“Written informed consent was obtained from the patient to
publish this report in accordance with the journal’s patient consent
policy”
Abstract
The iliac vein is an extremely rare site of metastasis for extraskeletal
mesenchymal chondrosarcoma (ESMC). Involvement of the veins usually
leads to an extremely dismal prognosis. Here, we report a 50-year-old
patient with retroperitoneal mesenchymal chondrosarcoma and initial
metastasis to the iliac bone, which further progressed to involve the
iliac vein. In the current study, we reviewed the major characteristics
of ESMC and the previously reported cases considering the rarity of
these tumors.
Keywords : Extraskeletal mesenchymal chondrosarcoma, iliac vein,
metastasis, magnetic resonance imaging
Introduction
Chondrosarcoma (CS) is a heterogeneous type of neoplasm which is
specified by cartilaginous matrix production through the tumor cells. CS
is the third most prevalent bone malignancy after myeloma and
osteosarcoma[1]. Different types of CS have been pathologically
identified, such as mesenchymal, conventional, dedifferentiated, myxoid,
and clear cell [2]. Mesenchymal chondrosarcoma (MCS) is a high-grade
type of CS that was evaluated for the first time in 1959 by Bernstein
and Lichtenstein in 1959[3]. The MCS is a rare and aggressive type
of conventional chondrosarcoma that accounts for nearly 1% of all CSs
and has a poor prognosis of 45–55% survival in 5 years [4]. 30%
to 50% of MCSs have an extraskeletal origin. [5]. Most cases are
diagnosed in the second decade of human life without any significant sex
predilection [6]. In this case report, we present a case of ESMC
with metastasis to the left iliac vein, which to the best of our
knowledge, has not been reported before.
Case presentation
A 50-year-old woman with previously diagnosed retroperitoneal
mesenchymal chondrosarcoma was referred to our hospital with the chief
complaint of left leg pain and swelling. The pain started four months
ago and increased gradually until the administration day. The pain was
localized in the lumbosacral and radiated through the lateral part of
her left lower limb, and it was not reduced by either rest or
consumption of any analgesics. Her limb edema started one week before
her admission, and she lost her ability to walk on her side. She did not
smoke, consume alcohol, or illicit drugs and underwent treatment with
chemotherapy. Her past drug history involved the consumption of daily
warfarin( due to prior history of deep vein thrombosis) and opioid
analgesics. On the physical examination, no ulceration or pigmentation
was observed. No lymphadenopathy was found in the inguinal region. She
has tender 4+ non-pitting edema on her left leg in conjunction with
tactile tenderness of her lumbosacral region. Her right leg had
limitations in mobility and movement. The ankle-brachial index was 1.02
in the left leg and 1.04 in the right leg.
The Computed Tomography (CT) images (Fig 1) displayed a minimally
enhancing heterogeneous retroperitoneal mass with extensive dense
popcorn-like calcifications located in the left paracervical area
extending to the ipsilateral distended external, internal and common
iliac veins
Magnetic resonance imaging (MRI) with and without gadolinium contrast
media (Fig 2) revealed a large lobulated hetrosignal lesion with
internal popcorn signal voids(calcifications) in the left paracervical
area invading to proximal of the left external/internal iliac veins and
the distal portion of the left common iliac vein. Both MRI and bone scan
(Fig 3) demonstrate multiple bone metastasis in pelvic and both femoral
bones,with largest deposit in right iliac wing,breaking through internal
and external cortices and expanding subperiosteally with aggressive and
massive periosteal reaction According to the MRI report, the tumor
developed metastasis to the iliac vein, a rare location for mesenchymal
chondrosarcoma to metastasis, and to the best of our knowledge, it has
never been reported previously.
Discussion
Extraskeletal mesenchymal chondrosarcoma(ESMC) comprises a rare type of
sarcoma originating from soft tissues, more frequently the meninges of
the cranial and spinal cord, extremities, orbit and the lower
extremities, particularly the thighs. Rarely, this tumor may arise from
the retroperitoneum, kidneys, pancreas and hand musculature [7].ESMC
account for about 1% of all chondrosarcomas[8]. Females are
slightly more affected than males, while males carry a higher
preponderance for extraskeletal and skeletal conventional
chondrosarcomas[7]. ESMC has two peak ages of incidence: ESMC
patients with involvement of the central nervous system, are generally
younger (23.5 years old, range of 5–48 years old), while soft tissue
and/or muscle tumor occur in older individuals (43.9 years old, range of
21-62 years old)[9]. Affected patients usually have unfavorable
prognoses due to high rates of regional and distant metastasis[10].
Retroperitoneal mesenchymal chondrosarcoma has been reported rarely in
the literature. In a cross-series study of Ghafoor et al. evaluating
skeletal and extraskeletal mesenchymal chondrosarcoma imaging features,
only 4 % of cases were located in the retroperitoneum [11]. ESMC
can rarely involve the veins, with the cases being reported in the
literature being as the primary site of the tumor. The iliac vein is an
extremely uncommon site for ESMC and patients with primary iliac vein
mesenchymal chondrosarcoma have a very poor prognosis [12]. The
femoral vein may also be the originating site for ESMC and it manifests
with lower limb swelling and deep vein thrombosis[13]. Moreover,
intravascular mesenchymal chondrosarcoma may also arise from the
pulmonary veins[14].
ESMC displays a strong tendency to locally and distantly metastasize,
which makes the clinical outcome extremely dismal, with a reported
10-year survival rate of 7-26 % [15]. In a study by Frezza et al.
conducted on 113 patients with ESMC, Seventeen patients (15%) presented
with distant metastasis at the time of diagnosis: seven patients (42%)
had pulmonary metastasis, two patients(11%) showed bone metastasis and
eight patients(47%) had metastasis to multiple organs[16]. Other
previously reported sites of metastasis include lymph nodes[17] and
the scalp[18], adrenal glands, pancreas and kidneys[11]. A
review of the literature, revealed one case of retroperitoneal
mesenchymal chondrosarcoma metastasizing to the vein. Juan Hu et al.
presented a 61-year-old female with unintentional weight loss,persistent
abdominal pain and nausea. Ultrasonography of the mass revealed two
large retroperitoneal masses located adjacent to the inferior vena cava.
The computed tomography scan showed dense and extensive, arc- and
ring-like calcifications in the retroperitoneal soft tissue mass.
Abdominal and pelvic magnetic resonance imaging(MRI) with gadolinium
enhancement was also performed, which showed hypointensity on
T1-weighted images and hyperintensity on T2-weighted images associated
with peripheral speculated enhancement consistent with calcification.
Subsequently, the histologic examination of the lesions revealed ESMC
[9]. Our case is unique in that it was previously diagnosed with
retroperitoneal mesenchymal chondrosarcoma with initial bone metastasis,
which further progressed to involve the iliac vein.(Table 1)
MRI of ESMC often demonstrates equal or low signal intensity on T1W1 and
heterogeneous hyperintense lesions on T2WI, as the intramural
non-calcified and calcified regions of EMCS tend to have high and low
intensity on T2WI, respectively, they are usually visible as areas of
high signal intensity around areas with low signal intensity or the
characteristic “ salt and pepper” appearance[19]. Moreover,
contrast-enhanced scanning of the lesions may show a diffuse nodular or
heterogeneous pattern of enhancement, with the non-calcified component
showing more homogenous enhancement and the calcified component showing
less pronounced and heterogeneous enhancement, which can be clearly
separated from each other in approximately 30 % of cases.
Calcifications appear predominantly as chondroid type arc- and ring-like
pattern in the majority of patients. In addition, T2-hyperintense
lobules, frequently seen in chondroid lesions, may be seen in 35% of
patients. Moreover, skeletal involvement is associated with cortical
destruction and extension into the surrounding soft tissue with
periosteal reaction in some lesions[11].In the present study, the
patient showed similar MRI findings.
Conclusion
The current study shows the first case of extraskeletal mesenchymal
chondrosarcoma with metastatic involvement of the iliac vein. ESMC
represents a rare entity of highly aggressive tumors with a propensity
to metastasize locally and distantly. MRI findings include hypointense
lesions on T1W1 and heterogeneously hyperintense lesions on T2WI.
Metastases are common and involvement of less commonly reported sites
may also occur.
Tables
Table 1. Summary of cases of mesenchymal chondrosarcoma with involvement
of the vein