Year : 2002 | Volume
: 36 | Issue : 4 | Page : 253--255
Tumoral Calcinosis - A case report
SMD Mujeeb, JK Jain, R Goldsmith, SS Gill
Department of Orthopaedic Surgery, Post graduate Institute of Medical Education and Research, Chandigarh, India
Department of Orthopaedic Surgery, Post graduate Institute of Medical Education and Research, Chandigarh
|How to cite this article:|
Mujeeb S, Jain J K, Goldsmith R, Gill S S. Tumoral Calcinosis - A case report.Indian J Orthop 2002;36:253-255
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Mujeeb S, Jain J K, Goldsmith R, Gill S S. Tumoral Calcinosis - A case report. Indian J Orthop [serial online] 2002 [cited 2019 Aug 18 ];36:253-255
Available from: http://www.ijoonline.com/text.asp?2002/36/4/253/35945
Tumoral calcinosis is a rare condition, characterized by localized massive accumulation of calcium phosphate, pyrophosphate and/or calcium carbonate in periarticular region in the absence of widespread vascular and visceral calcification. ,, Unfortunately, its etiopathology is poorly understood. It may be because of inborn error of phosphorus metabolism , or recurrent soft tissue micro trauma in genetically predisposed individuals. ,, It is also seen in patients with hyperphosphatemia of chronic renal failure or of prolonged hemodialysis. ,
Very few cases have been reported in the Indian literature. ,,, Most cases have been reported from African countries.  However, there is no report of comprehensive clinical pathological and radiological picture of this disease in a single patient. We are here presenting a case with its complete clinical, radiological, pathological parameters and successful management.
A 17 year old boy presented with complaints of multiple swellings, intermittent low grade fever with loss of appetite and weight. Five year ago the first swelling appeared in the region of right buttock, followed by similar swellings over the left scapula and right elbow. He was treated by a local doctor by incision and drainage of the swellings at multiple times and antitubercular drugs for one year. However, patient did not respond, rather he developed discharging sinuses on the right elbow and left shoulder.
At the time of presentation, patient was having swellings around the right elbow, left scapula, left axilla and left gluteal region with a size of 3 x 4 cm, 18 x 10cm, 8 x 6cm and 15 x 14 cm respectively. Discharging sinus was present over the right elbow and left axilla [Figure 1(a)]. All swellings were mildly tender, fluctuating masses with normal overlying skin and diffuse margins. Additionally dental caries was seen in two teeth.
Serum biochemistry including renal function test was within normal limits except persistent mild hyperphosphatemia (6.2 to 8.6 mg/dl). Serum parathyroid levels were low - - 5 ng/ml (normal 12-72 ng/ml). Casoni test was negative. FNAC of lymph node showed hyperplasia and was negative for AFB. Skin biopsy showed features of calcinosis. Large heterogenous soft tissue shadow with patchy calcification was seen in the radiograph of elbow and gluteal region [Figure 1(b)]. CT scan of scapular region and chest showed hypodense area with calcified margin in the trapezius and dense calcification involving the serratus anterior and subscapulris muscles on the left side. Ultrasonography of left buttock showed large multiple cysts of varying sizes with well defined wall. Underlying bones were not involved.Patient was put on antacid containing aluminium hydroxide 610 mg/10ml thrice a day and tablet acetazolamide 250 mg twice a day with low calcium and phosphate diet with daily antiseptic dressings. phosphate diet with daily antiseptic dressings. Subsequently about 300 ml to 500 ml viscous fluid was aspirated 5 times from the left gluteal swelling. Patient serum phosphate level decreased but lesions did not show any improvement so surgery was performed. Whole mass from the gluteal region and bursa around the elbow joints was excised with 150 ml of thick yellow fluid. Culture of the fluid was sterile on every occasion. Aspirated fluid consisted of calcium (26 mg/dl), phosphate (17.8 mg/dl) with protein (5.3 g/dl) on biochemical analysis and less than 200 cells/cc on microscopic examination. Patient had reoperation for recurrence of gluteal swelling. After one month patient was subsequently discharged from the hospital with syrup aluminium hydroxide 10 ml thrice a day and one tablet acetazolamide twice a day.
He was re-admitted after 2 years with painless globular cystic swelling of 20 x 20 cm in left gluteal region diffusely extending from greater trochanter to intergluteal fold. There was no other swelling in the body. At this admission patient had hyperphospatemia of 5.4 mg/dl. Radiograph of left hip joint shows large heterogenous soft tissue shadow with patchy calcification in margin around the gluteal area. MRI scan shows isointense lesion on T1 weighted image and bright on T2 weighted image with multiple septations. At surgery only partial excision of thick sac was possible because of its adherence to underlying muscle. About 1000 ml thick yellow fluid was drained. Histopathological examination showed large masses of calcium surrounded by giant cell reaction with fibrosis in the surrounding cyst wall [Figure 2]. Patient was again discharged from the hospital with same drugs as on last visit. At the latest follow after one year patient was asymptomatic.
Tumoral calcinosis is most commonly seen in coloured races.  While most common in second to third decade, cases have been reported from 3 months to 79 years of age. Painless, lobulated, calcified, cystic masses are located usually in the region of bursae around large joint such as hip. Elbow, shoulder and knee in decreasing order usually on the extensor aspect. ,,, the feet and hand are rarely involved. Lesions become very large, tends to ulcerate the skin and form a sinus tract that drain chalky, milk like fluid containing calcium phosphate and calcium carbonate. , Dental abnormalities  consists calcific deposits pulp chamber (pulpstones) with obliteration of pulp cavity. In addition, the roots are bulbous and short.
This is an autosomal dominant hereditary transmission with variable clinical expressibility.  Unaffected sibling may show raised level of serum phosphorous and serum 1,25 - - (OH)2 D. hyperphosphatemia and increased 1,25 vitamin D3 and low normal parathyroid hormone in patients have been reported in most of the series. ,,
These soft tissue lesion have a distinctive radiographic appearance comprising of multiple globular amorphous calcific component separated by radiolucent band.  Computerised tomography  showed variable appearance; usually mass consist of large cystic component and some high attenuation septae. Discrete thin layer of calcium outlined the wall of these cysts and most of the centre showed low attenuation. Usually there is no erosion of underlying bones. On MR imaging,  T2 weighted image showed inhomogenous signal intensity of mass but mostly high intensity despite the high calcific component. Two patterns of increased signal intensity have been observed : 1) nobular and very bright, 2) diffuse and less bright signal pattern. On T1 weighted image the masses appeared inhomogenous and showed low signal intensity.
On gross examination, it is usually a circumscribed mass with thick bands of fibrous and granulation tissue with intervening cavities full of semi-solid chalk like material.  On histopathological examination, , most of the lesions consist of multiple cystic spaces with irregular contour lined by mononuclear cells and giant cell reaction. Cyst is lined by dense hyalinized fibrous tissue and cyst lumen contain calcified material.
There are basically two methods of treatment - medical and surgical. Medical therapy consists of phosphate binding antacids, acetazolamide  (it inhibits proximal tubular reabsorption of phosphorus) and diet restriction for calcium and phosphorous.
In the literature all these methods of treatment have been tried individually without much success. Surgical excision of the swelling is usual method of treatment but recurrence is common. ,, Gregosiewicz  has tried successfully dihydroxyaluminium sodium carbonate in a dose of 900 mg per day in three divided doses for a period of 3 months with diet containing low phosphate and calcium in one patient of tumoral calcinosis without hyperphosphatemia. His patient was operated twice before putting on medical management and he had only small sizes swelling (1.52 cm). Patient responded completely within four week and swellings disappeared almost completely in size. Author has not found any recurrence even after 16 months of treatment. Mozaffrian et al  treated a patient with similar medical management.
In present case we started combination of syrup aluminium hydroxide and diet containing low amount of calcium and phosphate. Initial response showed decrease in serum phosphate level but no response in swelling. We excised the tumour because it was too big and interfering day to day activity of patient. After two year of medical treatment only one recurrence was noted in the left gluteal region. After repeat surgery we have not seen any recurrence and patient improved clinically. Hence we recommend combination of medical management by diet and drugs with surgical excision. Patient may require multiple surgeries for complete resolution.
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