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Uremic tumoral calcinosis: Acute hand presentations mimicking infection
Uremic Tumoral Calcinosis: Acute Hand Presentations Mimicking Infection Gregory F. Asuncion, MD, Chris D. Tzarnas, MD, FACS, Darby, Pennsylvania Tumoral calcium Acquired
condition
of the hand
salts in the soft tissues of the extremities.
calcinosis
The condition
tumoral
osteodystrophy with
tumoral
is an uncommon calcinosis,
also called
due to derangement calcification
onset
of presentation,
allow
early nonsurgical
of the hand
mimicking intervention
tumoral
in divalent acute
calcification,
infection.
Acute manifestations in the hand of metabolic divalent ion disorders are not commonly encountered. Tumoral calcinosis is an unusual condition characterized by soft tissue deposition of calcium salts.’ This may occur throughout the body, usually affecting periarticular regions. Involvement of the hand is infrequent .?-’ Different diagnostic names have been applied to numerous conditions resulting in dystrophic calcification, and the term tumoral calcinosis has also been applied to several different disease conditions with similar clinical presentations. A common cause of soft tissue calcification occurs as a variant of renal osteodystrophy. Tumoral calcifications are believed to be related to the solubility product of calcium and phosphate, although parathyroid hormone may enhance the accumulation of calcium in the soft tissues.8 An awareness of this condition may promote early diagnosis, allowing simple symptomatic relief without surgery. Two chronic hemodialysis patients with tumoral calcinosis of the hand are presented, illusFrom Mercy Catholic Medical Center, Darby, Pennsylvania. Received for publication Dec. 29, 1992; accepted in revised
form March 1I, 1994. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Chris D. Tzamas. MD, 1501 Lansdowne Avenue, Suite 206. Darby, PA 19023-1333.
by deposition
may be hereditary Two chronic
dialysis
These cases are unusual
Prompt function.
recognition (J Hand
of
or acquired.
is a rare manifestation
ion metabolism.
are presented.
to preserve
characterized
of renal patients
in their
rapid
of the condition
may
Surg 1994;
19A:809-812.)
trating the pitfalls in diagnosis and management that may occur. These cases are of particular interest because of the rapid onset of signs and symptoms, mimicking acute infection.
Case Reports Case 1 A 58-year-old woman on chronic hemodialysis awaiting transplantation was admitted for uremic pericarditis. During her hospital course, the proximal phalanx of the left thumb became swollen (Fig. 1). The thumb was painful and the swelling decreased joint motion. Firm nodular masses were also noted in the right elbow and shoulder. Antibiotic treatment had been initiated, before obtaining x-ray films, for a presumed infection of the thumb, and the patient was temporarily removed from the transplant list. X-ray films of the left hand revealed extensive soft tissue calcification of the proximal portion of the thumb without involvement of the joint (Fig. 2). Similar calcifications were seen in the right elbow and shoulder. The serum calcium was 11 (normal 9-11 mg/DL) and serum phosphorus was 7 (normal, 3-6 mg/DL), with a calculated calcium-phosphorus solubility product of 78. Serum uric acid level was 6 (normal, 2-6 mg/DL). Serum parathyroid hormone levels were normal. The Journal
of Hand
Surgery
809
810
Asuncion
and Tzarnas
/ Uremic
Tumoral
Calcinosis
Figure 1. Preoperative view of the soft tissue mass of the thumb due to tumoral calcification. The patient underwent surgical debulking of the soft tissue mass for symptomatic relief and to improve function of the thumb. Calcifications resembling the consistency of toothpaste and confined to the soft tissues were encountered on excision. No encapsulation was noted. Complete removal of all calcium deposition was not attempted as it was interspersed throughout the soft tissues. Resolution of symptoms resulted. Case 2 A 51-year-old woman on chronic hemodialysis presented with acute, painful soft tissue swelling of the ring fingers of both hands involving the distal and middle segments. This had developed acutely over 3 days, and antibiotic therapy had been initiated by the patient’s family physician without symptomatic improvement. Several soft, tender, erythematous masses of the digits of both hands were noted on physical examination during consultation. X-ray films demonstrated multiple calcifications within the periarticular soft tissue without joint involvement (Fig. 3).
Figure 2. Case 1. X-ray film demonstrating soft tissue calcifications. Note joint preservation.
Serum calcium was 11 mg/DL and serum phosphorus 7 mg/DL, with a calcium-phosphorus solubility product of 77. The nodules were aspirated with an 18 gauge needle. A creamy toothpaste-like material was withdrawn with immediate relief of symptoms (Fig. 4). Despite medical therapy consisting of a phosphaterestricted diet and phosphate-binding compounds, new calcifications developed 5 months later, again acutely. The recurrence was attributed, in part, to poor patient compliance with phosphorus restriction and phosphate binders. Needle aspiration was repeated. The patient eventually underwent successful renal transplantation with no further recurrence. Discussion Acute soft tissue calcification may occur with the derangement of calcium and phosphorus metabolism. The process of abnormal calcium deposition in various organs in rivu is varied, and the hypotheses to explain the phenomenon are specula-
The Journal
of Hand
Surgery
i Vol. 19A No. 5 September
Figure 4. Case 2. Needle aspiration moral calcifications of the finger.
Figure 3. Case 2. X-ray film of the hand demonstrating uremic tumoral calcinosis.
tive. Precipitation of calcium-phosphate salts within the soft tissues is thought to occur when the solubility product of calcium and phosphorus reaches a critical value, generally greater than 70. Proposed factors that predispose to precipitation include an increase in the calcium-phosphorus product, the degree of secondary hyperparathyroidism, level of blood magnesium, degree of acidosis, and presence of local tissue injury.’ An elevated vitamin D level in renal dialysis patients has also been suggested as an etiology.’ The differential diagnosis may include other conditions that affect calcium and phosphorus metabolism, such as familial tumoral calcinosis, calciphylaxis. vitamin D intoxication, primary or secondary hyperparathyroidism, and milk-alkali syndrome. Soft tissue calcifications are also observed in gout.
1994
811
of acute uremic tu-
calcinosis circumscripta or universalis. and myositis ossificans. Three distinct types of soft tissue calcification have been described in patients with end-stage renal failure: (I) calcification of medium-sized arteries, (2) tumoral calcifications, and (3) visceral calcifications. Extensive vascular calcifications are sometimes associated with ischemic skin lesions and the syndrome of calciphylaxis.“‘,” TUITIOrdl calcifications of renal osteodystrophy are clinically similar to the rare condition of familial tumoral calcinosis. The soft tissue calcium-salt deposition that may occur in renal osteodystrophy has been called tumoral calcification in most studies. A more precise term is tumoral calcinosis. denoting deposition of calcium salts in various tissues of the body.” A more accurate description is to consider whether the condition is acquired, as in renal osteodystrophy, or familial, as in the hereditary form of tumoral calcinosis.lj.14 On physical examination, the lesions of tumoral calcinosis (or tumoral calcification) are sometimes characterized as hard, chalky, calcific deposits
812
Asuncion
and Tzarnas
/ Uremic
Tumoral
Calcinosis
within the limits of subdermal tissue. They may also present as soft masses with a creamy, toothpastelike consistency. They may enlarge, sometimes quite rapidly as in the cases presented. With continued growth, ulceration into the overlying skin may occur, with possible secondary infection. In longstanding cases, a dense fibrous capsule may surround the lesion. X-ray films are distinctive. Soft tissue calcification is seen extrinsic to adjacent joints, bone erosion is absent, and joint spaces are maintained. Usually, large joints are involved, including the hip, elbow, and shoulder, and then hands and feet (in order of decreasing frequency). Laboratory studies usually reveal normal serum calcium and parathyroid hormone levels in the presence of an elevated serum phosphorus level. Tumoral calcifications may be reversed by phosphate restriction, with a lowering of the calciumphosphate product in plasma.15 Recurrence is common as treatment of the underlying metabolic derangement has been disappointing. Patients with secondary hyperparathyroidism should undergo parathyroidectomy. Renal transplantation for dialysis patients is curative for uremic tumoral calcinosis. However, calciphylaxis has been observed, even following successful renal transplantation.“” The cases presented are unusual because of involvement of the hand, an uncommon site for tumoral calcinosis. This presentation in renal dialysis patients illustrates the pathogenic role of acquired hyperphosphatemia with a resultant increased calcium-phosphorus product. For our two patients, the distinction of tumoral calcinosis from an infectious process was important, not only for correct diagnosis, but also to avoid delay in transplantation. Prompt recognition of this condition may permit aspiration of rapidly enlarging lesions. Once the nodules have become established, the surrounding inflammatory response within the
soft tissues precludes aspiration and surgical decompression is required for symptomatic relief.
References Brenner BM, Rector FC Jr. The kidney. 4th ed. Phila-
4.
5.
6. 7. 8.
9.
10.
11.
12. 13. 14. 15.
delphia: Saunders, 1991;2036-115. Knowles S, DeClerck G, Anthony P. Tumoral calcinosis. Br J Surg 1983;70: 105-7. Quarles L, Murphy G, Econs M, Martinez S, Lobaugh B, Lyles K. Uremic tumoral calcinosis: preliminary observations suggesting an association with aberrant vitamin D homeostasis. Am J Kidney Dis 1991; 18:706-10. Viegas S, Evans B, Calhoun J, Eng M, Goodwiller S. Tumoral calcinosis: a case report and review of the literature. J Hand Surg 1985;10A:744-8. Black J, Sladek G. Tumoral calcinosis in the foot and hand-a case report. J Am Podiatry Med Assoc 1983; 73: 153-5. Bogumill G, Lloyd R. Tumoral calcinosis in multiple digits: a case report. J Hand Surg 1985;10A:739-43. Gan K. Tumoral calcinosis: a case report and review of the literature. Br J Plast Surg 1982;35: 177-80. Mitnick P, Goldfarb S, Slatopolsky E. Lemann J, Gray R, Agus Z. Calcium and phosphate metabolism in tumoral calcinosis. Ann Intern Med 1980:92:482-7. Zerwekh J, Sanders L. Townsend J, Pak C. Tumoral calcinosis: evidence for concurrent defects in renal tubular phosphorus transport and in la, 25dihydroxycholecalciferol synthesis. Calcif Tissue Int 1980;32:1-6. Gipstein RM, Coburn JW, Adams DA et al. Calciphylaxis in man. Arch Int Med 1976;136: 1273-80. Khafif RA, DeLima C, Silverberg A, Frankel R. Calciphylaxis and systemic calcinosis. Arch Int Med 1990;150:956-9. Dorland’s Illustrated Medical Dictionary. 24th ed. Philadelphia: Saunders, 1965. 235. Inclan A. Tumoral calcinosis. JAMA 1943;121:490-5. Duret M. Tumeurs multiples et singulieres des bourses sereuses. Bull Sot Anat Paris 1899;74:725. Mozaffarian G, Lafferty F, Pearson 0. Treatment of tumoral calcinosis with phosphorus deprivation. Ann Int Med 1972;77:741-5.
condition
of the hand
salts in the soft tissues of the extremities.
calcinosis
The condition
tumoral
osteodystrophy with
tumoral
is an uncommon calcinosis,
also called
due to derangement calcification
onset
of presentation,
allow
early nonsurgical
of the hand
mimicking intervention
tumoral
in divalent acute
calcification,
infection.
Acute manifestations in the hand of metabolic divalent ion disorders are not commonly encountered. Tumoral calcinosis is an unusual condition characterized by soft tissue deposition of calcium salts.’ This may occur throughout the body, usually affecting periarticular regions. Involvement of the hand is infrequent .?-’ Different diagnostic names have been applied to numerous conditions resulting in dystrophic calcification, and the term tumoral calcinosis has also been applied to several different disease conditions with similar clinical presentations. A common cause of soft tissue calcification occurs as a variant of renal osteodystrophy. Tumoral calcifications are believed to be related to the solubility product of calcium and phosphate, although parathyroid hormone may enhance the accumulation of calcium in the soft tissues.8 An awareness of this condition may promote early diagnosis, allowing simple symptomatic relief without surgery. Two chronic hemodialysis patients with tumoral calcinosis of the hand are presented, illusFrom Mercy Catholic Medical Center, Darby, Pennsylvania. Received for publication Dec. 29, 1992; accepted in revised
form March 1I, 1994. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Chris D. Tzamas. MD, 1501 Lansdowne Avenue, Suite 206. Darby, PA 19023-1333.
by deposition
may be hereditary Two chronic
dialysis
These cases are unusual
Prompt function.
recognition (J Hand
of
or acquired.
is a rare manifestation
ion metabolism.
are presented.
to preserve
characterized
of renal patients
in their
rapid
of the condition
may
Surg 1994;
19A:809-812.)
trating the pitfalls in diagnosis and management that may occur. These cases are of particular interest because of the rapid onset of signs and symptoms, mimicking acute infection.
Case Reports Case 1 A 58-year-old woman on chronic hemodialysis awaiting transplantation was admitted for uremic pericarditis. During her hospital course, the proximal phalanx of the left thumb became swollen (Fig. 1). The thumb was painful and the swelling decreased joint motion. Firm nodular masses were also noted in the right elbow and shoulder. Antibiotic treatment had been initiated, before obtaining x-ray films, for a presumed infection of the thumb, and the patient was temporarily removed from the transplant list. X-ray films of the left hand revealed extensive soft tissue calcification of the proximal portion of the thumb without involvement of the joint (Fig. 2). Similar calcifications were seen in the right elbow and shoulder. The serum calcium was 11 (normal 9-11 mg/DL) and serum phosphorus was 7 (normal, 3-6 mg/DL), with a calculated calcium-phosphorus solubility product of 78. Serum uric acid level was 6 (normal, 2-6 mg/DL). Serum parathyroid hormone levels were normal. The Journal
of Hand
Surgery
809
810
Asuncion
and Tzarnas
/ Uremic
Tumoral
Calcinosis
Figure 1. Preoperative view of the soft tissue mass of the thumb due to tumoral calcification. The patient underwent surgical debulking of the soft tissue mass for symptomatic relief and to improve function of the thumb. Calcifications resembling the consistency of toothpaste and confined to the soft tissues were encountered on excision. No encapsulation was noted. Complete removal of all calcium deposition was not attempted as it was interspersed throughout the soft tissues. Resolution of symptoms resulted. Case 2 A 51-year-old woman on chronic hemodialysis presented with acute, painful soft tissue swelling of the ring fingers of both hands involving the distal and middle segments. This had developed acutely over 3 days, and antibiotic therapy had been initiated by the patient’s family physician without symptomatic improvement. Several soft, tender, erythematous masses of the digits of both hands were noted on physical examination during consultation. X-ray films demonstrated multiple calcifications within the periarticular soft tissue without joint involvement (Fig. 3).
Figure 2. Case 1. X-ray film demonstrating soft tissue calcifications. Note joint preservation.
Serum calcium was 11 mg/DL and serum phosphorus 7 mg/DL, with a calcium-phosphorus solubility product of 77. The nodules were aspirated with an 18 gauge needle. A creamy toothpaste-like material was withdrawn with immediate relief of symptoms (Fig. 4). Despite medical therapy consisting of a phosphaterestricted diet and phosphate-binding compounds, new calcifications developed 5 months later, again acutely. The recurrence was attributed, in part, to poor patient compliance with phosphorus restriction and phosphate binders. Needle aspiration was repeated. The patient eventually underwent successful renal transplantation with no further recurrence. Discussion Acute soft tissue calcification may occur with the derangement of calcium and phosphorus metabolism. The process of abnormal calcium deposition in various organs in rivu is varied, and the hypotheses to explain the phenomenon are specula-
The Journal
of Hand
Surgery
i Vol. 19A No. 5 September
Figure 4. Case 2. Needle aspiration moral calcifications of the finger.
Figure 3. Case 2. X-ray film of the hand demonstrating uremic tumoral calcinosis.
tive. Precipitation of calcium-phosphate salts within the soft tissues is thought to occur when the solubility product of calcium and phosphorus reaches a critical value, generally greater than 70. Proposed factors that predispose to precipitation include an increase in the calcium-phosphorus product, the degree of secondary hyperparathyroidism, level of blood magnesium, degree of acidosis, and presence of local tissue injury.’ An elevated vitamin D level in renal dialysis patients has also been suggested as an etiology.’ The differential diagnosis may include other conditions that affect calcium and phosphorus metabolism, such as familial tumoral calcinosis, calciphylaxis. vitamin D intoxication, primary or secondary hyperparathyroidism, and milk-alkali syndrome. Soft tissue calcifications are also observed in gout.
1994
811
of acute uremic tu-
calcinosis circumscripta or universalis. and myositis ossificans. Three distinct types of soft tissue calcification have been described in patients with end-stage renal failure: (I) calcification of medium-sized arteries, (2) tumoral calcifications, and (3) visceral calcifications. Extensive vascular calcifications are sometimes associated with ischemic skin lesions and the syndrome of calciphylaxis.“‘,” TUITIOrdl calcifications of renal osteodystrophy are clinically similar to the rare condition of familial tumoral calcinosis. The soft tissue calcium-salt deposition that may occur in renal osteodystrophy has been called tumoral calcification in most studies. A more precise term is tumoral calcinosis. denoting deposition of calcium salts in various tissues of the body.” A more accurate description is to consider whether the condition is acquired, as in renal osteodystrophy, or familial, as in the hereditary form of tumoral calcinosis.lj.14 On physical examination, the lesions of tumoral calcinosis (or tumoral calcification) are sometimes characterized as hard, chalky, calcific deposits
812
Asuncion
and Tzarnas
/ Uremic
Tumoral
Calcinosis
within the limits of subdermal tissue. They may also present as soft masses with a creamy, toothpastelike consistency. They may enlarge, sometimes quite rapidly as in the cases presented. With continued growth, ulceration into the overlying skin may occur, with possible secondary infection. In longstanding cases, a dense fibrous capsule may surround the lesion. X-ray films are distinctive. Soft tissue calcification is seen extrinsic to adjacent joints, bone erosion is absent, and joint spaces are maintained. Usually, large joints are involved, including the hip, elbow, and shoulder, and then hands and feet (in order of decreasing frequency). Laboratory studies usually reveal normal serum calcium and parathyroid hormone levels in the presence of an elevated serum phosphorus level. Tumoral calcifications may be reversed by phosphate restriction, with a lowering of the calciumphosphate product in plasma.15 Recurrence is common as treatment of the underlying metabolic derangement has been disappointing. Patients with secondary hyperparathyroidism should undergo parathyroidectomy. Renal transplantation for dialysis patients is curative for uremic tumoral calcinosis. However, calciphylaxis has been observed, even following successful renal transplantation.“” The cases presented are unusual because of involvement of the hand, an uncommon site for tumoral calcinosis. This presentation in renal dialysis patients illustrates the pathogenic role of acquired hyperphosphatemia with a resultant increased calcium-phosphorus product. For our two patients, the distinction of tumoral calcinosis from an infectious process was important, not only for correct diagnosis, but also to avoid delay in transplantation. Prompt recognition of this condition may permit aspiration of rapidly enlarging lesions. Once the nodules have become established, the surrounding inflammatory response within the
soft tissues precludes aspiration and surgical decompression is required for symptomatic relief.
References Brenner BM, Rector FC Jr. The kidney. 4th ed. Phila-
4.
5.
6. 7. 8.
9.
10.
11.
12. 13. 14. 15.
delphia: Saunders, 1991;2036-115. Knowles S, DeClerck G, Anthony P. Tumoral calcinosis. Br J Surg 1983;70: 105-7. Quarles L, Murphy G, Econs M, Martinez S, Lobaugh B, Lyles K. Uremic tumoral calcinosis: preliminary observations suggesting an association with aberrant vitamin D homeostasis. Am J Kidney Dis 1991; 18:706-10. Viegas S, Evans B, Calhoun J, Eng M, Goodwiller S. Tumoral calcinosis: a case report and review of the literature. J Hand Surg 1985;10A:744-8. Black J, Sladek G. Tumoral calcinosis in the foot and hand-a case report. J Am Podiatry Med Assoc 1983; 73: 153-5. Bogumill G, Lloyd R. Tumoral calcinosis in multiple digits: a case report. J Hand Surg 1985;10A:739-43. Gan K. Tumoral calcinosis: a case report and review of the literature. Br J Plast Surg 1982;35: 177-80. Mitnick P, Goldfarb S, Slatopolsky E. Lemann J, Gray R, Agus Z. Calcium and phosphate metabolism in tumoral calcinosis. Ann Intern Med 1980:92:482-7. Zerwekh J, Sanders L. Townsend J, Pak C. Tumoral calcinosis: evidence for concurrent defects in renal tubular phosphorus transport and in la, 25dihydroxycholecalciferol synthesis. Calcif Tissue Int 1980;32:1-6. Gipstein RM, Coburn JW, Adams DA et al. Calciphylaxis in man. Arch Int Med 1976;136: 1273-80. Khafif RA, DeLima C, Silverberg A, Frankel R. Calciphylaxis and systemic calcinosis. Arch Int Med 1990;150:956-9. Dorland’s Illustrated Medical Dictionary. 24th ed. Philadelphia: Saunders, 1965. 235. Inclan A. Tumoral calcinosis. JAMA 1943;121:490-5. Duret M. Tumeurs multiples et singulieres des bourses sereuses. Bull Sot Anat Paris 1899;74:725. Mozaffarian G, Lafferty F, Pearson 0. Treatment of tumoral calcinosis with phosphorus deprivation. Ann Int Med 1972;77:741-5.