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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
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121.4 Pathology
Hemangioblastoma is a highly vascular tumor composed of neoplastic stromal cells,
pericytes and endothelial cells. The neoplastic cell of the origin of CNS hemangioblastoma is the mesoderm-derived, embryologically arrested hemangioblast. There
is usually a syrinx, which is asymptomatic but as it enlarges symptoms will be
manifested.
121.5 Clinical Picture
The earliest features are those of the cerebellar and/or retinal. Spinal cord features
are rare to be the earliest. The clinical features of spinal hemangioblastoma associated with VHL disease are in four grades according to McCormik et al.:
• Grade I: neurologically normal, mild focal defi cit not signifi cantly affecting the
function of the affected limb, mild spasticity or refl ex abnormality. Gait is
normal.
• Grade II: Presence of sensorimotor defi cit affecting function of involved limb,
mild to moderate gait diffi culty, severe pain or dysesthetic syndrome impairing
patient’s quality of life, still functions and ambulates independently.
• Grade III: more severe neurological defi cit requires cane/brace for ambulation or
signifi cant upper limb impairment, may or may not function independently.
• Grade IV: severe defi cit requires wheelchair or cane/brace, bilateral upper limb
impairment, usually not independent.
Associated conditions of spinal hemangioblastoma are found in the article e.g.
renal cell carcinoma, cystic disease etc. In addition to the intracranial hemangioblastoma, the highest incidence of spinal cord hemangioblastoma is cervical, then
thoracic and least in the lumbar region. 86.5 % had syrinx.
121.6 Management
Total resection could be done in 95.7 %; subtotal removal in 2.1 %; partial removal
in 4.2 %. The results indicate the spinal cord hemangioblastoma can be removed in
the majority of cases without neurological deterioration. VHL patients frequently
have multiple spinal cord hemangioblastomas requiring multiple operations. It is
recommended to do through removal in the fi rst operation. For the functional outcome after resection of spinal cord hemangioblastoma associated with von Hippel
Landau disease. See article by Mehta et al. ( 2010 ) .
121 Spinal Hemangioblastomas in Von Hippel Lindau Disease
409
References
Kanno H, Yamamoto I, Nishikawa R, et al. Spinal cord hemangioblastomas in von Hippel-Lindau
disease. Spinal Cord. 2009;47(6):447–52.
Mehta GU, Asthagiri AR, Bakhtian KD, Auh S, Oldfi eld EH, Lonser RR. Functional outcome after
resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease.
J Neurosurg Spine. 2010;12(3):233–42.
References
411
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_122
122
Spinal Artery Steal Syndrome in Paget’s Disease of the Bone
122.1 Definition
This a condition of paraparesis or paraplegia in association with Paget’s disease of
the spine not due to cord compression but due to spinal artery steal syndrome due to
blood fl ow diversion in the bone and it is reversible after medical therapy of the
disease (Herzberg and Bayliss).
122.2 Incidence
The great majority of cases appear after 40 years of age. The asymptomatic form
has been identifi ed in 0.1–3 % of the population in the US and UK. The incidence of
neurological complications is probably more than recognized (Porrini et al.). It is
usually due to mechanical factors: enlarged vertebra, collapsed, or fractured vertebrae. Another pathological condition is spinal artery steal syndrome (Herzberg and
Bayliss). Slightly more males are involved.
122.3 Etiology
Pathologically speaking the disease is evolved in three phases:
• lytic
• osteogenic
• osteoblastic
Abstracted from Herzberg and Bayliss ( 1980 )
412
The resorbed bone is replaced by highly vascularized connective tissue. One of
the sequela is hemodynamic change. According to Rablin and Cattran (Pathologic
Bones of Medicine) there is vasodilatation in the skin and subcutaneous tissue overlying the involved bone. When there is 1/3 – ½ of the skeleton is affected there is
increased blood fl ow which may be associated with high output cardiac failure.
122.4 Pathology
Due to increased bony hemodynamics there may be deviation of some spinal arteries blood to the bone leading to paresis in absence of bone enlargement or bony
compression.
122.5 Clinical Picture
From the case described by Porrini et al.:
62 year-old male presented with paraparesis of the lower extremities, wheelchair
bound for 5 years, sensory level at T10, no sphincter dysfunction. X-rays showed
typical Paget’s disease. With collapse at T8 and L4, bone nuclear scan showed
increased uptake in the pelvic bones, dorsolumbar spine, left femur, and the skull.
CT scan showed no compression of the cord. Alkaline phosphatase was twenty
times the normal level at 820 mu/ml; serum Ca and P and acid phosphatase were
within normal. Urinary hydroxyproline was normal.
122.6 Management
Patient received salmon calcitonin 500 MCR 1 per day for 5 days a week and in 2
months his paraparesis was reversed slowly. He was continued on 100 MCR salmon
calcitonin twice a week. After 5 years he was completely asymptomatic.
Reference
Herzberg L, Bayliss E. Spinal-cord syndrome due to non-compressive Paget’s disease of bone: a
spinal-artery steal phenomenon reversible with calcitonin. Lancet. 1980;2(8184):13–5.
1
MCR is the Volume of biological fl uid completely cleared of drug metabolites as measured in unit
time. Elimination occurs as a result of metabolic processes in the kidney, liver, saliva, sweat, intestine, heart, brain, or other site.
122 Spinal Artery Steal Syndrome in Paget’s Disease of the Bone
413
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_123
123
Intramedullary Teratoma
123.1 Definition
Teratomas are lesions composed of a mixture of cell types derived from three germinal layers. On the basis of their histological features teratomas can be classifi ed
into:
• Immature
• Mature
• Malignant
They may occur in many locations in the body including the central nervous
system.
123.2 Incidence
According to Caruso and Colonnese ( 2006 ), 59 cases have been reported. They tend
to occur in children, particularly in midline sites e.g. in the pineal, sellar, suprasellar
sites and the fourth ventricle, excluding the neonatal sacrococcygeal teratoma.
Teratomas of the spinal cord are very rare; accounting for 0.2–0.5 % of all spinal
tumors. According to Fernández-Cornejo:
Intradural spinal teratomas accounts for approximately 0.1 % of all spinal tumors. In 1999,
Poeze et al. (
1999 ) published a literature review where only 83 cases of spinal teratomas
were found. Thirty-one were intramedullary (37 %), most of them occurring in adults
(58 %). In this survey, 42 % of cases were associated with several types of split cord malformations. Diastomyelia, myelomeningocele, tethered cord, and others have been reported in
relation to spinal teratomas. Patients are usually young adults and males (2.6:1).
Abstracted and reported by Fernández-Cornejo et al. ( 2004 )
414
Diplomyelia reported by Mut et al. In adults spinal cord teratomas are rare only
seven cases have been reported and reviewed by Nonomura et al. ( 2002 ), and added
two cases of their own. In adults cervical spinal intramedullary teratoma is quite
rare. According to Arvin et al., in (2009) (Arvin et al. 2009 ), only fi ve cases were
reported and they added the sixth case.
123.3 Pathogenesis
Teratomas are formed from remnants of all three primative germinal layers. The
exact pathogenic factor is not known. A traditional theory is that early in the
embryogenesis, pyramidal germ cells from the yolk sac become misplaced
because of alteration in the cell migration. These cells almost always are found in
the middle line structures where they gibe rise to germ cell tumors including
teratomas.
123.4 Pathology
Totipotential embryonic cells morphed from the yolk sac form the tumor. Teratomas
are of three types as mentioned above:
• Mature or benign: these contain mature tissue e.g. cartilage, squamous epithelium, mucous glands, neural elements—bundles of nerve fi bers. Fan et al.
(2001) (Fan et al. 2001 ), have recorded a case of carcinoid in mature
teratoma.
• Immature: This contains primitive cells of neurepithelial lineage and sometimes
mesenchymal elements. These tend to recur in almost all cases.
• Malignant: The tumor shows the characteristics of malignancy.
Intraspinal teratomas occur posteriorly at cervical or lower thoracic and upper
lumbar regions and may develop in the fi lum terminal, the conus, and the cauda
equina. The histological picture depends on the type of tumor whether mature,
immature, or malignant. They contain dermal, mesenchymal, and endodermal elements, well differentiated mature, primitive in immature.
123.5 Case Report
Depending on the level of the tumor, neurological symptoms are sensory, motor and
sphincteric usually of upper motor neuron type except those of cauda equina.
Associated congenital lesions may be present—especially dysraphism, syrinx,
tethered cord, etc. Skin stigmata e.g. dimples, may be seen.
123 Intramedullary Teratoma
415
123.5.1 Diagnosis
Diagnosis is based on the following criteria:
1. Clinical exam
2. Plain Radiography:
3. erosion of the vertebral body,
4. widening of the intrapedicular space,
5. vertebral anomalies e.g. spina bifi da,
6. vertebral body fusion,
7. asymmetry of vertebral bodies, diastomyelia,
8. soft tissue shadows of the tumor with calcifi cation
9. CT scan is useful as long as it shows variable densities and calcifi cation
10. MRI of the spinal cord: very useful because the tumor exhibits high and low
signal intensities, indicative of tissues heterogeneity, e.g. bone, fat, muscle,
cysts, etc.
123.6 Management
Total excision of the mass is advisable, provided no damage to the neural tissues
occur. Subtotal excision is also practiced and recurrence may not develop for many
years, since tumor regrowth is very rare. Intramedullary tumors are approached
through bilateral laminectomy. If the tumor is not visible posteriorly, intraoperative
ultrasound is important for localization not for embryogenic cyst. In children, laminectomy and laminoplasty are recommended. In the cauda equina laminoplasty is
recommended. Survival in adults with mature teratoma is good. Immature and
malignant teratomas have poor prognosis even with chemotherapy. Incomplete
removal of mature teratoma needs close followup with MRI. Surgery for dysraphism can follow later. When the tumor recurs a second surgery will be tried.
Chemotherapy is of no benefi t.
The operation is conducted under microscopy and somatosensory evoked
potentials for monitoring the integrity of the cord. The use of coagulants is routine. According to Caruso and Colonnese total removal of the tumor was only
possible in 12 out of 59 reported cases. The diffi culty for total resection is due
to teratoma adhesions between the tumor and healthy cord parenchyma. The
same authors share the opinion with Burduck and with controrre that lateral
removal of the tumor is unnecessary risk to the function of the sphincter. The
authors believe that the short- term growth of the tumor itself will apple to its
remnant. Their two cases lasted 7 years and 6 years without recurrence. In the
case of recurrence, the tumor is usually malignant or immature. According to
Mut et al., subtotal resection is mandatory if there are gross alterations in the
signals of SSEP.
123.6 M ana geme nt
416
References
Arvin B, Pohl U, David K. Intramedullary cervical teratoma in an adult. Spine
J. 2009;9(5):e14–8.
Caruso R, Colonnese C. Intramedullary teratomas: two case reports and a review of the literature.
Zentralbl Neurochir. 2006;67(4):213–8.
Fan X, Turner JE, Turner TM, et al. Carcinoid tumor development in an intramedullary spinal cord
mature teratoma. AJNR Am J Neuroradiol. 2001;22(9):1778–81.
Fernández-Cornejo VJ, Martínez-Pérez M, Polo-García LA, Martínez-Lage JF, Poza M. Cystic
mature teratoma of the fi lum terminale in an adult. Case report and review of the literature.
Neurocirugia (Astur). 2004;15(3):290–3.
Nonomura Y, Miyamoto K, Wada E, et al. Intramedullary teratoma of the spine: report of two adult
cases. Spinal Cord. 2002;40(1):40–3.
Poeze M, Herpers MJ, Tjandra B, Freling G, Beuls EA. Intramedullary spinal teratoma presenting
with urinary retention: case report and review of the literature. Neurosurgery.
1999;45(2):379–85.
123 Intramedullary Teratoma
Part VII
Toxic Causes of Myelopathy
419
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_124
124
Benzene Myelitis
124.1 Definition
This is a condition of myelopathy due to chronic exposure to benzene fumes.
124.2 Incidence
One case reported by P Herregods et al.
124.3 Etiology
Benzene (Bz) is the prototype aromatic hydrocarbon, since it is composed of only
the benzene ring. As such, and because of its physical properties, it is widely used
in the chemical industry as a feedstock for chemical reactions and less commonly as
a solvent. So frequent is its use as a constituent of commercial products that most
people probably have at least inhaled its fumes. It is a myelotoxin: chronic exposure
of humans and experimental animals to high concentrations results in blood dyscrasis including lymphocytopenia, thrombocytopenia, and pancytopenia or aplastic
anemia. It is also a carcinogen.
124.4 Pathology
Quoting from Herregods et al.
The neurotoxicity of benzene can be described as follows. The aromatic hydrocarbons have
a particular affi nity for nerve tissue because of their high lipid content. The presence of
Abstracted from Herregods et al. ( 1984 )