Tumoral Calcinosis of the Hand

Tumoral calcinosis is characterized by the deposition of calcium phosphate in periarticular tissues. In general, it involves large joints such as the hip, knee, shoulder, and elbow. The involvement of the hand is extremely rare and only a few cases have been reported.^1-3 This rare condition may lead to diagnostic confusion and maltreatments.⁴ The following case report involves tumoral calcinosis around the third metacarpo-phalangeal joint.

Figure 1: Standard radiograph showing multiple round to oval, well-demarcated masses of calcifications, located in the periarticular soft tissue of the third metacarpo-phalangeal joint. Figure 2: CT showing calcific focci in the soft tissue around the erosive lesions.

Case Report

A 43-year-old man presented with periodic attacks of severe pain, swelling, and redness over the dorsal aspect of the third metacarpophalangeal joint of his right hand. The pain repeated every 6 months and was relieved within 10 days with self-oriented antibiotics and nonsteriodal anti-inflammatory drugs. Post-medical history included a parachuting accident 22 years earlier with no specific involvement of his hand. After a 9-year interval, a painful and erythemateous mass had appeared. Two years later, the mass had been excised and diagnosed histopathologically as a benign lesion. There was neither a history of systemic disease nor a related family history.

During his initial presentation 10 days after the last attack, physical examination displayed swelling and slight tenderness over the dorsal aspect of the third metacarpophalangeal joint, just lateral to the insertion of the extensor tendon of the third finger of his right hand. The overlying skin was intact. The mobility of the adjacent metacarpophalangeal joint was unlimited. He had no lymphangitis or regional lymphadenopathy, and the rest of his hand functioned normally, including the range of movement and neurovascular status. There were no other such lesions within the body. He was afebrile and in good general health.

A standard radiograph showed multiple round to oval, well-demarcated masses of calcifications located in the periarticular soft tissue of the third metacarpophalangeal joint. An osteolysis of the ulnar aspects of the subarticular parts of both the third metatarsal and proximal phalanx had also recently appeared (Figure 1). Computed tomography showed calcific focci in the soft tissue around the erosive lesions (Figure 2). Laboratory tests that included the erythrocyte sedimentation rate (ESR), cell counts, electrolytes (serum calcium and phosphate), and uric acid were all within the normal range, with only a slight increase in cholesterol and triglyceride levels. There was no evidence of any underlying disease such as a metabolic or endocrine abnormality or connective tissue disease. Although laboratory findings were not completely supportive, the long, periodic, and relatively benign clinical course of the disease implied that there was a crystal deposition such as gouty tophus.

Surgical excision of the calcific deposits was performed through the dorsal approach to the third metacarpophalangeal joint. The excised material was pink-yellowish and chalky (Figure 3). It consisted of 4 parts, ranging in size from 2×2×0.4 cm to 0.3×0.2×0.1 cm. The cut surfaces of the nodules were yellow-white with chalky-white granular deposits.

Figure 3: Intraoperative view of the lesion (A, B). Figure 4: Granular calcium deposits (hematoxylen-eosin 310) (A). Fibroblastic vessel-rich stroma surrounding the deposits (hematoxylen-eosin 320) (B). Brown-black staining of calcium crystals (von kossa 310) (C). Red staining consistent with calcium deposition (alizarin red 320) (D).

In order to rule out gout disease, touch preparations were prepared and examined in a fresh, unstained state under a polarization microscope. The samples were also fixed in alcohol to preserve possible uric acid crystals and processed by formalin-omitted routine tissue protocol. On histologic examination, granules and deposits of calcium were seen in the soft tissue (Figure 4A). Deposits were located in a fibroblastic vessel-rich stroma, without a surrounding foreign body giant cell reaction (Figure 4B). Calcium deposition was also confirmed by histochemical studies such as von Kossa (Figure 4C) and alizarin red (Figure 4D) stains. Excised material consisted of precipitation of calcium salts without any evidence of gouty tophus and was diagnosed histopathologically as calcinosis.

At 4-year follow-up, the patient was pain free with no evidence of recurrence or similar lesions.

Discussion

Tumoral calcinosis is an uncommon disorder characterized by large periarticular deposition of calcium phosphate that resembles a neoplasm. The first report of tumoral calcinosis was by Duret⁵ in 1899 and termed endotheliome calcifie. The term tumoral calcinosis was proposed by Inclan⁶ in 1945 and was accepted worldwide.

Since Duret’s description, more than 250 cases have been reported, mostly located around large joints.⁷ However, the involvement of the hand in tumoral calcinosis is very rare. Approximately 10 cases have been reported.^2,3 Before 1967, Harkess and Peters⁸ researched the literature and reported a total of 33 tumoral calcinosis cases, including 6 of their own. Only 1 of the 33 cases reported involved the hand. In 2006, Kamath et al⁴ reported a case of tumoral calsinosis involving the second metacarpophalangeal joint. Also in 2006, Kim et al¹ reported 2 cases involving the metacarpophalangeal joints in 3 cases of tumoral calsinosis of the hand. The third case involved the fourth proximal interphalangeal joint.

Tumoral calcinosis is usually seen on the extensor surfaces of large joints such as the hip, knee, shoulder, and elbow in adolescents and young adults. The natural course is generally slow, with the progressive enlargement from a few weeks to many years. The clinical presentation is generally painless swelling. Chalky discharge and a secondary infection may arise. The electrolyte levels of these cases are normal. Standard radiography shows the aggregation of irregularly shaped lobules of varying sizes located in periarticular soft tissue. The adjacent joint is not involved, and the joint space is maintained for a long period of time. Our case involving a 13-year history presented only recently the erosion of adjacent surfaces of the third metatarsal and proximal phalanx.

The exact etiology of calcinosis is still unknown. However, an associated molecular abnormality has been shown in some cases of familial tumoral calcinosis. The elevation of fibroblast growth factor-23 in patients with a recessive form of familial tumoral calcinosis has been recently reported.⁹ A localized soft tissue alteration is proposed in the pathogenesis because patients with tumoral calcinosis often have a single calcification with no abnormal findings of mineral homeostasis.¹ An aberrant tissue response to local trauma has been rarely reported as the cause of tumoral calcinosis.^10,11 Although there was a history of a parachuting accident in our case, the long and asymptomatic interval did not allow us to claim any relationship. Minor repetitive trauma may also serve as a trigger mechanism, leading to a chain of events. It begins with a hemorrhage, fat necrosis, fibrosis, and collagenization, then ends with collagenolysis and ultimately massive calcification.⁷

Local inflammatory findings such as painful swelling, redness, and warmth implied an infection in our patient. However, he was afebrile and in good general health. White cell counts and ESR were ranged in normal limits. Sabesta et al² and Kamath et al⁴ also reported 2 cases with infection-like symptoms in the second metacarpophalangeal joint.

Other types of calcium deposition in soft tissues must be considered in a differential diagnosis. Our case had no laboratory evidence of any underlying disease such as a metabolic or endocrine abnormality or a connective tissue disease. Acute calcific deposition is the most common periarticular inflammatory disease leading to juxta-articular deposits of calcium hydroxyapatite in the hand. Histological findings are similar to those of tumoral calcinosis. The main characteristic of acute calcific deposition is spontaneous and relatively early (<4-month) resolution of the deposition. The inflammatory symptoms resolve spontaneously within a few days to a week, and the calcific deposition disappears radiographically within a short time. A conservative wait is all that is required. Acute calcific deposition is commonly involved in the shoulder, but the involvement of the hand has been reported.¹² With a 13-year history, periodic character of symptoms and persistence of deposition, this specific case was not consistent with acute calcific deposition.

Tophaceus pseudogout, also called tumoral calcium pyrophosphate dehydrate deposition disease, is characterized by a deposition of calcium pyrophosphate dehydrate in the periarticular tissues. Dagregorio and Saint-Cast¹³ reported a case with tumoral calcium pyrophosphate dehydrate deposition disease similar to the localization of our case. Tophaceus pseudogout is usually seen in elderly patients rather than middle-aged patients, as in our case. A crystalline appearance under a polarizing microscope is a characteristic of tophaceus pseudogout. In our case, tophaceus pseudogout was eliminated by the histological findings of excised material.

Finally, soft tissue chondromas are commonly seen in the hand, especially in middle-aged adults. These tumors are usually solitary and may adhere to tendons or joint capsules, but soft tissue chondromas, as in our case, can be easily differentiated by histological findings.

Surgical excision in tumoral calcinosis is the mainstream treatment; recurrence is rare. Kirk and Simon¹⁴ and Harkess and Peters⁸ reported the recurrence of tumoral calcinosis around the shoulder and elbow. These recurrences may have originated from incomplete excision. Researchers advocate meticulous excision. In our case, the reason for failure of the previous excision may have been an incomplete surgery.

Tumoral calcinosis should be considered in differential diagnoses of patients with painful masses in the hand. Its cure is possible with early and complete surgical excision.

References

1. Kim HS, Suh JS, Kim YH, Park SH. Tumoral calcinosis of the hand: three unusual cases with painful swelling of small joints. Arch Pathol Lab Med. 2006; 130(4):548-551.
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13. Dagregorio G, Saint-Cast Y. Tumoral calcium pyrophosphate dihydrate crystal deposition in the fifth metacarpophalangeal region. Orthopedics. 2006; 29(2):161-162.
14. Kirk TS, Simon MA. Tumoral calcinosis. Report of a case with successful medical management. J Bone Joint Surg Am. 1981; 63(7):1167-1169.

Authors

Drs Ozcelik, Aydogdu, and Sur are from the Department of Orthopedics and Traumatology, and Dr Doganavsargil is from the Pathology Department, Ege University Hospital, Izmir, Turkey.

Drs Ozcelik, Aydogdu, Doganavsargil, and Sur have no relevant financial relationships to disclose.

Correspondence should be addressed to: Semih Aydogdu, MD, Ege University Medical School Department of Orthopedic Surgery and Traumatology, Bornova, 35100, Izmir, Turkey.