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Medical and Pediatric Oncology 32:57–59 (1999)
Vertebra Plana Due to a Ewing Tumor
L. Kager, MD,1 A. Zoubek, MD,1 R. Kotz, MD,2 G. Amann, MD,3
P. Wiesbauer, MD,1 W. Dobrowsky, MD,4 and H. Gadner, MD1*
Key words: vertebra plana; Langerhans cell histiocytosis; Ewing tumor;
multimodal therapy
The radiological diagnosis ‘‘vertebra plana’’ indicates
the most severe degree of compression of the vertebral
body [1]. The collapse of the vertebral body can be
caused by various pathologic conditions. However, the
most common cause in children is Langerhans cell histiocytosis (LCH) [2]. Localized LCH of the bone is a
benign and often spontaneous regressing disorder. In
typical vertebra plana, biopsy confirmation of the presumptive diagnosis has been considered unnecessary by
some [3]. Our experience with a child with vertebra plana
of the third cervical vertebra due to a Ewing tumor is
relevant and illuminating.
The patient was an 8-year-old girl with neck pain of 2
months duration who was first admitted to another hospital. Physical examination demonstrated a tenderness to
percussion in the neck, but no neurological deficit. Plain
films of the cervical spine revealed a typical vertebra
plana of C3 (Fig. 1). The most likely diagnosis was LCH
of the spine and therefore no biopsy was performed.
Treatment with analgesic drugs and rest initially resolved
her complaints. Four months later, the girl was referred to
our hospital with rapid progressive tetraparesis. Magnetic
resonance imaging (MRI) of the cervical spine revealed
a collapsed C3 vertebral body and a large intraspinal
(C1–C4) mass (Fig. 2). A fine-needle biopsy was performed and revealed an MIC-2/CD99 positive, EWS/
Fli-1 rearranged small blue round cell tumor without
neural differentiation, compatible with the diagnosis of a
Ewing tumor. Extensive examination showed no other
tumor sites.
Chemotherapy according to the EICESS 92 protocol
[4] and radiotherapy were immediately started and neurological function improved quickly. After five cycles
(EVAIA) of a combination of etoposide, vincristine, ifosamide, and alternating doxorubicin and dactinomycin
and accelerated fractionated radiation of the third cervical vertebral body with 54 Gy, an impressive reduction in
tumor size was noted by MRI. Four months after diag© 1999 Wiley-Liss, Inc.
nosis, tumor resection by a ventral approach was performed. The third cervical vertebral body was resected,
followed by anterior fusion with autologous iliac crest
bone graft and plating. She then underwent resection 4
months later by a posterior approach with laminectomy
at C3 and removal of the posterior elements close to the
vertebral arteries on both sides. Posterior spinal fusion
from C2 to C4 was accomplished with bone grafts anchored by sublaminar wires (Fig. 3). Neither resected
specimen showed any viable tumor cells. The girl was
then treated with 7 cycles of EVAIA over 10 months.
Currently, she is 6 years off therapy and in good general
condition with no evidence of disease and without neurological deficits.
In 1925, Calvé [5] reported two children with radiologically detected flattened vertebral bodies and suggested aseptic necrosis of the involved bones as the
cause. Buchman [6] suggested the term ‘‘vertebra plana’’
for lesions characterized by a) total collapse of only one
vertebra, b) no involvement of the intervertebral disc, c)
the intravertebral space at least one third wider than normal, and d) increased density of the involved vertebra.
Later on it was proven histopathologically that acquired
vertebra plana in children was in most cases a manifestation of LCH [2,3]. Less common causes include trauma
[1], metabolic and endocrine disorders [1], infectious diseases [1], myofibromatosis [7], aneurysmal bone cyst [8],
neurofibromatosis [1], hemangiomas [8], and malignancies. Among the malignant disorders, osteosarcoma [9],
lymphoma [8], neuroblastoma [8], leukemia [8], rhabdo1
St. Anna Children’s Hospital, Vienna, Austria
Department of Orthopedics, University of Vienna, Vienna, Austria
Department of Pathology, University of Vienna, Vienna, Austria
Department of Radiotherapy, University of Vienna, Vienna, Austria
*Correspondence to: H. Gadner, Professor of Pediatrics, St. Anna
Children’s Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria. Email:
Received 29 June 1998; Accepted 7 July 1998
Kager et al.
Fig. 1. Conventional tomography of the cervical spine at initial presentation revealed a typical vertebra plana of the third cervical vertebra.
Fig. 2. Sagital T1 weighted MRI (after administration of gadolinium-GPTA) four months after initial presentation revealed again the
collapsed third cervical vertebra and additionally a large intraspinal
(C1–C4) mass.
myosarcoma [10], Hodgkin disease [10], and four cases
of Ewing sarcoma [3,8,11,12] have been described. The
few reported cases of vertebra plana caused by malignancies were often initially misdiagnosed as LCH, resulting in a regrettable diagnostic delay [9–12] since early
therapeutic interventions are highly desirable.
Conventional radiography of vertebra plana is unable
to identify the underlying disesase process. MRI, although also nonspecific, offers optimum detection of extraosseous tumor invasion of adjacent tissues; but ultimately a biopsy is needed to establish the diagnosis.
Fine-needle sampling often yields inadequate tissue
specimens [11]. Some authors therefore recommend
open biopsy [8,9].
Ewing tumor is the general term for a group of small
round-cell neoplasms that include Ewing sarcoma, extraosseus Ewing sarcoma (EES), and peripheral primitive
neuroectodermal tumors (pPNET) [13]. They are biologically characterized by an EWS-gene rearrangement with
either FLI-1, ERG, ETV1, E1A-F, or FEV and high expression of the MIC2/CD-99 antigen in at least 95% of
Primary Ewing tumor arising in the vertebral column
including the sacrum and paravertebral region is rare.
The incidence of primary vertebral involvement in Ewing sarcoma and pPNET is approximately 3% [12,14–
16], paraspinal pPNET occurs in about 5% [15], and 16
cases of primary spinal epidural EES have been reported
Almost all cases with ET of the spine at presentation
suffered from local pain that precedes the diagnosis by
2–8 months [12,16]. Neurological signs and symptoms
are reported to be present in 60–80% [12,16].
Almost all Ewing sarcoma patients published in the
last two decades were treated by a multimodal approach
to achieve local and systemic tumor control [12,16,17].
Systemic chemotherapy is the mainstay of treatment,
while the relative roles of surgery and radiotherapy in
local tumor control are still evolving. There is a high rate
of only partially resectable tumors in primary spinal epidural EES (about 64%) with a high rate of local recurrence [17]. These data suggest that complete surgical
removal may play an important role in local tumor control. Our experience does not help to resolve this issue.
On the one hand, excision was performed in two steps as
described; on the other, no viable tumor cells after chemoradiotherapy could be detected in the resected specimens.
Ewing Tumor and Vertebra Plana
EES, 63% died at a median 16 months after diagnosis
We conclude that the diagnosis of LCH should not be
assumed whenever vertebra plana is detected radiographically. This erroneous diagnosis led to a 4-month
delay in instituting appropriate therapy in our patient.
Thus, we recommend that when vetebra plana is detected
in a child with back pain and when the physical examination elicits additional neurologic signs, the lesion
should be regarded as malignant until proven otherwise.
Ewing tumor should be included in the differential diagnosis. Rapid diagnostic workup including biopsy is
needed. Ewing tumor of the spine is rare, and combined
modality treatment, as described in our case, can be effective.
Fig. 3. Plain radiograph taken 1 month following excision of C3 and
spine stabilization (see text).
Radiotherapy has been employed for definitive treatment irradiation or as an adjuvant in doses ranging from
30 to 60 Gy [12,16,17]. Prudence is needed because,
using conventional fractionation at doses above 45 Gy,
postirradiation myelopathy can occur. Also to be remembered is the 5–10% frequency of second malignancies in
irradiated sites within 20 years after treatment [13]. Our
patient was treated by an accelerated fractionated schedule and received a total of 54 Gy on the third vertebra.
Nonetheless, preoperative irradiation can play an important role when used to improve neurological impairment
quickly, as in our case.
Pilepich et al. [16] reported 100% local control and
86% 5-year disease-free survival of 14 patients diagnosed between 1973 and 1977 with Ewing sarcoma of
the spine proximal to the sacrum, treated according to the
Intergroup Ewing Sarcoma Study protocol. In that study,
eight patients with Ewing sarcoma of the sacrococcygeal
segments showed local control in 62.5% and a disease
free-survival in only 25% of the cases [16]. This influence of the primary site on prognosis could not be confirmed by Grubb et al. [12], who found a 5-year survival
rate of 33% in 36 patients with vertebral Ewing sarcoma
registered between 1951 and 1988, independent of the
primary site. In 16 patients with primary spinal epidural
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