Rare Oncology News
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Spotlight On
Adenoid cystic carcinoma
A relatively rare tumor of the minor and major salivary glands
Prevalence
Age of Onset
ICD-10
C06.9
Inheritance
This condition does not appear to have a clear pattern of inheritance.
5 Facts you should know
FACT
It is uncommon in the parotid gland while it is much more frequent in the submandibular gland and in minor salivary and mucinous glands
FACT
ACC has been traditionally subdivided into three histological groups - cribriform, tubular, and solid
FACT
Local recurrence occurs in approximately 50% of patients
FACT
Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing
FACT
Activation of the oncogenic transcription factor gene MYB is the key genomic event of ACC and seen in the vast majority of cases
Interest over time
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Common signs & symptoms
Swelling or lump
A noticeable lump or swelling in the affected area, typically in the salivary glands, head and neck region, or other sites where ACC may occur
Difficulty swallowing
Tumors in the throat or esophagus may lead to difficulty swallowing or a sensation of something stuck in the throat
Changes in voice
Tumors near the vocal cords or larynx may cause changes in voice quality, such as hoarseness or a raspy voice
Facial weakness or numbness
Tumors near facial nerves may cause facial weakness, paralysis, or numbness on one side of the face
Current treatments
The treatment of adenoid cystic carcinoma (ACC) typically involves a multidisciplinary approach and may include one or more of the following modalities:
Surgery
Surgical resection is often the primary treatment for localized ACC. The goal is to remove the tumor along with a margin of healthy tissue to reduce the risk of recurrence. Depending on the location and extent of the tumor, surgery may involve removing part or all of the affected organ (e.g., salivary gland, trachea)
Radiation therapy
Radiation therapy may be used before or after surgery to target any remaining cancer cells or to shrink tumors that cannot be surgically removed. It may also be used as a primary treatment for inoperable tumors or to relieve symptoms in advanced cases
Chemotherapy
Chemotherapy is not typically considered effective as a primary treatment for ACC. However, it may be used in combination with radiation therapy for certain cases, such as advanced or metastatic disease, to help control tumor growth and improve symptoms
Targeted therapy
Targeted therapies, such as molecularly targeted drugs or immunotherapy, are being investigated for the treatment of ACC. These treatments target specific molecular pathways involved in cancer growth and may offer new options for patients with advanced or recurrent disease