Abstract

Surgical excision is the mainstay of treatment. It should be undertaken early as it is difficult to remove a larger lesion and will necessitate more extensive surgical procedure. Recurrence after surgical excision is a problem, but can be managed by repeat excision.

Case Report

A 22-year-old housewife, who was apparently healthy, presented with complaints of severe pain and swelling over the second web space of seven days duration. She noticed swelling seven days back associated with pain, which worsened rapidly in next five days. There were no other such lesions in the body. Patient reported to a local general physician who suspected infection and referred the patient to us. The pain was severe enough to prevent her using the hand. But surprisingly, the patient was afebrile and in good general health.Atfirst instance it appeared to be infection but the absence of fever, and general good health raised suspicion in our mind. There were no signs of tenosynovitis, palmer space infection or flexor sheath infection. There was no regional lymphadenopathy. Movements at the metacarpo-phalangeal joint were possible, but range was decreased. Movements within the range possible were painless. There was no history of similar complaint in the past. There was no family history of similar problem.

Radiograph of the hand showed multiple round to oval, well-demarcated masses of calcification, located in the peri-articular soft tissue of the second metacarpo- phalangeal joint. The calcified masses appeared lobulated and seemed to consist of a conglomeration of multiple small and round opacities, unattached to bone. The joint was essentially normal with smooth articular margin andgoodjointspace.All the bloodinvestigationsincluding the ESR and cell counts were within the normal range and there was no evidenceof infection in the blood tests. Calcium, phosphorus & alkaline phosphatase levels also were normal.Surgical excision of the calcific deposits was done though the dorsalapproach to the second metacarpo-phalangealjoint. The calcific masses were hard and attached to the deeper tissues; theylayclosetothe tendon sheath of the flexortendons and at places were infiltrating it. On sectioning, the mass showed yellowish pasty calcareous material and there was a gritty sensation. There was no involvement of the metacarpo-phalangeal joint. We could excise the whole calcified mass withtedious dissection. Till two years of regular follow up, there was no evidence of any recurrence or similar lesions in any other part of the body.

Figure 1. Preoperative photographs of the patient showing the swelling in the region of the second web space.

Figure 2. Anteroposterior and lateral radio- graph of the hand showing the round to oval lobulated masses of calcification in the second web space.

Figure 3. Intraoperative photograph of thepatient showing the calcified masses.

Discussion

First report ofTumoralcalcinosiswas by Duret in 1899.⁴ He described a 17-year-old girl who had calcific tumoroftheelbowand buttocks and called it as‘endotheliomecalcifie’.Teutschlander (1935) described a case andthoughtthat the calcificationfollowedfat necrosis andcalledthelesion‘calciumtumor’ due to lipocalcinogranulomatosis.⁴ Other terminologies, which have beenassigned to this obscure condition, are: Calcifying bursitis; Calcifying Collagenolysis; Kikuyu bursa,⁵ but the most widely accepted terminology is ‘Tumoral Calcinosis’. It was Inclav in 1943, who first introduced the term ‘Tumoral Calcinosis’,³ to describe the deposition of prominent calcified masses overlying large joints, such as the hips, elbow and shoulders. He reported 3 cases in Negro boys.

Two theories have been put forward for its occurrence⁵-inborn error of phosphorus metabolism and mechanical trauma or repeated minor injuries. The disease is thought to be a result of metaplasia of connective tissue cells and not of disturbed calcium or phosphorus metabolism.⁶ The disease has a genetic background and the primary defect is in the collagen, which undergoes calcification.⁵

Tumoral calcinosis occurs in younger patients, affects multiple joints and has a familial tendency. Sites of preference¹ are lateral and superior aspect of shoulder, posterior aspect of elbow and laterally about the hips and buttocks. Less common sites are feet, retroscapular, acromioclavicular sacral and ischial region. It may occasional involve neck. Very rare sites are hand and distal femur. There are plenty of Tumoral calcinosis cases reported in the literature but hand involvement is only rarely reported, moreover isolated hand involvement is extremely rare.

Barton¹reported three cases of tumoral calcinosis but none of the cases hadinvolvement of the hand. Laffert⁷ reported 18 patients of tumoral calcinosis. One patient had involvement ofthewrist but none had handinvolvement.Smitand Schmaman⁸ reported two cases with extensive involvement but none had hands involved. Harkess 4 reviewed the literature before 1967 and reported total of 33 cases including six of his own. Only one patient out of 33 had involvement of the hand. Baldursson⁶ reportedfourcasesin a single family but hand involvement was absent in all. Hacihanefioglu⁹ reported 11 definite and 9 probable cases of tumoral calcinosis. Only one patient outofallhad involvement of finger. Malik and Acharya¹⁰ (1993) reported two cases of tumoral calcinosis involving the fingers. Sabesta, Kamineni and Dumont¹¹ in 2000 reported a case of idiopathic tumoral calcinosis of the index finger. Murali, Matsui and Nakamura¹² (2001) reported an interesting case of tumoral calcinosis with bilateral involvement of index fingers in a five-year-old child. Kuhlmann, Rambani, Dhillon and Aggarwal reportedacaseof multiple para-articular calcific masses involving the elbow, hand,back,kneeand the feet. They stressed that the distal location of the swellings in their case was an unusual feature. Mimoun and Baux¹³ (Article in French) in 2004 reportedacase of erosive type of tumoral calcinosis affecting the long finger.

Tumoral calcinosis classically has a very benign clinicalcourse.Itpresentsas asymptomatic and slowly growing masses. Its acute presentation, which mimics infection, is very atypical. Sabesta, Kamineni and Durmont11 reported a case of finger involvement, which appeared like infection and posed a diagnostic problem. Our case also presented as infection, but the general good health and normal blood investigations prompted us to look for other cause. All the serological investigations werenormal in the presented case.

Differential diagnosis of tumoral calcinosis includes: calcinosis universalis; calcinosis circumscripta; vitamin-D intoxication; Burnett’ssyndrome;renalosteodystrophy and pseudogout. It can be differentiated from the deposits in Pseudogout by the nature of crystals on X-ray, which are lobular in Tumoral calcinosis but granular and more delicate in Pseudogout. X-ray diffraction analysis is confirmatory.

The calcium deposits are characteristically peri-articualar (1-20 cm or more in size). They are dense, irregular and have a round or oval contour. Radiolucent fibrous septa separate clumps of calcium, givinga multinodular appearance. There are no bony abnormalities.

Pathology

Multilocular structure with fibrous connective tissue bands separating pasty, calcareous deposits. On microscopy macrophages, multinucleated giant cells and chronic inflammatory cells lining the fibrous bands are noted.

Complications are rare, buttheremay be ulceration of the overlying skin with secondary infection, fistula formation and discharge of yellow-white chalky fluid.

Surgical excision is the mainstay of treatment. It shouldbeundertakenearly as it is difficult to remove a larger lesion and will necessitate more extensive surgical procedure. Recurrence after surgical excision is a problem, but can be managed by repeat excision.

Summary

A Case of Tumoral Calcinosis affecting metacarpo-phalangeal joint region has been described. This is a very rare sitefor Tumoral calcinosis, which is classically known to affect larger joints. Most interesting thing about the case presented is its mode of presentation. The onset was very acute and at presentation it appeared tobe infection. Such an unusual presentation of tumoral calcinosis at an extremely rare site like can pose a diagnostic confusion. This report is to make hand surgeons aware of this unusual presentation of tumoral calcinosis in hand.

References

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