CLINICAL IMAGING 1992;16:49-51
49
TUMORAL CALCINOSIS CAUSING BONE EROSION IN A RENAL DIALYSIS PATIENT CAROLYN CIDIS MELTZER, MD, ELLIOT K. FISHMAN, AND WILLIAM W. SCOTT, JR., MD
Tumoral calcinosis is a rare disorder manifest by large calcific periarticular masses. Associated bone destruction has been described previously only once. The radiographic and computed tomographic (CT) findings of bone erosion in a case of tumoral calcinosis in a renal dialysis patient are presented. Although the presence of bone destruction may suggest a neoplasm, it does not exclude the diagnosis of tumoral calcinosis KEY WORDS:
Calcium; Kidney, failure; Soft tissues, calcification; Computed tomography
INTRODUCTION Although recognized since 1899 (l), Inclan (2) first used the term tumoral calcinosis to describe a hard, juxta-articular mass comprised of thick connective tissue septa and cavities filled with a dense milky substance. Although classically found in patients who are otherwise healthy, tumoral calcinosis does occur in the setting of chronic renal failure (3-6). One characteristic of this benign condition is the absence of bone destruction (7). We are aware of only one report in the world literature of bone destruction associated with tumoral calcinosis (8). Here we present an unusual case of tumoral calcinosis causing bone erosion in a dialysis patient. From the Russel H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, Baltimore, Maryland. Address reprint requests to: William W. Scott, Jr., M.D., Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, 600 N. Wolfe Street, Baltimore, MD 21205.
Received July 1991; revised August 1991. 1992 by Elsevier Science Publishing Co., Inc. 655 Avenue of the Americas, New York, NY 10010 0899-7071/921$5.00
0
MD,
CASE REPORT A 7%year-old black woman on chronic maintenance hemodialysis for 7 years presented with a 6-month history of worsening left hip pain that radiated down the leg. She also complained of progressive difficulty ambulating and numbness of the anterior left thigh. A fall 8 months earlier resulted in a fracture of the right superior and inferior pubic rami. Physical exam revealed a large palpable mass in the left groin. No muscle weakness was detected. Mildly decreased sensation in the distribution of the left femoral nerve was noted. The patient experienced pain with straight leg raising. Laboratory studies included an elevated creatinine of 12.7, and phosphorus of 8.1 mg/dL. The serum calcium level was normal at 8.4 mg/dL. Radiographs of the pelvis demonstrated a large, lobulated calcific mass near the left hip (Figure 1A). Healed fractures of the right pubis and ischium were also noted. Computed tomography (CT) demonstrated the multilocular nature of the mass with fluid-fluid levels (Figure 1B) as well as erosion of the left iliac wing adjacent to the mass (Figure 2). An excisional biopsy was performed. At surgery the mass was noted to contain a toothpaste-like material and clear fluid. Multiple biopsies of the mass over an 18-month interval were all diagnostic for tumoral calcinosis with no evidence of malignancy.
DISCUSSION Metastatic calcification due to secondary hyperparathyroidism is well known in chronic renal failure; however, large tumorous masses of the type described by Inclan (2) are rare. Metastatic calcification occurs when the calcium phosphorus product is elevated, usually in the setting of an elevated phosphorus and normal or low calcium (9). In the
50
MELTZER ET AL.
CLINICAL IMAGING VOL. 16, NO. 1
resection is commonly the treatment, although recurrence following incomplete removal is not infrequent (7). Dietary reduction in phosphorus and phosphate-binding antacids have also been successfully used (11). Pathologic findings in tumoral calcinosis are characteristic and most often diagnostic. The radiologic appearance is typically that of amorphous, lobulated calcified masses occurring near joints. A “sedimentation sign” produced by fluid levels due to layering of semisolid milk of calcium, as seen in this case, is an unusual but welldescribed feature that is almost always pathognomanic (13). Bone erosion in tumoral calcinosis is very rare. osteolysis Recently, Hawass et al. (8) demonstrated of the base of the terminal phalanx in a case of tumoral calcinosis of the finger. Meneghello (14) reported periarticular subchondral bone erosion near areas of soft-tissue calcification in patients with
FIGURE 2. (A, B) CT scans define the erosion of the iliac crest adjacent to the tumoral calcinosis.
B
FIGURE 1. (A) Anteroposterior radiograph demonstrates a large lobulated calcific mass near the left hip, extending up into the iliac bone. (B) CT scan at level beneath the left lesser trochanter demonstrates the nodular appearance of the lesion with calcified thin walls separating cystic spaces containing fluid-fluid levels.
idiopathic form of tumoral calcinosis, hyperphosphatemia due to a defect in renal tubular phosphorus transport has been postulated as a causative factor (10). Correction of the hyperphosphatemia in order to lower the calcium phosphorus product is a recognized treatment (4, 11).Tumoral calcinosis in the presence and absence of renal failure is clinically, radiologically, pathologically, and chemically similar (12). Large calcific periarticular masses have also been reported in milk-alkali syndrome, hypervitaminosis D, and sarcoidosis (4). These lesions predominately occur in the areas of the hip, elbow, and scapula (7). A history of trauma is variably present (3, 7). The masses generally are slow-growing
and often
become
quite
large. Surgical
B
IANLJARY-MARCH
BONE EROSION IN TUMORAL
1992
chronic renal failure. Destruction of adjacent bone may be the result of pressure erosion due to repeated microtrauma to the bone, caused by the large size of the mass and its periarticular location. In the presence of radiographic evidence of bone destruction a neoplasm, such as chondrosarcoma, must be included in the differential diagnosis. A sedimentation sign-most often appreciated on CT-if detected, is helpful in establishing the diagnosis of tumoral calcinosis.
51
and hypercalcemia in a hemodialysis patient without hyperparathyroidism: a case report. J Nucl Med 1990;31:10991103. 7. Bishop
AF, Destouet JM, Murphy WA, Gilula LA. Tumoral and review. Skeletal Radio1 calcinosis: case report 1982;8:269-274.
8. Hawass N-E-D, Kolawole T, Ismail AH, Pate1 PJ. Tumoral calcinosis: case reports from Saudi Arabia with a review of the literature. Trop Geogr Med 1988;40:58-63. 9. Eastwood JB, Bordier PJ, DeWardner HE. Some biochemical, histological, radiological, and clinical features of renal osteodystrophy. Kidney Int 1973;4:128-140. 10. Zerwekh JE, Sanders LA, Townsend J, Pak CYC. Tumoral calcinosis: evidence for concurrent defects in renal tubular phosphorus transport and in la,25-dihydroxycholecalciferol synthesis. Calcif Tissue Int 1980;32:1-6.
REFERENCES 1. Duret MH. Tumeurs multiples et singulieres reuses. Bull Sot Anat Paris 1899;725-731. 2. Inclan A. Tumoral calcinosis.
CALCINOSIS
des bourses se-
JAMA 1943;121:490-495.
3. Barton DL, Captain MC, Reeves RJ. Tumoral calcinosis: report of three cases and review of literature. AJR 1961;86:351-358. 4. Parfitt AM. Soft-tissue calcification Med 1969;124:544-556.
in uremia. Arch Intern
5. Suzuki K, Takahashi S, Ito K, Tanaka Y, Sezai Y. Tumoral calcinosis in a patient undergoing hemodialysis. Acta Orthop Stand 1979:50:27-31. 6. Eisenberg B, Tzamaloukas AH, Hartshorne MF, Listrom MB, Arrington ER, Sherrard DJ. Periarticular tumoral calcinosis
ll.
Manaster BJ, Anderson TM. Tumoral calcinosis: serial images to monitor successful dietary therapy. Skeletal Radio1 1982;8:123-125.
12.
Bosky AL, Vigorita VJ, Sencer 0, Stuchin SA, Lane JM. Chemical, microscopic, and ultrastructural characterization of the mineral deposits in tumoral calcinosis. Clin Orthop 1983:178:258-269,
13. Gordon LF, Arger PH. Dalinka MK, Coleman BG. Computed tomography in soft tissue calcification layering. J Comput Assist Tomogr 1984;8:71-73. 14. Meneghello A, Bertoli M, Romagnoli GF. Unusual complication of soft tissue calcifications in chronic renal disease: the articular erosion. Skeletal Radio1 1980;5:251-252.
49
TUMORAL CALCINOSIS CAUSING BONE EROSION IN A RENAL DIALYSIS PATIENT CAROLYN CIDIS MELTZER, MD, ELLIOT K. FISHMAN, AND WILLIAM W. SCOTT, JR., MD
Tumoral calcinosis is a rare disorder manifest by large calcific periarticular masses. Associated bone destruction has been described previously only once. The radiographic and computed tomographic (CT) findings of bone erosion in a case of tumoral calcinosis in a renal dialysis patient are presented. Although the presence of bone destruction may suggest a neoplasm, it does not exclude the diagnosis of tumoral calcinosis KEY WORDS:
Calcium; Kidney, failure; Soft tissues, calcification; Computed tomography
INTRODUCTION Although recognized since 1899 (l), Inclan (2) first used the term tumoral calcinosis to describe a hard, juxta-articular mass comprised of thick connective tissue septa and cavities filled with a dense milky substance. Although classically found in patients who are otherwise healthy, tumoral calcinosis does occur in the setting of chronic renal failure (3-6). One characteristic of this benign condition is the absence of bone destruction (7). We are aware of only one report in the world literature of bone destruction associated with tumoral calcinosis (8). Here we present an unusual case of tumoral calcinosis causing bone erosion in a dialysis patient. From the Russel H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, Baltimore, Maryland. Address reprint requests to: William W. Scott, Jr., M.D., Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, 600 N. Wolfe Street, Baltimore, MD 21205.
Received July 1991; revised August 1991. 1992 by Elsevier Science Publishing Co., Inc. 655 Avenue of the Americas, New York, NY 10010 0899-7071/921$5.00
0
MD,
CASE REPORT A 7%year-old black woman on chronic maintenance hemodialysis for 7 years presented with a 6-month history of worsening left hip pain that radiated down the leg. She also complained of progressive difficulty ambulating and numbness of the anterior left thigh. A fall 8 months earlier resulted in a fracture of the right superior and inferior pubic rami. Physical exam revealed a large palpable mass in the left groin. No muscle weakness was detected. Mildly decreased sensation in the distribution of the left femoral nerve was noted. The patient experienced pain with straight leg raising. Laboratory studies included an elevated creatinine of 12.7, and phosphorus of 8.1 mg/dL. The serum calcium level was normal at 8.4 mg/dL. Radiographs of the pelvis demonstrated a large, lobulated calcific mass near the left hip (Figure 1A). Healed fractures of the right pubis and ischium were also noted. Computed tomography (CT) demonstrated the multilocular nature of the mass with fluid-fluid levels (Figure 1B) as well as erosion of the left iliac wing adjacent to the mass (Figure 2). An excisional biopsy was performed. At surgery the mass was noted to contain a toothpaste-like material and clear fluid. Multiple biopsies of the mass over an 18-month interval were all diagnostic for tumoral calcinosis with no evidence of malignancy.
DISCUSSION Metastatic calcification due to secondary hyperparathyroidism is well known in chronic renal failure; however, large tumorous masses of the type described by Inclan (2) are rare. Metastatic calcification occurs when the calcium phosphorus product is elevated, usually in the setting of an elevated phosphorus and normal or low calcium (9). In the
50
MELTZER ET AL.
CLINICAL IMAGING VOL. 16, NO. 1
resection is commonly the treatment, although recurrence following incomplete removal is not infrequent (7). Dietary reduction in phosphorus and phosphate-binding antacids have also been successfully used (11). Pathologic findings in tumoral calcinosis are characteristic and most often diagnostic. The radiologic appearance is typically that of amorphous, lobulated calcified masses occurring near joints. A “sedimentation sign” produced by fluid levels due to layering of semisolid milk of calcium, as seen in this case, is an unusual but welldescribed feature that is almost always pathognomanic (13). Bone erosion in tumoral calcinosis is very rare. osteolysis Recently, Hawass et al. (8) demonstrated of the base of the terminal phalanx in a case of tumoral calcinosis of the finger. Meneghello (14) reported periarticular subchondral bone erosion near areas of soft-tissue calcification in patients with
FIGURE 2. (A, B) CT scans define the erosion of the iliac crest adjacent to the tumoral calcinosis.
B
FIGURE 1. (A) Anteroposterior radiograph demonstrates a large lobulated calcific mass near the left hip, extending up into the iliac bone. (B) CT scan at level beneath the left lesser trochanter demonstrates the nodular appearance of the lesion with calcified thin walls separating cystic spaces containing fluid-fluid levels.
idiopathic form of tumoral calcinosis, hyperphosphatemia due to a defect in renal tubular phosphorus transport has been postulated as a causative factor (10). Correction of the hyperphosphatemia in order to lower the calcium phosphorus product is a recognized treatment (4, 11).Tumoral calcinosis in the presence and absence of renal failure is clinically, radiologically, pathologically, and chemically similar (12). Large calcific periarticular masses have also been reported in milk-alkali syndrome, hypervitaminosis D, and sarcoidosis (4). These lesions predominately occur in the areas of the hip, elbow, and scapula (7). A history of trauma is variably present (3, 7). The masses generally are slow-growing
and often
become
quite
large. Surgical
B
IANLJARY-MARCH
BONE EROSION IN TUMORAL
1992
chronic renal failure. Destruction of adjacent bone may be the result of pressure erosion due to repeated microtrauma to the bone, caused by the large size of the mass and its periarticular location. In the presence of radiographic evidence of bone destruction a neoplasm, such as chondrosarcoma, must be included in the differential diagnosis. A sedimentation sign-most often appreciated on CT-if detected, is helpful in establishing the diagnosis of tumoral calcinosis.
51
and hypercalcemia in a hemodialysis patient without hyperparathyroidism: a case report. J Nucl Med 1990;31:10991103. 7. Bishop
AF, Destouet JM, Murphy WA, Gilula LA. Tumoral and review. Skeletal Radio1 calcinosis: case report 1982;8:269-274.
8. Hawass N-E-D, Kolawole T, Ismail AH, Pate1 PJ. Tumoral calcinosis: case reports from Saudi Arabia with a review of the literature. Trop Geogr Med 1988;40:58-63. 9. Eastwood JB, Bordier PJ, DeWardner HE. Some biochemical, histological, radiological, and clinical features of renal osteodystrophy. Kidney Int 1973;4:128-140. 10. Zerwekh JE, Sanders LA, Townsend J, Pak CYC. Tumoral calcinosis: evidence for concurrent defects in renal tubular phosphorus transport and in la,25-dihydroxycholecalciferol synthesis. Calcif Tissue Int 1980;32:1-6.
REFERENCES 1. Duret MH. Tumeurs multiples et singulieres reuses. Bull Sot Anat Paris 1899;725-731. 2. Inclan A. Tumoral calcinosis.
CALCINOSIS
des bourses se-
JAMA 1943;121:490-495.
3. Barton DL, Captain MC, Reeves RJ. Tumoral calcinosis: report of three cases and review of literature. AJR 1961;86:351-358. 4. Parfitt AM. Soft-tissue calcification Med 1969;124:544-556.
in uremia. Arch Intern
5. Suzuki K, Takahashi S, Ito K, Tanaka Y, Sezai Y. Tumoral calcinosis in a patient undergoing hemodialysis. Acta Orthop Stand 1979:50:27-31. 6. Eisenberg B, Tzamaloukas AH, Hartshorne MF, Listrom MB, Arrington ER, Sherrard DJ. Periarticular tumoral calcinosis
ll.
Manaster BJ, Anderson TM. Tumoral calcinosis: serial images to monitor successful dietary therapy. Skeletal Radio1 1982;8:123-125.
12.
Bosky AL, Vigorita VJ, Sencer 0, Stuchin SA, Lane JM. Chemical, microscopic, and ultrastructural characterization of the mineral deposits in tumoral calcinosis. Clin Orthop 1983:178:258-269,
13. Gordon LF, Arger PH. Dalinka MK, Coleman BG. Computed tomography in soft tissue calcification layering. J Comput Assist Tomogr 1984;8:71-73. 14. Meneghello A, Bertoli M, Romagnoli GF. Unusual complication of soft tissue calcifications in chronic renal disease: the articular erosion. Skeletal Radio1 1980;5:251-252.
