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Brown tumor in secondary hyperparathyroidism causing acute paraplegia
Brown Tumor in Secondary Hyperparathyroidism Causing Acute Paraplegia
MARK E. BOHLMAN, M.D. YOUNG CHOO KIM, M.D. JAMES EAGAN, M.D. EVERETT K. SPEES, M.D., Ph.D. Baltimore,
Maryland
The first known case of a “brown tumor” associated with secondary hyperparathyroidlsm causing paraplegia is described. A 6g-year-old white woman with chronic renal failure due to hypertension was admitted for back pain, and while she was under observation, paraplegia developed. A complete block was demonstrated by myelography. Computed tomography confirmed a mass at the level of obstruction, and results of biopsy were consistent with “brown tumor.” Neuroktgic symptoms were markedly improved with high-dose corticosteroids and a debulking procedure. This entity is important to recognize because prompt treatment of the hyperparathyroidism or decompression of the tumor mass by surgical means or corticosteroid administration can provide marked improvement in symptoms. Few cases of so-called “brown tumor” of primary hyperparathyroidism presenting with acute paraplegia have been reported [l-3]. In the last two decades, the association of “brown tumors” and secondary hyperparathyroidism has been described [4,5]. We report the first known case of “brown tumor” occurring in a patient with chronic renal failure and secondary hyperparathyroidism, presenting with acute paraplegia.
CASEREPORT
From the Francis Scott Key Medical Center and John Hopkins University School of Medicine, Baltimore. Maryland. Requests for reprints should be addressed to Dr. Mark E. Bohlman, Department of Radiology, Francis Scott Key Medical Center, 4940 Eastern Avenue, Baltimore, Maryland 2 1224. Manuscript accepted May 8, 1985.
A 69-year-old white woman was admitted to the surgical service for evaluation of abdominal pain thought to be secondary to cholelithiasis. She had a past history of chronic renal failure secondary to hypertension, and cholelithiasis. While she was under observation, progressive paraplegia developed. Chest radiography demonstrated bony resorption of the distal clavicle and an enlarged cardiac configuration. On thoracic spine radiography, an eroded right pedicle at the eighth thoracic vertrebral (T8) level was noted, with generalized osteopenia. Myelography (Figure 1, left and right) and computed tomographic scanning (Figure 2) demonstrated a lytic mass lesion expanding the pedicle of the eighth thoracic vertebral body, causing compression of the spinal cord. The serum calcium level was 4.5 meq/liter and the N-terminal parathormone level was 3,910 pg/ml (normal range 230 to 630 pg/ml). The patient underwent open biopsy of the posterior elements of the eighth thoracic vertebra. The pathologic specimen consisted of multiple fragments of red-brown tissue measuring approximately 4 by 4 by 1 cm. Histologic examination and the patient’s clinical data confirmed the diagnosis of “brown tumor.” The patient was then treated with large doses of steroids (40 mg dexamethasone every six hours) for eight days, followed by a slowly tapering dose. She had progressive return of left extremity function and sensation. An extensive metastatic work-up showed no abnormalities. A plan to debulk the spinal mass lesion was postponed because of numerous medical complications, which eventually led to her death.
September
1988
The American
Journal
of Medicine
Volume
81
545
BROWN
TUMOR
IN HYPERPARATHYROIDISM
CAUSING
PARAPLEGIA-BOHLMAN
ET AL
Figure 7. Lefl and the flow of contrast and below the level vertebra (T3). Note
right, obstruction to medium from above of the third thoracic the missing pedicle
(arrow).
primary hyperparathyroidism. A review of the literature revealed no cases of “brown tumor” associated with secondary hyperparathyroidism presenting with spinal cord compression. Among the complications of long-standing renal failure is hyperplasia of the parathyroid glands and a variety of skeletal lesions described as renal osteodystrophy [3]. The pathogenesis of renal osteodystrophy is still disputed, but it is generally accepted that in chronic renal failure, an acquired resistance to the effects of vitamin D on both intestine and bone results in hypocalcemia, skeletal abnormalities, and secondary parathyroid hyperplasia. Longstanding renal failure can result in a variety of manifestations in the skeleton. These changes include osteomalacia, osteitis fibrosa, and osteosclerosis, either alone or more commonly in various combinations [3,7]. Although in the past, hyperparathyroidism secondary to renal disease was considered relatively infrequent, the course of renal failure has been modified by the advent of long-term dialysis, and secondary hyperparathyroidism with renal osteodystrophy is now being seen with greater frequency. The frequency of brown tumors cited by Katz et al [4] was (1.5 percent) in 200 patients with chronic renal failure and secondary hyperparathyroidism, and 1.7 percent by Griffiths et al [5] in 225 renal transplant recipients, this being one of the more unusual roentgenographic changes identified. Demineralization of the spine was the most com-
Any case of osteoclastic tumor of the spine is rare. Lichtenstein [6] in 1959 could find only four cases in his series, none having hyperparathyroidism. Three cases of “brown tumor” causing spinal cord compression have since been reported [ 1,2,5], all occurring in patients with
Figure 2.
Computed tomographic scan through T3 showing the expansile mass lesion eroding the pedicle.
546
September
1966
The American
Journal
of Medicine
Volume
61
BROWN
TUMOR
monly reported finding (25 percent), with resorption of the distal clavicle (13 percent) and demineralization of the skull (IO percent) also common [4]. The common sites for brown tumors, in general, are the central medullary shafts of the long bones, metacarpals, ribs, pelvis, and mandible [5]. Radiographically, brown tumors usually appear sharply demarcated and lytic, and may occur in any part of the skeleton. The overlying cortex may be thinned or expanded. Healing may result in calcification, sclerosis, disappearance of the lesion, or persistence of a cystic defect [7,8]. A single case report of multiple brown tumors in the same patient has also been reported [9]. With the increasing incidence of renal osteodystrophy in patients undergoing long-term dialysis, the diagnosis of “brown tumor” of the spine causing acute paraplegia
IN HYPERPARATHYROIDISM
CAUSING
PARAPLEGIA-BOHLMAN
ET AL
should be added to the differential list of expansile lesions of the pedicle, causing paraplegia. Therapeutic implications are obvious. Prompt cord decompression with appropriate subsequent therapy of the patient’s hyperparathyroidism may lead to return of neurologic function. In at least one reported case with lesser symptoms of cord compression, initial surgical treatment of the patient’s primary hyperparathyroidism led to regression of neurologic symptoms [3]. Systemic steroid therapy may also be useful as was shown in this case. ACKNOWLEDGMENT We would like to express our appreciation to Miss Joyce Schaefer and Mrs. Dolores Kucharek for their assistance in the preparation of this manuscript.
REFERENCES 1.
2. 3.
4.
5.
Shaw MT, Davies M: Primary hyperparathyroidism presenting as spinal cord compression. Br Med J 1968; 4: 230231. Sundaram M, Scholz C: Primary hyperparthyroidism presenting with acute paraplegia. AJR 1977; 128: 674-676. Ganesh A, John L, Kurian S: Complete recovery of spinal cord compression following parathyroidectomy. Postgrad Med J 1981; 57: 652-653. Katz Al, Hampers CL, Merrill JP: Secondary hyperparathyroidism and renal osteodystrophy in chronic renal failure. Medicine (Baltimore) 1969; 48: 333-374. Griffiths HJ, Ennis JT, Bailey G: Skeletal changes following
September
6. 7. 8.
9.
1988
renal transplantation. Diag Radio1 1974; 113: 621-626. Lichtenstein L: Bone tumors, 3rd ed. St. Louis: Mosby, 1965. Robbins SL: Pathology, 3rd ed. Philadelphia: Saunders, 1967. Steinbach HL, Gordan GS, Eisenberg E, Crane JT, Silverman S, Goldman L: Primary hyperparathyroidism: a correlation of roentgen, clinical, and pathologic features. AJR 1961; 86: 329-343. Brown TW, Genant HK, Hattner RS, Orloff S, Potter DE: Multiple brown tumors in a patient with chronic renal failure and secondary hyperparathyroidism. AJR 1977; 128: 131-134.
The American
Journal
of Medicine
Volume
81
547
MARK E. BOHLMAN, M.D. YOUNG CHOO KIM, M.D. JAMES EAGAN, M.D. EVERETT K. SPEES, M.D., Ph.D. Baltimore,
Maryland
The first known case of a “brown tumor” associated with secondary hyperparathyroidlsm causing paraplegia is described. A 6g-year-old white woman with chronic renal failure due to hypertension was admitted for back pain, and while she was under observation, paraplegia developed. A complete block was demonstrated by myelography. Computed tomography confirmed a mass at the level of obstruction, and results of biopsy were consistent with “brown tumor.” Neuroktgic symptoms were markedly improved with high-dose corticosteroids and a debulking procedure. This entity is important to recognize because prompt treatment of the hyperparathyroidism or decompression of the tumor mass by surgical means or corticosteroid administration can provide marked improvement in symptoms. Few cases of so-called “brown tumor” of primary hyperparathyroidism presenting with acute paraplegia have been reported [l-3]. In the last two decades, the association of “brown tumors” and secondary hyperparathyroidism has been described [4,5]. We report the first known case of “brown tumor” occurring in a patient with chronic renal failure and secondary hyperparathyroidism, presenting with acute paraplegia.
CASEREPORT
From the Francis Scott Key Medical Center and John Hopkins University School of Medicine, Baltimore. Maryland. Requests for reprints should be addressed to Dr. Mark E. Bohlman, Department of Radiology, Francis Scott Key Medical Center, 4940 Eastern Avenue, Baltimore, Maryland 2 1224. Manuscript accepted May 8, 1985.
A 69-year-old white woman was admitted to the surgical service for evaluation of abdominal pain thought to be secondary to cholelithiasis. She had a past history of chronic renal failure secondary to hypertension, and cholelithiasis. While she was under observation, progressive paraplegia developed. Chest radiography demonstrated bony resorption of the distal clavicle and an enlarged cardiac configuration. On thoracic spine radiography, an eroded right pedicle at the eighth thoracic vertrebral (T8) level was noted, with generalized osteopenia. Myelography (Figure 1, left and right) and computed tomographic scanning (Figure 2) demonstrated a lytic mass lesion expanding the pedicle of the eighth thoracic vertebral body, causing compression of the spinal cord. The serum calcium level was 4.5 meq/liter and the N-terminal parathormone level was 3,910 pg/ml (normal range 230 to 630 pg/ml). The patient underwent open biopsy of the posterior elements of the eighth thoracic vertebra. The pathologic specimen consisted of multiple fragments of red-brown tissue measuring approximately 4 by 4 by 1 cm. Histologic examination and the patient’s clinical data confirmed the diagnosis of “brown tumor.” The patient was then treated with large doses of steroids (40 mg dexamethasone every six hours) for eight days, followed by a slowly tapering dose. She had progressive return of left extremity function and sensation. An extensive metastatic work-up showed no abnormalities. A plan to debulk the spinal mass lesion was postponed because of numerous medical complications, which eventually led to her death.
September
1988
The American
Journal
of Medicine
Volume
81
545
BROWN
TUMOR
IN HYPERPARATHYROIDISM
CAUSING
PARAPLEGIA-BOHLMAN
ET AL
Figure 7. Lefl and the flow of contrast and below the level vertebra (T3). Note
right, obstruction to medium from above of the third thoracic the missing pedicle
(arrow).
primary hyperparathyroidism. A review of the literature revealed no cases of “brown tumor” associated with secondary hyperparathyroidism presenting with spinal cord compression. Among the complications of long-standing renal failure is hyperplasia of the parathyroid glands and a variety of skeletal lesions described as renal osteodystrophy [3]. The pathogenesis of renal osteodystrophy is still disputed, but it is generally accepted that in chronic renal failure, an acquired resistance to the effects of vitamin D on both intestine and bone results in hypocalcemia, skeletal abnormalities, and secondary parathyroid hyperplasia. Longstanding renal failure can result in a variety of manifestations in the skeleton. These changes include osteomalacia, osteitis fibrosa, and osteosclerosis, either alone or more commonly in various combinations [3,7]. Although in the past, hyperparathyroidism secondary to renal disease was considered relatively infrequent, the course of renal failure has been modified by the advent of long-term dialysis, and secondary hyperparathyroidism with renal osteodystrophy is now being seen with greater frequency. The frequency of brown tumors cited by Katz et al [4] was (1.5 percent) in 200 patients with chronic renal failure and secondary hyperparathyroidism, and 1.7 percent by Griffiths et al [5] in 225 renal transplant recipients, this being one of the more unusual roentgenographic changes identified. Demineralization of the spine was the most com-
Any case of osteoclastic tumor of the spine is rare. Lichtenstein [6] in 1959 could find only four cases in his series, none having hyperparathyroidism. Three cases of “brown tumor” causing spinal cord compression have since been reported [ 1,2,5], all occurring in patients with
Figure 2.
Computed tomographic scan through T3 showing the expansile mass lesion eroding the pedicle.
546
September
1966
The American
Journal
of Medicine
Volume
61
BROWN
TUMOR
monly reported finding (25 percent), with resorption of the distal clavicle (13 percent) and demineralization of the skull (IO percent) also common [4]. The common sites for brown tumors, in general, are the central medullary shafts of the long bones, metacarpals, ribs, pelvis, and mandible [5]. Radiographically, brown tumors usually appear sharply demarcated and lytic, and may occur in any part of the skeleton. The overlying cortex may be thinned or expanded. Healing may result in calcification, sclerosis, disappearance of the lesion, or persistence of a cystic defect [7,8]. A single case report of multiple brown tumors in the same patient has also been reported [9]. With the increasing incidence of renal osteodystrophy in patients undergoing long-term dialysis, the diagnosis of “brown tumor” of the spine causing acute paraplegia
IN HYPERPARATHYROIDISM
CAUSING
PARAPLEGIA-BOHLMAN
ET AL
should be added to the differential list of expansile lesions of the pedicle, causing paraplegia. Therapeutic implications are obvious. Prompt cord decompression with appropriate subsequent therapy of the patient’s hyperparathyroidism may lead to return of neurologic function. In at least one reported case with lesser symptoms of cord compression, initial surgical treatment of the patient’s primary hyperparathyroidism led to regression of neurologic symptoms [3]. Systemic steroid therapy may also be useful as was shown in this case. ACKNOWLEDGMENT We would like to express our appreciation to Miss Joyce Schaefer and Mrs. Dolores Kucharek for their assistance in the preparation of this manuscript.
REFERENCES 1.
2. 3.
4.
5.
Shaw MT, Davies M: Primary hyperparathyroidism presenting as spinal cord compression. Br Med J 1968; 4: 230231. Sundaram M, Scholz C: Primary hyperparthyroidism presenting with acute paraplegia. AJR 1977; 128: 674-676. Ganesh A, John L, Kurian S: Complete recovery of spinal cord compression following parathyroidectomy. Postgrad Med J 1981; 57: 652-653. Katz Al, Hampers CL, Merrill JP: Secondary hyperparathyroidism and renal osteodystrophy in chronic renal failure. Medicine (Baltimore) 1969; 48: 333-374. Griffiths HJ, Ennis JT, Bailey G: Skeletal changes following
September
6. 7. 8.
9.
1988
renal transplantation. Diag Radio1 1974; 113: 621-626. Lichtenstein L: Bone tumors, 3rd ed. St. Louis: Mosby, 1965. Robbins SL: Pathology, 3rd ed. Philadelphia: Saunders, 1967. Steinbach HL, Gordan GS, Eisenberg E, Crane JT, Silverman S, Goldman L: Primary hyperparathyroidism: a correlation of roentgen, clinical, and pathologic features. AJR 1961; 86: 329-343. Brown TW, Genant HK, Hattner RS, Orloff S, Potter DE: Multiple brown tumors in a patient with chronic renal failure and secondary hyperparathyroidism. AJR 1977; 128: 131-134.
The American
Journal
of Medicine
Volume
81
547