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< Back Dr.. Serdar DEMIROZ Soft Tissue Sarcomas A diverse group of malignant tumours arising from mesenchymal tissues, commonly affecting extremities. Definition Soft tissue sarcomas (STS) are malignant tumors of mesenchymal origin , representing about 1% of all malignancies . They may arise in any anatomical site, most commonly the extremities and trunk . The clinical presentation is often insidious, with slow-growing, painless masses that delay diagnosis. Epidemiology Annual incidence: ~13,000 new cases in the U.S. Most occur in adults , with a slight male predominance. Deep-seated lesions are typical; superficial ones are less common. Etiology remains largely unknown, though some are linked to radiation, chronic lymphedema, or genetic syndromes (e.g., Li-Fraumeni, NF1). Clinical Features Painless, enlarging mass is the most frequent symptom. May reach substantial size before detection, especially in pelvic or retroperitoneal sites. Usually does not invade bone or major neurovascular structures . Imaging & Diagnosis, MRI : preferred for local staging, defining tumor margins, fascial plane involvement, and relation to vessels or bone. CT chest : screening for pulmonary metastases. Biopsy : core needle biopsy is the gold standard; excisional biopsy should only be done by the definitive surgical team. Molecular profiling increasingly aids subtype classification (>70 distinct subtypes). Management Multidisciplinary approach essential (orthopaedic oncology, pathology, radiology, medical oncology, radiotherapy). Wide surgical excision with negative margins is the cornerstone. Radiotherapy : indicated for large, deep, or high-grade tumors—either pre- or postoperatively. Chemotherapy : limited role; used selectively in high-risk or metastatic disease. Metastasis & Follow-Up Predominantly hematogenous spread to the lungs . Bone and liver metastases less frequent. Lymphatic spread rare but occurs in subtypes such as synovial sarcoma and epithelioid sarcoma . Postoperative surveillance: Clinical exam + chest imaging every 3–6 months (first 2 years), then annually. Prognosis Local control : ~85–90% with adequate margins. Local recurrence : 10–15%. Poor prognostic factors: Large size > 5 cm Deep location High histologic grade Positive margins Lymphovascular invasion Histologic Subtypes of Soft Tissue Sarcomas 1. Undifferentiated Pleomorphic Sarcoma (UPS) Previously Malignant Fibrous Histiocytoma (MFH) . High-grade pleomorphic tumor without identifiable line of differentiation. Common sites: thigh, buttock, shoulder girdle, retroperitoneum . Aggressive local behaviour; lung metastases common. Treatment: wide excision ± radiotherapy; chemotherapy in selected cases. 2. Malignant Peripheral Nerve Sheath Tumor (MPNST) Originates from peripheral nerves or pre-existing neurofibromas . Strongly associated with Neurofibromatosis type 1 (NF1) . Rapidly enlarging deep-seated mass along nerve pathways. S-100 and SOX10 positive in subset. Prognosis: poor; 5-year survival <50%. 3. Synovial Sarcoma Typically affects young adults (15–40 years) , often around large joints (especially knee, ankle). Despite name, does not arise from synovium . t(X;18)(p11;q11) translocation → SYT-SSX fusion. Histology: biphasic (epithelial + spindle cell) or monophasic. Treatment: surgery + radiotherapy ± chemo (if high-grade or metastatic). 4. Liposarcoma Most common soft tissue sarcoma in adults. Occurs in thigh and retroperitoneum . Subtypes: Well-differentiated (low grade, local recurrence) Myxoid/round cell (t(12;16), intermediate grade) Pleomorphic (high grade, aggressive) Treatment: wide excision; radiotherapy for high-grade disease. 5. Rhabdomyosarcoma Skeletal muscle–derived tumor; most common STS in children. Subtypes: embryonal, alveolar, pleomorphic. Markers: desmin, myogenin, MyoD1. Treatment: multimodal — surgery + radiotherapy + chemotherapy. 6. Fibrosarcoma Malignant spindle-cell tumor producing collagen . Often arises in deep soft tissues of extremities or trunk . Histology: herringbone pattern. Management: wide excision ± radiotherapy. Recurrence common; metastasis via hematogenous route. 7. Leiomyosarcoma Originates from smooth muscle cells (vessels, uterus, GI tract, soft tissue). Common in retroperitoneum and large veins . Markers: SMA, desmin, h-caldesmon positive. Prognosis: size and grade dependent. 8. Epithelioid Sarcoma Occurs in young adults , often distal upper extremities (hand, forearm). Mimics granulomatous or epithelial lesions. Loss of INI1 (SMARCB1) expression diagnostic. Tendency for local recurrence and lymphatic spread. 9. Angiosarcoma Malignant endothelial tumor. May arise spontaneously or post-radiation . Common sites: skin (scalp/face of elderly), breast, liver . CD31, CD34, ERG positive. Highly aggressive, poor prognosis. 10. Dermatofibrosarcoma Protuberans (DFSP) Low-grade, locally aggressive tumor of dermal fibroblastic origin . t(17;22) → COL1A1–PDGFB fusion. Slow-growing plaque or nodule on trunk or proximal extremities . Treatment: wide local excision or Mohs surgery . Recurrence common, metastasis rare. References – Soft Tissue Sarcomas WHO Classification of Soft Tissue and Bone Tumours, 5th Edition. IARC: Lyon, 2020. Casali PG, Abecassis N, Bauer S, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines. Ann Oncol. 2022;33(12):1348–1365. NCCN Clinical Practice Guidelines in Oncology: Soft Tissue Sarcoma, Version 2.2025. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. IARC, Lyon, 2013. Burningham Z, Hashibe M, Spector L, Schiffman JD. The epidemiology of sarcoma. Clin Sarcoma Res. 2012;2(1):14. Gronchi A, Miah AB, Dei Tos AP, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN–GENTURIS guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021;32(11):1348–1365. 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. J Clin Oncol. 1998;16(1):197–203. Thway K, Jones RL, Noujaim J, Fisher C. Updates in the histopathology and classification of soft tissue sarcomas. Histopathology. 2014;64(1):51–70. Stacchiotti S, Van Tine BA. Synovial sarcoma: current concepts and future perspectives. J Clin Oncol. 2018;36(2):180–187. References – Undifferentiated Pleomorphic Sarcoma & Histologic Subtypes Coindre JM. Histologic classification of soft tissue sarcomas (update and perspectives). Histopathology. 2014;64(1):51–70. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO Classification of Tumours of Soft Tissue and Bone, 5th Edition (2020). Coindre JM, Terrier P, Guillou L, et al. Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas. Cancer. 2001;91(10):1914–1926. Chibon F. The genetics of soft tissue sarcoma: from normal mesenchymal cells to malignant sarcomas. Nat Rev Cancer. 2013;13(8):545–558. Jo VY, Fletcher CDM. WHO classification updates on soft tissue and bone tumours. Histopathology. 2014;64(1):38–49. Widemann BC, Italiano A. Biology and management of malignant peripheral nerve sheath tumor. Neuro Oncol. 2018;20(6):763–773. Helman LJ, Meltzer P. Mechanisms of sarcoma development. Nat Rev Cancer. 2003;3(9):685–694. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 7th ed. Philadelphia: Elsevier, 2020. Thway K, Fisher C. Leiomyosarcoma: recent advances and diagnostic approach. Histopathology. 2015;67(5):701–711. Sirvent N, Maire G, Pedeutour F. Chromosome translocations in dermatofibrosarcoma protuberans. Hum Pathol. 2003;34(12):1293–1301. Rhabdomyosarcoma pathology Previous Next

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< Back Dr. Alper DUNKI Chondroblastoma Chondroblastoma is a rare, epiphyseal, benign bone tumor that exhibits locally aggressive behavior. It primarily affects skeletally immature individuals, most commonly males in their second decade of life. Most frequent locations include the distal femur, proximal tibia, proximal humerus, and less commonly the hip or calcaneus. Clinical Presentation Patients typically present with: Persistent joint-related pain Restricted range of motion Swelling or localized tenderness Due to its proximity to the joint, the symptoms often mimic inflammatory or mechanical arthropathy. Imaging Features Chondroblastomas typically present as well-circumscribed, lobulated lytic lesions located in the epiphysis or apophysis of long bones, most often around the knee or proximal humerus. On radiographs, they demonstrate geographic bone destruction with a thin sclerotic margin and may contain subtle internal calcifications reflecting a chondroid matrix. CT better delineates these calcifications and cortical integrity. On MRI, chondroblastomas usually appear heterogeneous, with intermediate signal intensity on T1-weighted and variable high signal on T2-weighted or fat-suppressed sequences, often surrounded by bone marrow and soft-tissue edema. A thin hypointense rim corresponding to reactive sclerosis is frequently seen. Post-contrast images show heterogeneous enhancement of the solid components and reactive tissues. Joint effusion or mild synovitis is common due to the subarticular location. Overall, the imaging appearance of chondroblastoma reflects a benign but locally active epiphyseal lesion in skeletally immature patients. X-Ray: Well-defined lytic lesion within the epiphysis; may show stippled or punctate calcification within the matrix. MRI: Demonstrates surrounding bone marrow and soft tissue edema. Lesion appears hypointense on T1 and heterogeneously hyperintense on T2 sequences. CT Scan: Can better define the mineralized matrix and thin sclerotic rim. Bone Scan: Typically shows increased uptake due to hypermetabolic activity. Histopathology Composed of round to polygonal chondroblasts with occasional multinucleated giant cells . The hallmark finding is “chicken wire” calcification , which refers to thin pericellular calcification encircling individual tumor cells. A "cobblestone " or lobulated architectural pattern may be observed. Differential Diagnosis Giant Cell Tumor (GCT): Usually affects skeletally mature individuals; tends to lack the calcified matrix. Clear Cell Chondrosarcoma: Typically occurs in the femoral head; may mimic chondroblastoma radiologically but differs in age group and clinical behavior. Chondromyxoid Fibroma (CMF): May resemble CB histologically but is more often metaphyseal and lacks typical pericellular calcification. Treatment and Prognosis The primary treatment is intralesional curettage . The resulting cavity may be filled with bone graft or bone cement . Radiofrequency ablation (RFA) has been explored in selected cases. The recurrence rate varies between 5% and 20%, depending on surgical technique and completeness of removal. Care must be taken to avoid damage to the physis and articular cartilage , particularly in younger patients. WHO Classification According to the 2020 WHO Classification of Bone Tumors, chondroblastoma is categorized as a benign chondrogenic tumor (ICD-O: 9230/0). References Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (eds). WHO Classification of Soft Tissue and Bone Tumours, 5th Edition. Lyon: IARC Press; 2020. Oliveira AM, Hsi BL, Weremowicz S, Rosenberg AE, Dal Cin P, Joseph N, et al. Aneurysmal bone cysts are clonal and characterized by specific chromosomal rearrangements. Am J Pathol. 2004;164(5):1639–1646. Lucas DR, Bridge JA. Chondroblastoma. In: Bone and Soft Tissue Pathology , 2nd ed. Elsevier; 2019. p. 275–283. Xu H, Nugent D, Monforte HL, Binitie O, Ahmed S, Letson GD, et al. Chondroblastoma of bone in the extremities: a clinical review of 177 cases. J Bone Joint Surg Am. 2015;97(11):925–931. Amanatullah DF, Clark TR, Lopez MJ, Borys D, Tamurian RM. Chondroblastoma of bone in the pediatric population. J Pediatr Orthop. 2014;34(4):421–425. Wu RT, O’Donnell RJ, Horvai AE. Histologic spectrum and molecular pathogenesis of chondroblastoma and related tumors. Arch Pathol Lab Med. 2020;144(1):26–35. Yoshida A, Ushiku T, Motoi T, et al. Recurrent IDH2 mutations in chondroblastoma and chondromyxoid fibroma: differential diagnostic and therapeutic implications. Mod Pathol. 2022;35(6):775–784. Bohman SL, et al. Treatment of chondroblastoma with extended curettage and adjuvants: long-term local control and functional outcomes. Clin Orthop Relat Res. 2019;477(5):1074–1082. Axial and coronal fat-suppressed proton-density MRI images of the distal femur demonstrate a well-defined lobulated lesion in the epiphyseal region, showing heterogeneous high signal with a thin hypointense rim and surrounding bone marrow and soft-tissue edema. The lesion abuts the subchondral bone without cortical destruction. Findings are consistent with a chondroblastoma in a skeletally immature patient. Previous Next

< Back Dr. Fevzi SAGLAM Limb Salvage vs Amputation Limb salvage surgery has replaced amputation as the preferred approach for most malignant bone and soft tissue tumors, provided that oncologic safety can be maintained. Advances in imaging, chemotherapy, and reconstructive techniques have enabled wide resection with functional preservation in appropriately selected patients. Absolute indications for limb salvage include the ability to achieve negative margins without compromising major neurovascular structures, whereas amputation remains essential for cases with extensive involvement, infection, or unresectable disease. Long-term survival is comparable between limb salvage and amputation when clear margins are achieved, but limb salvage offers superior functional and cosmetic outcomes at the cost of higher complication rates. Future developments such as 3D-printed implants and biologic reconstructions are expected to further improve results, yet oncologic safety must always remain the primary goal. Limb Salvage and Amputation Orthopedic oncology is a multidisciplinary specialty concerned with the diagnosis and management of bone and soft tissue tumors. A pivotal decision in this field is whether the affected limb can be preserved or requires amputation. Historically, amputation was the standard treatment for malignant bone tumors. However, advances in imaging modalities, chemotherapy, radiotherapy, and surgical techniques have allowed limb salvage surgery to become a safe and effective alternative in many cases. The primary objective is to achieve oncologically safe resection margins while preserving limb function and appearance. Development and Indications of Limb Salvage Surgery Limb salvage surgery involves complete tumor excision with negative margins, followed by reconstruction of the resulting defect. The success of this procedure depends on tumor location, proximity to neurovascular structures, and patient-specific factors such as overall health and rehabilitation potential. Absolute Indications: • Tumor can be resected with safe margins without compromising major vessels or nerves. • Tumor reduction after neoadjuvant chemotherapy allows safe surgical margins. • Localized disease with no distant metastasis. Relative Indications: • Limited soft tissue involvement. • Absence of active infection or impaired wound healing. • Patient’s physical and psychological capacity for rehabilitation. Reconstruction techniques include modular tumor prostheses, autografts or allografts, rotationplasty, and vascularized fibular grafts. These strategies aim to restore limb length and maximize post-operative function. Indications and Role of Amputation Despite the advantages of limb salvage, amputation remains necessary in selected patients to ensure oncologic safety and optimal quality of life. Absolute indications include encasement of major neurovascular structures, uncontrolled infection, extensive soft tissue necrosis, or inability to achieve negative margins. Absolute Indications: • Tumor involving major arteries or nerves. • Widespread infection or chronic osteomyelitis. • Recurrent tumors where reconstruction is not feasible. • Inability to achieve oncologically safe margins. Relative Indications: • Patients with comorbidities precluding long or complex surgery. • Technical limitations preventing reconstruction. • Patient preference or anticipated noncompliance with rehabilitation. Modern prosthetic technology has significantly improved post-amputation functional outcomes and mobility. Oncologic and Functional Outcome Comparison Several studies have demonstrated no significant difference in long-term survival between limb salvage and amputation, provided negative surgical margins are achieved. Local recurrence risk remains primarily dependent on margin status. Functionally, limb salvage generally yields superior outcomes. Functional scores such as the Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score (TESS) are typically higher in limb salvage patients (%70–80) compared to amputees (%50–60). However, limb salvage procedures are associated with higher rates of early complications including infection, prosthesis loosening, and mechanical failures. Amputation, conversely, presents fewer surgical complications but poses greater psychosocial adaptation challenges. Complications and Rehabilitation The most common complications after limb salvage surgery include wound healing problems, deep infections, implant loosening, and fractures. In pediatric patients, expandable prostheses are often employed to accommodate ongoing growth. After amputation, patients frequently encounter phantom limb pain, skin irritation, and challenges with prosthesis fitting. Rehabilitation requires a multidisciplinary approach involving physiotherapy, psychological support, and prosthetic training to optimize functional independence. Conclusion and Future Perspectives Limb salvage surgery in orthopedic oncology offers superior functional and aesthetic outcomes in appropriately selected patients. Individual patient assessment remains critical. Future advances, including 3D printing, biologic reconstruction, and improved understanding of chemoresistance mechanisms, are expected to enhance limb salvage success. Nonetheless, the fundamental principle remains unchanged: oncologic safety must always take precedence over functional preservation. References: 1. Simon MA, Aschliman MA, Thomas N, Mankin HJ. Limb-salvage treatment versus amputation for osteosarcoma of the distal end of the femur. J Bone Joint Surg Am. 1986;68(9):1331–1337. 2. Gonzalez, M. R., Mendez-Guerra, C., Goh, M. H., & Pretell-Mazzini, J. (2025). Principles of Surgical Treatment of Soft Tissue Sarcomas. Cancers , 17 (3), 401. 3. Grimer, R. J., Taminiau, A. M., & Cannon, S. R. (2002). Surgical outcomes in osteosarcoma. The Journal of Bone & Joint Surgery British Volume , 84 (3), 395-400. 4. Aksnes LH, Bauer HC, Jebsen NL, et al. Limb-sparing surgery preserves more function than amputation: a Scandinavian Sarcoma Group study of 118 patients. J Bone Joint Surg Br. 2008;90(6):786–794. 5. Chandrasekar CR, Grimer RJ, Carter SR, et al. Modular endoprosthetic replacement for tumours of the proximal femur. J Bone Joint Surg Br. 2009;91(1):108–112. 6. Malawer, M. M., & Sugarbaker, P. H. (Eds.). (2006). Musculoskeletal cancer surgery: treatment of sarcomas and allied diseases . Springer Science & Business Media. 7. Davis AM, Bell RS, Badley EM, et al. Evaluating functional outcome in patients with lower extremity sarcoma. Clin Orthop Relat Res. 1999;(358):90–100. 8. Cirstoiu, C., Cretu, B., Serban, B., Panti, Z., & Nica, M. (2019). Current review of surgical management options for extremity bone sarcomas. EFORT open reviews , 4 (5), 174-182. Previous Next

< Back Supracondylar (Peds) Supracondylar fractures are the most common elbow fractures in children and require careful assessment due to the risk of neurovascular compromise. These injuries typically occur from a fall on an outstretched hand. Gartland classification (Type I–III) guides management. Type I fractures are treated with immobilization, while Type II and III generally require closed reduction and percutaneous pinning. Complications include brachial artery injury, median nerve or anterior interosseous nerve injury, and cubitus varus (gunstock deformity). Prompt assessment and treatment are critical to avoid long-term dysfunction. supracondylar-fractures-peds Previous Next

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