Soft tissue sarcoma of the Soft tissue sarcoma of the extremities extremities - PDF Document

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  1. Soft tissue sarcoma of the Soft tissue sarcoma of the extremities extremities SUNY Downstate Medical Center SUNY Downstate Medical Center Brooklyn Veterans Hospital Brooklyn Veterans Hospital Valery Dronsky, M.D. Valery Dronsky, M.D.

  2. Soft Tissue Sarcomas of the Extremities

  3. Soft Tissue Sarcomas: Definition Soft Tissue Sarcomas: Definition ? ? Sarcomas are malignant tumors that Sarcomas are malignant tumors that arise from skeletal and extraskeletal arise from skeletal and extraskeletal connective tissue, mesenchymal cells, connective tissue, mesenchymal cells, including: including: ? ? adipose tissue adipose tissue ? ? bone bone ? ? cartilage cartilage ? ? smooth muscle smooth muscle ? ? skeletal muscle skeletal muscle

  4. Soft Tissue Sarcomas: Statistics Soft Tissue Sarcomas: Statistics ? ? Soft tissue sarcomas are rare and Soft tissue sarcomas are rare and unusual neoplasms unusual neoplasms ? ? about 1% of adult human cancers about 1% of adult human cancers ? ? 15% of pediatric malignancies 15% of pediatric malignancies ? ? Most commonly occur in the extremities Most commonly occur in the extremities (50%) (50%) ? ? Also common in the abdominal Also common in the abdominal cavity/retroperitoneum, trunk/thoracic cavity/retroperitoneum, trunk/thoracic region, and head and neck region, and head and neck

  5. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology Cell of origin Sarcoma type Adipocyte Liposarcoma Fibrohistiocyte Malignant fibrous histiocytoma Fibroblast Fibrosarcoma Smooth muscle Leiomyosarcoma Skeletal muscle Rhabdomyosarcoma Angiosarcoma, Kaposi’s Vascular Synovial Synovial sarcoma Melanocyte Malignant melanoma Unknown Ewing’s sarcoma, Epithelioid sarcoma

  6. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology ? ? Histopathology is Histopathology is determined by anatomic determined by anatomic site site ? ? Common subtypes in the Common subtypes in the extremity are liposarcoma extremity are liposarcoma and malignant fibrous and malignant fibrous histiocytoma histiocytoma ? ? In the retroperitoneal In the retroperitoneal location liposarcoma and location liposarcoma and leiomyosarcoma are the leiomyosarcoma are the most common histiotypes most common histiotypes ? ? In the visceral location, In the visceral location, gastrointestinal stromal gastrointestinal stromal tumors are found almost tumors are found almost exclusively exclusively

  7. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology ? ? Age as factor in histopathology Age as factor in histopathology ? ? In childhood, embryonal rhabdomyosarcoma is most In childhood, embryonal rhabdomyosarcoma is most common common ? ? Synovial sarcoma is more likely to be seen in young Synovial sarcoma is more likely to be seen in young adults (<35 years old) adults (<35 years old) ? ? An even distribution of liposarcoma and malignant An even distribution of liposarcoma and malignant fibrous histiocytoma as the predominant types in the fibrous histiocytoma as the predominant types in the older population older population

  8. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology ? ? The biologic behavior of The biologic behavior of sarcomas is extremely sarcomas is extremely variable variable ? ? Histologic grade is a Histologic grade is a major prognostic major prognostic determinant and is determinant and is based on degree of based on degree of mitosis, mitosis, cellularity cellularity, , presence of necrosis, presence of necrosis, differentiation, and differentiation, and stromal content stromal content

  9. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology ? ? Low Low- -grade sarcomas grade sarcomas- - better better- -differentiated, differentiated, less cellular, tend to less cellular, tend to resemble the tissue of resemble the tissue of origin to some extent, origin to some extent, cytologic abnormalities cytologic abnormalities are less prominent, are less prominent, mitotic rate is low mitotic rate is low ? ? Grow slower, low risk of Grow slower, low risk of metastasis, a high risk metastasis, a high risk of local recurrence after of local recurrence after surgical removal surgical removal

  10. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology ? ? High High- -grade sarcomas grade sarcomas- - highly cellular, poorly highly cellular, poorly differentiated differentiated mesenchymal cells with mesenchymal cells with marked nuclear marked nuclear abnormality, high abnormality, high mitotic rate and mitotic rate and anaplasia anaplasia ? ? Grow rapidly, show Grow rapidly, show extensive local invasion, extensive local invasion, metastasize early metastasize early through bloodstream, through bloodstream, usually fatal usually fatal

  11. Soft Tissue Sarcomas: Histology Soft Tissue Sarcomas: Histology ? ? Can be further Can be further characterized by cellular characterized by cellular appearance: appearance: ? ? well differentiated well differentiated ? ? undifferentiated undifferentiated ? ? myxoid myxoid ? ? round cell round cell ? ? pleomorphic pleomorphic

  12. Soft Tissue Sarcomas: Risk Factors Soft Tissue Sarcomas: Risk Factors ? ? Genetic Predisposition Genetic Predisposition ? ? Neurofibromatosis Neurofibromatosis - -von Recklinghausen's disease disease ? ? Li Li- -Fraumeni syndrome Fraumeni syndrome ? ? Retinoblastoma Retinoblastoma ? ? Gardner's syndrome (familial adenomatous Gardner's syndrome (familial adenomatous polyposis) polyposis) von Recklinghausen's 1 Barken D, Wright E, Nguyen D: Gene for von Recklinghausen neurofibromatosis is in the pericentromeric region of chromosome 17. Science 236:1100, 1987. 2 Li FP, Fraumeni JF: Soft-tissue sarcomas, breast cancer, and other neoplasms: A familial syndrome? Ann Intern Med 71:747, 1969. 3 Sorensen SA, Mulvihill JJ, Nielsen A: Long-term follow-up of von Recklinghausen neurofibromatosis: Survival and malignant neoplasms. N Engl J Med 314:1010, 1986

  13. Soft Tissue Sarcomas: Risk Factors Soft Tissue Sarcomas: Risk Factors ? ? Radiation Exposure Radiation Exposure1,2 ? ? Lymphedema Lymphedema ? ? Postsurgical Postsurgical ? ? Postirradiation Postirradiation ? ? Parasitic infection (filariasis) Parasitic infection (filariasis) ? ? Trauma Trauma ? ? Chemical Chemical ? ? 2,3,7,8 2,3,7,8- -Tetrachlorodibenzodioxin Tetrachlorodibenzodioxin ? ? Polyvinyl chloride Polyvinyl chloride ? ? Hemachromatosis Hemachromatosis ? ? Arsenic Arsenic 1,2 1 Brady MS, Gaynor JJ, Brennan MF: Radiation-associated sarcoma of bone and soft tissue. Arch Surg 127:1379, 1992. 2Brennan MF, Lewis JJ: Diagnosis and Management of Soft Tissue Sarcoma. London, Martin Dunitz, 2002.

  14. Soft Tissue Sarcomas: Pathogenesis Soft Tissue Sarcomas: Pathogenesis ? ? Specific genetic alterations : Specific genetic alterations : ? ? fusion genes due to reciprocal fusion genes due to reciprocal translocations translocations ? ? specific point mutations specific point mutations ? ? Nonspecific genetic Nonspecific genetic alterations : alterations : ? ? genetic losses and gains genetic losses and gains ? ? The tumor suppressor The tumor suppressor genes: genes: ? ? p53 p53 and and RB1 RB1

  15. Soft Tissue Sarcomas: Diagnosis Soft Tissue Sarcomas: Diagnosis ? ? Extremity sarcomas usually present with as a Extremity sarcomas usually present with as a painless mass painless mass ? ? However, pain is noted at presentation in up to However, pain is noted at presentation in up to 33% of patients 33% of patients ? ? Delay in diagnosis is common, with the most Delay in diagnosis is common, with the most common differential diagnosis for extremity and common differential diagnosis for extremity and trunk lesions being a hematoma or a "pulled" trunk lesions being a hematoma or a "pulled" muscle muscle

  16. Soft Tissue Sarcomas: Diagnosis Soft Tissue Sarcomas: Diagnosis ? ? Physical examination Physical examination ? ? should include assessment of the size of the mass should include assessment of the size of the mass and its relationship to neurovascular and bony and its relationship to neurovascular and bony structures structures ? ? Biopsy Biopsy ? ? Generally, in an adult, any soft tissue mass that is Generally, in an adult, any soft tissue mass that is symptomatic or enlarging, any mass that is larger symptomatic or enlarging, any mass that is larger than 5 cm, or any new mass that persists beyond 4 than 5 cm, or any new mass that persists beyond 4 weeks should be sampled weeks should be sampled Lewis J, Brennan MF: Soft tissue sarcomas. Curr Probl Surg 33:817, 1996

  17. Soft Tissue Sarcomas: Diagnosis Soft Tissue Sarcomas: Diagnosis ? ? Usually incisional or core biopsy Usually incisional or core biopsy preferred preferred ? ? Extremity masses should be biopsied Extremity masses should be biopsied through a longitudinal incision through a longitudinal incision ? ? Entire biopsy tract can be excised at Entire biopsy tract can be excised at the time of definitive resection the time of definitive resection ? ? The incision should be centered The incision should be centered over the mass in its most over the mass in its most superficial location superficial location ? ? No tissue flap should be raised No tissue flap should be raised ? ? Meticulous hemostasis to prevent Meticulous hemostasis to prevent cellular dissemination by hematoma cellular dissemination by hematoma

  18. Soft Tissue Sarcoma: Diagnosis Soft Tissue Sarcoma: Diagnosis ? ? Tru Tru- -cut core needle biopsy cut core needle biopsy ? ? Has been advocated as the first step in Has been advocated as the first step in diagnosis diagnosis ? ? Ease of performance Ease of performance ? ? Low cost Low cost ? ? Low complication rate Low complication rate ? ? Incisional biopsy may be reserved Incisional biopsy may be reserved for cases in which the core needle for cases in which the core needle biopsy is inconclusive biopsy is inconclusive ? ? Biopsy is indicated only if the actual Biopsy is indicated only if the actual treatment will be altered by a treatment will be altered by a definitive diagnosis definitive diagnosis Heslin MJ, Lewis JJ, Woodruff JM, Brennan MF: Core needle biopsy for diagnosis of extremity soft tissue sarcoma. Ann Surg Oncol 4:425, 1997.

  19. Soft Tissue Sarcomas: Diagnosis Soft Tissue Sarcomas: Diagnosis ? ? Excisional biopsy Excisional biopsy ? ? Only for small (< 3 cm) Only for small (< 3 cm) cutaneous or subcutaneous cutaneous or subcutaneous tumors tumors ? ? Fine Fine- -needle aspiration needle aspiration ? ? Limited role in diagnosing Limited role in diagnosing extremity soft tissue tumors extremity soft tissue tumors ? ? May be of value in the May be of value in the documentation of recurrence. documentation of recurrence.

  20. Soft Tissue Sarcomas: Diagnosis Soft Tissue Sarcomas: Diagnosis ? ? Imaging Imaging ? ? MRI preferred MRI preferred ? ? enhances the contrast enhances the contrast between tumor and between tumor and adjacent structures adjacent structures ? ? provides excellent provides excellent three three- -dimensional dimensional definition of fascial definition of fascial planes planes 1 Varma DG: Optimal radiologic imaging of soft tissue sarcomas. Semin Surg Oncol 17:2, 1999. 2 Panicek DM, Go SD, Healey JH, et al: Soft-tissue sarcoma involving bone or neurovascular structures: MR imaging prognostic factors. Radiology 205:871, 1997.

  21. Soft Tissue Sarcomas: Diagnosis Soft Tissue Sarcomas: Diagnosis ? ? Imaging: Relative value of Imaging: Relative value of MRI and CT MRI and CT ? ? No statistically significant No statistically significant difference between CT and difference between CT and MR imaging in determining MR imaging in determining tumor involvement of muscle, tumor involvement of muscle, bone, joints, or neurovascular bone, joints, or neurovascular structures structures ? ? Combination of CT and MR Combination of CT and MR images did not significantly images did not significantly improve accuracy improve accuracy Panicek DM, Gatsonis C, Rosenthal DI, et al: CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: Report of the Radiology Diagnostic Oncology Group. Radiology 202:237, 1997

  22. Soft Tissue Sarcomas: Workup Soft Tissue Sarcomas: Workup ? ? Evaluation for sites of potential metastasis: Evaluation for sites of potential metastasis: ? ? Lymph node metastases occur in less than 3% of Lymph node metastases occur in less than 3% of adult soft tissue sarcoma adult soft tissue sarcoma1 1 ? ? For extremity lesions, the lung is the principal site For extremity lesions, the lung is the principal site for metastasis of high for metastasis of high- -grade lesions ? ? For visceral lesions, the liver is the principal site For visceral lesions, the liver is the principal site3 3 ? ? Low Low- -grade lesions are assumed to have a low grade lesions are assumed to have a low <15% risk of subsequent metastasis <15% risk of subsequent metastasis ? ? High High- -grade lesions have a high >50% risk of grade lesions have a high >50% risk of subsequent metastasis subsequent metastasis grade lesions2 2 1 Fong Y, Coit DG, Woodruff JM, Brennan MF: Lymph node metastasis from soft tissue sarcoma in adults: Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg 217:72, 1993. 2 Gadd MA, Casper ES, Woodruff JM, et al: Development and treatment of pulmonary metastases in adult patients with extremity soft tissue sarcoma. Ann Surg 218:705, 1993. 3 DeMatteo RP, Lewis JJ, Leung D, et al: Two hundred gastrointestinal stromal tumors: Recurrence patterns and prognostic factors for survival. Ann Surg 231:51, 2000.

  23. Soft Tissue Sarcomas: Workup Soft Tissue Sarcomas: Workup ? ? Low Low- -grade extremity lesions require a grade extremity lesions require a chest radiograph chest radiograph ? ? High High- -grade lesions require a chest CT grade lesions require a chest CT ? ? Visceral lesions should have the liver Visceral lesions should have the liver imaged as part of the initial abdominal CT imaged as part of the initial abdominal CT or MRI or MRI

  24. Soft Tissue Sarcomas: Staging Soft Tissue Sarcomas: Staging ? ? Staging systems focus on: Staging systems focus on: ? ? Histologic grade of the tumor Histologic grade of the tumor ? ? Size of the primary tumor Size of the primary tumor ? ? Presence or absence of metastasis Presence or absence of metastasis ? ? Staging systems: Staging systems: ? ? apply to risk of metastasis apply to risk of metastasis ? ? disease disease- -specific survival specific survival ? ? overall survival overall survival almost exclusively confined to extremity lesions ? ? almost exclusively confined to extremity lesions 1 Brennan MF: Staging of soft tissue sarcomas. Ann Surg Oncol 6:8, 1999 2 Greene F, Page D, Fleming I, Fritz A, et al (eds): AJCC Cancer Staging Manual, 6th ed. Heidelberg, Springer-Verlag, 2002.

  25. Soft Tissue Sarcomas: Staging Soft Tissue Sarcomas: Staging

  26. Soft Tissue Sarcomas: Staging Soft Tissue Sarcomas: Staging

  27. Soft Tissue Sarcomas: Staging Soft Tissue Sarcomas: Staging

  28. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Surgical excision: Surgical excision: ? ? Greatest hope for cure Greatest hope for cure ? ? Whenever possible, function Whenever possible, function- - and limb procedures should be performed procedures should be performed ? ? As long as the entire tumor is removed, less radical As long as the entire tumor is removed, less radical procedures do not adversely affect local recurrence or procedures do not adversely affect local recurrence or outcome outcome ? ? Goal is complete removal of the tumor with negative Goal is complete removal of the tumor with negative margins and maximal preservation of function margins and maximal preservation of function and limb- -sparing sparing Rosenberg SA, Tepper J, Glatstein E, et al: The treatment of soft-tissue sarcomas of the extremities: Prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 196:305, 1982.

  29. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Excision Excision ? ? 1 to 2 cm margins 1 to 2 cm margins ? ? Amputation no longer Amputation no longer standard of care standard of care ? ? Meticulous dissection Meticulous dissection can prevent injury to can prevent injury to major neurovascular major neurovascular structures structures Williard WC, Collin C, Casper ES, et al: The changing role of amputation for soft tissue sarcoma of the extremity in adults. Surg Gynecol Obstet 175:389, 1992.

  30. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Wide excision alone Wide excision alone adequate for: adequate for: ? ? Any size low Any size low- -grade sarcoma sarcoma ? ? Subcutaneous or Subcutaneous or intramuscular high intramuscular high- -grade sarcoma < 5 cm sarcoma < 5 cm ? ? Otherwise adjuvant Otherwise adjuvant therapy is indicated therapy is indicated grade grade Yang JC, Chang AE, Baker AR, et al: Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16:197, 1998.

  31. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Adjuvant radiation Adjuvant radiation should be added to the surgical added to the surgical resection: resection: ? ? If the excision margin is close If the excision margin is close ? ? If extramuscular involvement is If extramuscular involvement is present present ? ? If a local recurrence would If a local recurrence would result in the sacrifice of a major result in the sacrifice of a major neurovascular bundle or neurovascular bundle or amputation amputation should be Yang JC, Chang AE, Baker AR, et al: Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16:197, 1998.

  32. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Adjuvant radiation Adjuvant radiation ? ? improves local control improves local control ? ? brachytherapy for high brachytherapy for high- - grade lesions grade lesions ? ? external external- -beam radiation beam radiation therapy for large (>5 cm) therapy for large (>5 cm) high high- - or low or low- -grade lesions grade lesions 1 Yang JC, Chang AE, Baker AR, et al: Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16:197, 1998. 2 Pisters PW, Harrison LB, Leung DH, et al: Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 14:859, 1996.

  33. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Adjuvant chemotherapy Adjuvant chemotherapy ? ? depends on the histologic depends on the histologic type of sarcoma; indicated type of sarcoma; indicated for: for: ? ? Ewing's sarcoma Ewing's sarcoma ? ? rhabdomyosarcoma rhabdomyosarcoma ? ? High risk of microscopic High risk of microscopic metastasis at diagnosis and good metastasis at diagnosis and good response to chemotherpy response to chemotherpy 1 Baldini EH, Demetri GD, Fletcher CD, et al: Adults with Ewing's sarcoma/primitive neuroectodermal tumor: Adverse effect of older age and primary extraosseous disease on outcome. Ann Surg 230:79, 1999. 2 Esnaola NF, Rubin BP, Baldini EH, et al: Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Ann Surg 234:215, 2001.

  34. Soft Tissue Sarcoma: Treatment Soft Tissue Sarcoma: Treatment ? ? Adjuvant chemotherapy Adjuvant chemotherapy ? ? Controversial for other Controversial for other histologic subtypes histologic subtypes ? ? Has no measurable impact Has no measurable impact on overall survival on overall survival ? ? Small (10% to 15%) Small (10% to 15%) improvement in disease improvement in disease- - free survival free survival 1 Bramwell V, Rouesse J, Steward W, et al: Adjuvant CYVADIC chemotherapy for adult soft tissue sarcoma-reduced local recurrence but no improvement in survival: A study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 12:1137, 1994. 2 Tierney JF, Stewart LA, Parmar MKB, et al: (Sarcoma Meta-analysis Collaboration): Adjuvant chemotherapy for localised resectable soft-tissue

  35. Soft Tissue Sarcoma Soft Tissue Sarcoma: : Recurrent Disease Disease Incidence of distant metastasis significant metastasis significant ? ? Billingsley, et al: Of 994 Billingsley, et al: Of 994 patients with primary extremity patients with primary extremity soft tissue sarcomas distant soft tissue sarcomas distant metastasis developed in 230 metastasis developed in 230 patients (23%) patients (23%)1 1 ? ? Median survival after the Median survival after the development of metastasis was development of metastasis was 11.6 months 11.6 months ? ? Lungs first metastatic site in Lungs first metastatic site in 73% 73% Recurrent ? ? Incidence of distant Billingsley KG, Lewis JJ, Leung DH, et al: Multifactorial analys Billingsley KG, Lewis JJ, Leung DH, et al: Multifactorial analysis of the survival of patients with distant metastasis arising from primary extremity patients with distant metastasis arising from primary extremity sarcoma. Cancer 85:389, 1999. 85:389, 1999. is of the survival of sarcoma. Cancer

  36. Soft Tissue Sarcoma Soft Tissue Sarcoma: : Recurrent Disease Disease Recurrent ? ? Predictors of survival: Predictors of survival: ? ? Extent of metastatic disease Extent of metastatic disease ? ? Length of the disease Length of the disease- -free interval interval ? ? Presence of a preceding local Presence of a preceding local recurrence recurrence ? ? Age Age free Billingsley KG, Lewis JJ, Leung DH, et al: Multifactorial analys Billingsley KG, Lewis JJ, Leung DH, et al: Multifactorial analysis of the survival of patients with distant metastasis arising from primary extremity patients with distant metastasis arising from primary extremity sarcoma. Cancer 85:389, 1999. 85:389, 1999. is of the survival of sarcoma. Cancer

  37. Soft Tissue Sarcoma Soft Tissue Sarcoma: : Recurrent Disease Disease Recurrent ? ? Local extremity recurrence Local extremity recurrence ? ? Presents as a nodular mass or Presents as a nodular mass or series of nodules arising in the series of nodules arising in the surgical scar surgical scar ? ? If isolated should undergo re If isolated should undergo re- - resection resection ? ? two thirds of these patients two thirds of these patients experience long experience long- -term survival benefit benefit ? ? Adjuvant radiation if feasible Adjuvant radiation if feasible term survival 1 1 Singer S, Antman K, Corson JM, Eberlein TJ: Long Singer S, Antman K, Corson JM, Eberlein TJ: Long- -term salvageability for patients with locally recurrent soft with locally recurrent soft- -tissue sarcomas. Arch Surg 127:548, 1992. tissue sarcomas. Arch Surg 127:548, 1992. 2 2 Brennan MF: The enigma of local recurrence. The Society of Surgi Brennan MF: The enigma of local recurrence. The Society of Surgical Oncology. Ann term salvageability for patients cal Oncology. Ann Surg Oncol 4:1, 1997. Surg Oncol 4:1, 1997.

  38. Soft Tissue Sarcoma Soft Tissue Sarcoma: : Recurrent Disease Disease ? ? Resection of pulmonary Resection of pulmonary metastases: metastases: ? ? Primary tumors controlled or Primary tumors controlled or controllable controllable ? ? No extrathoracic disease No extrathoracic disease ? ? Medically fit for thoracotomy Medically fit for thoracotomy ? ? Complete resection of all lung Complete resection of all lung disease appears possible disease appears possible ? ? 20% to 30% 3 20% to 30% 3- -year survival year survival rates after complete rates after complete resection resection Billingsley KG, Burt ME, Jara Jara E, et al: Pulmonary metastases from soft tissue E, et al: Pulmonary metastases from soft tissue sarcoma: Analysis of patterns of diseases and post sarcoma: Analysis of patterns of diseases and post- -metastasis survival. Ann Surg 229:602, 1999. 229:602, 1999. Recurrent 1 1 Billingsley KG, Burt ME, metastasis survival. Ann Surg

  39. Soft Tissue Sarcoma Soft Tissue Sarcoma: : Recurrent Disease Disease Role of chemotherapy ? ? Unresectable pulmonary metastases Unresectable pulmonary metastases ? ? Extrapulmonary metastatic sarcoma in Extrapulmonary metastatic sarcoma in more than a single site more than a single site ? ? Uniformly poor prognosis Uniformly poor prognosis ? ? DTIC (doxorubicin,ifosfamide,dacarbasine) and DTIC (doxorubicin,ifosfamide,dacarbasine) and MAID (mesna, ifosfamide, doxorubicin, MAID (mesna, ifosfamide, doxorubicin, dacarbasine) have a 47% response rate and a dacarbasine) have a 47% response rate and a 10% complete response rate 10% complete response rate ? ? Palliative, not curative, therapy Palliative, not curative, therapy Recurrent ? ? Role of chemotherapy Benjamin RS, Benjamin RS, Rouesse advanced sarcomas be treated with chemotherapy? advanced sarcomas be treated with chemotherapy? Eur Rouesse J, Bourgeois H, van J, Bourgeois H, van Hoesel Hoesel QG: Should patients with QG: Should patients with Eur J Cancer 34:958, 1998. J Cancer 34:958, 1998.

  40. Soft Tissue Sarcoma: Soft Tissue Sarcoma:Recurrent Disease Disease Recurrent ? ? Long Long- -term follow term follow- -up ? ? 9% of patients who were disease free at the 9% of patients who were disease free at the end of 5 years would have further recurrence end of 5 years would have further recurrence of the primary extremity sarcoma of the primary extremity sarcoma up Lewis JJ, Leung D, Casper ES, et al: Multifactorial analysis of Lewis JJ, Leung D, Casper ES, et al: Multifactorial analysis of long (more than 5 years) of primary extremity sarcoma. Arch Surg 134: (more than 5 years) of primary extremity sarcoma. Arch Surg 134:190, 1999 long- -term follow term follow- -up 190, 1999 up

  41. Conclusions Conclusions ? ? Sarcomas of the extremity can be Sarcomas of the extremity can be extremely variable in terms of their extremely variable in terms of their histologic make histologic make- -up, their natural up, their natural history, and their biological behavior history, and their biological behavior ? ? Surgical resection is the mainstay of Surgical resection is the mainstay of curative therapy curative therapy ? ? Multimodal treatment and salvage Multimodal treatment and salvage therapy have improved outcomes in a therapy have improved outcomes in a subset of patients subset of patients