a 52-year - old woman was referred to our department with a growing calcified mass in the left shoulder .
she had been previously diagnosed with interstitial lung disease and progressive systemic sclerosis eight and five years prior , respectively .
four years before admission , chest computed tomography first revealed a small calcified lesion in the sub - scapular area ( fig .
the calcified lesion increased in size , eventually becoming a large tumor - like mass ( fig .
1b ) . at presentation , the patient exhibited slightly limited motion in the affected joint and complained of mild pain and tenderness when leaning against a wall .
the patient had no family history of tumoral calcinosis and no history of trauma , with the exception of a previous thoracoscopic lung biopsy of the right lower lobe . on physical examination ,
no skin lesions were noted on the body surface , but the mass was palpable on the neck and back .
laboratory test results revealed that serum calcium , phosphorus , and vitamin d values were within normal limits .
x - ray imaging of the chest revealed a large , lobulated , and trapezoidal calcific plaque extending from the tip of the left scapula to the lower neck ( fig .
when the patient was referred to us , she was being treated with a steroid ( methylprednisolone , 2 mg ) and an immunosuppressive drug ( azathioprine , 100 mg ) for interstitial lung disease
. however , the patient received no specific therapy to control the progression of the calcification in the shoulder .
although the pain and disability associated with the affected joint were not severe , we surgically excised the chest wall mass because the mass continued to grow for several years , showing a tendency for more rapid growth in the years immediately before the decision to perform a surgical excision .
this was particularly problematic because the patient was completely dependent on a wheelchair due to polio - induced paralysis , and therefore , the preservation of arm function was essential for maintaining her quality of life .
moreover , we were concerned that further growth of the tumor was likely to produce skin ulceration or compression of the brachial plexus and axillary vessels , which could potentially lead to psychological trauma to the patient and the ultimate loss of shoulder function . in the operating room , the patient was positioned in a semi - prone position , and a skin incision approximately 15 cm in length was made along the medial border of the left scapula .
the mass was located between the rib cage and the scapula , and was tightly attached to the first and second intercostal muscles .
the mass was carefully dissected without damaging the neurovascular structures and completely excised ( fig .
the patient was discharged on the sixth postoperative day without any complications . in outpatient follow - up appointments
, she appeared well , and no recurrence was observed on a computed tomography scan 10 months after the surgery ( fig . 2c ) .
calcinosis , which refers to the deposition of calcium phosphate crystals in the soft tissues , is a reasonably frequent condition in patients with rheumatic disease or crest ( calcinosis , raynaud s phenomenon , esophageal motility disorders , sclerodactyly , telangiectasia ) syndrome .
the hands and fingers are affected in approximately 80% of patients with systemic sclerosis who experience calcinosis .
the superficial soft tissues overlying bony prominences are most commonly involved ; however , periarticular , intra - articular , or muscular involvement is also possible .
the calcinosis in systemic sclerosis is typically small and limited , although less common cases involving generalized or tumor - like calcifications have recently been reported [ 35 ] .
the term tumoral calcinosis was originally used to refer to a rare hereditary disease involving phosphate metabolic dysfunction with a pattern of autosomal recessive transmission .
this disease is characterized by multilobulated , densely calcified masses that are generally located on the extensor surfaces of large joints .
however , tumoral calcinosis gradually came to include huge mass - like calcifications that are secondary to various medical conditions such as renal failure , connective tissue disease , and hormonal imbalances .
although chronic tissue inflammation and macrophage activation causing tissue devitalization seem to play an important role in the pathogenesis of calcinosis in rheumatic disease , the precise mechanism has not been fully elucidated .
a few pharmacological agents such as warfarin , colchicine , diltiazem , and minocycline are occasionally beneficial in specific clinical circumstances ; however , their success rates are variable , and no large series of controlled studies have investigated the effects of these drugs . in general , surgical excision may be required when the lesion is large and causes severe nerve pain , deformity , disability , or ulceration of the affected joints .
the present case has some limitations in terms of its ability to provide general guidance for the surgical treatment of tumoral calcinosis .
first , attention was initially paid to the dominant pulmonary pathology , whereas the growing calcification in the shoulder was ignored as a typical epiphenomenon of systemic sclerosis .
second , medical treatment was thus not utilized to treat the early stages of the calcified mass . finally , our patient was paraplegic , stemming from a prior polio infection , and preservation of the function of the affected arm was critical for the patient s quality of life . despite these limitations ,
moreover , no pharmacological agent to date has demonstrated proven efficacy in the reduction or elimination of very large calcified masses , such as the mass observed in the present case . in light of the specific details of our case , we determined that a delay in surgery to allow for a possible response to medical treatment was potentially more risky than immediate surgical excision . in a previously reported case , a patient who did not undergo surgery eventually lost the functionality of his hand and the ability to engage in his occupation . in another case ,
a patient presented with a huge ulcerated mass that had not been treated for several years ; the painful mass limited his daily life and even resulted in depression . when determining the optimal timing for surgery , it is important to consider the size of the calcified mass , the time lapse between the onset of calcification and the start of treatment , the patient s symptoms and general condition , and the presence of impending complications .
although there is a risk of recurrence and further calcification due to surgical trauma , it may be beneficial to perform surgery before the condition progresses to become a very large , painful , disabling , and ulcerated mass .
 extensive tumoral calcinosis affecting a large joint is uncommon in patients with systemic sclerosis . 
 we report the case of a 52-year - old female patient referred for a growing calcified mass in the shoulder . 
 she was diagnosed with interstitial lung disease and progressive systemic sclerosis . 
 although the pain and disability associated with the affected joint was not severe , the patient underwent surgical excision because the mass continued to grow and was likely to produce shoulder dysfunction and skin ulceration . 
 the patient appeared well 10 months after surgery with no signs of recurrence . 
 this report highlights the timing and indication of surgical excision in similar cases . 
CASE REPORT
DISCUSSION