Adenoid Cystic Carcinoma (ACC)

  • Adenoid cystic carcinoma (ACC) accounts for approximately 10% of all salivary gland neoplasms:

    • This is the most common malignant disorder to arise in the submandibular, the sublingual, and the minor salivary glands.

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ACC of the hard palate
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ACC of the sublingual gland.
  • More than two thirds (65%) of them arise from the minor salivary glands:

    • Most commonly located within the oral cavity (palate), followed by the nasal cavity, and nasopharynx.

    • They were considered the most common malignant salivary gland tumor to involve the palate, but polymorphous low-grade adenocarcinoma is rapidly increasing in incidence.

  • ACC arise more often in women than in men and tend to affect adults in their fifth through seventh decades of life, often presenting as an otherwise asymptomatic mass.

  • Its natural history demonstrates a paradox:

    • First, tumor growth is slow, but its clinical course is unyielding and progressive.

    • Second, operative intervention is usually possible, but multiple local recurrences are the norm.

    • Third, metastatic spread to regional lymph nodes is rare, but distant spread to the lungs and bones is common (40% to 50% of the cases).

    • And fourth, 5-year survival rates are expectantly high, but 10 to 20-year survival rates are dismally low.

  • Tumor stage is considered the most reliable indicator of overall outcome, but some authors have questioned the importance of histologic sub-typing:

    • There is a strong positive correlation between site of origin and prognosis.

      • The more favorable outcome with major (relative to minor) salivary gland ACC is attributed to the earlier discovery of the neoplasm at these more accessible locations.

  • ACC is not encapsulated or partially encapsulated and infiltrates the surrounding tissue (the risk of local failure is approximately 50% with surgery alone).

  • Histologically they have a basaloid epithelium clustered in nests in a hyaline storm.

  • ACC can be categorized into three growth patterns cribriform, tubular, and solid patterns:

    • The most common histologic subtype is the cribriform type (44%), characterized by a “Swiss cheese” pattern of vacuolated areas:

      • The prognosis for the cribriform subtype is intermediate.

  • The tubular subtype (35%) carries the best prognosis and is characterized by cords and nests of malignant cells.

  • The solid subtype (21%) has the worst prognosis in terms of distant metastasis and long-term survival.:

    • Solid sheets of adenoid malignant cells characterize this subtype.

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Rodrigo Arrangoiz MS, MD, FACS a head and neck surgeon and is amember of Sociedad Quirúrgica S.C at the America British Cowdray Medical Center.

He is first author on some publications on oral cavity cancer:

Training:

• General surgery:

• Michigan State University:

• 2004 al 2010

• Surgical Oncology / Head and Neck Surgery / Endocrine Surgery:

• Fox Chase Cancer Center (Filadelfia):

• 2010 al 2012

• Masters in Science (Clinical research for health professionals):

• Drexel University (Filadelfia):

• 2010 al 2012

• Surgical Oncology / Head and Neck Surgery / Endocrine Surgery:

• IFHNOS / Memorial Sloan Kettering Cancer Center:

• 2014 al 2016

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