MAKALAH I

SARKOMA JARINGAN LUNAK

Berry Erida Hasbi
Dr. dr. Prihantono, Sp.B(K)Onk

Bagian Ilmu Bedah

Fakultas Kedokteran
Universitas Hasanuddin
Makassar
2018
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 1
 OUTLINE

•Insidens
•Etiologi
•Diagnosis
•Klasifikasi Histopatologi
•Status Penampilan
•Penatalaksanaan
•Prognosis & Follow Up
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 INSIDEN

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 INSIDEN

•Sangat jarang
•1% dari semua diagnosis kanker baru
•1, 66 juta kasus baru kanker di AS (2015)
 11. 930 Sarkoma Jaringan lunak

Hui Jane (2016). Epidemiology and Etiology of Sarcomas. Surg Clin N Am 96. 901-914
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25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 5
SEER Cancer Statistics Review 1975-2012, National Cancer Institute
 Burningham et al. Clinical Sarcoma Research 2012, 2:14
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 Burningham et al. Clinical Sarcoma Research 2012, 2:14
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ETIOLOGI

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 Etiologi pasti  Belum diketahui

Genetik Limfaedema Virus Lingkungan

Proses
Hormon Radiasi Penyebuhan
Kronik
Froehner, M., & Wirth, M. P. (2001). Etiologic Factors in Soft Tissue Sarcomas. Oncology Research and Treatment,
24(2), 139–142.
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 DIAGNOSIS
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 ANAMNESIS &
PEMERIKSAAN
FISIK

HISTOPATOLOGI IMAGING

• Suyatno, Pasaribu Emir (2014). Kanker Jaringan Lunak. Dalam: Suyatno, Pasaribu Emir, editor. Bedah Onkologi: Diagnosis dan Terapi. Jakarta: Sagung Seto. h.
195-232.
• Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker Solid PERABOI 2010. Jakarta: Sagung
Seto. h.165-93
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 11
 ADIPOCYTIC TUMOURS
Benign
Lipoma
Lipomatosis
Lipomatosis of nerve Lipoblastoma / Lipoblastomatosis
Angiolipoma
Myolipoma
Chondroid lipoma
Extrarenal angiomyolipoma Extra-adrenal myelolipoma
Spindle cell/
Pleomorphic lipoma Hibernoma

Intermediate (locally aggressive)

Atypical lipomatous tumour/
Well differentiated liposarcoma

Malignant
Dedifferentiated liposarcoma
Myxoid liposarcoma
Round cell liposarcoma
Pleomorphic liposarcoma
Mixed-type liposarcoma Liposarcoma, not otherwise
specified
Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology,
25/02/2019
clinical features, histopathological typing and grading. Fletcher CD, et
Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 12
al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
FIBROBLASTIC / MYOFIBROBLASTIC TUMOURS
Benign
Nodular fasciitis Proliferative fasciitis Proliferative myositis
Intermediate (rarely metastasizing)
Myositis ossificans
Solitary fibrous tumour
fibro-osseous pseudotumour of digits Ischaemic fasciitis
and haemangiopericytoma
Elastofibroma
(incl. lipomatous haemangiopericytoma)
Fibrous hamartoma of infancy Myofibroma / Myofibromatosis
Inflammatory myofibroblastic tumour Low grade
Fibromatosis colli
myofibroblastic sarcoma Myxoinflammatory
Juvenile hyaline fibromatosis Inclusion body fibromatosis
fibroblastic sarcoma Infantile fibrosarcoma
Fibroma of tendon sheath Desmoplastic fibroblastoma
Malignant
Mammary-type myofibroblastoma
Adult fibrosarcoma Myxofibrosarcoma
Calcifying aponeurotic fibroma Angiomyofibroblastoma Cellular
Low grade fibromyxoid sarcoma
angiofibroma Nuchal-type fibroma
hyalinizing spindle cell tumour Sclerosing epithelioid
Gardner fibroma Calcifying fibrous tumour Giant cell
fibrosarcoma
angiofibroma
Intermediate (locally aggressive)
Superficial fibromatoses (palmar / plantar) Desmoid-type
fibromatoses Lipofibromatosis

Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology,

25/02/2019
clinical features, histopathological typing and grading. Fletcher CD, et
Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 13
al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
 SO-CALLED FIBROHISTIOCYTIC TUMOURS
Benign
Giant cell tumour of tendon sheath Diffuse-type giant cell
tumour Deep benign fibrous histiocytoma
Intermediate (rarely metastasizing)
Plexiform fibrohistiocytic tumour Giant cell tumour of soft
tissues
Malignant
Pleomorphic ‘MFH’ / Undifferentiated pleomorphic sarcoma
Giant cell ‘MFH’ / Undifferentiated pleomorphic sarcoma
with giant cells
Inflammatory ‘MFH’ / Undifferentiated pleomorphic sarcoma
with prominent inflammation

Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology, clinical features, histopathological typing and grading. Fletcher CD, et al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
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 SMOOTH MUSCLE TUMOURS
Angioleiomyoma 8894/0
Deep leiomyoma 8890/0
Genital leiomyoma 8890/0
Leiomyosarcoma (excluding skin) 8890/3

PERICYTIC (PERIVASCULAR) TUMOURS

Glomus tumour (and variants) 8711/0
malignant glomus tumour 8711/3
Myopericytoma 8713/1

SKELETAL MUSCLE TUMOUR

Benign
Rhabdomyoma 8900/0 adult type 8904/0 fetal type 8903/0
genital type 8905/0
Malignant
Embryonal rhabdomyosarcoma 8910/3 (incl. spindle cell,
8912/3 botryoid, anaplastic) 8910/3
Alveolar rhabdomyosarcoma
(incl. solid, anaplastic) 8920/3
Pleomorphic rhabdomyosarcoma
Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology,
25/02/2019
clinical features, histopathological typing and grading. Fletcher CD, et
Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 15
al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
 VASCULAR TUMOUR

Benign
Haemangiomas of
subcut/deep soft tissue: 9120/0
capillary 9131/0
cavernous 9121/0
arteriovenous 9123/0
venous 9122/0 intramuscular 9132/0
synovial 9120/0
Epithelioid haemangioma 9125/0
Angiomatosis
Lymphangioma 9170/0
Intermediate (locally aggressive)
Kaposiform haemangioendothelioma 9130/1
Intermediate (rarely metastasizing)
Retiform haemangioendothelioma 9135/1
Papillary intralymphatic angioendothelioma 9135/1
Composite haemangioendothelioma Kaposi sarcoma
Malignant
Epithelioid haemangioendothelioma Angiosarcoma of soft
tissue
Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology,
25/02/2019
clinical features, histopathological typing and grading. Fletcher CD, et
Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 16
al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
 CHONDRO-OSSEOUS TUMOURS
Soft tissue chondroma Mesenchymal chondrosarcoma
Extraskeletal osteosarcoma

TUMOR OF UNCERTAIN DIFFERENTIATION

Benign
Intramuscular myxoma (incl. cellular variant)
Juxta-articular myxoma
Deep (‘aggressive’) angiomyxoma Pleomorphic hyalinizing
angiectatic tumour
Ectopic hamartomatous thymoma
Intermediate (rarely metastasizing)
Angiomatoid fibrous histiocytoma Ossifying fibromyxoid
tumour
(incl. atypical / malignant)
Mixed tumour/
Myoepithelioma/
Parachordoma

Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology,

25/02/2019
clinical features, histopathological typing and grading. Fletcher CD, et
Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 17
al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
 Malignant
Synovial sarcoma
Epithelioid sarcoma
Alveolar soft part sarcoma
Clear cell sarcoma of soft tissue Extraskeletal myxoid
chondrosarcoma
("chordoid" type)
PNET / Extraskeletal Ewing tumour
pPNET
extraskeletal Ewing tumour Desmoplastic small round cell
tumour Extra-renal rhabdoid tumour
Malignant mesenchymoma
Neoplasms with perivascular epithelioid
cell differentiation (PEComa)
clear cell myomelanocytic tumour
Intimal sarcoma

Fletcher CD, et al (2015). Soft tissue tumor: Epidemiology,

25/02/2019
clinical features, histopathological typing and grading. Fletcher CD, et
Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 18
al. WHO
Classification of Soft Tissue Tumors. USA. p. 1-18.
 T – Primary tumor

• T0 : No evidence of primary tumor

• T1 : Tumor <5 cm in greatest dimension
• T1a : Superficial tumor
• T1b : Deep tumor
• T2 : Tumor >5 cm in greatest dimension
• T2a : Superficial tumor
• T2b : Deep tumor
• Tx : Primary tumor cannot be assessed

N – Regional lymph nodes

• N0 : No regional lymph node metastasis

• N1 : Regional lymph node metastasis
• Nx : Regional lymph noeds cannot be assesed

M – Distant metastasis

• M0 : No distant metastasis
• M1 : Distant metastasis

Greene Frederick, Page David, Fleming Irvin, Fritz April, Balch Charles, et al (2002). Soft Tissue Sarcoma. Greene Frederick, Page David, Fleming Irvin, Fritz
April, Balch Charles, et al, editors. Ajcc Cancer Staging Manual. US: Springer. p.193-200
Edge, S. B., & Compton, C. C. (2010). The American Joint Committee on Cancer: the 7th Edition of the AJCC Cancer Staging Manual and the Future of
25/02/2019 TNM. Annals of Surgical Oncology, 17(6), 1471–1474. 19
 Stage Grouping
Stage IA Low grade T1a N0 M0
Low grade T1b N0 M0
Stage IB Low grade T2a N0 M0
Low grade T2b N0 M0
Stage IIA High grade T1a N0 M0
High grade T1b N0 M0
Stage IIB High grade T2a N0 M0
Stage III High grade T2b N0 M0
Stage IV Any Any T N1 M0
Any AnyT AnyN M1
Greene Frederick, Page David, Fleming Irvin, Fritz April, Balch Charles, et al (2002). Soft Tissue Sarcoma. Greene Frederick, Page David, Fleming Irvin, Fritz April, Balch Charles, et al, editors. Ajcc Cancer Staging
Manual. US: Springer. p.193-200
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Edge, S. B., & Compton, C. C. (2010). The American Joint Committee on Cancer: the 7th Edition of the AJCC Cancer Staging Manual and the Future of TNM. Annals of Surgical Oncology, 17(6), 1471–1474.
 Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker Solid PERABOI 2010. Jakarta:
25/02/2019
Sagung Seto. h.165-93 21
 Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker Solid PERABOI 2010. Jakarta: Sagung Seto.
h.165-93

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 SKOR KARNOFSKY
100- normal, tidak ada keluhan, tidak ada bukti penyakit
90-- mampu melakukan aktivitas normal, gejala dan tanda minor dari
penyakit
80-- aktivitas normal dengan usaha, gejala dan tanda penyakit (+)
70-- merawat diri, tidak mampu beraktivitas normal atau bekerja aktif
60-- kadang membutuhkan bantuan, tapi mampu merawat kebanyakan
kebutuhan pribadi
50-- membutuhkan bantuan dan perawatan medis rutin
40-- lumpuh, membutuhkan asisten dan perhatian lebih
30-- lumpuh parah, indikasi mondok, meski kematian belum dekat
20-- sangat sakit, butuh perawatan di RS, butuh terapi suportif aktif
10-- sekarat, proses yang fatal berlangsung cepat
0 -- mati
Peus Dominik, Newcomb Nocolas, Hofer Silvia (2013). Apprisal of the Karnofsky Performance Status and Proposal of a Simple Algorithmic System for its Evaluation.
Medical Informarics & Decision Making. 13; 72.

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 PENATALAKSANAAN

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 OPERASI SISTEMIK

RADIASI TARGET
Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker
Solid PERABOI 2010. Jakarta: Sagung Seto. h.165-93
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 25
 PEMBEDAHAN

• Modalitas utama  eksisi luas (G I-II, terlokalisir)

• 70 kasus dieksisi luas dengan margin negatif  4% rekurensi lokal (Rhydhom
et al)
• Tidak ada perbedaan pasien terapi bedah dengan radioterapi perioperatif
pada pasien Local Recurrent Free Surivival (Fiore Marco et al)
• Limb-sparing procedure  mempertahankan fungsi
• Amputasi
• Diseksi kelenjar getah bening regional
• Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan
Penatalaksanaan Kanker Solid PERABOI 2010. Jakarta: Sagung Seto. h.165-93
• Blay, Casali (2010). Soft Tissue Sarcomas: ESMO Clinical Practice Guideline for diagnosis, treatment and
follow-up. Annals of Oncology 21 (supplement 5): v198-v203
• Fiore, M., Ford, S., Callegaro, D., Sangalli, C., Colombo, C., Radaelli, S., et al (2018). Adequate Local Control
in High-Risk Soft Tissue Sarcoma of the Extremity Treated with Surgery Alone at a Reference Centre: Should
Radiotherapy Still be a Standard? Annals
25/02/2019 ofJaringan
Sarkoma Surgical Oncology,
Lunak, 25(6),
Bedah FK UNHAS 2018 1536–1543. 26
 MacNeill, A. J., Gupta, A., & Swallow, C. J. (2017). Randomized Controlled Trials in Soft Tissue
25/02/2019 Sarcoma: We Are Getting There! Surgical Oncology Clinics of North America, 26(4), 531–544 27
 Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker Solid PERABOI 2010.
25/02/2019 Jakarta: Sagung Seto. h.165-93 28
 RADIOTERAPI

•Tujuan: optimalisasi kontrol lokal

•Radiasi pasca operasi: bersifat individu
•Brachiterapi waktu lebih singkat 4-6 hari

Elsorafy Kareem, Ashfold Robert (June, 2015). Management soft tissue sarcoma. Orthopedics abd Trauma.
Volume 29, Issue 3. P 155-9
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 29
 RADIOTERAPI
• Keuntungan Preoperatif, Memungkinkan untuk penanganan
multidisiplin, Dosis radiasi lebih rendah, Ukuran lapangan
radiasi dan jumlah sendi yang terlibat lebih kecil
• Kerugian Radiasi Preoperatif: Tingginya insiden komplikasi luka
operasi, Sulit menentukan batas insisi, Diagnosis berdasarkan
biopsi saja, Pembedahan tertunda, Ekstensi tumor sulit dinilai
• Keuntungan Radiasi Pasca Operasi, Komplikasi luka rendah,
Pembedahan segera, Pemeriksaan histopatologis lengkap,
Ekstensi tumor dapat dinilai
• Kerugian Radiasi Pasca Operasi; Amputasi mungkin lebih besar

Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 30
Kanker Solid PERABOI 2010. Jakarta: Sagung Seto. h.165-93
 TARGETING THERAPY

•Olaratumab, antibodi monoklonal yang

menargetkan reseptor faktor pertumbuhan
yang berasal dari trombosit (PDGFR) -α,
ditunjukkan untuk memperpanjang
kelangsungan hidup keseluruhan pasien
sarkoma jaringan lunak dan disetujui pada
tahun 2016 di AS sebagai terapi terobosan.
25/02/2019 Nakano Kenji, Takahashi Shunji (2018). Current molecular therapies for Bone and Soft tissue sarcomas. Dept of Medical 31
oncology, Cancer Institute Hospital of Japanese.
 TERAPI SISTEMIK
• Adjuvan Kemoterapi  high grade sarkoma dengan diameter lebih 10
cm.
• Neoadjuvant kemoterapi  alternatif untuk mempertahankan
ekstremitas (limb sparing) pada pasien dengan kandidat untuk amputasi
• Obat: derivat antracyclin (doxorubicin, farmorubicin), antimetabolic
(methotrexate, bleomycin, 5 fluorouracyl), vinca alkaloid (vincristine,
etoposide), alkylating agent (dacarazine, cisplatin, cyclopjosphamide)
cm
• Isolated Limb Perfusion (ILP)  respon prosedur 18-80%, OS 50-70%

Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker Solid
PERABOI 2010. Jakarta: Sagung Seto. h.165-93
Elsorafy Kareem, Ashfold Robert (June, 2015). Management soft tissue sarcoma. Orthopedics abd Trauma. Volume 29, Issue
3. P 155-9
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 32
 REKURENSI LOKAL

• Pengaruh rekurensi lokal terhadap survival dan distant free

survival masih kontroversi.
• Rekurensi lokal diterapi dengan re-eksisi dengan batas bebas
tumor ditambah radiasi pasca re-eksisi.
• Pasien yang sebelumnya telah mendapatkan radioterapi dapat
dipertimbangkan untuk brachiterapi atau radiasi intraoperatif. [3,9]

Manuaba Tjakra W (2010). Sarkoma Jaringan Lunak (SJL). Manuaba Tjakra W, editor. Panduan Penatalaksanaan Kanker
Solid PERABOI 2010. Jakarta: Sagung Seto. h.165-93
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 33
METASTASIS

• Manajemen metastatik lokal atau metastatik jauh bersifat

kompleks dan memerlukan perawatan multidisiplin yang
disesuaikan secara individual oleh tim dokter spesialis.
• Terapi sistemik dapat diberikan dengan beberapa kemungkinan
tujuan, termasuk perpanjangan hidup, menghilangkan gejala, atau
perampingan untuk kemungkinan reseksi.

Blay, Casali (2010). Soft Tissue Sarcomas: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-
up. Annals of Oncology 21 (supplement 5): v198-v203

25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 34

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 Prognosis

• Rekurensi terjadi 2 tahun pertama

• M1 - Buruk

Follow up

• Anamnesis dan Pemeriksaan Fisik per 3 bulan

• Imaging per 3 bulan

MacNeill, A. J., Gupta, A., & Swallow, C. J. (2017). Randomized Controlled Trials in Soft Tissue Sarcoma: We Are Getting
25/02/2019 Sarkoma Jaringan Lunak, Bedah FK UNHAS 2018 41
There! Surgical Oncology Clinics of North America, 26(4), 531–544
KESIMPULAN
• Diagnosis  anamnesis pemfis, PA dan Imaging
• Tatalaksana  pembedahan, radioterapi,
sistemik dan targeting therapy
• Prognosis buruk dan angka rekurensi tinggi
• Follow up perlu dilakukan

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 TERIMA KASIH

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