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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
301
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_91
9 1
Amyloidosis
91.1 Definition
This a nodular mass of amyloid, without generalize amyloidosis.
91.2 Incidence
Solitary primary amyloidoma of the spine is very rare, 18 cases in the literature.
91.3 Etiology
The causative factors are unknown. There is a thought that the amyloidosis is a
result of proliferating plasma cells, and macrophages.
91.4 Pathology
The amyloidoma is a focal mass of amyloid tissue within the spinal canal, epidurally
leading to compression of the cord or cauda equina. It arises from the bone marrow
of the ventral body without involving disc. It is extruded into the epidural space. It is
more commonly found in the thoracic spine, followed by the cervical and lumbar.
The amyloidoma may cause bone destruction and may lead to fracture. The mass is
gray, nodular, fi rm, but may develop cyst formation. Interstitial hemorrhage may
occur. The amyloidoma is a benign lesion and does not change to malignancy.
Haridas et al. ( 2005 )
302
91.5 Microscopically
After Haridas et al., collection of eosinophilic material within fi brous and elastic
tissue AL protein is the type found in primary amyloidoma. The material shows
apple-green birefringence and congo-red staining and examination under polarized
light. There are occasional areas of hemosiderin and mononuclear cells. Under light
microscopy, amyloid appears as eosinophilic amorphous hyaline extracellular substance. Another picture from Aydin et al., fragmented cartilaginous tissue with lymphocytic and plasma cell infi ltrate. Deposition of eosinophilic acellular mass around
which foreign body giant cell response. In some foci blood vessel walls are thickened with deposition of eosinophilic material. Congo-red stain gave congophilic
response in the acellular material and the vessel walls. There is no mark of future
development of plasmacytoma. Under electron microscopy, according to
Iplicoeioglin et al., amyloidoma deposits appear as composed of rigid 7.5–10 nm
fi brils arranged in B-pleated sheets.
91.6 Clinical Picture
Primary amyloidoma is not accompanied by symptoms and causes systemic amyloidosis. The symptoms depend on the spinal segments involved. There is pain in
the back, may radiate to the neck and arms in cervical lesions, to the chest in thoracic amyloidoma and sciatic distribution in lumbar lesions. It is accompanied by
sensory changes, hypoesthesia, paraethesia, or sensory loss. There is weakness of
the extremities, which may end in para- or tetra- plegia. There may be sphincter
disturbances such as incontinence.
91.7 Diagnosis
Diagnosis based on:
1. Patient history
2. Physical exam, absence of systemic amyloidosis and its etiologies. There may be
tenderness over the affected vertebra, soft tissue swelling or a deformity.
3. Neurological exam sensory and motor defi cits, depending on the level of the
lesion. There is hypoesthesia, anaestheisa, increased muscle tone, hyperrefl exia,
weakness, paresis, or paralysis.
4. Plain radiography shows no abnormality. Or it may show a lytic areas in a verte-
bral body or bone distraction or fracture.
5. CT scan shows osteolytic areas in the vertebra and paraspinal soft tissue calcifi -
cation. Pathological fractures and bone destruction can be seen.
6. MRI usually shows low to intermediate signal intensity on both T1 and T2-weighted
images. There is variable degrees of epidural mass enhancement due to contract
91 Amyloidosis
303
administration on T1-weighted images. It should be differentiated from metastasis,
multiple myeloma, lymphoma, and chondrosarcoma.
7. Biopsy. Ultrasound or CT-guided biopsy or fi ne needle aspiration cytology with
special staining techniques, Congo-red under polarized light microscope or electron-
microscopy. Immunohistochemical studies may help diagnose amyloidoma.
91.8 Management
Primary solitary amyloidoma producing neurogenic symptoms and signs should be
removed, since there will be neurological recovery and if totally removed there will
be no recurrence. The surgical techniques vary according to the site of the amyloidoma. In general decompression, complete resection and stabilization are needed.
In their case, Haridas et al., L3-L4 laminectomies were done with complete resection of the amyloidoma. Abbas et al., had T9 vertebreotomy, which was collapsed
and insertion of mass cage. In the case laminectomy was done as a second procedure with removal of the mass. The fi rst procedure was just decompressive laminectomy. According to Haridas et al., if the mass is accidentally discovered and there
are no neurological symptoms or signs the patient can be treated conservatively,
with regular followup clinically and by imaging. As soon as neurological picture is
seen, surgery should be performed.
Reference
Haridas A, Basu S, King A, Pollock J. Primary isolated amyloidoma of the lumbar spine causing
neurological compromise: case report and literature review. Neurosurgery. 2005;57(1):E196;
discussion E196.
Reference
305
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_92
9 2
Acute Paraplegia Secondary to Brown Tumor
92.1 Definition
Brown tumor is a lytic bone tumor caused by hyperparathyroidism, either primary
or secondary. Synonyms: osteoclastoma, osteitis fi brocystica, EngelvonRecklinghausen.
92.2 Incidence
Brown tumors are more common in primary hyperparathyroidism than in secondary. There is an increased risk in patients with end-stage renal failure, 1.5–13 %
patients had brown tumors. In 2004, Vandenbussche, et al, in a review of the English
literature reported eight cases with spinal involvement. All of the patients were
women, and 6 out of 8 (75 %) were younger than 40 years old. Two (25 %) of the
cases involved the cervical, the other six (75 %) involved the thoracic. Four (50 %)
of the cases developed acute paraplegia.
92.3 Etiology
Primary hyperparathyroidism
• parathyroid adenocarcinoma in 85 % of the cases
• glandular hyperplasia associated with multiple endocrine neoplasia (Type I and
Type II)
– less than 0.5 % of cases are caused by parathyroid malignancies
– 10–20 % of the cases are caused by inherited hyperfunction of multiple para-
thyroid glands
Abstracted from multiple sources
306
• Secondary hyperparathyroidism
• chronic renal failure (CRF) in long term dialysis
92.4 Pathology
CRF leads to osteodystrophy, which entails osteomalacia, osteitis, or osteosclerosis either alone or in combination with brown tumors. Brown tumors occur
mostly in the maxilla, mandible, clavicle, ribs, sternum, pelvis, long bones- especially the femur and the vertebral column. there may be extra sklatal deposition
of calcium and phosphate. The brown tumor is a non-neoplastic, which is not
malignant. It appears as a reddish brown friable mass that replaces normal bone.
Histiologically, there is loss of bone trabeculae with accumulation of giant osteoclastic cells within fi brous tissue containing hemosiderin deposits. the tumor is
vascular with microhemorrages, there are microfractures of the trabeculae and
with its eventual loss. Progressive bone absorption leases to microscopic cysts,
which eventually coalesce, forming osteitis fi brosa cystica. The brown tumor is
almost indistinguishable from a giant cell tumor. The tumor may continue to
worsen despite a renal transplant. In the vertebral column it involves cancellous
bone marrow and may progress slowly casing compression of the spinal cord,
producing a space occupying effect. The cortex may be spared unless a pathological fracture occurs; in which case, it leads to acute paraplegia if it is in the
thoracic spine or tetraplegia if is located in the cervical spine. The pathophysiology according to Fineman, et al:
In osteitis fi brosa a brown tumor mass results from secondary hyperparathyroidism that is manifested by hyperplasia of the parathyroid chief cells in which elevated
serum parathyroid hormone (PTH) levels and osteoclast activation are produced.
The pathogensis of secondary hyperparathyroidism is linked to:
1. phosphate retention
2. altered metabolism of calcitrol (vitamin D)
3. skeletal resistance to PTH
4. impaired degradation of PTH
5. altered feedback regulation of PTH by calcium
Phosphate retention reduces the concentration of ionized calcium in extracellular fl uid due to increased binding of ionized calcium phosphate. there is also
decreased renal production of vitamin D in response to increased serum phosphate
concentration and decreased calcium mobilization from bone because of the direct
effects of phosphate on bone.
Serum PTH levels are further elevated by reduced production of vitamin
D. Elevated levels of phosphate inhibit the activity of the hyroxylase that is required
92 Acute Paraplegia Secondary to Brown Tumor
307
for conversion of 25(OH)D 3 to 1,25(OH) 2 D 3 (that is, calitrol, the active form of
vitamin D), which is necessary for intestinal absorption of calcium. this leads to
further decreased serum levels of ionized calcium and subsequent hyperstimulation
of the parathyroid glands.
Skeletal resistance to PTH in patients with CRF is responsible for the additional
reduction in serum ionized calcium. This results in further upregulation of parathyroid PTH production as an attempt to maintain adequate serum levels of ionized
calcium.
Additionally, because parathyroid hormone is metabolized in the liver and the
kidney, decreased renal function of end-stage renal disease may be responsible for
increased serum levels of PTH due to slowed catabolism.
Lastly, feedback regulation of PTH production in the parathyroid gland caused
by serum calcium is altered in cases of advanced renal disease that result from
decreased sensitivity to calcium; this in turn, leads to further increases in serum
levels of PTH.
92.5 Clinical Picture
Hyperparathyroidism may be asymptomatic, non-specifi c symptoms are lethargy,
confusion, nausea, fatigability, bone pain and weakness. When symptoms occur the
may be due to hypercalcemia and/or hyperparathyroid osteodystrophy. This is
focused on acute paraplegia to brown tumor of the vertebral column. the paralysis
may be a slow onset, due to slow invasion of the brown tumor into the spinal canal.
The pathological fracture will present acute onset paraplegia. There will be loss of
sensory motor function, as well as sphincter dysfunction.
92.6 Diagnosis
In primary hyperparathyroidism the diagnosis is based on persistant hypercalcemia,
which is confi rmed by repeated measurements of serum calcium concentration, and
an elevated PTH level. Parathyroid localization is done by technetium-99 m (
99m
Tc);
although, sestamibi scanning is the most accurate test for localization of the parathyroid. In secondary hyperparathyroidism there is commonly CRF. there will be an
elevation of creatinine, calcium, phosphate, Westergren sedimentation rate, and
PTH. Diagnosis of the bony lesion is confi rmed by computed tomography (CT)
guided biopsy; other imgaing tests especially magnetic resonance image (MRI) will
show spinal cord compression, and myelography may be indicated. Plain radiography will show generalized osteopenia, loss of the trabeculae, cystic formation and
may show a pathological fracture.
92.6 Diagnosis
308
92.7 Management
For patients with symptomatic primary hyperparathyroidism, a total or subtotal
parathyroidectomy should be performed by an expert surgeon and is more than 90 %
successful. For patients with secondary hyperparathryoidism one look at the medical treatment, which is:
1. initial control of serum phosphate by restriction of dietary phosphorus intake and
phosphate binding antiacids
2. adequate calcium intake
3. vitamin D therapy
If the medical therapy fails to halt the progression of the brown tumors, a subtotal
or total parathyroidectomy is performed to reduce the PTH levels. The indication
for a parathyroidectomy are:
• vertebral lesion with or without cord compression
• persistant hypercalcemia
• intractable pruritis
• extraskeletal clacifi cations
However, parathyroidectomy may only slow the progression of symptoms, then
one must look to spinal decompressive surgery. When the invading tumor is removed
spinal fusion by hardware and bone grafts are performed. The approach can be
either anterior or posterior; although, the latter is more favorable. Surgery should be
done as soon as possible to prevent permanent paralysis. Embolization of very vascular brown tumors may be indicated before surgery.
Bibliography
Fineman I, et al. Chronic renal failure causing brown tumors and myelopathy. Case report and
review of pathophysiology and treatment. J Neurosurg Spine. 1999;90(2):242–6.
Griffi ths HJ, Ennis JT, Bailey G. Skeletal changes following renal transplantation. Radiology.
1974;113(3):621–6.
Sargent MA, et al. Bone cysts and haemodialysis-related amyloidosis. Clin Radiol. 1989;40(3):
277–81.
Taniegra ED. Hyperparathyroidism. Am Fam Physician. 2004;69(2):333–9.
Vandenbussche E, et al. Brown tumor of the spine and progressive paraplegia in a hemodialysis
patient. Spine. 2004;29(12):E251–5.
92 Acute Paraplegia Secondary to Brown Tumor
309
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_93
9 3
Myelopathy due to Chondroblastoma of the Spine
93.1 Definition
Chondroblastoma is a rare benign tumor of the epiphyseal region of the long bones
and is very rare in the vertebral column. It is characterized by a sclerotic margin,
intralesional calcifi cation, rounded or polygonal chondroblast-like cells, cartilaginous intercellular matrix, and multinucleated giant cells.
93.2 Incidence
The most current statistics tabulated by (Vialle et al. 2005 ), 26 cases of spinal chon-
droblastoma have been reported in the past 50 years, only six of which were located
in the lumbar region, they added two cases of their own. The cervical is the most
common location affected. According to Lee et al., the age of diagnosis ranges from
9 to 59 years, with the majority in the second decade of life. As to the sex ratio men
are more commonly affected.
93.3 Etiology
No causative factor was detected.
93.4 Pathology
The tumor is benign mimicking malignancy, but tends to local recurrence and irradiation may precipitate malignancy. The tumor arises from the secondary ossifi cation center of the vertebra. The tumor has a chondroid appearance with cystic and
Abstracted from Vialle et al. ( 2005 )
310
hemorrhagic areas. Its consistency varies from soft to gritty depending on the
amount of calcifi cation or ossifi cation. Microscopically it consists of closely packed
polyhedral cells separated by a scanty interstitial matrix, giving the chondroid
appearance. Another classic feature is the presence of a lattice-like intercellular cellular calcifi cation resembling chicken-wire or the picket fence and the multinucleated giant cells. There is no mitosis or atypia. Hemorrhages may be seen as well as
sometimes aneurysmal blood cysts. When too big the tumor may extend to the soft
tissues around (muscles) and may ulcerate. However, the tumor does not metastasize. When invading the spinal cord it causes compression myelo-radiculopathy
without invading the dura.
93.5 Clinical Picture
The fi rst symptom is pain usually in the neck and dorsal region, rarely in the lumbar
region. The pain radiates depending on the site: in the cervical case the pain radiates
to the upper extremities; in the thoracic region it radiates to the chest; and in the
lumbar region it radiates to the abdomen and lower extremities. Sometimes the
chondroblastoma has a sciatica picture.
Swelling in early cases may be visible or palpable. With large tumors a swelling
on the back may be felt and it shows vascularity and pseudo-infl ammatory signs.
The consistency is heterogeneous, hard, fi rm, fl eshy, and cystic in parts. Extraspinal
soft tissue extension is also detectable in progressive cases.
Neurological signs in chondroblastoma vary; it is usually tetraparesis, paraplegia, or cauda equina syndrome depending on the site. In cervical cases extension to
the neck in the supraclavicular region and involvement of the brachial plexus is
possible. In the dorsal region may extend in the mediastinum and the lumbar region
may cause hydronephrosis
93.6 Diagnosis
Diagnosis based on:
1. Clinical assessment
2. Plain radiography: bone destruction (osteolytic lesion) usually in the vertebral
body and may be in the posterior elements, bone formation and calcifi cation are
usually detected
3. MRI: shows a bone tumor involving the vertebral body with soft tissue extension
to the muscles nearby. Extension into the spinal canal causes compression
myelopathy but the dura is not involved.
4. Gallium isotope scan: shows cystic fl uid and also by ultrasounography.
5. Needle aspiration biopsy: confi rms the nature of the tumor.
6. Neurophysiological studies: confi rms neurological defi cit.
93 Myelopathy due to Chondroblastoma of the Spine
311
93.7 Management
Because the tumor has a high tendency to recur wide excision and curettage are
required. After thorough curettage, fl ush irrigation and hemostasis packing with
polymethyl-methacrylate, may reduce recurrence rate. (Ramappa et al.) For extensive tumors combined anterior and posterior approach for corpectomy and removal
of extracorporeal tissue, bone graft and hardware fi xation. In recurrent cases another
attempt may be taken or possibly followed by irradiation, the latter may precipitate
malignant change. Early surgery is the key to avoid recurrence and may reverse
neurological defi cit.
Reference
Vialle R, Feydy A, Rillardon L, et al. Chondroblastoma of the lumbar spine. Report of two cases
and review of the literature. J Neurosurg Spine. 2005;2(5):596–600.
Reference