Sarcoma
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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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Sarcoma

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Sarcoma

Sarcoma


Objectives

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.


Sarcoma1

Sarcoma

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)

Types

  • 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

    Sites

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

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


Efts prognostic factors

EFTs Prognostic Factors

Pretreatment

  • 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

Symptoms

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

  • Lump or warmth

  • Fever of unknown origin

  • Pathologic fracture

    Diagnosis

  • 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).

    Treatment

  • 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

Treatment

  • 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

Treatment-related:

  • 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


References

References

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 http://www.cancer.gov/cancertopics/pdq/treatment/kaposis/HealthProfessional

National Cancer Institute. (2012a). Adult soft tissue sarcoma treatment PDQ®. Retrieved from http://www.cancer.gov/cancertopics/pdq/treatment/adult-soft-tissue-sarcoma/HealthProfessional/page8

National Cancer Institute. (2012b). Childhood rhabdomyosarcoma treatment PDQ®. Retrieved from http://www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/HealthProfessional

National Cancer Institute. (2012c). Ewing sarcoma family of tumors PDQ®. Retrieved from http://www.cancer.gov/cancertopics/pdq/treatment/ewings/HealthProfessional

National Cancer Institute. (2012d). Osteosarcoma and malignant fibrous histiocytoma of bone treatment PDQ®. Retrieved from http://www.cancer.gov/cancertopics/pdq/treatment/osteosarcoma/HealthProfessional

National Cancer Institute. (2012e). Uterine sarcoma treatment PDQ®. Retrieved from http://www.cancer.gov/cancertopics/pdq/treatment/uterinesarcoma/HealthProfessional

National Comprehensive Cancer Network. (2012). NCCN Clinical Practice Guidelines in Oncology: Soft tissue sarcoma [v.2.2012]. Retrieved from http://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf


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