Case Report Acute Paraparesis Caused by a Giant Cell Tumor of

台灣癌症醫誌 (J. Cancer Res. Pract.) 1(3), 260-266, 2014
DOI: 10.6323/JCRP.2014.1.3.11
journal homepage:www.cos.org.tw/web/index.asp
Case Report
Acute Paraparesis Caused by a Giant Cell Tumor of the Thoracic
Spine
Liang-Chun Chao1, Wei-Sheng Juan1, Che-Chao Chang1, Shih-Huang Tai1, Ming-Tsung Chuang2,
Chun-I Sze3, Yu-Chang Hung1, E-Jian Lee1*
1
Neurosurgical Service, Department of Surgery, National Cheng Kung University Hospital, College of Medicine,
National Cheng Kung University, Tainan, Taiwan
2
Department of Diagnostic Radiology, National Cheng Kung University Hospital, College of Medicine, National
Cheng Kung University, Tainan, Taiwan
3
Department of Pathology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung
University, Tainan, Taiwan
Abstract.
Giant cell tumor (GCT) is a benign but locally aggressive skeletal neoplasm of young
adults. GCT located in the spine is relatively rare and may need a combination of surgical and
adjunctive therapies. Here we present a patient who had intermittent thoracic back pain for two
weeks and experienced an acute episode of decreased muscle power of both lower limbs.
Magnetic resonance (MR) imaging examinations of the thoracic spine revealed that the patient
had severe spinal canal compression caused by pathological fracture due to a tumor within the
seventh thoracic vertebra. She underwent an emergent surgical intervention for total removal of
the tumor and spinal reconstruction with autologous rib grafts and instruments. Postoperatively,
the patient made an uneventful recovery of muscle power of bilateral lower limbs. She subsequently received adjuvant radiotherapy. In a follow-up period of 36 months, the patient had no
clinical or radiological evidence of tumor recurrence. Even though spinal location for GCT is a
rare event, it should be included in the differential diagnosis in patients with osteolytic lesions
or pathological fractures of the vertebra, especially in young female patients sustaining no
trauma who had a clinical history of persistent low back pain.
Keywords : acute paraparesis, pathological fracture, spinal giant cell tumor
病例報告
胸椎骨巨細胞瘤造成急性雙下肢癱瘓
趙亮鈞 1
阮威勝 1 張哲肇 1
戴世煌 1
莊明宗 2
司君一 3
洪裕昌 1
李宜堅 1*
1
國立成功大學醫學院附設醫院神經外科
國立成功大學醫學院附設醫院放射診斷部
3
國立成功大學醫學院附設醫院病理部
2
中文摘要
骨巨細胞瘤對年輕人來說是一種良性但具局部侵犯性的腫瘤，而發生在脊椎的骨巨
L. C. Chao et al./JCRP 1(2014) 260-266
261
細胞瘤相對來說是少見的，在治療上必須合併手術以及後續的放射線治療。在此我們提
出一個病例報告，病人經歷兩個禮拜的間歇下背痛且惡化成為急性雙下肢無力，胸椎核
磁共振檢查中發現胸椎第七節的腫瘤造成病理性骨折以及嚴重的脊髓壓迫，在施行緊急
的腫瘤移除以及前固定手術後，下肢的肌力恢復到正常。病人後續接受脊椎的放射線治
療，追蹤到目前並無復發的情形。儘管脊椎骨巨細胞瘤相當少見，我們仍然強調在診斷
脊椎的病理性骨折以及蝕骨性病灶時，必須將其列為鑑別診斷之一，尤其是在沒有外傷
病史卻有長期下背痛的年輕女性身上。
關鍵字: 急性下肢無力、病理性骨折、脊椎骨巨細胞瘤
INTRODUCTION
weakness accompanied with circumferential chest and
Giant cell tumor (GCT) is a solitary neoplasm
back pain. She was also found to have severe weak-
mostly involving the distal femur or proximal tibia.
ness (Motor Grade=2/5) of both lower limbs with
Spinal localization for GCT is a relatively rare event,
sparing of the urinary and bowel sphincter function.
and predominantly involves the vertebral body of the
Cranial nerve functions and other neurologic exami-
lumbosacral spine. It is estimated that the prevalence
nations were unremarkable. The MRI images of the
of spinal involvement in GCTs is less than 3%. In par-
thoracic spine showed a tumor-induced pathological
ticular, the involvement of GCT in the thoracic spine
fracture of the T7 vertebra with severe compression of
is extremely rare [1]. We, herein, report a patient who
the spinal cord (Figure 1). The patient was diagnosed
presented with acute paraparesis due to pathological
to have acute spinal cord compression caused by a
fractures of the seventh thoracic (T7) vertebra caused
metastatic lesion of the T7 vertebra. She subsequently
by a GCT. Following combined surgical and irradia-
underwent an emergent thoracotomy and T7 corpec-
tion therapies, this patient made a favorable neuro-
tomy for total excision of the tumor and, then, spinal
logic recovery. Demographic data, characteristic im-
reconstruction with autologous rib grafts (part of left
aging findings, treatment and outcome of the patient
7th and 8th ribs) and instruments with a vertebral
are discussed.
screwing and rod system (AO internal spine fixation
system, Spine-tech, Orion). Pathological examinations
CASE REPORT
showed that the lesion was a GCT of the T7 vertebra
A 27-year-old female patient who had no history
which revealed diffuse infiltration by numerous of
of systemic disease suffered from progressive intense
mononuclear cells with round to oval nuclei and oste-
posterior thoracic pain located in the midline around
oclast-like multinucleated giant cells (Figure 2).
the 7th and 8th thoracic vertebrae for two weeks, and
Postoperatively, the patient made an uneventful re-
the pain was reported to radiate to the anterior costal
covery. Her muscle power of both lower limbs gradu-
margin. On the day of admission, she experienced an
ally recovered to normal levels (muscle power scale=
acute onset of bilateral lower limbs numbness and
5/5, bilaterally) within 2 weeks after the operation.
The patient was examined with positron emission tomography (PET) examinations which revealed that the
*Corresponding author: E-Jian Lee M.D., Ph.D.
patient did not have other detectable lesions. The pa-
*通訊作者：李宜堅醫師
tient then received focal external beam radiotherapy
Tel: +886-6-2353535 ext.5204
up to 50 Gy in 25 fractions. The patient tolerated the
Fax: +886-6-2766676
treatment well. She remained progression-free and did
E-mail: [email protected]
not have notable clinical or MRI evidence of recur-
262
L. C. Chao et al./JCRP 1(2014) 260-266
A
B
C
D
Figure 1. The T2-weighted MR imaging demonstrated (A) pathological fracture caused by a low intensity lesion
within the 7th thoracic vertebra. In the axial sections (B), the tumor was noted to have bilateral extension to posterior spinal elements that resulted in severe spinal cord compression. After contrast enhancing(C,D), the tumor showed heterogeneous enhancement, showing possibility of myeloma, lymphoma,
Langerhans cell histiocytosis in differential diagnosis
L. C. Chao et al./JCRP 1(2014) 260-266
263
Figure 2. Pathological examinations of the giant cell tumor (A, B) revealed a characteristic background network
of stromal mononuclear cells incorporated with many multinucleated giant cells (asterisks)
Figure 3. The follow-up MR images (A, B) revealed incorporation of rib grafts and instruments and satisfactory
consolidation of fusion mass. There was no radiological evidence of tumor recurrence up to 36 months
after surgery
rence, now 36 months after the combined treatment
(Figure 3). She was symptom-free, having returned
her previous work one month after surgery.
DISCUSSION
GCT is a relatively rare, benign osteolytic skeletal
neoplasm of young adults with predominance in females. It mostly occurs in the long bones near articu-
264
L. C. Chao et al./JCRP 1(2014) 260-266
lations, and usually has a benign clinical presentation.
in many tumors. The tumor may breach the cortex and
GCT localized in the spine is uncommon but may
extend into soft tissues. Microscopically, GCTs are
present with a locally aggressive clinical course due to
comprised of multinucleated, osteoclastic giant cells,
a complex anatomical configuration of the vertebrae [1].
which are thought to arise from mononuclear cells of
Shankman and colleagues demonstrated that the prev-
macrophage origin [12]. The tumor exhibited promi-
alence of spinal localization was 2.7 % out of 1277
nent vascularity and hemorrhage. It is generally ac-
reported patients with a GCT. Most spinal GCTs oc-
cepted that the characteristic large osteoclastic giant
cur in the sacrum, followed in order of decreasing
cells are not neoplastic. The mononuclear cells, which
frequency by lumbar, cervical, and thoracic segments
represent the neoplastic component, are thought to
[2,3]. Actually, we have extensively reviewed the
arise from primitive mesenchymal stromal cells [13].
English literature in the past 10 years, and have found
Histologic grading does not appear to be of value in
only twenty one reported cases that had presented with
predicting either locally aggressive behavior or the
low back pain and were diagnosed to have a GCT in
development of metastases. The stromal cells show no
the thoracic vertebra [3-6]. Therefore, it is evident that
cytologic features suggestive of malignancy, unless
thoracic spine GCT is a very rare event leading to
associated with malignant transformation.
acute paraparesis and need emergent decompressing
surgery.
Radiographically, GCT of bone usually manifests
itself as a destructive, osteolytic lesion on plain films.
Patients with a spinal GCT most commonly pre-
The CT and MRI studies can provide more infor-
sent with persistent back pain [7]. A lumbar spine
mation on the extent of bony involvement and the
GCT with the involvement of the sacral plexus may
degree of surrounding soft tissue involvement [3].
induce bladder and bowel symptoms [8,9]. A large
The characteristic MR findings for spinal GCTs in-
GCT tumor of the cervical/thoracic vertebrae will re-
clude a hypervascular mass with cystic changes with
sult in sensory and motor deficits, or even paraplegia
heterogeneous, low to intermediate signal intensity
of extremities secondary to spinal cord compression
on T1-weighted images and low to similar signal in-
[10]. It has been proposed that female hormones, es-
tensity relative to the normal spinal cord on T2-
pecially estrogen and progesterone, may account for
weighted images. In contrast enhanced image, GTC
rapid tumor growth of GCTs, because a patient with
shows heterogeneous accentuating of the extent of
pregnancy has been found to have rapid recurrence of
paraspinal and epidural disease. Hemorrhage may
spinal GCT [11]. In our case, the patient suffered from
occur and cause heterogeneous signals due to hemo-
persistent back pain for only two weeks prior to her
siderin. Compression fractures may also be observed
acute onset of paraparesis. The exact mechanisms by
with tumors at the thoracolumbar spine. These MR
which the tumor has a course of rapid expansion is not
findings are essential to make a differential diagnosis
currently known, since our patient actually presented
from metastastic tumors because most metastases
with normal levels of circulating estrogen and also did
show high signal intensity [8,12]. Both tumors,
not have pregnancy or other hormone-secreting tu-
however, may need adjuvant radiation following sur-
mors. Thus, the spectrum of disease behaviors in spi-
gical excision because these tumors usually arise
nal GCTs may be highly variable and unpredictable,
from the vertebral body but their expansive or infil-
especially in young female patients, despite the gener-
trative parts may involve epidural space or posterior
ally benign nature of the GCTs.
elements.
Grossly, GCT is a fleshy, reddish, and homoge-
Campanacci et al proposed a grading system for
nous tumor. Focal hemorrhage or necrosis can be seen
GCTs that is based on the radiographic appearance of
L. C. Chao et al./JCRP 1(2014) 260-266
265
Table 1. Campanacci radiological grading system for giant cell tumors of bone
Grade
Description
1
Intramedullary lesion confined to bone
2
Thinned, expanded cortex
3
Cortical breakout
Table 2. Enneking classification of giant cell tumors of bone
Stage
Description
Clinical presentation
I (latent)
Confined totally by bone
Asymptomatic
Histologically benign
Inactive on bone scan
Expanded cortex but no breakthrough
Symptomatic
Histologically benign
Active on bone scan
II (active)
Pathological fracture
III (aggressive)
Malignant
Cortical perforation with soft tissue mass
Symptomatic
Histologically benign
Extensive active on bone scan
Sarcomatous lesion
May metastasis
the tumors (Table 1) [14]. It is similar to that proposed
mains to be the gold standard in the management of
by Enneking for benign bone tumors (Table 2) [15],
GCT, because an en bloc resection with wide safe
but lower correlation exists between the grading sys-
margins provides the possibility for surgical cure [12].
tems and the incidence of local recurrence or metasta-
In the management of spinal GCTs, the complex con-
ses. The Enneking classification combines radiologi-
figuration of vertebrae with the involvement of vital
cal and histological characters and corresponds more
structures, however, precludes the attempt to obtain an
closely to clinical presentations. In the present case,
en bloc resection with wide safe margins. In such a
the tumor extended bilaterally to the posterior spinal
case, radiotherapy is a reasonable alternative option to
elements, pre-vertebral area and epidural space with
decrease the local recurrence rate with preservation of
cortical destruction in MRI studies. So, it can be de-
spinal function, because GCT is a radiosensitive tu-
fined as a grade 3 GTC by the Campanacci Radiolog-
mor [8,16]. With the rapid advances in radiation ther-
ical Grading System. According to the pathological
apy and new techniques, disadvantages of the radio-
report, there was no obvious evidence of malignant
therapy may further be minimized, including local
change. Thus, it can be defined as a stage III (aggres-
radiation effects or the potential for sarcomatous
sive) GTC by the Enneking classification and pre-
transformation.
sented a rapid growing and symptomatic mass as expected.
In summary, we report a rare case presenting with
acute onset of paraparesis due to a GCT originated
The management of GCT is predicated on the an-
from the T7 thoracic vertebra. Through a combination
atomic site of the tumor, the extent of bone destruc-
of emergent surgical excision and adjuvant radiother-
tion, and the mitotic potential in pathological exami-
apy, the patient made an uneventful recovery. We
nations. It needs to be emphasized that surgery re-
emphasize that spinal loocalization for the GCT, alt-
266
L. C. Chao et al./JCRP 1(2014) 260-266
hough rare, should be included in the differential di-
mid-to-long-term results. Spine 1 32: 1107-15,
agnosis for patients with pathological fractures of the
2007.
vertebrae, especially in those non-traumatized young
patients.
8. Leggon RE, Zlotecki R, Reith J, et al. Giant cell
tumor of the pelvis and sacrum: 17 cases and
analysis of the literature. Clin Orthop Relat Res
REFERENCES
1. Ozaki T, Liljenqvist U, Halm H, et al. Giant cell
tumor of the spine. Clin Orthop Relat Res:
194-201, 2002.
2. Shankman S, Greenspan A, Klein MJ, et al. Giant
cell tumor of the ischium. A report of two cases
and review of the literature. Skeletal Radiol 17:
46-51, 1988.
3. Refai D, Dunn GP, Santiago P. Giant cell tumor of
the thoracic spine: case report and review of the
literature. Surg Neurol 71: 228-33, 2009.
4. Matsumoto M, Ishii K, Takaishi H, et al. Extensive total spondylectomy for recurrent giant cell
(423): 196-207, 2004.
9. Randall RL. Giant cell tumor of the sacrum.
Neurosurg Focus 15: E13, 2003.
10. Ross AE, Bojescul JA, Kuklo TR. Giant cell tumor: a case report of recurrence during pregnancy.
Spine 30: E332-5, 2005.
11. Kwon JW, Chung HW, Cho EY, et al. MRI findings of giant cell tumors of the spine. AJR Am J
Roentgenol 189: 246-50, 2007.
12. Luther N, Bilsky MH, Härtl R. Giant cell tumor
of the spine. Neurosurg Clin N Am 19: 49-55,
2008.
13. Salerno M, Avnet S, Alberghini M, et al. Histo-
tumor in the thoracic spine. J Neurosurg Spine 6:
genetic characterization of giant cell tumor of
600-5, 2007.
bone. Clin Orthop Relat Res 466: 2081-91,
5. Yang SC, Chen LH, Fu TS, et al. Surgical treatment for giant cell tumor of the thoracolumbar
spine. Chang Gung Med J 29: 71-8, 2006.
6. Doita M, Miyamoto H, Nishida K, et al. Giantcell tumor of the tendon sheath involving the thoracic spine. J Spinal Disord Tech 18: 445-8,
2005.
7. Hosalkar HS, Jones KJ, King JJ, et al. Serial arterial embolization for large sacral giant-cell tumors:
2008.
14. Campanacci M, Giunti A, Olmi R. Metaphyseal
and diaphyseal localization of giant cell tumors.
Chir Organi Mov 62: 29-34, 1975.
15. Ennecking W. Musculoskeletal Tumor Surgery.
New York, NY Churchill Livingstone, 1983.
16. Sanjay BK, Sim FH, Unni KK, et al. Giant-cell
tumours of the spine. J Bone Joint Surg Br 75:
148-54, 1993.

Download Report