- Email: [email protected]
Primary solitary amyloidosis of the spine: A case report and review of the literature
ELSEVIER
Inflammatory
PRIMARY SOLITARY AMYLOIDOSIS THE SPINE: A CASE REPORT AND REVIEW OF THE LITERATURE
OF
Kevin J. Mullins, M.D.,* Steven P. Meyers, M.D.,t Ann Marie Kazee, M.D.,? James M. Powers, M.D.,? and Paul K. Maurer, M.D.* *Division of Neurological Surgery, TDepartments of Radiology, and $Pathology and Laboratory Medicine, Strong Memorial Hospital, University of Rochester School of Medicine, Rochester, New York
management and prognostication
of each case. 0 1997
Mullins KJ, Meyers SP, Kazee AM, Powers JM, Maurer PK. Primary solitary amyloidosis of the spine: a case report and review of the literature. Surg Neuroi 1997;48:405-8.
by Elsevier Science Inc.
BACKGROUND
Amyloidoma, primary solitary amyloidosis, spine.
KEY
Amyloidosis is a term that encompasses a group of disorders that have as their common feature the intercellular deposition of the amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia. We present the second reported case of a cervical spine amyloidoma and discuss its presentation and management. METHODS
man presented with a l-year history of intermittent chest pain that would radiate into both legs precipitated by valsalva maneuvers. A magnetic resonance imaging (MRI) of the cervical spine revealed a homogenously enhancing lesion focally involving the C-7 vertebral level with significant spinal cord compression. He underwent combined anterior and posterior decompressive procedures with instrumentation for spinal stabilization. Histopathology revealed amyloid deposits and This 5gyearold
a systemic work-up
WORDS
T
he term amyloidosis encompasses a group of disorders that have as their common feature the intercellular deposition of the fibrillary protein, amyloid, by several different pathogenetic mechanisms [lo]. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia (multiple myeloma).
In this report,
we discuss
the case
of a focal cervical spine amyloidoma, its presentation and management, and review the literature on amyloidosis and vertebral amyloidoma.
was negative for amyloidosis.
RESULTS
The patient is free of any tumor recurrence and has a stable spine construct.
at 24 months
CONCLUSIONS
Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Diagnosis requires special stains and therefore a degree of suspicion for the disease. Management of vertebral amyloidoma involves aggressive local resection of the tumor when
feasible and stabilization of the spine as mandated by the degree of tumor involvement. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the
Address reprint requests to: Paul K. Maurer, M.D., Division of New* logical Surgery, Box 670, University of Rochester School of Medicine, Rochester, NY 146424670. Received December 20, 1995; accepted September 26, 1996. 0 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York,
NY 10010
CASEREPORT PRESENTATION The patient is a 58year-old right-handed man who was in good health until May 1993, when he began experiencing chest discomfort in the midsternal and epigastric region. He noticed that coughing or other valsalva maneuvers precipitated the chest discomfort that would radiate from his chest into both legs. The pain was associated with tingling in his distal legs along with a sense of leg weakness and incoordination. This symptom complex varied in its severity over the course of 1 year but had significantly worsened during the 4 weeks prior to evaluation. 009~3019/97/$17.00 PII S009~3019(96)00488-0
406
Mulhns et al
Surg Neurol 1997;48:405-8
Postoperative plain lateral X ray of the cervical spine H illustrating anterior and posterior decompression/ fusion and instrumentation with rectangle and Hartshill
Synthes plate.
Sagittaf T,-weighted MRI after Iv gadolinium shows IIbody abnormal marrow enhancement of the C-7vertebral and posterior elements (arrows). The signal of normal fatty tissues, including marrow, have been sup pressed to increase the conspicuity of the lesion. Expansion of the C-7 spinous process is observed. Epidural enhancement is seen proximal and distal to the C-7 vertebra.
EXAMINATION In May 1994, the patient underwent a neurologic examination that revealed a sensory level at ap proximately T-7, decreased vibration sense in both legs, hyperreflexic knee and ankle jerks, and a spas-
tic tandem gait. The rest of the neurologic and general examination was within normal limits. A magnetic resonance imaging (MRl) of the cervical spine revealed a lesion involving the C-7 vertebral level with significant cord compression (Figure 1). SURGERY Due to the extensive involvement of the anterior and posterior elements, the patient underwent a combined approach involving decompression and stabilization of the anterior and posterior spine.
The initial procedure was a bilateral C6-7 decompressive laminectomy and fusion utilizing Hartshill instrumentation from C-4 to T-Z and iliac crest bone graft (The Hurtshill instrument is a preformed stainless steel rectangle that is made in varying centimeter lengths and is secured into place using a combination of facet, spinous process, and sublaminar cables). This procedure was combined with an anterior C-6 and C-7 corpectomy with iliac crest strut graft and Synthes plate placement (Rgure 2). HISTOPATHOLOGY Multiple specimens of the lesion involving both the bone and epidural space were taken and underwent pathologic examination. Microscopic examination revealed amorphous, eosinophilic deposits with numerous multinucleated giant cells and histiocytes phagocytizing the foreign material (Figure 3). Dispersed among the histiocytes were lymphocytes and occasional plasma cells. Special stains revealed focally strong congophilia with Congo red stain for amyloid. Examination under polarized light revealed focal apple-green birefringence. The modified Puchtler technique (pretreatment of Congo red dye with potassium permanganate) was suggestive
Primary Solitary Amyloidosis
of the Spine
Photomicrograph of amyloid deposits (stars) with Q lymphocytic and histiocytic inflammation and multinucleated giant cells (arrowheads). Congo red stain, original magnification,
400X.
of primary amyloidosis. Stains for bacteria, fungi, acid-fast bacilli, and uric acid stains were negative. POSTOPERATIVE COURSE The patient subsequently underwent a systemic evaluation for amyloidosis that included a chest computed tomography (CT), abdominal CT, echocardiogram, bone marrow biopsy, and bone scan that were negative. Urine and serum protein electrophoresis and immunoelectrophoresis did not reveal a monoclonal gammopathy. A follow-up examination at 12 months revealed a stable spine construct and no evidence of recurrent disease.
DISCUSSION Amyloidosis is a disease complex resulting from the extracellular deposition of amyloid, an insoluble proteinaceous material with a beta-pleated sheet configuration [22]. Amyloid deposits consist of fine, rigid, nonbranching fibrils approximately 7.5-10.0 nm in diameter [4]. It was first named by Virchow in 1854 on the basis of its color after staining with iodine and sulfuric acid [3,26]. Amyloid deposits cause their pathologic destruction by progressive intercellular accumulation and pressure atrophy of adjacent cells. The exact etiology and pathogenesis of amyloidosis is unclear but appears to be multifactorial in many cases. Certain types of amyloidosis are thought to be related to an underlying immune cell dyscrasia in which “amyloidogenic” species of Bence-Jones proteins are cleaved into amyloid proteins [9,10]. Accurate diagnosis requires tissue for special staining with Congo red stain and examination under a polarized light. Amyloid deposits may be focal or systemic and
Surg Neurol 1997;48:405-8
407
related (reactive/secondary amyloidosis) or unrelated (primary amyloidosis) to an underlying infectious or inflammatory process. The recent WHO-IUIS nomenclature subcommittee reclassified amyloid and amyloidosis into 15 types based on the amino acid sequence of the specific amyloid protein involved in each disease type [8,12,13]. The two most clinically relevant forms of amyloidosis include the amyloid light chain (AL) type associated with multiple myeloma and primary amyloidosis and the amyloid associated (AA) type found in secondary/reactive amyloidosis due to chronic infectious and inflammatory conditions [3]. Prognosis for patients with amyloidosis is dependent on the specific form of amyloidosis. In reactive/secondary amyloidosis, treatment of the underlying disease can slow or actually reverse the progression of amyloid disease with 5-year and lo-year survival after diagnosis not uncommon [ 111. Patients with immunocytic derived amyloidosis have the worst prognosis with less than 1 year survival [ 111. Primary solitary amyloidosis carries the best prognosis with apparent long-term survival and no increased risk of developing other forms of amyloidosis following diagnosis and treatment [ 2,221. However, the natural history of this variant of amyloidosis cannot be clearly defined due to the limited number of case reports and lack of published long term follow-up studies. Neurologic manifestations of amyloidosis commonly involve the development of primary cerebral amyloid angiopathy and peripheral neuropathy due to focal amyloid deposition. Recent attention in the neurosurgical literature has focused on systemic amyloidosis of the Beta-2 microglobulin type that is found in patients on long-term hemodialysis and results in a destructive spondyloarthropathy that may cause radiculopathy, myelopathy, or acute spinal cord compression [6,17,19,27]. Rare case reports of amyloidoma involving the central nervous system (CNS) include the gasserian ganglion, pituitary gland, cerebellopontine angle, jugular foramen, and cerebral hemisphere [ 5,14,23,24]. Primary solitary amyloidosis of the vertebral column is a rare entity with only seven previously reported cases [7,15,16,18,20,21,25]. The majority of spinal column amyloid deposits are associated with multiple myeloma or systemic amyloidosis that was excluded in our case. Our report is the second involving the cervical spine; the first was reported by Dickman et al. in 1988 [ 71. The differential diagnosis of an amyloidoma is diverse and includes metastatic disease, plasmacytoma, infection, primary bone tumor, and inflammatory processes. Imaging studies including CT and MRI can
408
Mullins et al
Surg Neurol 1997;48:405-8
be suggestive but not diagnostic of amyloidoma. Diagnosis requires specific staining of tissue for amyloid and therefore, requires a degree of suspicion for the disease. Management includes obtaining as complete a resection as is possible of the tumor with stabilization of the spine as necessitated by the degree of tumor involvement. Our case involved the entire anterior-posterior extent of the C-7 vertebral level and therefore, required a combined stabilization procedure. The limited number of case reports describing amyloidomas of the vertebral column indicate that local resection results in an excellent prognosis similar to that found in patients with primary solitary amyloidosis involving other regions of the body [7,15,25]. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the management and prognostication of each case.
CONCLUSION Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Involvement of the spinal column is rare and is commonly manifested by symptoms of pain and signs of paraparesis/myelopathy. Imaging studies are nondiagnostic and management includes prompt decompression and stabilization of the spine followed by a full work-up of systemic signs of primary or secondary systemic amyloidosis. REFERENCES 1. Bauer WH, Kuzma JF. Solitary “tumors” of atypical amyloid baramyloid). Am J Clin Path01 1949;19:1097112. 2. Cathcart E, Ritchie R, Cohen AS, Brandt K. Immuneglobulins and amyloidosis: an immunologic study of 62 patients with biopsy proved disease. Am J Med 1972;52:93-101. 3. Cohen AS. Amyloidosis. In: Harrison’s principles of internal medicine, vol. 2, 13th ed. New York: McGraw Hill, 1994:1625-30. 4. Cotran RS, Kumar V, Robbins SL. Robbins pathologic basis of disease, 5th ed. Philadelphia: W.B. Saunders Company, 1994231-g.
5. Daly DD, Love JG, Dockerty MB. Amyloid tumor of the gasserian ganglion: report of case. J Neurosurg 1956; 14134762. 6. Davidson GS, Montanera WJ, Fleming JF, et al. Amyloid destructive spondyloarthropathy causing cord
7. 8.
9. 10. 11. 12. 13. 14. 15. 16. 17.
compression: related to chronic renal failure and dialysis. Neurosurgery 1993;33:519-22. Dickman CA, Sonntag VKH, Johnson P, et al. Amyloidoma of the cervical spine: a case report. Neurosurgery 1988;22:419-22. Gertz MA, Greipp PR, Kyle RA. Classification of amyloidosis by the detection of clonal excess of plasma cells in the bone marrow. J Lab Clin Med 1991;118: 33-9. Glenner GG, Terry WD, Isersky C. Amyloidosis: its nature and pathogenesis. Semin Hematol 1973;10:6586. Glenner GG. Amyloid deposits and amyloidosis: the BFibrilloses. N Engl J Med 1980;302:1283-92. Glenner GG. The amyloidosis. In: Internal medicine, 4th ed. St. Louis: Mosby Year-Book Inc. 1994:251 l-4. Husby G. Nomenclature and classification of amyloid and amyloidosis. J Int Med 1992;232:511-2. Husby G. Nomenclature of amyloid and amyloidosis. Bullet World Health Organ 1993;71:105-8. Kuratsu JI, Matsukado Y, Miura M. Prolactinoma of pituitary with associated amyloid-like substances: case report. J Neurosurg 1983;59:1067-70. Leeson MC, Rechtine GR, Makely JT, et al. Primary amyloidoma of the spine: a case report and review of the literature. Spine 1985;10:303-6. Mandl J. Ueber lokales amyloid im bereiche der brustwirbelsaule. Virchows Arch [A] (Path01 Anat) 1924;253:639-55. Mazenac K, McClure J, Bartley CJ, et al. Systemic amyloidosis of B-2 microglobulin type. J Clin Path01
1992;45:832-3. 18. McAnena OJ, Feely MP, Kealy WF. Spinal cord compression by amyloid tissue. J Neurol Neurosurg Psych 1982;45:1067-9. 19. Naidich JB, Mossey RT, McHeffey-Atkinson B, et al. Spondyloarthropathy from long-term hemodialysis. Radiology 1988;167:761-4. 20. Pawar S, Kay CJ, Anderson HH, et al. Primary amyloidoma of the spine. J Comput Assist Tomogr 1982; 6:1175-7. 21. Prasad BK, Andrews K. Localised amyloid deposit producing paraplegia. Br Med J 1981;283:1087. 22. Scott PP, Scott WW, Siegelman SS. Amyloidosis: an overview. Semin Roentgen 1986;21:103-12. 23. Townsend JJ, Tomiyasu U, MacKay A, et al. Central nervous system amyloid presenting as a mass lesion: report of two cases. J Neurosurg 1982;56:439-42. 24. Unal F, Hepgul K, Bayindir C, et al. Skull base amyloidoma: case report. J Neurosurg 1992;76:303-6. 25. Villarejo F, Perez Diaz C, Perla C, et al. Spinal cord
compression 1176-81.
by amyloid
deposits. Spine 1994;19:
26. Virchow R. Zur cellulose-frage. Virchows Arch [A] (Path01 Anat) 1854;6:416-31. 27. Welk LA, Quint DJ. Amyloidosis of the spine in a
patient on long-term hemodialysis. Neurorad 1990;32: 334-6.
Inflammatory
PRIMARY SOLITARY AMYLOIDOSIS THE SPINE: A CASE REPORT AND REVIEW OF THE LITERATURE
OF
Kevin J. Mullins, M.D.,* Steven P. Meyers, M.D.,t Ann Marie Kazee, M.D.,? James M. Powers, M.D.,? and Paul K. Maurer, M.D.* *Division of Neurological Surgery, TDepartments of Radiology, and $Pathology and Laboratory Medicine, Strong Memorial Hospital, University of Rochester School of Medicine, Rochester, New York
management and prognostication
of each case. 0 1997
Mullins KJ, Meyers SP, Kazee AM, Powers JM, Maurer PK. Primary solitary amyloidosis of the spine: a case report and review of the literature. Surg Neuroi 1997;48:405-8.
by Elsevier Science Inc.
BACKGROUND
Amyloidoma, primary solitary amyloidosis, spine.
KEY
Amyloidosis is a term that encompasses a group of disorders that have as their common feature the intercellular deposition of the amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia. We present the second reported case of a cervical spine amyloidoma and discuss its presentation and management. METHODS
man presented with a l-year history of intermittent chest pain that would radiate into both legs precipitated by valsalva maneuvers. A magnetic resonance imaging (MRI) of the cervical spine revealed a homogenously enhancing lesion focally involving the C-7 vertebral level with significant spinal cord compression. He underwent combined anterior and posterior decompressive procedures with instrumentation for spinal stabilization. Histopathology revealed amyloid deposits and This 5gyearold
a systemic work-up
WORDS
T
he term amyloidosis encompasses a group of disorders that have as their common feature the intercellular deposition of the fibrillary protein, amyloid, by several different pathogenetic mechanisms [lo]. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia (multiple myeloma).
In this report,
we discuss
the case
of a focal cervical spine amyloidoma, its presentation and management, and review the literature on amyloidosis and vertebral amyloidoma.
was negative for amyloidosis.
RESULTS
The patient is free of any tumor recurrence and has a stable spine construct.
at 24 months
CONCLUSIONS
Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Diagnosis requires special stains and therefore a degree of suspicion for the disease. Management of vertebral amyloidoma involves aggressive local resection of the tumor when
feasible and stabilization of the spine as mandated by the degree of tumor involvement. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the
Address reprint requests to: Paul K. Maurer, M.D., Division of New* logical Surgery, Box 670, University of Rochester School of Medicine, Rochester, NY 146424670. Received December 20, 1995; accepted September 26, 1996. 0 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York,
NY 10010
CASEREPORT PRESENTATION The patient is a 58year-old right-handed man who was in good health until May 1993, when he began experiencing chest discomfort in the midsternal and epigastric region. He noticed that coughing or other valsalva maneuvers precipitated the chest discomfort that would radiate from his chest into both legs. The pain was associated with tingling in his distal legs along with a sense of leg weakness and incoordination. This symptom complex varied in its severity over the course of 1 year but had significantly worsened during the 4 weeks prior to evaluation. 009~3019/97/$17.00 PII S009~3019(96)00488-0
406
Mulhns et al
Surg Neurol 1997;48:405-8
Postoperative plain lateral X ray of the cervical spine H illustrating anterior and posterior decompression/ fusion and instrumentation with rectangle and Hartshill
Synthes plate.
Sagittaf T,-weighted MRI after Iv gadolinium shows IIbody abnormal marrow enhancement of the C-7vertebral and posterior elements (arrows). The signal of normal fatty tissues, including marrow, have been sup pressed to increase the conspicuity of the lesion. Expansion of the C-7 spinous process is observed. Epidural enhancement is seen proximal and distal to the C-7 vertebra.
EXAMINATION In May 1994, the patient underwent a neurologic examination that revealed a sensory level at ap proximately T-7, decreased vibration sense in both legs, hyperreflexic knee and ankle jerks, and a spas-
tic tandem gait. The rest of the neurologic and general examination was within normal limits. A magnetic resonance imaging (MRl) of the cervical spine revealed a lesion involving the C-7 vertebral level with significant cord compression (Figure 1). SURGERY Due to the extensive involvement of the anterior and posterior elements, the patient underwent a combined approach involving decompression and stabilization of the anterior and posterior spine.
The initial procedure was a bilateral C6-7 decompressive laminectomy and fusion utilizing Hartshill instrumentation from C-4 to T-Z and iliac crest bone graft (The Hurtshill instrument is a preformed stainless steel rectangle that is made in varying centimeter lengths and is secured into place using a combination of facet, spinous process, and sublaminar cables). This procedure was combined with an anterior C-6 and C-7 corpectomy with iliac crest strut graft and Synthes plate placement (Rgure 2). HISTOPATHOLOGY Multiple specimens of the lesion involving both the bone and epidural space were taken and underwent pathologic examination. Microscopic examination revealed amorphous, eosinophilic deposits with numerous multinucleated giant cells and histiocytes phagocytizing the foreign material (Figure 3). Dispersed among the histiocytes were lymphocytes and occasional plasma cells. Special stains revealed focally strong congophilia with Congo red stain for amyloid. Examination under polarized light revealed focal apple-green birefringence. The modified Puchtler technique (pretreatment of Congo red dye with potassium permanganate) was suggestive
Primary Solitary Amyloidosis
of the Spine
Photomicrograph of amyloid deposits (stars) with Q lymphocytic and histiocytic inflammation and multinucleated giant cells (arrowheads). Congo red stain, original magnification,
400X.
of primary amyloidosis. Stains for bacteria, fungi, acid-fast bacilli, and uric acid stains were negative. POSTOPERATIVE COURSE The patient subsequently underwent a systemic evaluation for amyloidosis that included a chest computed tomography (CT), abdominal CT, echocardiogram, bone marrow biopsy, and bone scan that were negative. Urine and serum protein electrophoresis and immunoelectrophoresis did not reveal a monoclonal gammopathy. A follow-up examination at 12 months revealed a stable spine construct and no evidence of recurrent disease.
DISCUSSION Amyloidosis is a disease complex resulting from the extracellular deposition of amyloid, an insoluble proteinaceous material with a beta-pleated sheet configuration [22]. Amyloid deposits consist of fine, rigid, nonbranching fibrils approximately 7.5-10.0 nm in diameter [4]. It was first named by Virchow in 1854 on the basis of its color after staining with iodine and sulfuric acid [3,26]. Amyloid deposits cause their pathologic destruction by progressive intercellular accumulation and pressure atrophy of adjacent cells. The exact etiology and pathogenesis of amyloidosis is unclear but appears to be multifactorial in many cases. Certain types of amyloidosis are thought to be related to an underlying immune cell dyscrasia in which “amyloidogenic” species of Bence-Jones proteins are cleaved into amyloid proteins [9,10]. Accurate diagnosis requires tissue for special staining with Congo red stain and examination under a polarized light. Amyloid deposits may be focal or systemic and
Surg Neurol 1997;48:405-8
407
related (reactive/secondary amyloidosis) or unrelated (primary amyloidosis) to an underlying infectious or inflammatory process. The recent WHO-IUIS nomenclature subcommittee reclassified amyloid and amyloidosis into 15 types based on the amino acid sequence of the specific amyloid protein involved in each disease type [8,12,13]. The two most clinically relevant forms of amyloidosis include the amyloid light chain (AL) type associated with multiple myeloma and primary amyloidosis and the amyloid associated (AA) type found in secondary/reactive amyloidosis due to chronic infectious and inflammatory conditions [3]. Prognosis for patients with amyloidosis is dependent on the specific form of amyloidosis. In reactive/secondary amyloidosis, treatment of the underlying disease can slow or actually reverse the progression of amyloid disease with 5-year and lo-year survival after diagnosis not uncommon [ 111. Patients with immunocytic derived amyloidosis have the worst prognosis with less than 1 year survival [ 111. Primary solitary amyloidosis carries the best prognosis with apparent long-term survival and no increased risk of developing other forms of amyloidosis following diagnosis and treatment [ 2,221. However, the natural history of this variant of amyloidosis cannot be clearly defined due to the limited number of case reports and lack of published long term follow-up studies. Neurologic manifestations of amyloidosis commonly involve the development of primary cerebral amyloid angiopathy and peripheral neuropathy due to focal amyloid deposition. Recent attention in the neurosurgical literature has focused on systemic amyloidosis of the Beta-2 microglobulin type that is found in patients on long-term hemodialysis and results in a destructive spondyloarthropathy that may cause radiculopathy, myelopathy, or acute spinal cord compression [6,17,19,27]. Rare case reports of amyloidoma involving the central nervous system (CNS) include the gasserian ganglion, pituitary gland, cerebellopontine angle, jugular foramen, and cerebral hemisphere [ 5,14,23,24]. Primary solitary amyloidosis of the vertebral column is a rare entity with only seven previously reported cases [7,15,16,18,20,21,25]. The majority of spinal column amyloid deposits are associated with multiple myeloma or systemic amyloidosis that was excluded in our case. Our report is the second involving the cervical spine; the first was reported by Dickman et al. in 1988 [ 71. The differential diagnosis of an amyloidoma is diverse and includes metastatic disease, plasmacytoma, infection, primary bone tumor, and inflammatory processes. Imaging studies including CT and MRI can
408
Mullins et al
Surg Neurol 1997;48:405-8
be suggestive but not diagnostic of amyloidoma. Diagnosis requires specific staining of tissue for amyloid and therefore, requires a degree of suspicion for the disease. Management includes obtaining as complete a resection as is possible of the tumor with stabilization of the spine as necessitated by the degree of tumor involvement. Our case involved the entire anterior-posterior extent of the C-7 vertebral level and therefore, required a combined stabilization procedure. The limited number of case reports describing amyloidomas of the vertebral column indicate that local resection results in an excellent prognosis similar to that found in patients with primary solitary amyloidosis involving other regions of the body [7,15,25]. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the management and prognostication of each case.
CONCLUSION Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Involvement of the spinal column is rare and is commonly manifested by symptoms of pain and signs of paraparesis/myelopathy. Imaging studies are nondiagnostic and management includes prompt decompression and stabilization of the spine followed by a full work-up of systemic signs of primary or secondary systemic amyloidosis. REFERENCES 1. Bauer WH, Kuzma JF. Solitary “tumors” of atypical amyloid baramyloid). Am J Clin Path01 1949;19:1097112. 2. Cathcart E, Ritchie R, Cohen AS, Brandt K. Immuneglobulins and amyloidosis: an immunologic study of 62 patients with biopsy proved disease. Am J Med 1972;52:93-101. 3. Cohen AS. Amyloidosis. In: Harrison’s principles of internal medicine, vol. 2, 13th ed. New York: McGraw Hill, 1994:1625-30. 4. Cotran RS, Kumar V, Robbins SL. Robbins pathologic basis of disease, 5th ed. Philadelphia: W.B. Saunders Company, 1994231-g.
5. Daly DD, Love JG, Dockerty MB. Amyloid tumor of the gasserian ganglion: report of case. J Neurosurg 1956; 14134762. 6. Davidson GS, Montanera WJ, Fleming JF, et al. Amyloid destructive spondyloarthropathy causing cord
7. 8.
9. 10. 11. 12. 13. 14. 15. 16. 17.
compression: related to chronic renal failure and dialysis. Neurosurgery 1993;33:519-22. Dickman CA, Sonntag VKH, Johnson P, et al. Amyloidoma of the cervical spine: a case report. Neurosurgery 1988;22:419-22. Gertz MA, Greipp PR, Kyle RA. Classification of amyloidosis by the detection of clonal excess of plasma cells in the bone marrow. J Lab Clin Med 1991;118: 33-9. Glenner GG, Terry WD, Isersky C. Amyloidosis: its nature and pathogenesis. Semin Hematol 1973;10:6586. Glenner GG. Amyloid deposits and amyloidosis: the BFibrilloses. N Engl J Med 1980;302:1283-92. Glenner GG. The amyloidosis. In: Internal medicine, 4th ed. St. Louis: Mosby Year-Book Inc. 1994:251 l-4. Husby G. Nomenclature and classification of amyloid and amyloidosis. J Int Med 1992;232:511-2. Husby G. Nomenclature of amyloid and amyloidosis. Bullet World Health Organ 1993;71:105-8. Kuratsu JI, Matsukado Y, Miura M. Prolactinoma of pituitary with associated amyloid-like substances: case report. J Neurosurg 1983;59:1067-70. Leeson MC, Rechtine GR, Makely JT, et al. Primary amyloidoma of the spine: a case report and review of the literature. Spine 1985;10:303-6. Mandl J. Ueber lokales amyloid im bereiche der brustwirbelsaule. Virchows Arch [A] (Path01 Anat) 1924;253:639-55. Mazenac K, McClure J, Bartley CJ, et al. Systemic amyloidosis of B-2 microglobulin type. J Clin Path01
1992;45:832-3. 18. McAnena OJ, Feely MP, Kealy WF. Spinal cord compression by amyloid tissue. J Neurol Neurosurg Psych 1982;45:1067-9. 19. Naidich JB, Mossey RT, McHeffey-Atkinson B, et al. Spondyloarthropathy from long-term hemodialysis. Radiology 1988;167:761-4. 20. Pawar S, Kay CJ, Anderson HH, et al. Primary amyloidoma of the spine. J Comput Assist Tomogr 1982; 6:1175-7. 21. Prasad BK, Andrews K. Localised amyloid deposit producing paraplegia. Br Med J 1981;283:1087. 22. Scott PP, Scott WW, Siegelman SS. Amyloidosis: an overview. Semin Roentgen 1986;21:103-12. 23. Townsend JJ, Tomiyasu U, MacKay A, et al. Central nervous system amyloid presenting as a mass lesion: report of two cases. J Neurosurg 1982;56:439-42. 24. Unal F, Hepgul K, Bayindir C, et al. Skull base amyloidoma: case report. J Neurosurg 1992;76:303-6. 25. Villarejo F, Perez Diaz C, Perla C, et al. Spinal cord
compression 1176-81.
by amyloid
deposits. Spine 1994;19:
26. Virchow R. Zur cellulose-frage. Virchows Arch [A] (Path01 Anat) 1854;6:416-31. 27. Welk LA, Quint DJ. Amyloidosis of the spine in a
patient on long-term hemodialysis. Neurorad 1990;32: 334-6.