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Radiological-pathological correlation of pleomorphic liposarcoma of the anterior mediastinum in
a 17-year-old girl
Saeed, Maysa; Plett, Sara; Kim, Grace E.; Daldrup-Link, H.; Courtier, J.
Postprints, UC Santa Cruz
Liposarcoma is a soft-tissue sarcoma typically seen in adults. It is extremely rare in children. It
most often occurs in the extremities or in the retroperitoneum. We present a very rare case of
an anterior mediastinal liposarcoma of the pleomorphic subtype in a 17-year-old girl, along with
radiological and pathological correlation. The location, patient age and histological subtype are
exceedingly uncommon for this tumor.
Radiological-pathological correlation of pleomorphic
liposarcoma of the anterior mediastinum
in a 17-year-old girl
Maysa Saeed & Sara Plett & Grace E. Kim &
H. Daldrup-Link & J. Courtier
Received: 9 February 2010 /Revised: 30 June 2010 /Accepted: 13 July 2010 /Published online: 9 September 2010
# The Author(s) 2010. This article is published with open access at Springerlink.com
Abstract Liposarcoma is a soft-tissue sarcoma typically
seen in adults. It is extremely rare in children. It most often
occurs in the extremities or in the retroperitoneum. We
present a very rare case of an anterior mediastinal lip-
osarcoma of the pleomorphic subtype in a 17-year-old girl,
along with radiological and pathological correlation. The
location, patient age and histological subtype are exceed-
ingly uncommon for this tumor.
Keywords Liposarcoma.Anterior mediastinum.Child.
Liposarcoma is a soft-tissue sarcoma that is extremely rare
in children. It is classified as a mesenchymal malignant
neoplasm with fatty tissue differentiation. Liposarcomas are
commonly found in the retroperitoneum and the extremi-
ties. We report a case of a large (20 cm×8 cm) liposarcoma
localized in the mediastinum of a 17-year-old girl. This
tumor was treated surgically with resection, followed by
adjuvant chemotherapy and radiation therapy.
A 17-year-old girl was referred by her primary care physician
because of an abnormality on an echocardiogram from
another hospital. The echocardiogram reported a 20-cm
anterior mediastinal mass.
The girl’s symptoms prompting the echocardiogram were
shortness of breath, chest discomfort and an unusual heart
murmur. She also reported fatigue and a 6-lb. weight loss.
She did not experience night sweats, fevers or chills. In light
of the echo results and clinical presentation, the patient was
referred to pediatric oncology. A follow-up chest CT scan
revealed a 20 cm × 8 cm heterogeneous tumor with various
areas of soft-tissue density and elements of fat tissue (Fig. 1).
Her left main stem bronchus was compressed by the mass.
Increased displacement of the vessels was noted.
Over the next month, the girl’s chest discomfort worsened.
ovoid22 × 16 × 5cmmassweighed941gandhadasolid,but
lobulated, yellow-orange to pink cut surface (Fig. 2).
Microscopically, the neoplastic cells ranged from small
to larger polygonal cells with abundant eosinophilic
granular cytoplasm, many of which had variably prominent
cytoplasmic vacuoles. They were arranged in sheets of
trabeculae and nests (Fig. 2). These epithelioid-appearing
cells had multiple small vacuoles dispersed within the
granular cytoplasm in some areas, while other areas had up
to a few large vacuoles pushing the nucleus to the side
M. Saeed (*)
Undergraduate, University of California, Santa Cruz,
70 Pinto Lane,
Novato, CA 94947, USA
G. E. Kim
Department of Pathology, University of California San Francisco,
San Francisco, CA, USA
S. Plett:H. Daldrup-Link:J. Courtier
Department of Radiology, University of California San Francisco,
San Francisco, CA, USA
Department of Radiology,
Columbia University College of Physicians and Surgeons,
New York, NY, USA
Pediatr Radiol (2010) 40 (Suppl 1):S68–S70
Fig. 2 Histology. a The gross appearance of the epithelioid variant
of pleomorphic liposarcoma depicts a portion of the large
(22 × 16 × 5 cm), solid, multilobated mass with yellow-orange and
pink-white areas. b Hematoxylin and eosin stain: Low magnification
shows most of the tumor cells had abundant granular eosinophilic
cytoplasm (right upper and lower pink areas), but focal zones of the
tumor also had varying amounts of cytoplasmic lipid (central to left
pink white area) confirmed with an oil red O stain (not shown). c
Hematoxylin and eosin stain: This higher magnification shows the
trabecular arrangement of the granular epithelioid tumor cells with
distinct cytoplasmic borders separated by capillaries resembling an
epithelioid neoplasm. d Hematoxylin and eosin stain: This higher
magnification reveals prominent cytoplasmic vacuolation, including
signet ring-like lipoblasts (black arrows), but convincing multi-
vacuolated lipoblasts were not identified. e Hematoxylin and eosin
stain: Only focal nuclear pleomorphism was present
Fig. 1 Chest CT. a An axial image of the chest status after
administration of IV contrast (2 cc/kg Omnipaque 350, GE Healthcare
Inc., Princeton, N.J.) at the level of the superior mediastinum
demonstrates multiple enlarged, tortuous collateral vessels. b Level
of the carina demonstrating an enlarged heterogeneous mass with
areas of fat attenuation in the anterior mediastinum. The mediastinal
contents are displaced posteriorly. No mediastinal lymphadenopathy is
present. c A scan at the level of the superior pulmonary veins
demonstrates a more extensive soft-tissue component of the anterior
mediastinal mass. d A scan at the level of the inferior pulmonary veins
demonstrates the inferior margin of the anterior mediastinal mass
Pediatr Radiol (2010) 40 (Suppl 1):S68–S70S69
(signet ring-like lipoblasts, Fig. 2). In one distinct focus, the Download full-text
cells had larger hyperchromatic pleomorphic nuclei with
abundant finely granular cytoplasm without vacuolated
cytoplasm (Fig. 2).
The morphological features were reminiscent of an
epithelial tumor, such as adrenal cortical carcinoma, or a
hepatocellular neoplasm with fatty change. The differential
diagnosis included epithelial neoplasms and other tumors,
such as hepatoid yolk sac tumor, perivascular epithelioid
cell tumor and the epithelioid variant of pleomorphic
liposarcoma. A histochemical stain for oil red O confirmed
that the vacuoles contained lipid (not shown). Immunohisto-
chemical stains were negative for synaptophysin, chromogra-
nin, inhibin and Melan A, excluding a neuroendocrine tumor
or adrenal cortical carcinoma. Despite the hepatoid appear-
ance, the mass was not reactive for HepPar1, glypican-3 and
AFP and, therefore, unlikely to be a a hepatoid variant of yolk
sac tumor. These as well as other carcinomas were further
excluded by the negative staining for pancytokeratin and
EMA. Negative stain for HMB45 provided no support for a
perivascular epithelioid cell tumor. Other negative stains
included S100 protein, SMA, desmin, caldesmon, myogenin,
CDK4, CD34 and CD31. An epithelioid variant of pleomor-
phic liposarcoma was diagnosed based on the morphological,
histochemical and immunohistochemical findings.
Surgery was followed by adjuvant chemotherapy with
doxorubicin (total dose 375 mg/m2) and ifosfomide (total
dose 54 mg/m2) as well as radiation therapy (total dose
55.8 Gy) to the anterior mediastinum.
Liposarcoma is a rare soft-tissue malignant tumor derived
from primitive embryonic tissues. It represents approximately
1% of all soft-tissue malignancies in adults. Most commonly,
these tumors are found in the lower extremities; mediastinal
involvement is exceedingly rare, with fewer than 150 cases
reported in the literature . Typically, liposarcomas present
in adults in their 50s or 60s; patients often remain
asymptomatic until the tumor reaches a critical size. Patients
with mediastinal involvement clinically present with chest
pain, dyspnea and possible superior vena cava syndrome.
There may also be fatigue and weight loss. Interestingly, rare
cases of mediastinal liposarcoma in children as young as
12 years old have also been reported .
Histologically, liposarcoma is divided into three major
subtypes: well-differentiated myxoid/round cell, dediffer-
entiated and pleomorphic . Myxoid liposarcoma report-
edly accounts for 40–50% of these tumors . These
histological subtypes correlate with potential for metastasis;
well-differentiated liposarcomas are considered to be the
least aggressive, while myxoid liposarcomas have been
reported to disseminate to pleural, pericardial and diaphrag-
matic surfaces .
The histology of this case was specifically of the
pleomorphic type, epithelioid variant. It is the rarest of the
subtypes and its recognition requires careful histopathological
examination for pleomorphic lipoblasts for accurate diagnosis
; however, in the original description of this tumor
Miettinen et al. describes that the lipoblastic differentiation
was most often of the signet ring type .
Pleomorphic liposarcoma was first described in adult
patients and is considered a high-grade sarcoma with
significant risk of metastasis . The primary locations
were the extremities and chest wall.
Typical imaging findings of mediastinal liposarcoma
include mediastinal widening on chest radiography with
possible tracheal deviation. Depending on the degree of
differentiation, mediastinal liposarcoma may appear on CT
or MR as a fatty mass with a variable soft-tissue component.
The solid components may enhance and there may be
infiltration or displacement of adjacent structures .
The classical treatment for mediastinal liposarcoma is
surgical resection, often with adjuvant chemotherapy and
radiation therapy. Same-site recurrence following surgery is
common, often due to an incomplete surgical resection,
thus adjuvant therapy is recommended.
In conclusion, we document a case of a primary anterior
mediastinal pleomorphic liposarcoma in a 17-year-old girl.
To our knowledge, the radiological appearance of this rare
subtype of liposarcoma is unique.
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