summary Soft Tissue Sarcomas are rare, malignant soft tissue tumors comprising of a variety of subtypes distinguished by histological findings. The condition usually presents in patients > 15 years old with a slow-growing, painless soft tissue mass. Diagnosis is made by biopsy and histological findings. Treatment usually involves mass excision and radiation therapy. Epidemiology Incidence 7000 new case/year in the United States Demographics males > females 85% occur in patients >15 years old Anatomic location 60% occur in extremities Etiology Genetics classic translocations t(X;18); SYT-SSX fusion synovial sarcoma t(2:13) rhabdomyosarcoma (alveolar) t(12;16)(q13:p11) myxoid liposarcoma Associated conditions neurofibromatosis type-1 malignant peripheral nerve sheath tumor (MPNST) Stuart-Treves syndrome angiosarcoma Classification Classification of STS is based on histology, with over 50 types recognized all STS have the same presentation, imaging, differential diagnosis, and treatment histologic findings and molecular signatures are used to distinguish individual types commonly tested soft tissue sarcomas include undifferentiated pleomorphic sarcoma (UPS; previously malignant fibrous histiocytoma) malignant peripheral nerve sheath tumor (MPNST) synovial sarcoma liposarcoma rhabdomyosarcoma fibrosarcoma leiomyosarcoma epithelioid sarcoma angiosarcoma dermatofibrosarcoma protuberans clear cell sarcoma alveolar soft part sarcoma (ASPS) AJCC staging system is used for interdisciplinary communication AJCC Staging System for Soft Tissue Sarcoma of the Extremity or Trunk Stage Size Lymph Node Involvement Metastasis Grade IA < 5 cm None/unknown None Low IB > 5 cm None/unknown None Low II < 5 cm None/unknown None Intermediate/high IIIA 5-10 cm None/unknown None Intermediate/high IIIB > 10 cm None/unknown None Intermediate/high IVA Any Regional None Any IVB Any Any Present Any Presentation History may be a history of trauma that draws attention to the mass Symptoms slow growing, painless mass incidental trauma often draws attention to mass Physical exam inspection mass may be palpable lack of ecchymosis suggests an encapsulated mass could contradict diagnosis of hematoma, which typically presents with ecchymosis after trauma palpable soft tissue lesion differentiation of whether a mass is above or below fascia has prognostic importance size > 5 cm in cross-section is a poor prognostic factor can be confused with more common pathology lipoma hematoma Imaging Radiographs indications obtain plain radiographs in at least two planes for initial workup CT chest indications obtain to assess for metastatic disease MRI indications mandatory to evaluate soft tissue lesions in the extremities and determine treatment algorithm findings T1: low signal intensity (isointense with muscle) T2: high signal intensity IV gadolinium: peripheral enhancing zone and non-enhancing necrotic center can be diagnostic for the following benign lesions lipoma neurilemoma (schwannoma) intramuscular myxoma if MRI is diagnostic and the mass is benign and symptomatic, then it can be removed without a biopsy indeterminate MRI if MRI is indeterminate or suggestive of sarcoma, then a core needle or open biopsy must be obtained before further treatment is initiated soft tissue sarcomas can look similar to hematomas, so be cautious of a "hematoma" which occurs without trauma Studies Histology determined by type of sarcoma undifferentiated pleomorphic sarcoma malignant peripheral nerve sheath tumor synovial sarcoma liposarcoma rhabdomyosarcoma fibrosarcoma leiomyosarcoma epithelioid sarcoma angiosarcoma dermatofibrosarcoma protuberans Differential Differentiate from benign soft tissue masses with MRI and biopsy Hematoma Lipoma Intramuscular Myxoma Hemangioma of Soft Tissue Calcifying Aponeurotic Fibroma Plantar Fibromatosis (Ledderhose Disease) Extra-abdominal Desmoid Tumor Nodular Fasciitis Neurofibroma Neurilemmoma Neuroma Treatment Operative biopsy indications mass concerning for malignancy after clinical and radiological workup outcomes best results if done at the institution where definitive surgery will take place, under a multidisciplinary team wide surgical resection & radiation therapy indications standard of care in most cases treatment must be based on a tissue diagnosis unless images are diagnostic (i.e. lipoma) radiation is an important adjunct to surgery decreasing local recurrence chemotherapy is controversial for soft tissue sarcomas considered standard of care for rhabdomyosarcoma and Ewing sarcoma outcomes reduces risk of local recurrence to less than 10% amputation indications when otherwise unable to resect tumor with negative margins most frequently in the foot resection would result in irreversible damage to major nerves patient comorbidities limit potential for recovery after limb-sparing surgery outcomes good prognosis in absence of metastatic disease surgical resection of lung metastases indications first-line for pulmonary metastases if the preop evaluation shows that complete resection is possible outcomes curative in up to 25% of patients Techniques Biopsy approach longitudinal incision transverse incisions are contraindicated due to a need for wider resection at time of surgery technique use anatomic route that limits contamination of compartments and neurovascular structures maintain meticulous hemostasis use knife or curette to remove tissue drains should be placed distal and in line with the surgical incision to allow removal of drain site with extensile incision Wide resection & radiation therapy approach elliptical incision used to incorporate biopsy and drain sites technique resection use of tourniquet without exsanguination maintain meticulous hemostasis meticulous dissection to ensure wide margins, must confirm border free of disease with histology frozen section can be used to ensure margins are free of tumor radiation 50 Gy pre-operative and 66 Gy postoperative is the standard dose for soft-tissue sarcomas timing of radiation does not impact overall survival Amputation approach level and incision guided by advanced imaging to ensure negative margins technique use of tourniquet without exsanguination maintain meticulous hemostasis meticulous dissection to ensure wide margins, must confirm border free of disease with histology frozen section can be used to ensure margins are free of tumor Surgical resection of lung metastases technique wedge resection lobectomy Complications Radiation induced pre-operative radiotherapy is associated with a > 30% risk for wound complications post-operative radiotherapy is associated with greater radiation-induced morbidity and an increased risk of radiation-induced sarcoma early effects: desquamation, delayed wound healing, infection late effects: fibrosis, post-radiation fracture, possible secondary sarcoma postradiation sarcoma Recurrence local recurrence <10% with radiation and surgery following resection, the most common location for recurrence of a low-grade STS is locally Unplanned excision CT chest, abdomen, pelvis to exclude metastasis MRI of the limb to determine the degree of contamination, post-operative changes, and to assess margins revision surgery limb salvage + radiation therapy amputation unplanned excision is associated with higher plastic reconstruction of the affected region Prognosis Natural history of disease often slow-growing painless mass metastasis is most commonly to the lung 5% lymph node metastasis (most commonly epithelioid, synovial, angiosarcoma, rhabdomyosarcoma, clear cell) Prognostic variables tumor stage is most important prognostic factor poor prognostic factors include high grade metastatic disease size > 5 cm tumor location below the deep fascia delay in diagnosis unplanned excision most common error in STS treatment referral to orthopaedic oncologist for multidisciplinary workup and repeat excision is critical Survival with treatment <10% local recurrence with surgery and radiation mortality up to 50% with high-grade disease
Technique Guide Technique guides are not considered high yield topics for orthopaedic standardized exams including ABOS, EBOT and RC. Wide Resection of Thigh Soft Tissue Sarcoma Team Orthobullets (D) Pathology - Soft Tissue Sarcoma
QUESTIONS 1 of 28 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ12.57) A 58-year-old man presents to your clinic with a 4-cm deep mass in his left thigh. It has been slowly expanding over the last 6 months. Radiographs show no evidence of bony involvement or soft tissue calcifications within the lesion. Which of the following is most appropriate at this time? QID: 4417 Type & Select Correct Answer 1 Repeat clinical examination and radiographs in 6 months 1% (55/4333) 2 Contrast-enhanced MRI scan 87% (3789/4333) 3 Fine needle aspiration 2% (89/4333) 4 Core biopsy 5% (228/4333) 5 Excisional biopsy 3% (148/4333) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ11.234) A 56-year-old male undergoes resection of a mass that was suspected to be a simple lipoma. The mass was contained within his sartorius muscle and shown in Figure A. The final pathology came back as a high grade soft tissue sarcoma. The treating surgeon operated completely within the sartorius compartment and did not violate any other compartments, however the final pathology showed positive margins. Staging studies show isolated disease in his right thigh. What is the most appropriate treatment for this patient? QID: 3657 FIGURES: A Type & Select Correct Answer 1 Amputation 1% (49/3522) 2 Systemic chemotherapy 1% (35/3522) 3 Radiation therapy 1% (44/3522) 4 Wide excision of the tumor bed 20% (722/3522) 5 Wide excision of the tumor bed followed by radiation therapy 75% (2656/3522) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ09.69) A 24-year-old male presents to your office with ecchymosis and a mass in the posterolateral aspect of his thigh. He states he noticed the mass approximately 6-months ago and that it has slowly been growing. He denies constitutional symptoms, is otherwise healthy, and denies any associated trauma. MRI report suggests the mass may be an organizing hematoma. Core needle biopsy in the office yields only blood. What is the next appropriate treatment option? QID: 2882 Type & Select Correct Answer 1 Conservative treatment - rest, ice, compression, elevation 10% (225/2285) 2 Repeat core needle biopsy 6% (130/2285) 3 Consult interventional radiography to place a drain under image guidance 3% (69/2285) 4 Irrigation and debridement with initiation of postoperative antibiotics 2% (54/2285) 5 Open surgical biopsy 79% (1796/2285) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ06.248) Which treatment factor has been shown to be most important for five-year disease-free survival in patients with subcutaneous sarcomas? QID: 259 Type & Select Correct Answer 1 Dose of chemotherapy 1% (10/1029) 2 Addition of adjuvant radiation 16% (165/1029) 3 Size of operative margin 80% (821/1029) 4 Length of chemotherapy course 1% (15/1029) 5 Addition of radiofrequency ablation 1% (13/1029) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (OBQ06.225) A surgeon resects a small, superficial, mobile lesion from the thigh. Pathological review of the specimen suggests it is a low-grade soft tissue sarcoma with positive surgical margins. After staging the patient, no other sites of disease are identified. What is the next most appropriate treatment for this patient? QID: 236 Type & Select Correct Answer 1 Observation 3% (64/1965) 2 Systemic chemotherapy 3% (67/1965) 3 Radiotherapy 8% (155/1965) 4 Tumor bed re-excision 85% (1661/1965) 5 Amputation 0% (9/1965) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ05.231) Sixty Gy is a typical radiation dose for which of the following indications? QID: 1117 Type & Select Correct Answer 1 Prevention of post-operative heterotopic ossification 29% (320/1119) 2 Neoadjuvant radiation therapy for a high-grade chondrosarcoma 6% (70/1119) 3 Adjuvant radiation therapy for a high-grade osteosarcoma 10% (113/1119) 4 Adjuvant radiation therapy for a soft-tissue sarcoma 44% (497/1119) 5 Palliative therapy for a painful adenocarcinoma metastasis in the thoracic spine 10% (107/1119) L 4 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ04.106) A 52-year-old male presents with 6 months of swelling and pain in his leg. He states the lesion has not changed in size for several months and doesn't bother him. He is otherwise healthy and has no other complaints. Representative photograph and MRI are shown in Figures A through C. What is the best initial step in his management? QID: 1211 FIGURES: A B C Type & Select Correct Answer 1 Follow-up in 6 months with repeat radiographs 1% (12/1337) 2 Biopsy 89% (1194/1337) 3 Marginal excision 4% (57/1337) 4 Radiation therapy 0% (5/1337) 5 Neoadjuvant chemotherapy and wide excision 5% (62/1337) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ04.184) What is the correct treatment for an incompletely excised high grade superficial sarcoma on the volar aspect of the forearm? QID: 1289 Type & Select Correct Answer 1 Chemotherapy 1% (23/2195) 2 Radiation therapy 4% (92/2195) 3 Marginal resection of the tumor bed to spare the neurovascular bundles 5% (99/2195) 4 Wide resection of the tumor bed and coverage as necessary 89% (1963/2195) 5 Palliative care 0% (7/2195) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (OBQ04.260) A 39-year-old male is diagnosed with myxoid liposarcoma of the thigh. Radiation therapy is being considered in adjunct to surgical excision. Which of the following is true regarding radiation therapy in soft tissue sarcoma? QID: 1365 Type & Select Correct Answer 1 Compared to post-operative radiation, pre-operative radiation is associated with a reduced rate of wound dehiscence 7% (141/1955) 2 Radiation therapy reduces the risk of local recurrence 73% (1421/1955) 3 Radiation therapy improves overall survival 8% (166/1955) 4 Compared to post-operative radiation, pre-operative radiation is associated with increased rates of radiation induced morbidity such as post-radiation fibrosis 10% (201/1955) 5 Radiation therapy decreases the chances of successful limb-salvage 1% (16/1955) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic
FREE PDF OKU- MSK 3 The Evaluation and Diagnosis of Soft Tissue Masses Mayerson Pathology - Soft Tissue Sarcoma Mayerson, 1 views 0.0 UptoDate.com Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma. Ryan CW Meyer J Pathology - Soft Tissue Sarcoma Ryan CW, 1 views 0.0
All Videos (4) Podcasts (1) Login to View Community Videos Login to View Community Videos Cleveland Combined Hand Fellowship Lecture Series 2018-2019 Soft Tissue Tumors of the Upper Extremity Part II - Timothy Fei, MD Timothy Fei Pathology - Soft Tissue Sarcoma D 10/1/2020 56 views 0.0 (0) Login to View Community Videos Login to View Community Videos Cleveland Combined Hand Fellowship Lecture Series 2018-2019 Tumor like Lesions & Benign Tumors of the Hand & Wrist - Deepak Ramanathan, MD Deepak Ramanathan Pathology - Soft Tissue Sarcoma D 9/29/2020 33 views 0.0 (0) Login to View Community Videos Login to View Community Videos California Orthopaedic Association Annual Meeting - 2018 Appropriate Work-Up of Commonly Found Lesions How to Decide When to Refer to an Orthopaedic Oncologist Avoiding Unplanned Resection of Sarcoma/Disease Spread - Jeffrey Krygier, MD (COA 2018, 7.1) Pathology - Soft Tissue Sarcoma A 8/30/2018 501 views 4.6 (8) Pathology | Soft Tissue Sarcoma Pathology - Soft Tissue Sarcoma Listen Now 18:0 min 10/15/2019 433 plays 4.8 (4) See More See Less
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