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Sarcoma PowerPoint PPT Presentation

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Sarcoma. Objectives. The learner will be able to: Identify the different types of sarcomas. Discuss symptom management issues associated with primary treatment modalities for sarcoma management. Sarcoma.

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The learner will be able to:

  • Identify the different types of sarcomas.

  • Discuss symptom management issues associated with primary treatment modalities for sarcoma management.


Sarcoma is cancer of the bone, fat, cartilage, blood vessels, and connective or supportive tissues.

Types of Sarcoma

  • Ewing sarcoma: Of bone, extraosseous (EOE), and primitive neuroectodermal (PNET) tumors; called EFTs (Ewing family of tumors)

  • Kaposi: AIDS-related;more likely to occur in people with HIV

  • Osteosarcoma: Primary bone cancer

  • Rhabdomyosarcoma: In striated muscle

  • Soft tissue: Begins in muscle, fibrous tissue, blood vessels, fat, or supportive tissue

  • Uterine: Rare;leiomyosarcoma or endometrial stromal

Ewing Sarcoma Family of Tumors(EFTs)


  • Classic type

  • Primitive neuroectodermal (PNET)

  • Askin (PNET of the chest wall)

  • Extraosseous (EOE): Growing in tissue other than bone

    Risk factors

  • Teenage; median age is 15 years.

  • Male, Caucasian


  • Most common: Lower extremity (41%), pelvis (26%)

  • Most common EOE locations: Trunk (32%), extremity (26%)

EFTs Prognostic Factors


  • Best prognosis: Distal extremity, lower tumor volume, < age 15, female, lower serum lactate dehydrogenase (LDH) levels, absence of metastasis, other favorable morphologic characteristics

    Treatment response

  • Those with minimal or no residual tumor after presurgical chemotherapy have greater overall event-free survival.

EFTs Symptoms and Diagnosis


  • Pain/swelling (arms, legs, back, chest, pelvis)

  • Lump or warmth

  • Fever of unknown origin

  • Pathologic fracture


  • History/physical, CBC, blood chemistries

Treatment: EFTs

Localized disease

  • Surgery and/or radiation

  • Vincristine, doxorubicin, cyclophosphamide (VAdriaC or VDC), alternating with ifosfamide and etoposide (IE)

  • Clinical trials

    Metastatic disease

  • VDC/IE, radiation; overall cure rate is 20%.

  • High-dose regimens

  • Clinical trials

Kaposi Sarcoma (KS)

  • Initially rare

  • 1981: First reported as part of AIDS epidemic

  • 95% of all cases of epidemic KS in the United States are diagnosed in homosexual or bisexual men.

  • Lesions involve skin, mucosa, lymph nodes, and visceral organs (GI, lung, liver, spleen).


  • Localized lesions treated by electrodessication or surgery/local radiation therapy

  • Depressed immunologic status limits use of chemotherapy.

  • Interferon-alphas show promising response rates.

  • Clinical trials in progress

Osteosarcoma/Malignant Fibrous Histiocytoma (MFH) of Bone

Osteosarcoma: About 5% of childhood tumors

Risk factors: Adolescent/young adult

Site is significant prognostic indicator:

  • Extremities: Distal sites more favorable

  • > 50% arise from the long bones around the knee.

  • Axial skeleton sites have poorer outcomes.

  • Pelvic: Survival rates are 20%47% with complete resection.

  • Craniofacial/head/neck: Mandibular tumors have better prognosis than extragnathic (skull) tumors.

Osteosarcoma/Malignant Fibrous Histiocytoma (MFH) of Bone

Staging: Now uses American Joint Committee on Cancer (AJCC) staging (TNM IIV)

Localized tumor treatment:

  • Surgery: > 80% can be limb-sparing procedures.

  • Chemotherapy: Combination regimens/clinical trials

    Metastatic disease treatment:

  • Lung metastasis only: Resection of lesions if possible, after neoadjuvant chemotherapy

  • Bone only or with lung metastasis: Neoadjuvant chemotherapy, followed by surgical resection of primary tumor, resection of lung lesions, and postoperative chemotherapy

  • Clinical trials

Childhood Rhabdomyosarcoma

  • Embryonal: Head/neck, genital/urinary (most common)

  • Alveolar: Arms/legs, abdomen, genitals, anal area (typically occur in teen years)

  • Anaplastic: Rare in children

  • Genetic risk factors exist.

  • Symptoms: Lump/swelling (bulging eye, headache, difficulty urinating or defecating, blood in urine, bleeding)

Childhood Rhabdomyosarcoma


  • Surgery

  • Radiation therapy

  • Chemotherapy: Neoadjuvant or adjuvant

  • Clinical trials

  • High-dose chemotherapy with stem cell transplant

  • Immunotherapy

  • Targeted therapy

Soft Tissue Sarcomas

  • Can occur in adults and children

  • Adult risk factors associated with gene mutations, prior radiation therapy, exposure to some chemicals

  • Often asymptomatic at early stages, or painless lump

  • Multiple cellular classification types

  • Graded by TNM system; stages IIV define prognostic groups.

Soft Tissue Sarcomas: Treatment

  • Stage I: Resection, possible pre- or post-radiation (PORT); clinical trials

  • Stage II/node negative stage III: Resection with pre-XRT or PORT; resection alone; high-dose radiation if unresectable. Some cases may include presurgery chemotherapy; clinical trials.

  • Advanced stage III (N1): Resection + lymphadenectomy + PORT; adjuvant chemotherapy; clinical trials

  • Stage IV: Possible resection of lung metastasis; chemotherapy (doxorubicin; ifosfamide); clinical trials

Uterine Sarcoma

  • Rare: Uterine muscles or fibrous tissue

  • Risk factors: Prior radiation therapy; tamoxifen

  • Stage I: Hysterectomy, bilateral salpingo-oophorectomy, lymphadenectomy only; surgery + radiation therapy; or surgery + chemotherapy

  • Stage II: Surgery alone, with or without radiation; with or without chemotherapy

  • Stage III: Clinical trial surgery + radiation; or clinical trial surgery + chemotherapy

  • Stage IV: Clinical trial chemotherapy

Symptom Management: Sarcomas

Disease-related: QOL changes, pain, distress, limited physical functioning


  • Postoperative: Pulmonary management, pain management

  • Radiation: Depending on location: mucositis, esophagitis, skin changes, fatigue

  • Chemotherapy: Drug and dose dependent: nausea, vomiting, diarrhea, alopecia, immunosuppression/infection risk, fatigue


Granda-Cameron, C., Hanlon, A.L., Lynch, M.P., & Houldin, A. (2011). Experience of newly diagnosed patients with sarcoma receiving chemotherapy. Oncology Nursing Forum, 38, 160169.

National Cancer Institute. (2012). Kaposi sarcoma treatment PDQ®. Retrieved from

National Cancer Institute. (2012a). Adult soft tissue sarcoma treatment PDQ®. Retrieved from

National Cancer Institute. (2012b). Childhood rhabdomyosarcoma treatment PDQ®. Retrieved from

National Cancer Institute. (2012c). Ewing sarcoma family of tumors PDQ®. Retrieved from

National Cancer Institute. (2012d). Osteosarcoma and malignant fibrous histiocytoma of bone treatment PDQ®. Retrieved from

National Cancer Institute. (2012e). Uterine sarcoma treatment PDQ®. Retrieved from

National Comprehensive Cancer Network. (2012). NCCN Clinical Practice Guidelines in Oncology: Soft tissue sarcoma [v.2.2012]. Retrieved from

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