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Case Reports in Orthopedics

Volume 2022, Article ID 9195529, 8 pages
https://doi.org/10.1155/2022/9195529

Case Report
Pleomorphic Liposarcoma of Femur: A Rare Soft Tissue Sarcoma
Metastasized to the Bone—Case Report and Review of Literature

Thanate Poosiripinyo,1 Thanapon Chobpenthai ,2 Taweechok Wisanuyotin,3

and Winai Sirichativapee3
1
Khon Kaen Hospital, Department of Orthopaedics, Khon Kaen, Thailand
2
Chulabhorn Royal Academy, Princess Srisavangavadhana College of Medicine, Bangkok, Thailand
3
Department of Orthopaedics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand

Correspondence should be addressed to Thanapon Chobpenthai; dseero@gmail.com

Received 2 May 2021; Accepted 15 February 2022; Published 7 March 2022

Academic Editor: Kaan Erler

Copyright © 2022 Thanate Poosiripinyo et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.

Background. Pleomorphic liposarcoma (PLPS) accounts for less than 5% of liposarcoma, and its metastasis to bone is rare. As a
high-grade tumor, PLPS is reported to be more invasive with high local recurrence and distant metastasis. Here, we report a case
of PLPS of the femur and undertake a review of the literature. Case Presentation. A 58-year-old man presented with a big mass at
posterior aspect of his left thigh. The computed tomography of the chest for staging revealed two nodules at the left upper lung
field. Wide resection of the soft tissue mass at the left thigh was performed by a general surgeon. Thoracotomy and wedge
resection of the lung nodules was carried out by a cardiothoracic surgeon. Pathologic diagnosis suggested PLPS. Three years
later, he was presented with sudden right hip pain after he slipped. The plain radiograph revealed an osteolytic lesion at the
right proximal femur with minimally displaced pathological fracture. The MRI showed the presence of a tumor at the proximal
part of the right femur and its soft tissue invasion. The patient underwent en bloc proximal femur wide resection followed by
cemented long-stem bipolar hemiarthroplasty. The final histopathology report from definite surgery specimen revealed
pleomorphic spindle, round, and polygonal cells arranged in sheets, short fascicles, and storiform arrays. There were no
complications, adverse outcomes, or recurrence reported at six months after surgery. The patient could walk without gait aid
and had good functional outcomes according to the TESS questionnaire. Conclusion. PLPS is a highly aggressive tumor with a
high distant metastatic rate. The definite diagnosis of PLPS is made on the basis of histopathology. Surgical treatment
involving wide resection that aims to achieve a negative margin is the best option currently available, and we recommend
treating bone metastasis from PLPS as the primary site of the tumor. The effect of chemotherapy and radiotherapy in
preventing postoperative recurrence is still unclear and requires further studies.

1. Introduction groups and five types: well-differentiated/dedifferentiated

Soft tissue sarcoma (STS) constitutes around 20% of all the
adult sarcomas, and liposarcoma is its common subtype
[1]. According to the 4th AFIP fascicle, by definition, lipo-
sarcoma is a malignant tumor that has differentiation
towards adipocytes [2]. Based on adipocyte differentiation,
World Health Organization classified liposarcoma into three
liposarcoma (WDL/DDL), myxoid/round-cell liposarcoma
(MRC), and pleomorphic liposarcoma (PLPS) [3]. PLPS
accounts for less than 5% of liposarcoma, and its metastasis
to bone is rare [4]. As a high-grade tumor, PLPS is reported
to be more invasive with high local recurrence and distant
metastasis in 30–50% of cases [5, 6]. The commonest site
for this tumor is the lower extremity, and less common
2 Case Reports in Orthopedics

Figure 1: Specimen of soft tissue tumor (the primary tumor) after wide resection.

Figure 2: Plain radiograph revealed osteolytic lesion at right proximal femur with minimally displaced pathological fracture at the
intertrochanteric region.

anatomical sites are the retroperitoneum, lungs, mediasti-
num, and bones [5, 7]. According to the literature reviews,
there are few bone metastasis reports from PLPS [8, 9],
and the treatment strategies are still unclear. Although surgi-
cal resection is the mainstay of treatment, there is substantial
uncertainty about histological diagnosis and the best suitable
treatment of this rare tumor [10].
n this case report, we provided an overview of the clini-
cal, radiological, and pathological findings in a case of
metastatic PLPS in the proximal femur of a 58-year-old
male. We also reviewed the literature about PLPS of bone
and proposed revised diagnostic criteria as well as treatment
options according to our findings.
2. Case Presentation
A 58-year-old man presented with a big mass at posterior
aspect of his left thigh. The computed tomography (CT) of
the chest for staging revealed two nodules at the left upper
lung field. Wide resection of the soft tissue mass at the left
thigh was carried out by a general surgeon (Figure 1). Car-
diothoracic surgeon performed thoracotomy and wedge
Case Reports in Orthopedics
(a)
(c)
Figure 3: The MRI revealed the tumor at right proximal femur with soft tissue extension, especially at posteromedial aspect: MRI coronal
T1 weight (a), coronal T2 weight (b), coronal T1 + contrast (c), and axial T1 + contrast (d).
resection of the lung nodules. The pathological report of
mass at left thigh suggested PLPS, and tumor breakthrough
capsule and lung nodules were metastatic PLPS. The patient
was treated postoperatively with radiotherapy at left thigh
for local control and chemotherapy with 6 cycles of doxoru-
bicin and ifosfamide regimen for systemic control.
Three years later, he presented with sudden right hip pain
after he slipped and was brought to the hospital by an ambu-
lance. The physical examination revealed that he could not
bear weight on his right hip. There was no apparent defor-
mity and palpated mass, and neurovascular was also intact.
However, there was tenderness at greater trochanter and lim-
ited range of motion due to pain. All of his laboratory inves-
tigations were normal. The plain radiograph revealed an
osteolytic lesion at the right proximal femur with a minimally
3
(b)
(d)
displaced pathological fracture at the intertrochanteric
region (Figure 2). The MRI showed the presence of tumors
at proximal part of the right femur and soft tissue invasion,
especially at the posteromedial aspect (Figures 3(a)–3(c)).
Femoral neurovascular structures and sciatic nerves were
spared from the tumor (Figure 3(d)). The chest CT revealed
right upper lung mass with no other skeletal metastasis
shown by bone scan. The right upper lung mass was lung
metastasis, which was confirmed by a pathologist from core
needle biopsy of the lung. The thoracotomy and right upper
lobectomy were taken. The patient underwent incisional
biopsy through a direct lateral approach. The histopathology
report suggested metastatic PLPS. The patient underwent en
bloc proximal femur wide resection (Figure 4). Owing to
financial problems, the patient was unable to copay for
4 Case Reports in Orthopedics
Figure 4: Specimen of proximal femur includes biopsy tract after
en bloc resection.
endoprosthesis, and the allograft was not available at that
time. The surgeon considered to opt reconstruction by
cemented long-stem bipolar hemiarthroplasty (ECHELON
revision hip stem, Smith and Nephew Company). Poly-
methyl methacrylate (PMMA) was used for implant fixation
at the proximal femur. In order to restore hip abduction, soft
tissue reconstruction was performed, specifically for gluteus
medius muscle, using Dacron vascular graft covering
PMMA. Figure 5 shows the postoperative radiographs after
the en bloc resection and reconstruction of the proximal
femur.
The final histopathology report from definite surgery
specimen revealed pleomorphic spindle, round, and polygo-
nal cells arranged in sheets, short fascicles, and storiform
arrays. Severe nuclear atypia with multinucleation and
numerous mitoses, including atypical forms of intracellular
and extracellular eosinophilic droplets, was presented. Vari-
able size of pleomorphic adipocytes and lipoblasts was iden-
tified with vascular invasion. All surgical margins were free
from the tumor cell (Figures 6(a)–6(d)). The patient received
adjuvant radiotherapy and chemotherapy after the surgery,
as per tumor board decision.
The patient was advised for early ambulation and full
weight-bearing after the right bipolar hemiarthroplasty.
The postoperative course was uneventful, and the patient
was discharged within one week of surgery. There were no
complications, adverse outcomes, or recurrence reported at
6 months after surgery, and he could walk without gait aid.
On the Thai version of the Toronto Extremity Salvage Score
(TESS) for patients with bone and soft-tissue sarcoma, he
had good functional outcomes with a score of 74.1% [11].
3. Discussion
PLPS is a rare soft tissue sarcoma. Bone is the second com-
mon site of metastasis of pleomorphic liposarcoma [12].
On the contrary, most skeletal metastases were prostate
and breast cancer (up to 70%) [13]. PLPS is the rarest type
of liposarcoma [14], accounting for only 5-10% of all lipo-
sarcoma subtypes, but is considered as a high-grade malig-
nancy with a high rate of metastasis, local recurrence, and
poor prognosis [4–6]. The highest prevalence of PLPS is
found in the 6th and 7th decade of life, and it affects both
sexes equally [7, 15]. However, some previous studies have
also reported cases of younger patients, with the youngest
reported case was 8 years of age [16]. Table 1 summarizes
the clinical and demographic features of cases of PLPS
reported in the literature. In this report, we presented a
known case of PLPS of the femur in a 58-year-old male.
In PLPS, there is a rapid growth of a painless mass,
which is easily neglected until the mass is large enough or
produce symptoms due to compression of surrounding
structures [17]. Three distinct morphological features of
PLPS have been described in the literature, such as malig-
nant fibrous histiocytoma-like liposarcoma, epithelioid
structure like neoplasia, and fusiform/spindle-shaped cell
neoplasia [16]. PLPS most commonly arise in the deep soft
tissues of the proximal part of the lower extremity, with
thigh as the commonest site reported in 34% of the cases;
however, it could also occur in the retroperitoneum, lungs,
mediastinum, and bones [12, 17]. In our case, the patient
presented with a big mass at the posterior aspect of his left
thigh in the first episode. After recurrence, he presented with
sudden right hip pain after he slipped and was brought to
the hospital by an ambulance. The final histopathology
report from definite surgery specimen revealed pleomorphic
spindle, round, and polygonal cells arranged in sheets, short
fascicles, and storiform arrays. Preliminary studies have
shown that plain radiographs of the bone in PLPS presented
with osteolytic lesions [10]. These findings are in accordance
with our study, where the plain radiograph revealed osteoly-
tic lesion at the right proximal femur with a minimally dis-
placed pathological fracture at the intertrochanteric region.
Radiological investigations like MRI and CT are impor-
tant to plan surgical resection in order to determine the size
of the tumor and degree of invasion in neighboring tissues.
[17]. However, it is challenging to differentiate PLPS from
other types of liposarcoma and sarcoma, merely on the basis
of these radiological examinations. The definite diagnosis of
PLPS is made on the pathological examination. The presence
of lipoblasts is necessary for diagnosis [14, 17]. In our case,
the MRI showed the presence of a tumor at the proximal
part of the right femur and its soft tissue invasion, especially
at the posteromedial aspect. On histopathological analysis,
variable size of pleomorphic adipocytes and lipoblasts was
identified with vascular invasion. The differential diagnosis
was metastasis carcinoma because the age of the patient
was more than 40 years old and presented with painful
Case Reports in Orthopedics 5

(a) (b)

Figure 5: Postoperative radiographs after en bloc resection and reconstruction of the proximal femur: anteroposterior view (a) and trans
inguinal view (b).

(a) (b)

(c) (d)

Figure 6: Pathological diagnosis of tissue from right proximal femur: metastatic pleomorphic liposarcoma, with the presence of vascular
invasion and all surgical resected margins appear free of tumor. 20× (a, b), 40× (c), and 60× (d).
6 Case Reports in Orthopedics

Table 1: Clinical, demographic features, type of treatments, results and outcomes of the present case, and reported cases of pleomorphic
liposarcoma in the literature.

Age
Study (years)/ Location Clinical data Treatment Result Follow-up
sex
Torok Wide resection, radiation, Resolution of the
34/M Right femur _ Alive. 12 months follow-up.
et al. [23] chemotherapy. symptoms
Complete
Patient presented with Neoadjuvant MAP
Metaphysis resolution of the 12 months. Patient is well
Tiemeier a six-month history of chemotherapy
18/M of the left metastases with no evidence of
et al. [10] pain and swelling in Surgical resection of the
tibia following recurrence.
the left leg tumor
chemotherapy
Rasalkar Chemotherapy, surgical Resolution of No recurrence at 13 months
13/M Femur _
et al. [24] wide resection. symptoms follow-up
Hamlat Thoracic Backache Laminectomy T7-T8 and 19 months. Gradual
45/F Relieve from pain
et al. [7] spine Paraplegia radiotherapy deterioration of disease
Resection of L4, L3 to L5
Barra de At 18 months,
Lumbar Lumbosciatica on the arthrodesis.
Moraes 60/F neither pain nor 3 years. Lung metastasis
spine left side Radiotherapy and
et al. [15] recurrence
chemotherapy
Dehiscence and a
Bilateral
Morales- deep wound 2 months. Local recurrence,
Lumbar lumbosciatica. En bloc resection in L1, L2,
Codina 61/M infection. hepatic metastasis, extensive
spine Motor deficit in the and L3
et al. [14] Inflammatory thrombosis. Death
lower limbs.
polymyopathy.
En bloc proximal femur
wide resection followed by All surgical 6 months. Patient is well and
Patient presented with
Present cemented long-stem bipolar margins were free could walk without gait aid.
54/M Right femur a sudden right hip
case hemiarthroplasty. from the tumor No evidence of recurrence or
pain after he slipped.
Radiotherapy and cell metastases.
chemotherapy

osteolytic lesion and bone metastasis from soft tissue sar-
coma is rare.
Surgery, especially wide resection that aims to achieve
a negative surgical margin, is the main treatment for PLPS
(Table 1) [17]. It is an important palliative treatment for
PLPS patients with remote metastasis and local infiltration
to alleviate the compressive manifestations [17]. Besides,
the use of adjuvant chemotherapy and radiotherapy still
remains controversial. Miura et al. reported that conven-
tional chemotherapy was not useful for the sarcoma
patient [18]. Some reports have suggested that the tumor
volume and the local recurrence rate were reduced by
radiotherapy, but on the other hand, the histopathology
might be changed due to radiotherapy [17, 19].
On the contrary, a study shows poor outcomes in PLPS
following surgery alone and recommends the use of neoad-
juvant MAP chemotherapy [10]. In our case, surgical treat-
ment involving wide resection that aimed to achieve a
negative margin exhibited good oncological and functional
outcomes in the patient. However, the effect of chemother-
apy and radiotherapy in preventing postoperative recurrence
is still unclear and requires further studies.
The data from previous studies show that metastasis
from STS is considered as a hematogenous spreading and
an end-stage of patients [20]. The distant metastasis rate in
PLPS is 30–50% [5]. Bone metastasis from PLPS is not the
most common site, reported in less than 10% of PLPS
patients [21]. With regard to outcomes, PLPS is a highly
aggressive tumor with a 5-year survival rate of only about
29% [17]. In our case, the tumor arose in the thigh of the
patient at first and then metastasized to lungs, followed by
recurrence and metastasis into the bone (femur). Radical
resection of femoral metastasis gives good treatment results
if a general health condition allows surgery [22].
4. Conclusion
PLPS is a rare high-grade sarcoma, more invasive tumor
with high local recurrence and distant metastatic rate.
However, the metastasis of PLPS to the bone is not the
most common site. The definite diagnosis of PLPS is made
based on histopathology. Surgical treatment involving wide
resection that aims to achieve a negative margin is the best
available option currently available, and we recommend
treating bone metastasis from PLPS as the primary site
of the tumor. The effect of chemotherapy and radiother-
apy in preventing postoperative recurrence is still unclear
and requires further studies.
Case Reports in Orthopedics
Data Availability
The data supporting this study’s findings are available from
the corresponding author upon reasonable request.
Ethical Approval
This retrospective chart review study involving human
participants was in accordance with the ethical standards
of the institutional and national research committee and
with the 1964 Helsinki Declaration and its later amend-
ments or comparable ethical standards. The Human
Research Ethics Committee of the Khon Kaen University
acknowledged and approved this study (Approval Num-
ber: IRB00001189).
Consent
All patients acknowledged and signed informed consent
regarding publishing their data and photographs.
Conflicts of Interest
The authors declare that they have no competing interests.
Authors’ Contributions
TP did the conceptualization, methodology, validation, for-
mal analysis, investigation, data curation, writing—original
draft, visualization, supervision, and project administra-
tion. TC did the validation, formal analysis, investigation,
writing—original draft, supervision, and project adminis-
tration. TW did the methodology, validation, formal anal-
ysis, and writing—review and editing. WS did the
investigation, data curation, visualization, and writing—re-
view and editing.
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Liposarkoma Pleomorfik Pada Femur : Sarkoma Jaringan Lunak Langka yang Bermetastasis
ke Tulang - Laporan Kasus dan Tinjauan Pustaka

Latar Belakang : Pleomorphic liposarkoma (PLPS) memiliki angka kejadian kurang dari 5% dari jumlah
liposarkoma, dan kejadian metastasisnya ke tulang pun jarang terjadi. Sebagai sebuah tumor yang
memiliki tingkatan tinggi, PLPS telah dilaporkan memiliki kemampuan invasif yang lebih dengan
kekambuhan lokal yang cukup tinggi dan metastasis yang jauh. Pada kesempatan kali ini, kami
melaporkan kasus PLPS tulang paha dan kami juga melakukan kajian literatur. Presentasi Kasus :
Seorang pria berusia 58 tahun datang dengan massa (benjolan) besar di posterior paha kirinya. Pada
alat Computed tomography di dada yang berfungsi untuk penentuan stadium, menunjukkan terdapat
dua nodul di permukaan paru kiri sebelah atas. Reseksi secara luas pada massa jaringan lunak di paha
kiri dilakukan oleh seorang dokter bedah umum. Torakotomi dan reseksi irisan nodul di paru dilakukan
oleh ahli bedah kardiotoraks. Diagnosis patologis mengarah pada PLPS. Tiga tahun kemudian, pasien
tiba-tiba mengalami nyeri pinggul kanan setelah terpeleset. Foto polos menunjukkan lesi osteolitik
pada femur proksimal kanan dengan fraktur patologis, yang jenis frakturnya tergeser secara minimal.
Pemeriksaan MRI menunjukkan adanya tumor di bagian proksimal tulang paha kanan beserta invasi
jaringan lunaknya. Pasien menjalani reseksi secara luas pada tulang paha proksimal en bloc serta
diikuti dengan hemiartroplasti bipolar berbentuk batang panjang yang disemen. Laporan akhir
histopatologi dari spesimen pembedahan secara pasti menunjukkan adanya sel-sel pleomorfik
gelondong, bulat, dan poligonal yang tersusun dalam lembaran, fasikula yang pendek, dan susunan
storiform. Tidak ada komplikasi, hasil yang merugikan, atau kekambuhan yang dilaporkan pada enam
bulan setelah operasi. Pasien bisa berjalan tanpa alat bantu dan memiliki hasil fungsional yang baik
menurut kuesioner TESS. Kesimpulan : PLPS adalah tumor yang sangat agresif dengan tingkat
metastasis yang jauh dan tinggi. Diagnosis pasti PLPS dibuat berdasarkan hasil pemeriksaan
histopatologi. Perawatan bedah dengan reseksi secara luas yang bertujuan untuk mencapai margin
yang negatif adalah pilihan terbaik yang tersedia saat ini, dan kami merekomendasikan perawatan
metastasis tulang dari PLPS sebagai suatu tumor yang utama. Efek kemoterapi dan radioterapi dalam
mencegah kekambuhan pasca operasi masih belum diketahui secara lanjut dan masih memerlukan
penelitian lebih lanjut.

1. Pendahuluan

Sarkoma jaringan lunak (STS) terdiri dari 20% dari semua jenis sarkoma dewasa dan liposarkoma
merupakan subtipe yang umum. Menurut fasikula AFIP ke-4, secara definisi, liposarkoma adalah
tumor ganas yang memiliki diferensiasi menjadi adiposit. Berdasarkan diferensiasi menjadi adiposit,
WHO mengklasifikasikan liposarkoma menjadi tiga kelompok dan lima jenis: liposarcoma
berdiferensiasi baik/ terdiferensiasi (WDL/DDL), liposarkoma myxoid/sel bulat (MRC), dan
liposarkoma pleomorfik (PLPS). PLPS berjumlah kurang dari 5% dari liposarkoma yang lain , dan
metastasis menuju tulangpun jarang terjadi. Sebagai tumor yang memiliki derajat tinggi, PLPS telah
dilaporkan memiliki kemampuan invasive yang lebih dengan angka kekambuhan lokal yang tinggi dan
metastasis yang jauh pada 30-50% kasus. Tempat yang paling umum terjadi pada tumor ini adalah
ekstremitas bawah, dan dan jarang terjadi pada tempat yang secara anatomi adalah retroperitoneum,
paru-paru, mediastinum, dan tulang. Menurut ulasan beberapa literatur, ada beberapa laporan
tentang metastasis PLPS pada tulang dan strategi pengobatannyapun masih belum diketahui secara
pasti. Meskipun reseksi bedah menjadi pengobatan yang utama atau diandalkan, tetapi terdapat
ketidakpastian tentang diagnosis pemeriksaan histologis dan pengobatan yang paling cocok untuk
tumor langka ini. Dalam laporan kasus ini, kami memberikan kesimpulan temuan klinis, radiologis, dan
patologis dalam kasus PLPS metastatik pada femur sebelah proksimal pada pasien pria berusia 58
tahun. Kami juga meninjau literatur tentang PLPS pada tulang dan kami pun mengusulkan kriteria
diagnostik yang telah direvisi atau diperbaharui, serta pilihan pengobatan yang sesuai dengan temuan
kami.

2. Presentasi Kasus / Laporan Kasus

Seorang pria berusia 58 tahun datang dengan sebuah massa (benjolan) besar di sebelah posterior paha
kirinya. Pada alat Computed tomography (CT) di dada untuk penentuan stadium menunjukkan adanya
dua buah nodul di permukaan paru kiri atas. Reseksi secara luas pada massa jaringan lunak di paha
kiri dilakukan oleh seorang dokter bedah umum (Gambar 1). Ahli bedah kardiotoraks melakukan
torakotomi dan reseksi pada nodul di paru-paru. Hasil pemeriksaan patologis pada massa di paha kiri
menunjukkan adanya PLPS, kapsul tumor dan pada nodul di paru-paru menunjukkan adanya PLPS
metastatik. Pasien dirawat pasca operasi dengan radioterapi di paha kiri untuk control secara lokal
dan kemoterapi dengan rejimen doxorubicin dan ifosfamide 6 siklus untuk control secara sistemik.
Tiga tahun kemudian, pasien tiba-tiba mengalami nyeri pinggul kanan setelah terpeleset dan dibawa
ke rumah sakit dengan ambulans. Pada pemeriksaan fisik menunjukkan bahwa pasien tidak dapat
menahan beban di pinggul kanannya. Tidak ada kelainan bentuk dan massa yang teraba, dan
neurovaskular juga utuh. Namun, terdapat nyeri tekan pada trokanter mayor dan rentang gerak yang
terbatas karena nyeri. Semua pemeriksaan laboratoriumnya normal. Foto polos menunjukkan adanya
lesi osteolitik pada femur bagian proksimal kanan dengan fraktur patologis yang minimal di daerah
intertrochanteric (Gambar 2). Pemeriksaan MRI menunjukkan adanya tumor pada bagian proksimal
femur kanan dan adanya invasi jaringan lunak, terutama pada bagian posteromedial (Gambar 3(a)–
3(c)). Struktur neurovaskular femoralis dan saraf siatik tidak terkena tumor (Gambar 3(d)). CT pada
dada menunjukkan adanya massa pada paru kanan atas tanpa metastasis ke kerangka lain yang
ditunjukkan oleh pemindaian tulang. Massa paru kanan atas adalah metastasis paru, yang
dikemukakan oleh ahli patologi dari biopsy denga jarum pada inti paru. Torakotomi dan lobektomi
kanan atas telah dilakukan. Pasien menjalani biopsi insisi melalui pendekatan disebelah lateral secara
langsung. Laporan histopatologi menunjukkan adanya PLPS metastatik. Pasien menjalani reseksi luas
di femur sebelah proksimal en bloc (Gambar 4). Karena adanya masalah keuangan, endoprosthesis,
dan allograft tidak tersedia pada saat itu, Dokter ahli bedah memberikan pertimbangan untuk memilih
rekonstruksi dengan hemiartroplasti bipolar yang bebrbentuk batang panjang yang disemen (batang
pinggul , ECHELON, Smith dan Nephew Company). Polimetil metakrilat (PMMA) digunakan untuk
fiksasi implan pada femur sebelah proksimal. Untuk memulihkan pergeseran pinggul, dilakukan
rekonstruksi jaringan lunak, khusus untuk otot gluteus medius, menggunakan cangkok vaskular
Dacron yang menutupi PMMA. Gambar 5 menunjukkan radiografi pasca operasi setelah reseksi en
bloc dan rekonstruksi femur sebelah proksimal. Laporan akhir histopatologi dari spesimen
pembedahan yang pasti menunjukkan adanya sel-sel pleomorfik berbentuk gelondong, bulat, dan
poligonal yang tersusun dalam lembaran, fasikula yang pendek, dan susunan storiform. Adanya atypia
nuklir yang parah dengan multinukleasi dan banyaknya mitosis, termasuk bentuk atipikal dari tetesan
eosinofilik intraseluler dan ekstraseluler, tururt disajikan pada pemeriksaan ini . Ukuran variabel
adiposit pleomorfik dan lipoblast diidentifikasi dengan invasi vaskular. Semua daerah bedah bebas
dari sel tumor (Gambar 6(a)–6(d)). Pasien menerima radioterapi dan kemoterapi tambahan setelah
operasi, sesuai keputusan polo bagian tumor. Pasien juga disarankan untuk ambulasi secara dini dan
latihan menahan beban setelah hemiartroplasti bipolar kanan. latihan pasca operasi sudah lancar, dan
pasien akhirnya dipulangkan dalam waktu satu minggu setelah dioperasi. Tidak ada komplikasi, hasil
yang merugikan, atau kekambuhan yang dilaporkan pada 6 bulan setelah operasi, dan dia bisa berjalan
tanpa alat bantu. Menurut hasil penilaian menggunakan Skor Penyelamatan Ekstremitas Toronto
(TESS) versi negara Thailand, pasien ini yang sebelumnya memiliki sarkoma tulang dan jaringan lunak,
telah menunjukkan hasil fungsional yang baik dengan hasil skor 74,1%.

3. Diskusi

PLPS adalah sebuah sarkoma jaringan lunak yang langka. Tulang adalah tempat kedua yang umum
pada metastasis liposarkoma pleomorfik. Sebaliknya, sebagian besar metastasis tulang adalah kanker
prostat dan payudara (hingga 70%). PLPS adalah jenis liposarkoma yang paling langka, terhitung hanya
5-10% dari semua subtipe liposarkoma, tetapi dianggap sebagai keganasan tingkat tinggi dengan
tingkat metastasis yang tinggi pula, kekambuhan local yang tinggi, dan prognosis yang buruk.
Prevalensi tertinggi PLPS ditemukan di 6 dan 7 dekade kehidupan, dan itu sama-sama mempengaruhi
kedua jenis kelamin. Namun, beberapa penelitian sebelumnya juga melaporkan kasus pasien yang
lebih muda, dengan kasus termuda yang dilaporkan adalah usia 8 tahun. Tabel 1 merangkum
gambaran klinis dan demografis dari kasus PLPS yang dilaporkan dalam literatur. Dalam laporan ini,
kami mempresentasikan kasus PLPS femur yang diketahui pada pria berusia 58 tahun. Pada PLPS,
terjadi pertumbuhan massa yang cepat tanpa rasa sakit, yang diabaikan hingga massa tersebut sudah
cukup besar atau menimbulkan gejala akibat kompresi struktur di sekitarnya. Tiga fitur morfologis
yang berbeda dari PLPS telah dijelaskan dalam literatur, seperti liposarkoma seperti histiocytoma
berserat ganas, struktur epiteloid seperti neoplasia, dan neoplasia sel fusiform / berbentuk gelondong.
PLPS paling sering timbul pada jaringan lunak bagian dalam dari bagian proksimal ekstremitas bawah,
dengan paha sebagai tempat yang paling sering dilaporkan pada 34% kasus. Namun, bisa juga terjadi
di retroperitoneum, paru-paru, mediastinum, dan tulang. Dalam kasus studi kami, pasien memiliki
massa yang besar di aspek posterior paha kirinya pada waktu yang pertama kali. Setelah kambuh, dia
merasakan nyeri pinggul kanan secara tiba-tiba setelah terpeleset dan dibawa ke rumah sakit dengan
ambulans. Laporan akhir histopatologi dari spesimen pembedahan secara pasti menunjukkan adanya
sel-sel pleomorfik gelondong, bulat, dan poligonal yang tersusun dalam lembaran, fasikula yang
pendek, dan susunan storiform. Studi awal telah menunjukkan bahwa radiografi polos pada tulang di
PLPS menunjukkan adanya lesi osteolitik. Temuan ini sesuai dengan penelitian kami, di mana
radiografi polos menunjukkan lesi osteolitik pada femur proksimal kanan dengan fraktur patologis
yang tergeser secara minimal pada regio intertrochanteric. Investigasi radiologis seperti MRI dan CT
penting dilakukan untuk merencanakan reseksi bedah sekaligus menentukan ukuran tumor dan
tingkat invasi pada jaringan di sekitarnya. Namun, sulit untuk membedakan antara PLPS pada
liposarkoma dengan sarkoma jenis lain, hanya bisa dilakukan berdasarkan pemeriksaan radiologis ini.
Diagnosis pasti PLPS dibuat pada pemeriksaan patologis. Keberadaan lipoblast diperlukan untuk
mendiagnosis . Dalam kasus kami, MRI menunjukkan adanya tumor di bagian proksimal tulang paha
kanan dan invasi jaringan lunaknya, terutama pada aspek posteromedial. Pada analisis histopatologis,
ukuran variabel adiposit pleomorfik dan lipoblas diidentifikasi dengan adanya invasi vaskular. Kejadian
lesi osteolitik dan metastasis tulang dari sarkoma jaringan lunak jarang terjadi. Pembedahan,
khususnya reseksi luas yang bertujuan untuk mencapai margin tepi jaringan secara negatif,
merupakan pengobatan utama untuk PLPS (Tabel 1). Hal ini adalah pengobatan paliatif yang penting
untuk pasien PLPS dengan metastasis yang jauh serta infiltrasi lokal untuk meringankan manifestasi
kompresi. Selain itu, penggunaan kemoterapi dan radioterapi adjuvant masih kontroversial Miura et
al. melaporkan bahwa kemoterapi konvensional tidak berguna untuk pasien sarkoma. Beberapa
laporan menunjukkan bahwa volume tumor dan tingkat kekambuhan lokal berkurang dengan
radioterapi, tetapi di sisi lain, hasil histopatologi dapat berubah karena radioterapi. Sebaliknya, sebuah
penelitian menunjukkan hasil yang buruk pada PLPS setelah menjalankan operasi saja dan
merekomendasikan penggunaan kemoterapi MAP neoadjuvant. Dalam kasus kami, perawatan bedah
yang menggunakan reseksi secara luas yang bertujuan untuk mencapai batas jaringan yang negatif
menunjukkan hasil onkologis dan fungsional yang baik pada pasien. Namun, efek kemoterapi dan
radioterapi dalam mencegah kekambuhan pasca operasi masih belum jelas dan memerlukan
penelitian lebih lanjut. Data dari penelitian sebelumnya menunjukkan bahwa metastasis dari STS
dianggap hasil penyebaran secara hematogen dan tahap akhir kehidupan pasien. Tingkat metastasis
yang jauh pada PLPS adalah 30-50% . Metastasis pada tulang dari PLPS bukanlah yang paling umum
terjadi, dilaporkan hasilnya kurang dari 10% jumlah pasien PLPS. Berkenaan dengan hasil, PLPS adalah
tumor yang sangat agresif, dengan tingkat kelangsungan hidup selama 5 tahun hanya berkisar 29%.
Dalam kasus kami, tumor muncul di paha pasien pada awalnya dan kemudian bermetastasis ke paru-
paru, diikuti dengan kekambuhan dan metastasis ke tulang (femur). Reseksi radikal metastasis
femoralis memberikan hasil pengobatan yang baik jika kondisi kesehatan umum pasien
memungkinkan dilakukannya pembedahan.

4. Kesimpulan

PLPS adalah sarkoma berlevel tinggi yang cukup langka, tumor yang lebih invasif dengan kekambuhan
lokal yang tinggi dan tingkat metastasis yang jauh. Namun, metastasis PLPS ke tulang bukanlah tempat
yang paling umum terjadi. Diagnosis pasti PLPS dibuat berdasarkan histopatologi. Perawatan bedah
yang melibatkan reseksi luas yang bertujuan untuk mencapai margin ( tepi jaringan ) yang negatif
adalah pilihan terbaik yang tersedia saat ini, dan kami merekomendasikan bahwa metastasis tulang
pada PLPS sebagai lokasi utama tumor. Efek kemoterapi dan radioterapi dalam mencegah
kekambuhan pasca operasi masih belum jelas dan masih memerlukan penelitian lebih lanjut.