Presentation
IC presents in juxta-articular tissue in a tumour-like fashion is known as idiopathic tumoral calcinosis (ITC). Recurrent trauma and chronic pressure are thought to be the triggering factors. It usually presents in adolescents and adults. [ncbi.nlm.nih.gov]
Case Presentation: A 54‐year old African American man with history of dialysis‐dependent renal failure secondary to diabetes, presented with a large, painful swelling in his right buttock. [shmabstracts.com]
Entire Body System
- Collapse
When a hydatid cyst collapses, heals, and scars, the result is an amorphous mass, quite unlike the lobulated, septate appearance of tumoral calcinosis. Fig. 29.14. Tuberculous debris around a healed tuberculous hip joint. [isradiology.org]
Skin
- Dermatitis
J Am Acad Dermat. 1995; 33:693-706. 2. Reiter N, El-Shabrawi L, Leinweber B, et al. Calcinosis cutis: Part I. Diagnostic pathway. J Am Acad Dermatol. 2011;65:1–12. 3. Yang JH, Kim JW, Park HS, Jang SJ, Choi JC. [webmedcentral.com]
[…] aldolase, AST, LDH • Electromyogram: short and small polyphasic motor units, fibrillations, acute positive waves, repetitive discharges of high frequency • Skin changes: rash in heliotrope with periorbital edema, Gottron sign (erythematous and squamous dermatitis [elsevier.es]
Musculoskeletal
- Arthralgia
Reduced joint mobility and arthralgia may be the associated features and compression of the adjoining structures may give rise to neurovascular symptoms [ 3 ]. [academic.oup.com]
[…] weight loss, lymphadenopathy and hepatosplenomegaly.23 The organs most affected in children are the skin (malar rash, photosensitivity, vascular lesions, palmar and plantar erythema, Raynaud's phenomena and annular erythema), musculoskeletal (arthritis, arthralgias [elsevier.es]
myalgia, skin thickening/hardening, sclerodactyly, Raynaud phenomenon, esophageal dysmotility, telangiectasia, & pulmonary, renal, or cardiac disease) Other features of systemic lupus erythematosus (arthralgia, myalgia, fever, malar erythema, Raynaud [ncbi.nlm.nih.gov]
- Myalgia
During this time the patient had no fever, weight loss, weakness, myalgias, pain or limitation of movement of the affected joints. She received two schemes of antibiotics without improvement. [elsevier.es]
He had general myalgia, with pain and stiffness in calves and forearms after slight activity. [bmcgenet.biomedcentral.com]
skin thickening/hardening, sclerodactyly, Raynaud phenomenon, esophageal dysmotility, telangiectasia, & pulmonary, renal, or cardiac disease) Other features of systemic lupus erythematosus (arthralgia, myalgia, fever, malar erythema, Raynaud phenomenon [ncbi.nlm.nih.gov]
- Bone Pain
The diagnosis of TIO is usually suspected when serum phosphate levels are chronically low in the setting of bone pain, fragility fractures and muscle weakness. [natureasia.com]
When there is calcific myelitis, there may be bone pain. 4. The tumors are likely to be multiple, although not necessarily occurring simultaneously. [isradiology.org]
When hyperostosis is symptomatic (i.e., causes pain, erythema, edema, and warmth), long-bone radiographs show hypermineralization of cortical bone, patchy sclerosis of the medullary cavities, diaphysitis, and marked periosteal reaction ( Figure 2C ) [ [ncbi.nlm.nih.gov]
- Myopathy
[…] disorders of glycosylation; TIEF, transferrin isoelectric focusing; ApoC-III, apolipoprotein C-III; IEF, isoelectric focusing; Ga1NAc, N-acetylgalactosamine; NeuAc, sialic acid; FTC, familial tumoral calcinosis (MIM 211900); HIBM, hereditary inclusion body myopathy [medical-dictionary.thefreedictionary.com]
Juvenile dermatomyositis is the most common juvenile idiopathic inflammatory myopathy. It is an autoimmune disease characterized by a systemic vasculopathy with myositis, progressive muscle weakness and dermatological findings. [elsevier.es]
- Musculoskeletal Pain
Firm lumps that don’t cause pain (some lumps may be tender to touch). Musculoskeletal pain in bones or joints. Diagnosis and Tests How is a tumoral calcinosis diagnosed? An orthopaedist diagnoses and treats musculoskeletal problems. [my.clevelandclinic.org]
Eyes
- Angioid Streaks of the Retina
Patients may also have dental abnormalities, vascular calcifications, cortical hyperostosis and angioid streaks of the retina. Two types of FTC have been recognised, hyperphosphatemic FTC (HFTC) and normophosphatemic FTC (NFTC). [exeterlaboratory.com]
Angioid streaks of the retina. Patients with angioid streaks of the retina should be followed by an ophthalmologist. [ncbi.nlm.nih.gov]
Hetrotrophic calcification hyperphosphatemia and angioid streaks of the retina. Am. J. Med. 31 :488, 1961 Google Scholar Copyright information © Société Internationale de Chirurgie 1989 Authors and Affiliations V. L. N. Prasad 1 K. N. Naresh 1 S. [link.springer.com]
PubMed View Article Google Scholar McPhaul JJ, Engel FL: Heterotopic calcification, hyperphosphatemia and angioid streaks of the retina. Am J Med. 1961, 31: 488-492. 10.1016/0002-9343(61)90131-0. [bmcgenet.biomedcentral.com]
Neurologic
- Seizure
Degeneration Epilepsy, Occipital Calcifications Facial Dysmorphism, Selective Tooth Agenesis, and Choroid Calcification Greenberg Dysplasia Hemorrhagic Destruction of the Brain, Subependymal Calcification, and Cataracts Hyperphosphatemia, Polyuria, and Seizures [rgd.mcw.edu]
- Stroke
In rare instances, the condition affects your blood vessels or brain, increasing your risk for heart attack, strokes and other serious health issues. The condition can also cause: Corneal calcifications or angioid streaks (red streaks) in your eyes. [my.clevelandclinic.org]
Workup
A computed tomography (CT) scan was performed as part of his workup, and a posterior cervical neck mass was detected. CT-guided fine needle aspiration was performed with an immediate cytologic interpretation of tumoral calcinosis. [ncbi.nlm.nih.gov]
X-Ray
- Soft Tissue Calcification
Soft tissue calcification associated with tumoral calcinosis is rare in the head and neck. The present case describes a 65- year-old male who had been on hemodialysis for 3 years. [ncbi.nlm.nih.gov]
Radiographically, idiopathic tumoral calcinosis demonstrates lobulated, cloudy soft-tissue calcifications near a joint (A). [jaocr.org]
- Muscle Calcification
Calcific myonecrosis (benign calcified mass in leg muscles). Calcific tendonitis. Calcinosis circumscripta (calcium deposits in the skin and tissue of the hands and feet). [my.clevelandclinic.org]
Computed tomography (CT) scan showing calcifications in an adult with HFTC including shoulder and hip soft tissue calcifications (white arrows), aortic calcification (dashed arrow), papillary muscle calcification (black arrow), and submucosal gut calcifications [ncbi.nlm.nih.gov]
Treatment
It can be considered an effective alternative treatment for tumoral calcinosis if surgical treatment is not feasible. [ncbi.nlm.nih.gov]
Nonoperative observation indications treatment of choice for non-symptomatic lesions Operative complete surgical excision indications treatment of choice for symptomatic lesions technique complete surgical excision is required to decrease the rate of [orthobullets.com]
Surgical excision of the masses has been the only partially effective treatment. [annals.org]
Prognosis
[…] milky fluid Microscopic • Nodules have large areas of amorphous or granular calcium masses • May have interspersed calcopherites • Calcium crystals exhibit hydroxyapatite • Macrophage infiltration, osteoclast-like giant cells, inflammatory cells present PROGNOSIS [tumorsurgery.org]
This sub-typing is closely related to the prognosis and to the response to treatment. References 1. Maize J, Metcalf. Metabolic Diseases of the skin. In: Elder D (editor). [ijdvl.com]
Although the pathogenesis of the calcification process in tumoral calcinosis is still controversial, surgical removal remains the mainstay treatment with a satisfactory prognosis. References 1. Weiss, SW.; Goldblum, JR. [ccmbm.com]
Outlook / Prognosis What is the outlook for someone with tumoral calcinosis? Most people with tumoral calcinosis enjoy full, active lives. It’s rare for the condition to cause serious health problems. [my.clevelandclinic.org]
Infectious complications of these lesions are associated with poor prognosis. [4] Tumoral calcinosis is a rare inherited metabolic disorder characterized by massive calcium and phosphate deposits. [5] It is rare in dialyzed patients, [6] and its pathogenesis [sjkdt.org]
Etiology
TC is a disorder of obscure etiology with typical clinical, radiologic, cytologic and histopathologic features. [ncbi.nlm.nih.gov]
TERMINOLOGY Synonyms Lipid calcinosis Calcifying collagenolysis Tumoral lipocalcinosis Definitions Extraskeletal soft tissue calcification with granulomatous response ETIOLOGY/PATHOGENESIS Etiology Theories Inborn error of phosphorus metabolism Associated [basicmedicalkey.com]
Epidemiology
Chondrocalcinosis Allowable Qualifiers English : BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications CN congenital DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]
Epidemiology Demographics more common in females and African-Americans onset in childhood or early adolescence Anatomic location around joints, primarily the hip (trochanteric bursa is most common location) > shoulder Etiology Pathophysiology unknown [orthobullets.com]
Pathophysiology
Identification of this pathophysiology extends the etiologic spectrum of hyperphosphatemic tumoral calcinosis and suggests that immunomodulatory therapy may be an effective treatment. Authors Mary Scott Roberts, Peter D. [jci.org]
The recent identification of a fusion of fibronectin and fibroblast growth factor receptor 1 (FGFR1) as a molecular driver in some tumours not only sheds light on the pathophysiology of TIO but also opens the door to a better understanding of the transcription [natureasia.com]
Epidemiology Demographics more common in females and African-Americans onset in childhood or early adolescence Anatomic location around joints, primarily the hip (trochanteric bursa is most common location) > shoulder Etiology Pathophysiology unknown [orthobullets.com]
Tumoral calcinosis revisited: pathophysiology and treatment. Rheumatol Int. 2005; 25: 55-9. Martínez S, Vogler J, Harrelson J, Lyles K. Imaging of tumoral calcinosis: new observations. Radiology. 1990; 174: 215-22. [archivo.bc.una.py]
Tumoral calcinosis revisited: pathophysiology and treatment. Rheumatol Int. 2005; 25(1):55-9. 7. Kim HS, Suh JS, Kim YH, Park SH. Tumoral calcinosis of the hand: three unusual cases with painful swelling of small joints. [oapublishinglondon.com]
Prevention
A concise review of the pertinent literature focusing on the diagnosis, treatment options, and preventive measures also is reported. This journal requires that authors assign a level of evidence to each article. [ncbi.nlm.nih.gov]
Determine whether calcitonin reduces or prevents tumor recurrence. III. Determine whether hyperphosphatemia abolishes the normal circadian pattern of serum phosphorus. IV. [clinicaltrials.gov]