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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
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
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
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
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
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
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/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 bone1
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
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 rhabdomyosarcoma1
Childhood Rhabdomyosarcoma


  • Surgery
  • Radiation therapy
  • Chemotherapy: Neoadjuvant or adjuvant
  • Clinical trials
  • High-dose chemotherapy with stem cell transplant
  • Immunotherapy
  • Targeted therapy
soft tissue sarcomas
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
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
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
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