Thoracic osteochondroma with spinal cord

Thoracic osteochondroma with spinal cord compression
1. Vaquero; R. Martínez; J. C. Gómez-Angulo and P. Aragonés
Service of Neurosurgery. Puerta de Hierro Clinic. Autonomous University. Madrid
Summary
«familial osteochondromatosis», and «hereditary multipIe exostoses».(1.7.12.16)
A case of osteochondroma at T5 level, impinging on
The cause of hereditary multiple osteochonthe spinal cord in a patient previously diagnosed of
dromatosis is unknown, males are predominantly, and
is presented. Spinal el 21/11/2016.
hereditary osteochondromatosis,
Documento descargado de http://www.revistaneurocirugia.com
Copia para uso personal, se prohíbe la transmisión de este documento por cualquier m
malignant
neoplastic transformation occur in 5 % to
compression is an infrequent event in the course of this
25 % of patients. (1.3, 6, 7, 9. 13-17)
disease. Nevertheless, one must consider this possibility
Osteochondromas usually arise at the end of long
in the follow-up of the patients with this relatively combones,
but they can involve the spine in 7% of the
mon bone dysplasia.
cases,'3) and although has been reported that spinal
osteochondromas are 10cated mainly at the thoracic and
KEY WORDS: Osteochondroma, Spinal cord, Spine.
lumbar areas,(l3) a recent review of 94 cases of spinal
osteochondromas showed that 39 % arose in the cervical spine, 28% in the lumbar spine, 29% in the
thoracic spine, and 3% in the sacrum.(14) In any case,
Resumen
neurological complications due to spinal cord compression are rare,<2· 4.8,12.13.15,17) appearing in 0,5% of the paSe presenta un caso de osteocondroma espinal, a nivel
tients, roughly(l2)
T5, en una paciente previamente diagnosticada de
In the present report, we describe a case of thoracic
osteocondromatosis hereditaria. Aunque la aparición de
spinal cord compression due to an osteochondroma,
un cuadro de compresión medular por osteocondromas
in a patient with hereditary multiple osteochonraquídeos es relativamente infrecuente en el curso de la
dromatosis.
osteocondromatosis hereditaria, su posibilidad debe ser
tenida en cuenta en el control evolutivo de los pacientes
con esta displasia ósea.
Case report
PALABRAS CLAVE: Osteocondroma, Médula espinal,
Raquis.
Introduction
Multiple osteochondromatosis is a relatively common bone dysplasia which is usually hereditary. According to O'Connor and Roberts (12) it was first
described by Stanley in 1849. Later, this entity was
extensively reviewed by Ehrenfried, in 1917,(5) who
called the process «hereditary deforming chondrodysplasia». It also has been designated as
«diaphyseal aclasis», «multiple cartilaginous exostoses»,
This 29-year-old woman had been previously
diagnosed of hereditary multiple osteochondromatosis.
She was hit in the back approximately 18 months prior
to admission. Initially, she suffered back pain, but the
symptoms gradually progressed to pain radiating to the
mid-scapular region, and weakness of both legs
developed.
Neurological exarnination showed muscle strenght
of 4/5 on both legs. Her reflexes were hyperreactives on both legs, and Babinski sign was present
bilaterally.
Plain spine X-ray showed a lesion at the right side
of the T5 vertebral body that suggested an ostochondroma. Numerous osteochondromas were radiologically disclosed at the ribs and left wrist. Myelography and
125
Neurocirugía
Thoracic osteochondroma with spinal cord compression
subsequent CT-scan showed a calcified mass impinging on the spinal cord (Figo 1 and 2)0 The lesion
was originated in the head of the right rib at TS
level. MRI study confirmed the lesion, impinging
on the spinal cordo The tumor was hypointense, with
zones of increased signal on T2-weighted images
(Fig. 3).
The patient underwent surgery via a posterior approach to the thoracic spine; through a laminectomy
a mostly calcified epidural tumor was removed by drill-
Documento descargado de http://www.revistaneurocirugia.com el 21/11/2016. Copia para uso personal, se prohíbe la transmisión de este documento por cualquier m
Fig. 2.-CT-scan after myelography, showing the tumor within the spinal
canal and the spinal cord compression.
Fig. 3.-Magnetic Resonance, showing the hypointense tumor, with areas
of hyperintense signal, on T2 weighted images.
Fig. l.-Myelographic study with intratecal metrizamide, showing the
calcified tumor and the spinal cord compression (arrows).
Discussion
ing. The postoperative course was unremarkable, and
she returned home within one week.
Pathological study showed hyaline cartilage, bone
and marrow with fat tissue (Fig. 4)0 A diagnosis of
osteochondroma was establishedo
126
Recently, a case of osteochondroma of the thoracic
spine was reported by Roblot et al.(14) and after an excellent review of the literature, these authors collect
on1y 94 previous cases of spinal osteochondroma. According to this review also, osteochondromas are rarely
Thoracic osteochondroma with spinal cord compression
Neurocirugía
seen on the spine, and this localization represents between
Many authors consider osteochondromas as
2,5 % and 3,3 % of the localizations of osteochondromas.
developmental anomalies rather than true tumors. These
Nevertheless, osteochondromas represent 7,6 % of the
lesions begin in early childhood, and they grow at the
benign cartilaginous neoplasms of the spine. (14)
same time as the host bone until the nearest epiphyseal
center ossifiesY' 12) Nevertheless, it is obvious that, in
Our present case is added to the previous reports about
spinal cord compression in hereditary osteochondromasorne cases, osteochondromas with spinal involvement
may produce spinal cord compression. They usually
tosis. In our patient, the osteochondroma arose from
become symptomatic during the 2nd or 3rd decades, (8)
the head of a rib, and caused spinal cord compression.
Pathologically, osteochondromas occur within the
like in our present case. Surgical removal usually obperiosteum of bones with endochondral ossification,
tains excellent clinical results in these patients.
and usually grow progressively by endochondral
ossification of their cartilaginous caps. They are surrounded by a thin layer of hyaline cartilage, and
References
covered by the periosteum of the parent bone. (1, 7) The
1. AERGERTER, E.; K!RKPATRICK, 1. A.: Orthopaedíc Díseases, ed
most part ofDocumento
the tumor
is composed of cancellous bone
4. Philadelphia.
WBpersonal,
Saunders,
1975, lapp.
121-128.de este documento por cualquier m
descargado de http://www.revistaneurocirugia.com el 21/11/2016.
Copia para uso
se prohíbe
transmisión
2. BUCKLER, R. A.; CHAO, D. A.; SMITH, T. W.; DAVIDSON, R. I.:
interspersed with marrow. (1, ll) In our case microscopical
SCIATICA: An early manifestation of thoracic vertebral osteochondroma.
study disclosed a great amount of fat within the marNeurosurgery, 1987; 21:98-100.
3. CARMEL, P. W.; CRAMER, F. J.: Cervical cord compression due
row, and this fact led us to explain the increase signal
to exostosis in a patient with hereditary multiple exostoses. Case report
within the tumor on MRI images.
J. Neurosurg. 1968; 28:500-503.
Fig. 4.-Histological aspect ofthe resected tumor, showing cartilage, bone,
and marrow wíth fat cells. H. E. techníque, original magnification x250.
As previously reported cases of spinal osteochondroma have demonstrated, CT-scan is useful for
establishing the origin of the lesion and for delineating
the tumor from the spinal cord. (2,8, 10,13,14)
In 75 % of patients with multiple osteochondromatosis
a strong familial inheritance is disclosed, and when farnily
trees are studied, a clear predominance of males is present. (12,14) A single autosomal-dominant gene may be
responsible for the clinical disease in heterozygote patients
while it could be suppressed by an independently
segregated autosomal-dominant gene, this gene would be
frequently latent in females, and this could be the reason
by which the sex ratio líeavily favours the males. (9,12)
4. CHIURCO, A. A.: Multiple exostoses of bone with fatal spinal
cord compression. Report of a case and review of the literature. Neurology,
1970; 20:275-278.
5. EHRENFRIED, A.: Hereditary deforming chondrodysplasiamultiple cartilaginous exostoses. A review of the American literature and
report of twelve cases. JAMA, 1917; 68:502-508.
6. FIELDING, 1. W.; RATZAN, S.:Osteochondroma of the cervical
spine, J. Bone Joint, Surg. (Am) 1973; 55: 640-641.
7. JAFFE, M. L.: Tumors and Tumorous Conditions of Bones and
¡omts. Philadelphia, Lea and Febiger, 1958, pp. 150-165.
8. KARIAN, J. M.; DEFILIPP, G.; BUCHHEIT, W. A.; BONAKDARPUR, A.; ECKHARDT, W.: Vertebral osteochondroma causing spinal cord
compression: Case report, Neurosurgery, 1984; 14:483-484.
9. KROOTH, R. S.; MACKLIN, M. T.; HILBISH, T. F.: Diaphysial
aclasis (multiple exostoses) on Guam. Am. J. Hum. Genet. 1961;
13:340-347.
10. LOFTus, C. M.; ROZARlO, R. A.; PRAGER, R.; SCOTT, R. M.:
Solitary osteochondroma of T4 with thoracic cord compression. Surg.
Neurol. 1980; 13:355-357.
11. MAC CEE, E. E.: Osteochondroma of the cervical spine: a cause
of transient quadriplegia. Neurosurgery, 1979; 4:259-260.
12. O'CONNOR, G. A.; ROBERTS, T. S.: Spinal cord compression by
an osteochondroma in a patient with multiple osteochondromatosis. J.
Neurosurg. 1984; 60:420-423.
13. PALMER, F. J.; BLUM, P. W.: Osteochondroma with spinal cord
compression: Report of three cases. J. Neurosurg. 1980; 52:842-845.
14. ROBLOT, P.; ALCALAY, M.; CAZENAVE-RoBLOT, F.; LEVY, P.;
BONTOUX, D.: Osteochondroma of the thoracic spine. Report of a case and
review of the literature. Spine, 1990; 15:240-243.
15. SLEPIAN, A.; HAMBY, W. B.: Neurologic complications
associated with hereditary deforming chondrodysplasia. Review of the
literature and report on two cases occurring in the same family. 1.
Neurosurg. 1951; 8:529-535.
16. TUREK, S. L.: Orthopedics, ed 3. Philadelphia. J. P. Lippincott,
1977, pp. 321-322.
17. VINSTEIN, A. L.; FRANKEN, E. A. JR.: Hereditary multiple exostoses. Report of a case with spinal cord compression. Am. J, Roentgenol.
1971; 112:405-407.
Vaquero, J.; Martínez, R.; Gómez-Angulo, J. C. and
Aragonés, P.: Thoracic osteochondroma with spinal cord
compression. Neurocirugía, 1991; 2: 125-127.