clinical therapeutic trials.     

Classification of breast microcalcifications: radiological-pathological correlation

Background Microcalcifications play a very important role in detection of breast cancer, especially early stage breast cancer. However, ambiguity still exists in understanding the relationship between radiological and pathological characteristics of microcalcifications. The definitive indication of a biopsy has not been established. The purpose of this study is to evaluate the relationship of classification of breast microcalcifications using full-field digital mammography to the pathological characteristics. Methods For all the women an open biopsy had been conducted. One hundred and three mammographs showing clustered microcalcifications from 98 consecutive patients were reviewed along with their pathological records. To investigate the value of each criterion for the detection of cancer, univariate and multivariate analyses were performed on the entire sample and then on morphological subgroups.Results Pathological examination showed 67 malignant lesions (65.05%) and 36 benign lesions (34.95%). In the univariate analysis, four radiological variables were significant: morphological type (P=0.001), complicated by a mass (P=0.002), number of microcalcifications per cluster (P=0.02) and linear or triangular distribution of clusters (P=0.009). In the multivariate analysis, two criteria remained significant: morphological type (P<0.001) and complicated by a mass (P=0.001). The percentage of malignancy was 37.0%, 60.0%, 78.8%, and 88.9%, respectively, for type 2 (regularly punctiform), type 3 (dusty), type 4 (irregularly punctiform) and type 5 (vermicular) microcalcifications (Le Gal’s classification). The malignancy was 78.6% for microcalcifications complicated by a mass and 48.9% without a mass. The difference was significant (P<0.05). The relationship between morphological types of microcalcifications and the pathological characteristics was also studied. In subgroups, type 3 (dusty) microcalcifications complicated by a mass (P=0.001) or with the number of microcalcifications more than 10 (P=0.024); and type 2 (regularly punctiform) with a diameter of the area over 20 mm (P=0.024) or complicated by a mass (P=0.025) were statistically significant as criteria for malignant tumour. Conclusions Most cases of microcalcifications of type 4 or 5; type 3 complicated by a mass or with the number of microcalcifications more than 10; type 2 complicated by a mass or with a diameter of the area over 20 mm; are indicative of cancer. Open biopsy is recommended to acquire definitive pathological diagnosis for these cases. For the remainder of the morphological types, stereotaxic biopsy or followup should be considered.     

Two cases of dystrophic calcinosis cutis in burn scars

Dystrophic calcinosis cutis is defined as the abnormal deposition of insoluble calcium salts in dead or degenerated cutaneous tissues in the absence of abnormal serum calcium or phosphate concentrations. Although dystrophic calcification can occur in various diseases, its occurrence on a burn scar has rarely been reported in the dermatologic literature. Herein we describe two patients who presented with a solitary non-healing ulcer in a postburn scar, with histopathologic evidence of calcium deposition in the dermis.     

Excessive myocardial calcinosis in a chronic hemodialyzed patient

Summary Secondary oxalosis in chronic hemodialyzed patients is caused by impaired renal excretion and inadequate removal of oxalic acid during hemodialysis. Ascorbic acid is a precursor of oxalic acid. We report a parathyroidectomized patient with chronic renal failure, on hemodialysis, who received over a period of several months a total dose of 91.0 g ascorbic acid i.v. The plasma oxalic acid level in this patient was 14-fold higher than in healthy persons. Increased oxalic acid synthesis from its precursor ascorbic acid may be responsible for hyperoxalemia, high content of oxalic acid in myocardium, aorta and lung, and calcium oxalate deposition in soft tissues. Application of high doses of ascorbic acid should be avoided in hemodialysed patients with chronic renal failure.Abbreviations PTH parathyroid-hormone - RDT regular dialysis treatment     

钙化灶在超声诊断乳腺肿块中的意义

【目的】探讨钙化灶在超声诊断乳腺肿块中的价值。【方法】检查 2 5 6例乳腺肿块 ,其中乳腺癌 130例 (A组 ) ,乳腺良性肿块 12 6例 (B组 )。使用配备高频线阵探头 (12MHz和 10MHz)的电脑彩色超声仪 ,注意观察乳腺肿块中的钙化灶。所有病例诊断均经病理检查证实。【结果】在乳腺癌组和乳腺良性肿块组中钙化灶的检出率分别为 5 2 %和 9.5 % ,两者比较差异有非常显著性意义 (P <0 .0 1)。乳腺癌组以微小钙化灶最多 ,占76 % (5 2例 ) ,微小钙化灶在乳腺良性肿块中仅占 33% (4例 ) ,两组差异亦有非常显著性意义 (P <0 .0 1)。【结论】超声检出乳腺肿块内钙化灶有助于对肿块良恶性的判断 ;密集分布的微小钙化灶常提示乳腺癌。     

Tumoral calcinosis of the scalp: An unusual site for a rare tumor

Tumoral calcinosis is a rare calcifying disorder that is associated with deposition of calcium crystals in the periarticular tissues. The mass is most often around the hips, elbows, shoulders, and feet but may be occasionally found elsewhere. We report a case of multiple sporadic tumoral calcinoses in an adult male over the scalp. The scalp as a site of tumoral calcinosis has not been previously reported in adults. Previous surgical excisions done on two occasions had resulted in recurrence of the tumors. This report highlights the need to include tumoral calcinosis in the differential diagnosis of tumors of the scalp.     

A failure of mucocutaneous lymphangiogenesis may underlie the clinical features of lipoid proteinosis

Lipoid proteinosis (LiP) (OMIM 247100) is a rare autosomal recessive disease caused by loss of function mutations in the extracellular matrix protein 1 gene, ECM1, on chromosome 1q21. LiP is characterized clinically by hoarseness in early infancy, followed by waxy papules and plaques on the face and body along with pox-like and acneiform scars. We studied a 20-year-old Japanese woman with LiP. She was born of consanguineous parents. Biopsy specimens obtained from a nodule on the elbow were used for histopathology, immunohistology and electron microscopy. Exons 6 and 7 of ECM1 were amplified by polymerase chain reaction (PCR) from genomic DNA from the proband, her parents, her brother and an unrelated person. PCR products were sequenced to detect the mutation. Histopathological examination revealed an irregular mass of calcium beneath deposits of a hyaline material in the dermis. Immunofluorescence double staining showed that the CD31-positive microvascular density was increased but that staining for the lymphatic-specific hyaluronan receptor LYVE-1 was drastically diminished in lesional compared with nonlesional skin of the patient and with normal skin. Electron microscopy revealed marked concentric reduplication of basal laminae not only around blood vessels but also around solitary dermal cells positive for Weibel-Palade bodies scattered in the hyaline material. Sequencing of the PCR products revealed a homozygous frameshift mutation, 507delT, in exon 6. This led to a premature stop codon 23 bp downstream. The results of immunopathological and ultrastructural characterization suggest that a failure of mucocutaneous lymphangiogenesis may underlie the clinical features of LiP. Identification of mutation 507delT in a Japanese patient with LiP further supports the thesis that this mutation represents a recurrent mutation in ECM1 in patients with LiP. To our knowledge, this case represents the first report of calcinosis cutis occurring in LiP.     

The clinical utility of 99<Superscript>m</Superscript>Tc-HMPAO SPECT in Fahr’s disease

Fahr's disease is a rare neurodegenerative syndrome, characterized by massive symmetrical intracerebral calcifications of the basal ganglia, dentate nuclei of the cerebellum, and the adjacent parenchyma. Computerized tomography (CT) is considerably more sensitive to detect these intracranial calcifications than other imaging modalities. The clinical, CT scan, and 99(m)Tc-D,L-hexamethylpropylene amine oxime (99(m)Tc-HMPAO) brain perfusion single-photon emission computerized tomography (SPECT) findings in a 42-year-old woman with Fahr's disease are reported, and the clinical utility of 99(m)Tc-HMPAO SPECT findings in Fahr's disease is discussed in this article. In conclusion, 99(m)Tc-HMPAO brain perfusion SPECT seems to be useful in the clinical approach to Fahr's disease, and may provide more specific and clinically relevant information when compared with anatomical imaging.