Types of Bone Cancers

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Latest Edit: Hector 2014-02-18 (EDT)

The following is a review of the different types of bone cancers.

Sarcomas

Sarcomas are malignant neoplasms arising from connective tissue (fat, bone, cartilage, smooth muscle, fibroblasts, etc), resulting in a tumor. The combined incidence in 2012 of both soft tissue sarcoma and bone/joint sarcoma is 14,170 with the combined deaths from these two is 5,310. [1].

There are exceptions included in soft tissue sarcomas such as a tumor arising in the blood vessels, called angiosarcoma, and primary nerve sheath tumors called primary neuroectodermal tumor, or Ewing's sarcoma.

The optimal conventional oncology management of a sarcoma includes complete surgical resection with or without radiotherapy. In spite of this 50% of patients with stage III Soft Tissue Sarcomas (large, deep, high-grade) develop metastasis. While the role of chemotherapy remains unclear, the most common chemotherapeutic single agents used in soft tissue sarcoma treatment are Doxorubicin, Ifosfamide, Dacarbazine, and Trabectedin. Sarcoma treatment is determined histologically, and can be used in combination with Gemcitabine, Taxanes, and Tyrosine Kinase inhibitor chemotherapy drugs. [2]


Assessment:

  • History and physical examination.
  • Lab values including CBC w Diff, liver function including ALP and LDH.

Imaging studies:

  • Plain film x-ray of affected bone and a chest x-ray (PA and lateral view).
  • CT scan of the chest, abdomen and pelvis.
  • MRI to determine tumor extent, invasion to surrounding tissue or structures.
  • Bone scan to determine lesions elsewhere from primary site.

Histology:

  • Bone marrow aspirate assessment.
  • Bone biopsy is essential to diagnosis as imaging studies do not provide histological information for staging. The information about the malignant cells on histological examination is linked to the type of treatment.

Soft Tissue Sarcomas

Soft Tissue Sarcomas are given a crazy group of names not related to biology or therapy. Often a clear histological diagnosis is difficult because of the tissue of origin (mesenchyme) has several histological elements that can appear in any given sarcoma. The categories of Soft Tissue Sarcomas and their sub-classes are listed below. [3]. The high-grade malignancies are the Osteogenic sarcomas, Rhabdomyosarcomas, Ewing's sarcomas, and Synovial sarcomas [4].

  • Skeletal Muscle Tumors: there are two types:

1. Rhabdomyosarcoma - 4 categories:

    • a. embryonal
    • b. botyroid
    • c. spindle cell
    • d. alveolar
    • e. pleomorphic


2. Rhabdomyosarcomas with ganglionic differentiation (ectomesenchymoma).


  • Liposarcoma - arising in the deep adipose tissue. There are 4 subtypes:
    • 1. Well-Differentiated
    • 2. Dedifferentiated
    • 3. Myxoid or round cell
    • 4. pleomorphic


  • Smooth Muscle Tumors - Leiomyosarcoma and epitheliod lymomyosarcoma.
  • Fibrosarcoma - arising in the connective tissue at the ends of the long bones of the arms and lower legs.

There are three classes:

      1. infantile or congenital fibrosarcoma
      1. adult form of fibrosarcoma, which more invasive and requires extensive treatment than infantile fibrosarcoma
      1. inflammatory fibrosarcoma
  • Pleomorphic Sarcoma or Malignant Fibrous Histiocytoma (MFH) - In 2002, the World Health Organization (WHO) declassified MFH as a formal diagnostic entity and renamed it as an undifferentiated pleomorphic sarcoma not otherwise specified, NOS [5] This is an soft tissue tumor that is most currently proposed to be a result of a final common process of tumor progression, and is the reason for why it is not easily classified histologically. It is very rare and most commonly occurs in the thigh, arm and retroperitoneum(the space behind the peritoneum, containing the kidneys and related structures, and is behind the stomach, pancreas, liver and spleen) of people aged 50-70 years of age.
  • Myxoid Liposarcoma - a subtype of MFH, this is less agressive and is the second most common form of MFH.
  • Neural Tumors

1.Ewing Sarcoma or Malignant Peripheral/Primary Nerve Sheath Tumor - This is a rare tumor showing up in bone marrow from early neural elements. It is causal to about 10% of bone sarcomas. These tumors are radiosensitive and respond to radiation therapy. They can be of glandular or epithelioid classification and can also be present with rhabdomyosarcoma, which is called a Malignant Triton Tumor. Ewing's Sarcoma are rare tumors arising in the bone marrow from primitive neural elements and constitute approximately 10% of bone sarcomas. The incidence is 5% of all childhood bone tumors in 2005 with a median age of 14 years, and slightly higher prevalence in males. There are subtypes of Ewing's Sarcoma: a. Classic Ewing's Sarcoma b. Primitive Neuroectodermal Tumor

They are distinguished by immunohistochemistry from other pediatric tumors by genetic expression of MIC2 translocation t(11:22)(q24;q12). [6]


2. Malignant granular cell tumor

3. Primitive Neuroectodermal Tumor (with three sub-classes):

      • a. neuroblastoma
      • b. ganglioneuroblastoma
      • c. neuroepithelioma (peripheral ectodermal tumor)


  • Sarcoma Tumors of the Blood and Lymph Nodes:
    • 1. Epithelioid Hemangioendothelioma
    • 2. Angiosarcoma and Lymphangiosarcoma
    • 3. Kaposi's Sarcoma


  • Miscellaneous Soft Tissue Sarcomas:
    • 1. Epithelioid sarcoma - affects hands and forearms followed by lower legs and upper arms. Most tumors present as firm-to-hard palpable masses, either in the deep soft tissue or in the skin.
    • 2. Alveolar Soft Part Sarcoma - a rare, symptom-less and slow-growing tumor that most often metastasizes to the brain in adults and children by the time it is detected. It originates in many different types of tissues such as bone, the mediastinum, gentialia, bladder, breast and gastrointestinal tract.
    • 3. Synovial sarcoma - this commonly arises in the joints of the arms and legs but is not from the synovial tissue in the joints. It can appear in any part of the body. It is also known as a malignant giant cell tumor of the tendon sheath.
    • 4. Clear cell sarcoma - arising from flat broad connective tissue (tendons) in the feet and hands, the skin, or in the gastrointestinal connective tissues. There is a genetic mutation of either EWSR1/ATF1 or a EWSR1/CREB1 translocation that causes clear cell sarcoma.
    • 5. Gastro-Intestinal Stromal Tumor (GIST). This is the most common soft tissue sarcoma.
    • 6. Extra-skeletal Ewing's Sarcoma
    • 7. Malignant Extrarenal Rhabdoid Tumor

The known causes of soft tissue sarcomas include:

  1. Ionizing Radation, dose-dependent fashion, with a long latency (over 10 years), causing osteosarcoma, angiosarcoma or malignant fibrous histiocytoma.
  2. Chronic Lymphedema - currently less than 1% of breast cancer patients, is known as Steward-Treves Syndrome or lymphangiosarcoma.
  3. Vinyl Chloride, Thorium Dioxide, Dioxin, Arsenic, and Androgen Exposure - Hepatic Angiosarcoma
  4. HHV-8, Cytomegalovirus and HIV-1 - Kaposi Sarcoma.
  5. Radium, post-mastectomy irradiation, Paget's disease - Osteosarcoma
  6. Post-irradiation, Paget's disease - Fibrosarcoma
  7. HIV-1 in children - Leiomyosarcoma
  8. Genetic translocations causing chromosomal abberations in DNA transcription are being defined. These are found in nearly all sarcomas.

Trauma is not causative in the development of soft tissue or bone sarcomas. [7]

Primary Malignant Bone Tumors

Actual cancer of the bone is rare; there are 400 estimated diagnoses of osteosarcoma per year in the USA [8], and a total of 2760 estimated diagnoses of primary malignant bone tumors per year in the USA. [9]

When bone cancer arises as a result of metastasis it is referred to as "(name of) cancer metastasized to the (name of) bone". For example: Breast cancer metastasized to the left second rib. This is not bone cancer as the bones are affected after a primary cancer (breast) has been diagnosed, the the bone tissue biopsy reveals the same malignant cells as was found in the breast biopsy. It is rare to have two primary cancer sites and it is highly unlikely that they would be diagnosed at the same time.

Osteosarcoma

  • Osteosarcomas are classified into 4 main classes:
  1. Conventional- intramedullary, high grade,
  1. The Conventional class has 6 sub-classes:
    a) Osteoblastic
    b) Chondroblastic
    c) Fibroblastic
    d) Mixed
    e) Small cell
    f) Other: Telangiectactic, Epithelioid, and Giant cell
  2. Intramedulary, low grade
  3. Periosteal
  4. Parosteal

Chondrosarcoma

This type of cancer is the second most common sarcoma of the bone. It can come from a benign lesion called an enchondroma, and causes destructive effects locally. It spreads most often from the pelvis and femur to the shoulder, ribs and skull.

There are two types: a. Myxoid Chondrosarcoma b. Mesenchymal Chondrosarcoma

Surgery is the only useful option and hence the standard of care for a vast majority of chondrosarcomas, with radiation of limited and chemotherapy with inactive effects.

[10]

References

  1. Siegel, R., Naishadham, D. and Jemal, A. (2012), Cancer statistics, 2012. CA: A Cancer Journal for Clinicians, 62: 10–29
  2. Frustaci et al. JCO March 1, 2001 vol. 19 no. 5 1238-1247
  3. http://sarcomahelp.org/asps.html
  4. Casciato DA. Manual of Clinical Oncology 5th ed. Lippincott Williams & Wilkins 2004. Pp 370-382
  5. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. Edited by Fletcher CDM, U. K., Mertens F., Lyon, France, IARC Press, 2002.
  6. Walshe J, Fojo T and Helman L.(2007). Sarcomas. In Boyiadzis MM Editor, Lebowitz PF Editor, Frame JN Editor, and Fojo, T. Editor (Eds.) Hematology - Oncology Therapy; (pp 437-465). New York, USA: McGraw-Hill Medical
  7. Dickson MA. Memmorial Sloan-Kettering Cancer Center Oncology Review 2013.
  8. American Cancer Society: Cancer Facts and Figures 2005 http://cancer.org
  9. Walshe J, Fojo T and Helman L.(2007). Sarcomas. In Boyiadzis MM Editor, Lebowitz PF Editor, Frame JN Editor, and Fojo, T. Editor (Eds.) Hematology - Oncology Therapy; (pp 437-465). New York, USA: McGraw-Hill Medical
  10. Walshe J, Fojo T and Helman L.(2007). Sarcomas. In Boyiadzis MM Editor, Lebowitz PF Editor, Frame JN Editor, and Fojo, T. Editor (Eds.) Hematology - Oncology Therapy; (pp 437-465). New York, USA: McGraw-Hill Medical