Keywords: Meningeal hemangiopericytoma; isolated metastasis; radiotherapy
Most hemangiopericytomas are located in the musculoskeletal system and the skin. They are more
common in men than in women. Intracranial hemangiopericytomas
are rare and are often located supratentorially.[
The rates of metastatic disease vary from 23.4%[
In this report, we present a rare case of an isolated
metastatic recurrence of an intracranial meningeal
hemangiopericytoma 5.5 years after the first diagnosis.
In the medical literature, there are six reported cases
of intracranial hemangiopericytomas with an isolated
bone metastasis, and our case is the seventh. However,
our patient is the first case with an isolated femur metastasis.
The aim of this report is to present a very rare
case and discuss the clinical features and treatment of
this tumor.
Upon histopathological examination, microscopically,
it was discovered that the whole specimen was
composed of a tumor with extensively branched, thinwalled
vessels. The tumor cells, which had enlarged nuclei with moderate pleomorphism and scanty cytoplasm,
were arranged as nested solid sheets around the
vessels. Immunohistochemically, the tumor cells were
positive for vimentin, CD34, and Bcl-2, but the cells
were negative for CD31, desmin, pancytokeratin, S100
protein, Factor VIII, smooth muscle actin, and alpha
epithelial membrane antigen. Ki-67 showed a proliferation
in around 10% of tumor cells. The diagnosis of
WHO Grade II hemangiopericytoma was accepted in
accordance with these findings (Figure
No further treatment was offered by neurosurgeons and the patient attended follow-up visits. Radiological assessments with magnetic resonance imaging (MRI) of her brain showed no residual or recurrent tumor, and her neurological examinations were normal until 44 months after surgery.
In April 2013, the patient was asymptomatic, but
a cranial MRI showed a lesion, which was 20 mm in
diameter, in the left frontal lobe near the previous tumor
site (Figure
During the first year after radiotherapy, the patient
had no neurological symptoms, and radiologic followup
was normal. One year after radiotherapy, she presented
with a painful left hip. A lytic lesion, which was
4x4.5 cm in diameter, was seen on an X-ray of the proximal
left femur (Figure
In February 2015, the patient underwent a resection
of the left femur head, and prosthetic replacement
surgery was performed. The pathological diagnosis
was compatible with metastatic meningeal
hemangiopericytoma (Figure
Written informed consent was obtained from the patient who participated in this study.
Surgery is the mainstay of treatment for meningeal
hemangiopericytomas. For unfavorably located
tumors, conservative surgery with postoperative radiotherapy
has been suggested. A dose of 50?60 Gy of
radiotherapy is suggested to improve local control. The
presented patient was treated and followed in surgical
clinic after primary surgery. Since she was referred to
our clinic after recurrence surgery, she has not received
any radiotherapy. They have a strong tendency to recur
locally and to develop distant metastases. Mena et al.
reported that 57 patients developed local recurrence
(60.6%) and 22 patients developed single or multiple
metastases (23.4%) in their review, which consisted of
94 cases of CNS hemangiopericytoma.[
In the literature review, we used PubMed to identify
cases of bone metastases originating from intracranial
meningeal hemangiopericytoma. The key words "hemangiopericytoma", "cranial", "meningeal", "bone",
"metastasis", "radiotherapy", "surgery" were searched
and cross-referenced. Articles including metastatic patients
without bone metastasis were excluded. Twenty
english-language articles were found, including 28 described
cases of bone metastasis originating from an
intracranial meningeal hemangiopericytoma. In three
articles, the sites of the bone metastasis were not noted.
Table
A bone metastasis can be seen as a lytic lesion with
a sclerotic margin on plain roentgen film. On plain
roentgen film and CT scans, the involved bones could
show thinning and expansion.[
Metastases typically occur 63-99 months after the
initial diagnosis of hemangiopericytomas. When a single
bone metastasis is determined, synchronous multiple
metastases can be observed in bones or in different
organs, such as the lungs, liver, pancreas, and kidneys.
The median survival rates of patients with metastases
are paradoxically longer than expected. One study reported
that the median survival rates of patients with
and without systemic metastases were 202 months and
99 months, respectively.[
Outcomes of chemotherapy for the management of
metastatic disease, have been disappointing. The most
frequent symptom at the time of diagnosis of bone metastases
is pain. The total surgical excision of a bone
metastasis may be beneficial for patients with limited
distant disease, but the long-term benefits of this excision
are unclear. Radiotherapy for bone metastases is
effective for palliation. The most commonly used radiotherapy
regimen is 30 Gy/10 Fr. The aim of radiotherapy
for bone metastases is not only pain relief but also functional preservation and maintenance of a patient"s
quality of life. Besides, palliative radiotherapy is given
as standart procedure to eradicate the microscopic tumor
cells that may be left behind after metastasectomy.
Consequently, 30 Gy (3 Gy/fraction daily and 5 fractions
per week) of radiotherapy was given to metastasectomy
area in concordance with the literature.
To the best of our knowledge, this report is the
first reported case of an isolated femur metastasis that
originated from an intracranial meningeal hemangiopericytoma
5.5 years after the initial surgery. The metastatic
potential of this rare tumor should be considered.
Long-term follow-up is mandatory due to the high recurrence
rates many years after the surgical resection
of the primary tumor. Since it is a rare disease, further
multi-institutional studies are strongly needed to understand
the clinical behavior and treatment modalities
of this disease.
Disclosure Statement
The authors declare no conflicts of interest.
