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Soft Tissue Sarcoma
Presentor : Dr Ajit H Moderator : Dr Praveen M - Sarcomas represent a heterogenous group of rare malignant tumours
- Mesenchymal origin
- Occur anywhere
- Account for less than 1% of malignant tumours
- Sarcomas include more than 70 histologic subtypes
- Extermity is the most common location ( 50-
60%) , followed by trunk (19%)
- Then retroperitoneum (15%) and head and
neck (9%). History - In 1761 , Giovanni Battista Morgagni, an Italian anatomist reported first case of retroperitoneal sarcoma(RPS)- a large lipomatous tumour during the autopsy of a 60- year-old female Retroperitoneum divided into three spaces –
- Anterior pararenal space
- Perirenal space
- Posterior pararenal space
Anterior pararenal space:
- It is anterior to renal fascia , but posterior to
peritoneum
- Contains the ascending and descending colon,
the duodenum, and the pancreas. Perirenal space :
- Lies posterior to anterior pararenal space
- Contains inferior vena cava, the aorta, kidneys,
and adrenal glands. Posterior pararenal space :
- Posterior pararenal space is in continuity with
preperitoneal fat of the anterior abdomen. Why surgeons need to know? - High rates of recurrence and poor overall survival.
- The overall 5-year survival rate is 33% to 39%
and at least 70 % of cases will relapse
- Most succumb to lung metastasis - within 2 to
3 years after initial diagnosis Subtypes of STS and frequencies SUBTYPES PERCENTAGE
Liposarcoma 15
Leiomyosarcoma 12
Unclassified sarcoma 11 Synovial sarcoma 10
Malignant peripheral nerve 6
sheath tumor
Rhabdomyosarcoma 5
Fibrosarcoma 3
Ewing sarcoma 2 Angiosarcoma 2
Osteosarcoma 1
Epithelioid sarcoma 1
Chondrosarcoma 1
Clear cell sarcoma 1
Alveolar soft part sarcoma 1
Malignant hemangiopericytoma 0.4
Epidemiology • 1) Radiation exposure : It is a rare but well established risk factor with a risk of 8 to 50 times greater than the general-population.
The most common histologic types were osteogenic
sarcoma, pleomorphic undifferentiated sarcoma, angiosarcoma, and lymphangiosarcoma • 2) Occupational exposure : Herbicides such as phenoxyacetic acids and to wood preservatives containing chlorophenols – increase risk
Others: thorium oxide (Thorotrast),
vinylchloride, and arsenic - hepatic angiosarcomas • 3) Trauma : Patient often give a history of trauma No causal relationship identified The most frequent retroperitoneal sarcoma subtypes are 1) Liposarcoma
2) Leiomyosarcoma
3) MFH( malignant fibrous histiocytoma) –
currently referred as undifferentiated pleomorphic sarcoma. • LIPOSARCOMA - Most common retroperitoneal sarcoma subtype
- Appears as large sized , well rounded mass
with homogenous fatty content
- Pathogenesis : amplification of chromosome
12q leading to upregulation of MDM2 and CDK4 • Divided into
1) Locally aggressive – well differentiated type
2) Malignant type – dedifferentiated ,
pleomorphic, myxoid and round cell type • Well differentiated and dedifferentiated : retroperitoneum location
- Single point mutation in gene encoding gene encoding KIT receptor tyrosine kinase or platelet-derived growth factor receptor-α (PDGFRA) 4) Complex genomic rearrangements Neurofibromatosis type 1 • 10% risk- malignant peripheral nerve sheath tumor Li- Fraumeni syndrome • Risk of rhabdomyosarcoma, undifferentiated pleomorphic sarcoma, and pleomorphic sarcoma Familial adenomatous polyposis • Risk of developing desmoid tumour Initial assessment • Clinical behavior determined by –
1) Histologic subtype
2) Grade of aggressiveness
3) Size - Average age at presentation : 54 years
- Male and Female ratio : 1:1
- Main pathway of metastasis : hematogenous to
the lungs Clinical features – - Mainly asymptomatic
- If symptoms present , variable presentation
and includes : abdominal pain, weight loss, early satiety, nausea, vomiting , back or flank pain, paresthesias, and weakness. - Testicular examination in men
- Differential diagnosis : 1) Benign lipoma ( 100 times more common)
2) Retroperitoneal lymphadenopathy: lymphoma,
germ cell tumor, or metastasis from gastrointestinal primary
10 cm in greatest dimension More than 10 cm and less than or equal to T3 15 cm in greatest dimension More than 15 cm in greatest dimension T4 Regional Lymph Nodes (N)
Sarcomas closely resembling normal adult mesenchymal tissue 1
Sarcomas for which histologic typing is certain 2
Embryonal and undifferentiated sarcomas, sarcomas of doubtful 3
types , synovial sarcomas, soft tissue osteoarcoma, Ewing sarcoma/ primitive neuroectodermal tumour of soft tissue FNCLCC(Fédération Nationale des Centres de Lutte Contre Le Cancer) Histologic grade
G G DEFINITION
GX Grade cannot be assessed
G1 Total differentiation, mitotic count, necrosis score – 2 or 3
G2 Total differentiation, mitotic count, necrosis score – 4 or 5
G3 Total differentiation, mitotic count, necrosis score – 6,7,8
AJCC prognostic stage grouping T N M GRADE STAGE T1 N0 M0 G1,GX IA
T2, T3, T4 N0 M0 G1,GX IB
T1 N0 M0 G2,G3 II
T2 N0 M0 G2,G3 III A
T3, T4 N0 M0 G2,G3 III B
ANY T T1 M0 ANY G III B
ANY T ANY N M1 ANY G IV
Imaging studies • Ultrasound Advantages : - inexpensive - in patients with metallic implants - adjunct to MRI when findings are indeterminate and for delineating adjacent vascular structures -postoperative surveillance and to guide biopsies. • Disadvantage :
- low inter- and intra-observer agreement
- operator’s technique related skills.
Computed Tomography It is the preferred imaging technique for retroperitoneal sarcoma 1) Identify and characterize the masses in terms of location, size and extension in the retroperitoneal space
2) Spectrum of differential diagnosis
(solid/cystic, neoplastic/nonneoplastic) 3) Guide the percutaneous biopsy
4) Plan surgery for complete surgical resection -
local soft tissue infiltration and relationship with critical neurovascular structures
5) Identify postoperative complications
6) Assessing response to neoadjuvant treatment
7) Follow-up and restaging
Also a CT chest is to be done – lung metastasis
When ? At presentation and before radical treatment • Liposarcoma :
- Differentiated liposarcoma : smooth margins
with lobular contour , thick septa , nodular component with low contrast enhancement - Dedifferentiated liposarcoma : - Non lipomatous mass surrounded by fatty tissue - Calcifications present - Nodular or irregularly linear septa within the lesion - Increase enhancement after contrast • Leiomyosarcoma: Large mass with well defined margin iso attenuating to muscle
intra-abdominal disease 4) Assess early treatment response
5) For biopsy • Disadvantage :
1) No clear role for diagnosis
2) Poor discrimination between low-grade
tumors and benign lesions Biopsy examination - CT-guided core needle biopsy is appropriate to provide a tissue diagnosis
- But not mandatory
- Also sampling bias – giving false information
Treatment - For localized sarcoma- Complete surgical resection is the mainstay of treatment
- To improve local control and potentially
overall survival- extended surgical approach (en bloc tumor resection with adherent organs and structures) advocated Extent of resection - Before going for multivisceral resection – a balance between margin adequacy and morbidity should be considered
- Generally , en bloc resection of ipsilateral
colon, kidney, adrenal, and psoas muscle is done to get a negative margin - In some patients , resection of pancreas and spleen or a pancreaticoduodenectomy or vascular resection is required
- Surgical access : a midline incision is the most
common
- For better exposure and vascular control-
transverse/ oblique extension or thoracoabdominal incision (1) Midline laparotomy incision
(2) Subcostal extension
(3) Extension transversally to the flank
(4) Ilioinguinal incision
(5) Right thoraco-phreno-laparotomy
• Leiomysarcoma : - Surgical resection with negative margin is first line of therapy
- If metastasis present , non resectable:
- Olaratumab (PDGFR alpha antibody ) + Doxorubicin Role of radiotherapy - Complex
- Tumours are large and close to radiosensitive
structures
- Several techniques used : preoperative and
postoperative external-beam radiation therapy, intraoperative radiation therapy, brachytherapy • Pre op radiation therapy
- Better tolerated as toxic effects are less
- Tumor borders are definable
- Tumor displaces radiosensitive viscera away
from the treatment field - Effective doses of radiation may be lower preoperatively
- Thus, for patients with large, high-grade tumors
or recurrent low-grade tumors : Total pre-op dose of 50 Gy followed by surgical resection is considered Role of chemotherapy - Controversial
- As the trials are inconclusive on the role of
chemotherapy and no consclusions can be drawn (European Society for Medical Oncology , European Organisation for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group) Treatment of recurrence - Recurrence rates are high .
- Eg ; Retroperitoneal leiomyosarcomas- recur
locally and metastasize to the lungs and liver.
- Retroperitoneal sarcomas recur diffusely
throughout the peritoneal cavity - Treatment : extended surgical resection
- But probability of resectability declines with
each recurrence. Metastatic sarcomas
- First-line therapy : Single or combination
therapy with anthracyclines
- Second-line therapy : gemcitabine and
docetaxel Immunotherapy agents - Use of immunotherapy agents – fail to give good results
- But , undifferentiated pleomorphic sarcoma or
dedifferentiated liposarcoma have response when treated with pembrolizumab, a programmed death-1 inhibitor. - Other Novel agents are undergoing further study such as trabectedin, tyrosine kinase inhibitors, MDM2 antagonists, peroxisome proliferator-activated receptor gamma agonists, and CDK4 antagonists. Studies In 1954 Pack and Tabah , 120 patients treated for retroperitoneal tumors at the Memorial Sloan Kettering Cancer showed results :
- Complete excision of the tumor was achieved
in 21% of cases
- Operative mortality of 10.8%
In 1973, Kinne published results of 34 RPS at the Memorial Hospital for Cancer and Allied Diseases of New York. - 32.4% : complete resection with/without radiation therapy - 44.1% : partial resection and radiation therapy - 23.5% : biopsy and radiation therapy.
- Operative mortality was 11.7%.
In 1984, McGrath published results for 47 RPS patients who underwent surgery at the Medical College of Virginia.
- Complete resection was performed in 38% of
patients
- In 68% of cases, curative surgery required
multivisceral resection - Kidney (32%), colon (25%) and adrenal gland (18%) : most commonly resected
-The complication rate was up to 32%;
In 1998, Lewis et al demonstrated that the overall survival was better with complete resection in a study of 500 patients treated over a 15-year period with a postoperative mortality rate of 4% HPR showed : dedifferentiated liposarcoma pT4NxMx . Stage IIIB • References 1) Sabsiton textbook of surgery 21 st edition 2) Schwartz’s principles of surgery 11 th edition 3) Current treatment of retroperitoneal sarcomas- a joint effort with the Italian society of surgical oncology- 2018 EDITION 4) Fischer’s master of surgery 6th edition • THANK YOU
