sarcoma overview by dr mohamed elmalki.pptx

Elobied teaching hospital-sudan

1. Sarcoma dr : Mohamed elmalki

2. Understanding Sarcoma: A Comprehensive overview Sarcomas are a heterogeneous group of cancers that arise from mesenchymal cells or mesoderm-derived elements . Including muscle , fat , nerve sheath , cartilage , blood vessels , bone and other connective tissues Clinical behavior and prognosis are largely defined by anatomic location , tumor grade , size and ability to achieve complete surgical resection. The Greek word sarkoma meaning fleshy excrescence is the origin for the term sarcoma . Modern foundations for the description and histogenic descriptions of sarcoma are attributed to the work of James Ewing who over the course of his pathology career refined the classification of sarcomas including the importance of grade in disease outcome.

3. ………………………………………………… Types of Sarcoma Bone Sarcomas Bone sarcomas are cancers that originate in the bones. The most common types are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. These typically develop in the long bones of the arms and legs, but can also occur in the pelvis, spine, and other bones. Bone sarcomas are often aggressive and can spread quickly to other parts of the body if not treated promptly. Soft Tissue Sarcomas Soft tissue sarcomas are a diverse group of cancers that develop in the soft tissues of the body, such as muscle, fat, blood vessels, and connective tissue. Common types include liposarcoma , leiomyosarcoma, and rhabdomyosarcoma. These tumors can occur anywhere in the body, but are most often found in the arms, legs, chest, and abdomen. Soft tissue sarcomas can be challenging to diagnose and treat, as they can resemble other, more common types of tumors. Gastrointestinal Stromal Tumors (GISTs) GISTs are a type of soft tissue sarcoma that originates in the gastrointestinal tract, most often in the stomach or small intestine. These tumors develop from specialized cells found in the lining of the digestive system. While GISTs can be non-cancerous, they have the potential to become malignant and spread to other parts of the body. Kaposi Sarcoma Kaposi sarcoma is a type of cancer that causes abnormal growth of the cells that line blood vessels. It typically appears as purple, red, or brown lesions on the skin, but can also occur in the mouth, throat, lungs, and other internal organs. Kaposi sarcoma is most commonly associated with HIV/AIDS, but can also occur in people with weakened immune systems from other causes.

4. Epidemiology : Soft tissue sarcoma can occur in all age groups and are equally distributed between genders . While the median age at diagnosis varies by histopathologic subtype, sarcomas are relatively rare in the adult population. However, sarcomas are among the most common cancers seen in children and young adults, representing approximately 15% of pediatric malignancies and often occurring in children under 5 years of age.6 There are nearly 75 distinct histopathologic subtypes. Further, sarcomas can occur at any anatomic site , though most arise in the extremities and trunk, and accordingly, anatomic site influences treatment and outcomes STS are named based on their tissue which it resembles . Liposarcoma—fat; fibrosaroma—fibroblast ; malignant fibrous histiocytoma—mesenchyma/ histiocytes; leiomyosarcoma—smooth muscle; rhabdomyosarcoma—skeletal muscle; chondrosarcoma— chondroblast ; Angiosarcoma — blood vessels

5. Clinical presentation : The most common presentation of a soft tissue sarcoma is that of an asymptomatic mass. Sarcomas tend to grow in a centrifugal fashion, usually pushing surrounding structures away rather than directly invading them. Encasement of structures can occur, but again, this largely occurs in the absence of direct invasion. Compression generally does not produce pain, swelling, or obstructive symptoms until the tumors become quite large. Because of surrounding anatomic structures, some tumors of the extremities tend to be detected at a relatively smaller size, whereas tumors of the retro-peritoneum are infrequently smaller than 10 cm at the time of presentation . Even very large abdominal or retroperitoneal sarcomas present with nonspecific abdominal symptoms such as fullness , early satiety, or minor abdominal discomfort. In general, the vast majority of soft tissue masses tend to be benign, but concerning features which should prompt a higher index of suspicion for malignancy include large size (>5 cm), deep location (sub fascial, intramuscular, or intra-abdominal), variations in texture on examination, immobile nature or noted fixation to underlying structures, or changes to an existing lesion (increasing size or worsening compressive symptoms). No tumor markers for sarcomas exist, so serum bloodwork is generally not useful in the evaluation of soft tissue masses.

6. E tiology : - Genetics factors . -immunological factors . - Chemicals —PVC, tetrachlorodibenzodioxin, arsenic. - Viral —HIV in Kaposi’s sarcoma, cytomegalovirus. - Ionizing radiation . - Lymphangiosarcoma in post-mastectomy lymphedema. - Osteogenic sarcoma in Paget’s disease of bone/exposure to radium Retinoblastoma associated sarcoma. Trauma . - Gorlin’s syndrome. ???

7. Diagnosis : clinical history and physical examination #Diagnostic Imaging : 1 Accurate radiologic imaging is critical in the diagnostic workup of sarcoma to provide information about the precise location and extent of the primary tumor. Computed tomography (CT) scans and magnetic resonance imaging (MRI) are the most important studies for evaluating the resectability of soft tissue sarcomas , providing definition of the primary tumor in relation to bone, muscle, neurovascular structures, and adjacent organs. Plain radiographs of bones and radionuclide bone scans rarely provide useful information regarding invasion of bone by the tumor. H owever CT and MRI can provide critical information for treatment planning . #Diagnostic Biopsy : 2 Properly performed biopsies are critical in directing a multimodality treatment approach. Image-guided techniques are increasingly being applied so that open biopsy is not mandatory . Fine-needle aspiration (FNA) is frequently used for the evaluation of enlarged lymph nodes, thyroid nodules, or breast masses. However , FNA often does not provide sufficient materials for definitive histopathologic diagnosis. FNA may be useful to demonstrate recurrent disease. -Core needle biopsy (CNB) is considered the initial procedure of choice for diagnosis of soft tissue sarcomas. CNB retrieves sufficient material for immunohistochemically staining, and when necessary, for cytogenetic analysis or flow cytometry. Image-guided CNB, either using CT or ultrasound, allows for the biopsy of deep masses that may not be easily palpable and can help target suspicious areas in a heterogeneous field for better diagnostic value . -Excision biopsy is done only if the tumor size is < 3 cm which is cutaneous or subcutaneous where in wide local re-excision is possible. Otherwise excision biopsy should be avoided as it may contaminate the tumor bed and restricts the therapeutic options . - Incision biopsy is the most reliable method of diagnosis. It provides adequate tissue sample.

8. Staging and Grading 1 Staging Because of the prognostic importance of staging, stage classification of the primary tumor is based on both clinical and histologic information. The usual TNM classification used by the AJCC45 for other solid tumors is modified to a GTNM system for soft tissue sarcomas. 2 Grading In addition to staging, sarcomas are also graded based on the appearance and behavior of the cancer cells under a microscope. Sarcoma grading is determined by factors such as the number of dividing cells, the degree of cell differentiation, and the presence of necrosis (cell death). Sarcomas are typically graded on a scale of 1 to 3, with grade 1 sarcomas being the least aggressive and grade 3 sarcomas being the most aggressive. Tumor grade, along with stage, plays a crucial role in determining the prognosis and guiding the most appropriate treatment approach for each patient. By far the most common site of metastasis is the lungs though metastases to other sites do occur. Metastases to the liver should be considered with GIST or leiomyosarcoma.

9. Principles of Surgical Resection Complete surgical resection remains the cornerstone of treatment for soft tissue sarcomas. Performing a wide excision of the tumor with negative margins is the goal of resection with curative intent. Simple enucleation, if even possible, usually results in inadequate resection and should be avoided. Technical aspects of resection must take into consideration the anatomic location and extent of disease. Often, soft tissue sarcomas are surrounded by a zone of compressed reactive tissue that forms a pseudo capsule. Care should be taken to avoid entry into the tumor and pseudo capsule during course of dissection . Enucleation commonly results in microscopically positive margins and resection can be considered. though anatomic location may preclude effective clearance of margins even with a second operation. Metallic clips placed in the tumor bed following resection can help define the limits of resection and aid in surveillance as well as the planning of future treatment. -Suction drainage catheters are routinely used to obviate postoperative seroma formation following resection of extremity or truncal sarcomas. Drains should be placed close to the incision so that the site can be included in a postoperative radiation field and to minimize the extent of proximal involvement if amputation ever becomes necessary. A unique characteristic of sarcoma is the lack of metastasis to regional lymph nodes. If regional lymphadenopathy is discovered in conjunction with a diagnosis of sarcoma, therapeutic lymphadenectomy can be considered since clearance of disease may be associated with improved outcomes. For selected histopathologic subtypes, in patients with clinically negative node examination, there may be a role for sentinel lymph node biopsy to identify occult micrometastatic disease. -Taken together, sarcomas of the upper and lower extremities and trunk make up the majority of soft tissue sarcomas. Large truncal tumors may require reconstruction with a mycutaneous flap or prosthetic materials if resultant defects cannot be closed primarily Largely , radical amputations were once the mainstay of treatment for extremity sarcoma, but modern surgical approaches involve limb-sparing procedures , which maximize functional outcomes . -Radical amputations, such as hemipelvectomy, hip disarticulation, or forequarter amputation, are now reserved for patients who are not suitable candidates for limb-sparing approaches, usually because of extent of disease, bony or joint invasion. TREATMENT :

10. Adjuvant and Neoadjuvant Therapies - The role of external beam radiotherapy is best defined by a trial comparing limb-sparing surgery alone with limb-sparing surgery with adjuvant radiation, showing improved 10-year local recurrence rates with radiation. In high-grade sarcomas, the recurrence rate was 0% versus 22%, respectively, though no significant effect on overall survival was appreciated. Certainly, in the setting of involved surgical margins which could not otherwise be resected, adjuvant radiation therapy is associated with improved local control. -there is one major disadvantage to preoperative radiation and that is its detrimental effect on wound healing. Some groups have used an intraoperative or postoperative boost dose of radiation to the resection bed if there are concerns that margins are involved.

11. Chemotherapy : Doxorubicin mg/m2/cycle Dacarbazine 800 mg/m2/cycle Ifosfamide 6 g/m2/cycle -every 2 weeks with mesna and filgrastim support following surgical resection of the primary sarcoma

12. Complications of treatment for extremity sarcoma :

13. - liposarcoma : -It is the commonest type of soft tissue sarcoma arising from the fat cells. - Common Sites 1. Thigh—commonest site. 2. Retroperitoneum. 3. Back. 4. Shoulder . -Treatment is wide excision or radiotherapy with surgical debulking in places where complete removal of tumor is not possible like in retroperitoneal liposarcoma . - Fibrosarcoma : -It can arise from the bone or from soft tissues . - It is common between 30-55 years; common in deep soft tissues of lower extremities with intact overlying skin . It is arising from fibroblasts. Intramuscular and inter-muscular fibrous tissue, fascial envelops, aponeurosis and tendons are common origin . Commonest site is thigh

14. -MALIGNANT FIBROUS HISTIOCYTOMA (MFH) : - It is group of malignant soft tissue tumors with a fibro histiocytic appearance . - MFH is one of the most common STS in adult (18–20%). - 70% occur in skeletal muscles. - Lower extremity is the common site. - It presents as solitary, multilobular lesion. - It spreads along the fascial planes or between muscle fibers which is the probable reason for local recurrence. - Myxoid/giant cell/inflammatory / angiomatoid /pleomorphic are the types

15. LEIOMYOSARCOMA : - It arises from smooth muscle. Cut section shows whorled appear ance. - common after 60 years . Two third occurs in women. - It is undetermined grade—aggressive. - It is common in retro-peritoneum and viscera, but can occur in limbs and skin. Uterus is also common site. - Recurrence is common. It has got poor prognosis. - It can occur in the piloerector muscle of skin; inferior vena cava; pulmonary artery. RHABDOMYOSARCOMA : - It arises from striated muscle. - It is common in head and neck , upper thigh and arm. - It is commonest sarcoma in children. - It can occur in retroperitoneum, pelvis and genitourinary tract. CHONDRO SARCOMA : - It arises from chondroblasts. - It attains large size with slow growing nature. - Common sites are ribs, flat bones.

16. HAEMANGIOSARCOMA : - It originates from blood vessel endothelium. LYMPHANGIOSARCOMA : - It arises from lymph vessel endothelium. - It commonly occurs after radical lymph node dissection Stewart-Treves syndrome . SYNOVIAL SARCOMA : Origin need not be from synovium. - 85–90 % occurs in lower limb; head, neck and shoulder are the next common site. - It is common in thigh, leg, shoulder, hand and foot. - Occasionally it can occur in the abdominal wall and retroperitoneum. - It occurs adjacent to joint but uncommon to involve the synovial sheath of the joint. - It spreads both through blood as well as through lymph nodes.

17. MALIGNANT PERIPHERA L NERVE SHEATH TUMOUR (MPNST ) : - It arises from peripheral nerves. - It shows differentiation along nerve elements. - MPNST replaced older terms—malignant schwannoma, neurofi brosarcoma, neurogenic sarcoma, malignant neurilemmoma. - MPNST is an exception to sarcoma will not arise from benign precursor. Common sites are major/proximal nerve trunks . KAPOSI’S SARCOMA : - It is malignant blood vessel tumor of multicentric origin arising from vascular smooth muscle or pericytes. - It is seen commonly in HIV patients due to immunosuppression. - Primary tumor commonly occurs in skin, mucous membrane, lymph nodes or viscera. It is linked with Human Herpes Virus 8 (HHV8) as causative agent. CF : Multiple reddish-blue nodules in the skin with ulceration over the nodule. - Lymph node enlargement.

18. RECURRENCE : About two-thirds of soft tissue sarcoma recurrences occur in the first 2 years after diagnosis and treatment, though recurrences may be seen at any time. Recurrences may be classified as loco-regional or distant. Though no standardized follow-up regimens exist , routine history and physical examination (clinical follow-up) and interval chest imaging are recommended for the first 2 to 3 years. Site-specific imaging guidelines are less clear. Directed cross-sectional imaging studies are helpful if location of tumor precludes early detection of recurrence on clinical evaluation alone, the choice of imaging study depends on availability. - If chest radiography is used, abnormal findings should be followed with a CT scan of the chest. Low-grade lesions infrequently metastasize in the absence of local recurrence, though can recur locally up to 20 years after original resection.

19. Clinical Trials and Research 🧪🔬 Ongoing clinical research and trials are critical for advancing the understanding and treatment of sarcoma. Sarcoma is a relatively rare type of cancer, which makes it challenging to conduct large-scale studies. However, researchers around the world are dedicated to identifying new and more effective ways to diagnose, treat, and ultimately cure these complex and diverse cancers. 100+ Ongoing clinical trials 🧑‍⚕️👨‍⚕️👩‍⚕️ $300M Annual funding for sarcoma research 💰 15 New sarcoma drugs in development 💊💉 — Advancing Sarcoma Care 🚀

20. KEY POINTS : 1- Sarcomas are a rare and heterogeneous group of cancers that arise from mesoderm derived elements such as muscle, fat, nerve/nerve sheath, cartilage, blood vessels, bone , and other connective tissue. They are histologically distinct from cancers of epithelial origin. 2- Clinical behavior and prognosis are largely defined by anatomic location, tumor grade , size, and ability to achieve complete surgical resection . 3- For extremity sarcomas, radiotherapy is indicated for high-risk tumors to decrease disease relapse and improve survival. Limb-sparing procedures are favored in order to maximize functional outcomes . 4- Surgical resection is the cornerstone of treatment for intra-abdominal or retroperitoneal sarcomas. Complete resection may require en bloc resection of adjacent or involved organs, most commonly of the kidney and colon. 5- Increasing use of multimodality therapies including surgical resection, chemotherapy, and radiotherapy has dramatically improved outcomes for sarcomas such as osteosarcoma and Ewing sarcoma.

21. -Why preoperative evaluation is done ? Increase risk of local recurrence ? Increase risk of distant metastasis ? DX for Sarcoma? Sarcomas spread to lymph nodes ?

22. THANK YOU ! Your dedication to nurturing the next generation of medical professionals is truly commendable – unit B