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Neck Cancers

Determining the Type of Neck Cancer

The best way to classify neck cancers is to determine if it is a cancer that started in the neck or has spread from another site.

Primary cancers of the neck include the following:

  • Salivary gland cancer: The submandibular gland is technically located in the neck. Therefore, a submandibular gland cancer can present as a neck mass just under the jawbone that doesn’t go away. Also, the tail of the parotid gland is actually located in the upper neck just behind the angle of the jawbone, so a cancer there can also show up as a neck mass. Finally, a lymph node with a salivary gland cancer (metastatic lymph node) is another way that salivary gland cancer could show up in the neck. See Salivary Gland Cancer and Metastatic Lymph Nodes to read more about those.
  • Thyroid cancer: The thyroid gland is located in the central neck, just under the voice box (larynx) and over the top of the windpipe (trachea). The thyroid gland can usually be felt in that area. Patients or doctors typically discover thyroid cancer when they feel a lump in the thyroid gland or if an imaging test for some unrelated reason reveals a lump in the thyroid gland. More rarely, a lump somewhere else in the neck (not in the thyroid gland itself) is biopsied and turns out to be a lymph node with thyroid cancer. See Advanced Thyroid Cancer to learn more.
  • Parathyroid cancers: These cancers are exceedingly rare. They are associated with very high levels of calcium and parathyroid hormone in the blood stream.
  • Sarcomas: A sarcoma is cancer in the neck that arises not in a lymph node but from some other soft tissue such as bone, cartilage, muscle or fat. Some examples include Ewing’s sarcoma, rhabdomyosarcoma, liposarcoma and fibrosarcoma. According to the World Health Organization, the classification list of sarcomas is as follows2 Fletcher CDM, Rydholm A, Singer S, Sundaram M, Coindre JM. Soft Tissue Tumours. In: Barnes EL, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization classification of tumours: pathology & genetics WHO Classification. Lyon: IARCPress; 2005.:

Types of sarcomas

  • Adipocytic tumors
    • Dedifferentiated liposarcoma
    • Myxoid/round cell liposarcoma
    • Pleomorphic liposarcoma
  • Fibroblastic/myofibroblastic tumors
    • Fibrosarcoma
    • Myxofibrosarcoma, low grade
    • Low-grade fibromyxoid sarcoma
    • Sclerosing epithelioid fibrosarcoma
  • So-called fibrohistiocytic tumors
    • Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma
  • Smooth muscle tumors
    • Leiomyosarcoma
  • Skeletal muscle tumors
    • Rhabdomyosarcoma
  • Vascular tumors
    • Epithelioid hemangioedothelioma
    • Angiosarcoma of soft tissue
  • Tumors of peripheral nerves
    • Malignant peripheral nerve sheet tumor
  • Chondro-osseous tumors
    • Extraskeletal chondrosarcoma
    • Extraskeletal osteosarcoma
  • Tumors of uncertain differentiation
    • Synovial sarcoma
    • Epithelioid sarcoma
    • Alveolar soft part sarcoma
    • Clear cell sarcoma of soft tissue
    • Extraskeletal myxoid chondrosarcoma
    • Primitive neuroectodermal tumor (PNET)
    • Desmoplastic small round cell tumor
    • Extrarenal rhabdoid tumor
    • Undifferentiated sarcoma, sarcoma NOS
    • Intimal sarcoma
  • Rhabdomyosarcoma: This is the most common soft tissue cancer of childhood (soft tissue cancers do not include blood-based cancers such as lymphoma). The most common site for rhabdomyosarcoma is the orbit, but they can be found just about anywhere in the head and neck region (and even other parts of the body). There are several subtypes of rhabdomyosarcoma, including embryonal, alveolar and pleomorphic. The main treatment for rhabdomyosarcoma is chemotherapy, but other methods such as surgery and radiation may play a role in select cases.

In addition, there are a number of tumors in between benign (non-cancerous) and malignant (cancerous) or intermediate state (either locally aggressive or rarely metastasizing). An example of this is Kaposi’s sarcoma.

  • Kaposi’s sarcoma (KS): This is a cancer linked with a virus called HHV-8. It can present anywhere in the body, including the skin, lining of the mouth or other parts of the breathing or eating tracts. There can be a single site of involvement or multiple sites. There are several subtypes, including classic, endemic, immunosuppression-associated and AIDS-associated. Treatment involves getting the immune system into better shape (such as starting anti-retrovirals for AIDS-associated KS, or chemotherapy if it is spread throughout the body).
  • Lymphomas: This is a cancer of white blood cells called lymphocytes. Lymphoma is the most common cancer of the neck in children.4 Cunningham MJ, Myers EN, Bluestone CD. Malignant tumors of the head and neck in children – a 20 year review. International Journal of Pediatric Otorhinolaryngology. 1987;(13)3:279-292. Non-Hodgkin lymphoma is a term that includes about 30 different types of lymphomas. Hodgkin lymphoma is considered a different type of lymphoma because it behaves so differently (in terms of epidemiology and survival). Surgery might be necessary to help with the diagnosis, but treatment is typically either radiation or chemotherapy.
  • Hodgkin lymphoma: This typically presents in teenagers or young adults (aged 15 to 34) or people over the age of 55. It is a cancer of lymphoid cells and is spread via adjacent lymph node regions. Staging for Hodgkin lymphoma is based on what regions of the body have cancerous lymph nodes or bone marrow (see below). The chances of cure are quite good. There are a few different subtypes of Hodgkin lymphoma based on World Health Organization (WHO) criteria:
    • Nodular lymphocyte predominance
    • Nodular sclerosing
    • Mixed cellularity
    • Lymphocyte depletion
  • Non-Hodgkin lymphoma: This describes about 30 different types of lymphomas. Treatment consists of a chemotherapy regimen. There is a form of non-Hodgkin lymphoma that can be found in the lungs, stomach and intestines and the outer lining of the eyes in lymphoid tissue that is associated with the mucosa (MALT tissue).
  • Skin cancers: Obviously, since the neck is covered with skin, skin cancers can appear there as well. This is particularly important because the neck is often exposed to sun, which is a major cause of skin cancer. See Skin Cancers to learn more.
  • Aggressive benign tumors: You should also probably be aware of a few types of benign tumors that can behave aggressively and should therefore be treated. These tumors are usually treated by surgery, but in some cases radiation or chemotherapy are warranted. For example, desmoid tumors, paragangliomas, vascular malformations and hemangiomas are all aggressive benign tumors that your doctor may treat as if they were cancerous.

Having understood those cancers, the next set of cancers are those that have spread into lymph nodes in the neck from another site. These are called metastatic lymph nodes and are the most common types of cancers in the neck.

  • Metastatic lymph nodes: Spread to neck lymph nodes from the head and neck region.
    • Cancer from the mouth, throat, nose, face or scalp
    • Cancers from the skin, thyroid or salivary glands
    • Cancers from an unknown primary source
  • Distant metastatic cancer: Cancers spread to neck lymph nodes from a region below the collarbones such as the lungs, stomach, kidney, testicles or just about anywhere else.
References

1 Gurney JG, Young JL, Roffers SD, Smith MA, Bunin GR. SEER pediatric monograph – soft tissue sarcomas. National Cancer Institute. Page 111. http://seer.cancer.gov/publications/childhood/softtissue.pdf.

2 Fletcher CDM, Rydholm A, Singer S, Sundaram M, Coindre JM. Soft Tissue Tumours. In: Barnes EL, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization classification of tumours: pathology & genetics WHO Classification. Lyon: IARCPress; 2005.

3 Zhang MQ, El-Mofty SK, Dávila RM. Detection of human papillomavirus-related squamous cell carcinoma cytologically and by in situ hybridization in fine-needle aspiration biopsies of cervical metastasis: a tool for identifying the site of an occult head and neck primary. Cancer. 2008;114(2):118-23.

4 Cunningham MJ, Myers EN, Bluestone CD. Malignant tumors of the head and neck in children – a 20 year review. International Journal of Pediatric Otorhinolaryngology. 1987;(13)3:279-292.

5 Edge SB, et al. The AJCC Cancer Staging Manual – Seventh Edition. American Joint Committee on Cancer 2010. Page 611.

6 Referenced with permission from The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Head and Neck Cancers V.2.2013. © National Comprehensive Cancer Network, Inc 2013. All rights reserved. Accessed June 20, 2013. To view the most recent and complete version of the guideline, go online to www.nccn.org. NATIONAL COMPREHENSIVE CANCER NETWORK®, NCCN®, NCCN GUIDELINES®, and all other NCCN Content are trademarks owned by the National Comprehensive Cancer Network, Inc.

7 Referenced with permission from The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.1.2013. © National Comprehensive Cancer Network, Inc 2013. All rights reserved. Accessed July 17, 2013. To view the most recent and complete version of the guideline, go online to www.nccn.org. NATIONAL COMPREHENSIVE CANCER NETWORK®, NCCN®, NCCN GUIDELINES®, and all other NCCN Content are trademarks owned by the National Comprehensive Cancer Network, Inc.

8 Ries LAG, Young JL, Keel GE, Eisner MP, Lin YD, Horner M-J (editors). SEER Survival Monograph: Cancer Survival Among Adults: U.S. SEER Program, 1988-2001, Patient and Tumor Characteristics. National Cancer Institute, SEER Program, NIH Pub. No. 07-6215, Bethesda, MD, 2007.