New method of local adjuvant therapy with bicarbonate Ringer’s solution for tumoral calcinosis: A case report

BACKGROUND Tumoral calcinosis is a condition characterized by deposits of calcium phosphate crystals in extra-articular soft tissues,occurring in hemodialysis patients.Calcium phosphate crystals are mainly composed of hydroxyapatite,which is highly infilt-rative to tissues,thus making complete resection difficult.An adjuvant method to remove or resolve the residual crystals during the operation is necessary.CASE SUMMARY A bicarbonate Ringer’s solution with bicarbonate ions(28 mEq/L)was used as the adjuvant.After resecting calcium phosphate deposits of tumoral calcinosis as much as possible,while filling with the solution,residual calcium phosphate deposits at the pseudocyst wall can be gently scraped by fingers or gauze in the operative field.A 49-year-old female undergoing hemodialysis for 15 years had swelling with calcium deposition for 2 years in the shoulders,bilateral hip joints,and the right foot.A shoulder lesion was resected,but the calcification remained and early re-deposition was observed.Considering the difficulty of a complete rection,we devised a bicarbonate dissolution method and excised the foot lesion.After resection of the calcified material,the residual calcified material was washed away with bicarbonate Ringer’s solution.CONCLUSION The bicarbonate dissolution method is a new,simple,and effective treatment for tumoral calcinosis in hemodialysis patients.

Review of tumoral calcinosis: A rare clinico-pathological entity

Tumoral calcinosis(TC) has long been a controversial clinico-pathological entity. Its pathogenesis and genetic background have been gradually unravelled since its first description in 1943. According to the presence or absence of an underlying calcifying disease process, TC has been divided into primary and secondary varieties. Two subtypes of the primary variety exist; a hyperphosphatemic type with familial basis represented by mutations in Gal NAc transferase 3 gene(GALNT3), KLOTHO or Fibroblast growth factor 23(FGF23) genes, and a normo-phosphatemic type with growing evidence of underlying familial base represented by mutation in SAMD9 gene. The secondary variety is mainly associated with chronic renal failure and the resulting secondary or tertiary hyperparathyroidism. Diagnosis of TC relies on typical radiographic features(on plain radiographs and computed tomography) and the biochemical profile. Magnetic resonance imaging can be done in difficult cases, and scintigraphy reflects the disease activity. Treatment is mainly surgical for the primary variety; however, a stage-oriented conservative approach using phosphate binders, phosphate restricted dietsand acetazolamide should be considered before the surgical approach is pursued due to the high rate of recurrences and complications after surgical intervention. Medical treatment is the mainstay for treatment of the secondary variety, with failure warranting subtotal or total parathyroidectomy. Surgical intervention in these patients should be kept as a last resort.

Recuperation of severe tumoral calcinosis in a dialysis patient: A case report

BACKGROUND One of the common late sequela in patients with end-stage renal disease(ESRD)is the calcium phosphate disorder leading to chronic hypercalcemia and hyperphosphatemia causing the precipitation of calcium salt in soft tissues.Tumoral calcinosis is an extremely rare clinical manifestation of cyst-like soft tissue deposits in different periarticular regions in patients with ESRD and is characterized by extensive calcium salt containing space-consuming painful lesions.The treatment of ESRD patients with tumoral calcinosis manifestation involves an increase in or switching of renal replacement therapy regimes and the adjustment of oral medication with the goal of improved hypercalcemia and hyperphosphatemia.CASE SUMMARY We describe a 40-year-old woman with ESRD secondary to IgA-nephritis and severe bilateral manifestation of tumoral calcinosis associated with hypercalcemia,hyperphosphatemia and tertiary hyperparathyroidism.The patient was on continuous ambulatory peritoneal dialysis and treatment with vitamin D analogues.After switching her to a daily hemodialysis schedule and adjusting the medical treatment,the patient experienced a significant dissolution of her soft tissue calcifications within a couple of weeks.Complete remission was achieved 11 mo after the initial diagnosis.CONCLUSION Reduced patient compliance and subsequent insufficiency of dialysis regime quality contribute to the aggravation of calcium phosphate disorder in a patient with ESRD leading to the manifestation of tumoral calcinosis.However,the improvement of the treatment strategy and reinforcement of patient compliance enabled complete remission of this rare disease entity.

Calcinosis circumscripta in a captive African cheetah(Acinonyx jubatus)

This article reports a first case of calcinosis circumscripta in a captive African cheetah(Acinonyx jubatus).Histopathology demonstrated well defined multiple cystic structures containing granular,dark basophilic materials with peripheral granulomatous reaction,characterized by presence of multinucleated giant cells surrounded by a varying amounts of fibrous connective tissues.Special staining with von Kossa revealed black stained deposits confirming the presence of calcium salts.

Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. We report the favorable outcome of rituximab on severe calcinosis in 4 JDM patients and present their clinical data. Patients and Methods: A retrospective chart review of 4 children with JDM and severe calcinosis who received rituximab for relapsing or polycyclic JDM course. Diagnosis and follow up of calcinosis was clinically and by X-ray. Review data included: age of patients at onset of JDM symptoms and diagnosis, clinical and laboratory criteria at diagnosis, disease course and duration of follow up. Data about calcinosis onset, sites, severity and its progression were also included. Further data about rituximab therapy included: dosage, side effects, other treatment used before, during or after this drug and outcome and duration of follow up of calcinosis after therapy. Results: 4 patients (2 male, 2 female), interval between onset of symptoms and diagnosis was 6 - 12 months, course of JDM was polycyclic or relapsing, duration of follow up was 5 - 7 years. Calcinosis was severe causing ulceration, recurrent skin infections and joint limitation. It was not improving despite treatment with different DMARDs and/or bisphosphonates, colchicine and warfarin. Reason to start rituximab was inadequate disease control with conventional DMARDs. All patients received steroids and more than one DMARD before starting rituximab and were continued thereafter, follow up after rituximab was 3 to 5 years. All patients had improvement in disease activity and frequency of admission especially due to complications of calcinosis. One patient had complete clearance of calcinosis for the last 5 years. Others had significant improvement in calcinosis with no new lesions, decreased sites and density and decreased calcinosis related contractures. There were no serious side effects to rituximab. Conclusion: Our study showed the favorable effect of rituximab in treatment of calcinosis in 4 patients with JDM-associated severe calcinosis when it was used with other conventional DMARDs.

Iatrogenic calcinosis cutis in a child affected by Acute Lymphoblastic Leukemia

Iatrogenic calcinosis cutis is a rare disorder that can be due to the intravenous administration of calcium or phosphate-containing infusions such as calcium gluconate or calcium chloride with extravasation. Fortunately, the course of calcinosis cutis is benign in immunocompetent children. The treatment remains supportive therapy. After about 6 months, there is no evidence of tissue calcification. We describe a 4-year-old girl with Acute Lymphoblastic Leukemia (ALL) who developed severe calcinosis cutis in the left humerus after extravasation of calcium gluconate during the treatment for the tumor-lysissyndrome-related hypocalcaemia. Surgical debridement and local wound care were not successful, and so a temporary suspension of chemotherapy was necessary to achieve complete healing of the lesion. Notwithstanding this complication, her ALL is in complete hematological remission after 8 months from the diagnosis. No functional or sensitive impairment due to the cutis has persisted.

Dystrophic Calcinosis in the Hands of a Patient with Rheumatoid Arthritis and Secundary Sjogren’s Syndrome

Salts of calcium phosphate and inorganic phosphate are normally found in serum and extracellular fluids, balancing through poorly understood factors that prevent abnormal tissue deposition of these minerals. However, in those tissues that are injured, especially due to chronic inflammatory processes, a predisposition to the deposition of these minerals is developed, triggering what has been called Dystrophic Calcinosis (DC), common in different Connective Tissue Diseases (CTD), especially dermatomyositis and scleroderma, but there is no a frecuent association with diseases like Rheumatoid Arthritis (RA) and Sj?gren Syndrome (SS). We report a case of a female patient of 63 years old with RA and Secundary SS who presents with DC in the hands and no evidence of other connective tissue.

Calcinosis cutis in a young man with dermatomyositis

A 25-year-old man with known dermatomyositis was admitted to the University of Leipzig Medical Centre for the first time.The diagnosis was originally made abroad years ago.Current laboratory studies showed elevated C-reactive protein and creatine kinase.Antinuclear antibodies,anti-Mi-2 antibodies,anti-Jo-1 antibodies,and anti-Scl-70 antibodies were negative,and the rheumatoid factor was positive.Nailfold video capillaroscopy(NVC)revealed numerous arborized(or“bushy”)capillaries reflecting dermatomyositis-typical neoangiogenesis.Physical examination showed extensive calcinosis cutis on arms,legs,and trunk.

From variome to phenome:Pathogenesis,diagnosis and management of ectopic mineralization disorders

Ectopic mineralization- inappropriate biomineralization in soft tissues- is a frequent finding in physiological aging processes and several common disorders, which can be associated with significant morbidity and mortality. Further, pathologic mineralization is seen in several rare genetic disorders, which often present life-threatening phenotypes. These disorders are classified based on the mechanisms through which the mineralization occurs: metastatic or dystrophic calcification or ectopic ossification. Underlying mechanisms have been extensively studied, which resulted in several hypotheses regarding the etiology of mineralization in the extracellular matrix of soft tissue. These hypotheses include intracellular and extracellular mechanisms, such as the formation of matrix vesicles, aberrant osteogenic and chondrogenic signaling, apoptosis and oxidative stress. Though coherence between the different findings is not always clear, current insights have led to improvement of the diagnosis and management of ectopic mineralization patients, thus translating pathogenetic knowledge(variome) to the phenotype(phenome). In this review, we will focus on the clinical presentation, pathogenesis and management of primary genetic soft tissue mineralization disorders. As examples of dystrophic calcification disorders Pseudoxanthoma elasticum, Generalized arterial calcification of infancy, Keutel syndrome, Idiopathic basal ganglia calcification and Arterial calcification due to CD73(NT5E) deficiency will be discussed. Hyperphosphatemic familial tumoral calcinosis will be reviewed as an example of mineralization disorders caused by metastatic calcification.

Panniculitis,an Uncommon Skin Manifestation in Adult Dermatomyositis

Objective:The skin manifestations of dermatomyositis(DM)are complex and diverse,with some rashes having diagnostic significance,while others have a certain suggestive effect on the patient’s condition,visceral damage,and prognosis.This case-cohort study was performed to investigate the skin manifestations of 205 adult inpatients with DM.We then analyzed the association between the clinical characteristics and panniculitis in adult patients with DM.Methods:This study was conducted at the First Medical Center of PLA General Hospital,Beijing,China from March 1,2016 to December 30,2020 with follow-up until December 31,2021.This case-cohort study involved 205 eligible participants with DM who met the criteria for idiopathic inflammatory myopathy.Clinical data,cutaneous manifestations,and lesion locations were screened and analyzed.Serum samples were collected,and immunoblotting was used to detect 16 types of myositis-specific antibodies and myositis-associated antibodies.Results:The results showed that 16(7.8%)of the 205 adult patients with DM had panniculitis.The panniculitis was mainly distributed in the subcutaneous fat of the abdomen,buttocks,and thighs.This condition was often accompanied by skin ulcers,atrophic scars,and calcinosis.Moreover,9(72.7%)of the 16 patients with panniculitis were positive for antibodies to nuclear matrix protein 2(anti-NXP2 antibodies).Conclusion:The data showed that panniculitis is an uncommon skin manifestation in adult patients with DM,with a positivity rate of 7.8%.The appearance of panniculitis is likely associated with anti-NXP2 antibodies.

Classification of breast microcalcifications: radiological-pathological correlation

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.

Detection of multiple clustered microcalcifications by mammography following breast-conserving surgery

Breast cancer is the most common cause of cancer related death among women around the world. Each year, 1.1 million more women are diagnosed with it, representing more than 10% of all new cancer cases annually. With more than 410 000 deaths each year, the disease accounts for more than 1.6% of all female deaths worldwide. Prompt diagnosis and appropriate treatment may increase life expectancy and recovery rate.