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PubMed2005

Severe vascular disturbance in a case of familial brain calcinosis.

Here we present the first neuropathological study of a case of autosomal dominant brain calcinosis in a family followed through five generations. The 71-year-old female who came to autopsy had unusually severe and extensive bilateral brain calcifications. The process appeared to start with deposition of minute calcium-positive spheroids of less than 1 mum in diameter in capillaries that otherwise appeared normal. These could be observed extending to areas distant from the main pathology. In more advanced stages, larger spheroids completely covered some capillaries while sparing others. In heavily affected regions, ghost capillaries were observed where only calcium spheroids remained after endothelial cells and basement membranes had disappeared. Vessels of all sizes were affected, and large accretions were observed in the basal ganglia, thalamus and cerebellum. Combined scanning electron microscopy and X-ray spectrometry of these large deposits revealed a dominant presence of calcium and phosphorous, plus carbon and oxygen indicative of organic material, and small amounts of sodium, potassium, sulfur, and magnesium. Reactive astrocytes and reactive microglia accumulated around the calcified deposits, indicating a mild ongoing inflammatory process. The results suggest that severe vascular impairment and mild inflammation contribute to the slow but inexorable progression of hereditary brain calcinosis.

Aged↗
PubMed2006

Unusually diffuse idiopathic calcinosis cutis.

Calcifications in idiopathic calcinosis cutis are most commonly localized to one area. We herein report a 66-year-old woman with idiopathic calcinosis cutis who unusually exhibited widespread calcific deposits. In this report, we will also briefly discuss the pathogenesis, differential diagnosis, and current treatment of this disease.

Aged↗
PubMed1989

Idiopathic scrotal calcinosis: a possible aetiology reaffirmed.

Idiopathic scrotal calcinosis is characterised by the presence of multiple firm scrotal nodules of unknown or disputed aetiology. Only 55 cases have been reported in the literature since the first description by Lewinski (1883). We report an additional five patients. We have reviewed the literature exhaustively and our findings support the suggestion that the lesions of "idiopathic" scrotal calcinosis result simply from dystrophic calcification of epidermal cysts.

Adult↗
PubMed1989

Tumoral calcinosis with unusual dental radiographic findings.

Tumor calcinosis is a rare disease of unknown cause, manifesting itself as abnormal calcifications of cystic masses in the fibrous tissues adjacent to, but not involving, the joint spaces. A family with multiple siblings affected by tumoral calcinosis and found to have unusual dental radiographic findings consistent with varying degrees of expression of dentinal dysplasia is described.

Adolescent↗
PubMed1995

Nonfamilial tumoral calcinosis associated with chronic renal failure and secondary hyperparathyroidism: report of two cases with clinicopathological, immunohistochemical, and electron microscopic findings.

The clinical and pathological findings, including those of immunohistochemical and ultrastructural studies, of two cases of tumoral calcinosis-like lesion (TCL) are described. Both cases were associated with chronic renal failure and hyperparathyroidism. One case presented as a rapidly growing 20-cm multiloculated, cystic, calcific gluteal mass on the sacrum, which was not clinically suspected to be related to hyperparathyroidism. The other case presented as a 2.5-cm calcific mass on the right foot that recurred after surgical excision as a 7-cm mass, which was clinically presumed to be related to secondary hyperparathyroidism. Light microscopic and immunohistochemical studies on both cases and ultrastructural studies on one case indicated that the calcifying process involved histiocytes and osteoclast-like giant cells of histiocytic origin lining the cystic cavities. Hydroxyapatite crystal formation and calcification appeared to develop predominantly from intracytoplasmic membrane bound vesicles and also from mitochondria. These findings are similar to those recently reported for familial tumoral calcinosis, which support its having a mechanism of calcification comparable with that of a TCL.

Aged↗
PubMed1979

Idiopathic calcinosis of scrotum.

A forty-three-year-old man complained of painless, firm scrotal nodules which had first appeared at age sixteen. These were diagnosed clinically as multiple epidermoid inclusion cysts and were excised. Histologically they were composed of calcified, amorphous, granular material, characteristic of idiopathic calcinosis of the scrotum. This is a rare, benign condition without any recognized underlying metabolic abnormalities. The cause of idiopathic calcinosis of the scrotum is unknown, but we believe it is due to dystrophic calcification of dartoic muscles.

Adult↗
PubMed1994

Uremic tumoral calcinosis: acute hand presentations mimicking infection.

Tumoral calcinosis is an uncommon condition of the hand characterized by deposition of calcium salts in the soft tissues of the extremities. The condition may be hereditary or acquired. Acquired tumoral calcinosis, also called tumoral calcification, is a rare manifestation of renal osteodystrophy due to derangement in divalent ion metabolism. Two chronic dialysis patients with tumoral calcification of the hand are presented. These cases are unusual in their rapid onset of presentation, mimicking acute infection. Prompt recognition of the condition may allow early nonsurgical intervention to preserve function.

Calcinosis↗
PubMed1995

Cerebral calcinosis and learning disabilities in hypoparathyroidism: a cause and effect or a coincidence?

A 12-year-old child is described in whom the presentation of learning difficulties and history of previous cataract removal led to a diagnosis of primary hypoparathyroidism. Cranial computed tomography revealed extensive calcinosis, previously described only in adults with long-standing hypoparathyroidism, and neuropsychologic evaluation revealed a marked symmetrical decrease in intellectual function. The possibility of the severe complication of brain calcinosis necessitates prompt diagnosis and treatment of hypoparathyroidism.

Brain Diseases↗
PubMed1995

Successful treatment of hyperphosphatemic tumoral calcinosis with long-term acetazolamide.

We describe a patient with tumoral calcinosis, in which acetazolamide (ACZ) was, for the first time, tested for its therapeutic efficacy. The 19-year-old Japanese man had been suffering from multiple recurrent calcific masses with tenderness around the finger, knee, and toe joints since 10 months of age. Radiographs revealed several calcific subcutaneous masses around the finger joints, and calcific myelitis around the right knee joint and in the calvarium. The patient had hyperphosphatemia with elevated maximal threshold of renal phosphate excretion in the presence of normal kidney function and normocalcemia, suggesting a reduced ability to excrete phosphorus in the urine. A delay of disappearance of orally administered phosphate from the blood stream was found. A serum parathyroid hormone (PTH) level was normal, and responses to PTH and ACZ were also normal regarding the induction of phosphaturia. Since the masses tended to recur easily despite repeated surgical resections, we started medical treatment with phosphorus deprivation by oral aluminum hydroxide. However, the drug alone had no effect on hyperphosphatemia or calcific lesions, and ACZ was added in expectation of making the patient's phosphorus balance negative by its phosphaturic effect. Fourteen years of administration of the two drugs apparently improved the patient's symptoms, the biochemical findings, and the calcific lesions on radiographs. Thus, ACZ appeared to be useful for tumoral calcinosis resistant to phosphorus deprivation by aluminum hydroxide alone.

Acetazolamide↗
PubMedDate not supplied

Tumoral calcinosis-like metastatic calcification in a patient on renal dialysis.

Tumoral calcinosis-like metastatic calcification in a patient with uremia on dialysis has rarely been reported in the radiological literatures. This report describes the radiographic, scintigraphic, ultrasound, and CT findings of this condition in a 54-year-old man who had a clinical history of renal dialysis for 6 years. Elevation of serum phosphate and parathyroid hormone levels was noted. Physical examination revealed a hard, movable, and tender mass over his left arm. Plain radiographs of the left shoulder showed an amorphous massive calcification in the upper arm. Scintigraphy showed increased tracer accumulation in the soft tissue of the proximal portion of the left arm and both thighs, where CT scan revealed multilocular, amorphous, calcified masses in these areas. Scintigraphy can help in screening multiple lesions, and CT scan may further help in identifying the extent of a localized lesion such as joint involvement. Ultrasound showed localized multiloculated fluid accumulation within hyperechoic masses and perifocal interstitial fluid collection, which can help to determine the activity of the lesion. Ultrasound findings of tumoral calcinosis have not been reported before.

Arm↗
PubMed2004

Scrotal calcinosis: is the cause still unknown?

Scrotal calcinosis is a rare benign entity of calcified nodules within the scrotal skin. We describe a healthy 25-year-old man with multiple asymptomatic calcified nodules restricted to the scrotum. Histologic studies of multiple nodules showed calcium deposition, and basophilic material with sparse inflammation surrounded by a fibrous capsule. Because the intensive evaluation of our patient failed to reveal a cause, an analysis of all 123 cases found in the literature was conducted. Several plausible origins for scrotal calcinosis are disclosed; however, pathogenesis of this condition remains equivocal and controversial. The most common treatment option is excision of the affected nodules. Our report underscores the need for further investigation of this florid and bizarre disorder.

Adult↗
PubMed1995

Hyperphosphataemic tumoral calcinosis in Bedouin Arabs--clinical and radiological features.

In the first report of Bedouin Arabs with tumoral calcinosis, three Saudi Arabian male siblings and their female first cousin with the condition are described. In tumoral calcinosis the majority of cases are familial. These patients had associated hyperphosphataemia with normal renal function. The characteristic appearance of periarticular soft tissue calcified masses was present. Two of the cases developed calcific myelitis of long bones--a recognized complication of the disease. Two have widespread arterial calcification, which has not previously been described. Two cases have other unusual features; one has a calcified mass which has destroyed the blade of the scapula, whilst another developed a large tumoral mass on the posterior aspect of the knee.

Adolescent↗
PubMed2003

Tumoral calcinosis of the gluteal region in a child: case report with overview of different soft-tissue calcifications.

Tumoral calcinosis is a distinct clinical and histologic entity that is characterized by a large deposition of calcium that resembles a neoplasm and is found predominantly in adolescents and young adults in the periarticular tissues of large joints. The authors describe the clinical presentation of a 15-year-old boy admitted at our pediatric day-surgery center for surgical management of tumoral calcinosis of the left gluteal region. Complete surgical excision is the treatment of choice.

Adolescent↗
PubMed2004

[Tumoral calcinosis of the plantar forefoot: a case report and review of the literature].

Tumoral calcinosis of the plantar forefoot is an uncommon benign condition resulting from deposits of hydroxyapatite crystals in periarticular soft tissue. The pathogenic mechanism remains to be clarified. This condition is generally observed in young subjects and a hereditary pattern is often discovered. We report the clinical, radiological, and biological findings in a case of right forefoot tumoral calcinosis observed in a sixteen-year-old girl and describe the surgical treatment and pathology results. Based on this case and a review of the literature, we discuss the different pathogenic hypotheses as well as the appropriate diagnostic and therapeutic approach.

Calcinosis↗
PubMed2001

Scrotal calcinosis: is it idiopathic?

Idiopathic calcinosis of the scrotum is a rare condition, and its pathogenesis remains unknown. We recently examined 3 patients in whom scrotal epidermoid cysts in varying stages of inflammation coexisted with scrotal calcinosis. Some cysts revealed calcification of their keratin contents with little evidence of active inflammation. Other cysts showed rupture of their epithelial walls associated with the presence of keratin fibers and calcium granules in the surrounding dermis. We found that this disorder is not idiopathic and that epidermal inclusion cysts constitute the basic abnormality.

Calcinosis↗
PubMed2003

A unique presentation of calcinosis cutis in a patient with cystic fibrosis after double lung transplants.

Calcinosis cutis is the deposition of insoluble calcium salts in the skin and subcutaneous tissue. We report the case of a 28-year-old Caucasian woman with cystic fibrosis in whom strikingly symmetrical and reticulate calcinosis cutis developed on the lower extremities, which was noted on histology to spare the eccrine glands. Careful review of the literature fails to reveal any previous report with these remarkable cutaneous and histologic manifestations.

Adult↗
PubMed1988

Idiopathic calcinosis of the scrotum: histopathologic observations of fifty-one nodules.

A 29-year-old man had a 2-year history of multiple, asymptomatic, firm, subcutaneous nodules on the scrotal skin, which sometimes discharged a chalky material. Fifty-one nodules were observed in the histopathologic examination, which revealed, in addition to the typical findings of idiopathic calcinosis of the scrotum, various forms of intact cysts: epidermal (some calcified), pilar (calcified), hybrid (calcified), and indeterminate cysts with diffusely calcified keratinous content and attenuated walls. A mixture of calcified keratinous material and inflammatory infiltrates was detected, with or without remnants of the cyst wall. These findings suggest that idiopathic calcinosis of the scrotum derives from the dystrophic calcification of cysts.

Adult↗