Symptoms of Adenoid Cystic Carcinoma

 Signs and Symptoms

Adenoid cystic carcinoma has three histological patterns of tumor growth: cribriform, tubular, and solid. The sieve-like growth pattern is the most common, and it appears as a "Swiss cheese" pattern on histological staining. The sieve and tubular growth patterns are less aggressive. Tumors with solid patterns are more likely to spread and have a worse prognosis.

 ACC most commonly occurs in the salivary glands or other areas within the head and neck area. Symptoms of ACC in the salivary glands may include numbness in the lower lip and / or other facial areas; nerve damage leading to weakness in certain facial muscles; persistent pain; and / or other related anomalies. The specific symptoms observed vary from patient to patient, depending on the size of the tumor and the specific salivary glands and nerves affected by the malignant tumor.

 The lacrimal glands are glands that produce tears. Symptoms of lacrimal adenoid cystic carcinoma include exophthalmos (bulging eyeballs) or vision changes. Although lacrimal ACC appears to occur primarily in adulthood, there have been some reports of the disease in children and adolescents. Some researchers have pointed out that when this form of ACC occurs in young people, its aggressiveness is slightly lower.

 ACC may also appear in certain areas of the skin (primary skin ACC). Such tumors mainly occur on the scalp and external auditory canal and can cause pain, pus and/or blood discharge and/or other symptoms. The development of single or multiple red (erythema) nodules or plaques of varying sizes usually characterizes the ACC of the skin. ACC of the skin is due to abnormal cell growth and tends to actively invade local soft tissues and bones. Other important sites where tumors develop include the arms or legs and the trunk. Although the associated symptoms may vary, the results may include pain, increased sensitivity, or the perception of pain from stimuli that are not normally associated with pain. Also, people with scalp involvement may experience hair loss in the area where the tumor grows. Cutaneous adenoid cystic carcinoma can be aggressive and may be related to nerve infiltration. In rare cases, it can cause distant metastases. Additionally, many affected individuals may experience a local recurrence months to years after surgical removal of the initial lesion.

ACC can also appear in certain organs of the upper or lower respiratory tract, breast, esophagus, cervix (female), and prostate (male). A description of these forms of ACC is provided in the following paragraphs.

Lower respiratory tract adenoid cystic carcinoma occurs most frequently in the mucous glands of the trachea, especially in the upper third. In patients with tracheal ACC, tumor growth gradually leads to obstruction of the trachea, causing difficulty or difficulty in breathing, hoarseness, and / or high-pitched breath sounds (wheezing) when inhaling. Other symptoms may include physical discomfort (malaise), weight loss, pain, repeated lung inflammation (pneumonia), and / or coughing up blood.

In lower respiratory tract ACC, tumors tend to infiltrate regional lymph nodes and may extend along nerves to bones, especially the spine (vertebrae). More rarely, the site of metastasis may include the lung, liver, brain, kidney, or other areas.

In some individuals, ACC may also appear in the mucous glands of the throat, which is located between the throat and trachea. The ACC of the larynx most often occurs in the area below the glottis, which is the slit-like opening between the vocal cords. In addition, these tumors can locally invade the vocal cords. Tumor growth in the subglottic area gradually leads to dyspnea, shortness of breath, and eventually airway obstruction during fatigue. When a malignant tumor develops above the opening, between the vocal cords, it can eventually cause persistent hoarseness, speech changes, difficulty swallowing, and a sore throat. In some people with ACC in the larynx, a lump can be seen in the neck area. Because this malignant tumor tends to infiltrate the nerves, some affected individuals may also experience related pain or discomfort. ACC in the larynx can spread through the bloodstream and nerves. Metastatic disease occurs most often in the lungs; however, other areas may include the bones or the brain.

ACC from the esophagus is extremely rare and has the same cellular structure and composition as ACC from the salivary glands and other parts of the body. Like ACC of the salivary glands, esophageal ACC is a slow-growing malignant tumor that can be prone to perineural infiltration, local recurrence, and distant metastasis. Initially, affected people may have trouble swallowing solids. As tumor growth increases, they may have trouble swallowing soft foods and liquids, and in some cases, even saliva. This usually results in reflux of food and fluids, with associated weight loss.

ACC can also appear in the breast. However, the course of the disease may be significantly different from that of primary ACC in other parts of the body. For example, breast ACC is considered to be a less aggressive tumor that is unlikely to invade regional lymph nodes, metastasize, or recur locally. Compared with other forms of breast cancer, ACC is of low grade and progresses slowly. The researchers attributed these characteristics to several possible factors, including slower overall tumor growth, relatively small tumor size, and increased ability to remove all traces of such malignant tumors through surgery. However, this malignant tumor tends to invade adjacent tissues and infiltrate nerves. In addition, although it is considered extremely rare, local recurrence and metastatic disease may occur after surgical removal of the primary tumor. The most common site of metastatic disease is the lung. Other less common areas of metastasis include lymph nodes, soft tissues, bones, brain, and kidneys. Incomplete resection of the primary tumor leads to rare cases of local recurrence and metastasis. Less than 0.1% of breast cancers are diagnosed as adenoid cystic carcinoma.

According to reports in the medical literature, only one breast is affected. To date, no cases of ACC in both breasts have been reported. Breast ACC is characterized by the overgrowth of two specific cell types (luminal and basal cells) in a specific pattern.

Unlike other forms of breast cancer, patients will develop slowly expanding movable masses that can cause tenderness or pain. Tumors tend to develop in the nipple or areola area (the round, pigmented skin area surrounding the nipple). Findings related to other malignancies in the area include bloody discharge, nipple depression, and / or tumor invasion of the breast muscles. Conditions related to adenoid cystic carcinoma do not appear to be common.

In women, ACC can also appear on the cervix, especially after menopause. Initial symptoms include watery or bloody discharge or vaginal bleeding, accompanied by a relatively large cervical mass. Cervical ACC often recurs locally, spreads to lymph nodes / vascular and peripheral spaces, and metastasizes to distant organs. ACC accounts for 0.1% of all cervical cancer cases and is very aggressive.

In men, ACC can occur in the prostate. This rare ACC is considered a subtype of prostate cancer, which is a common type of prostate cancer. Symptoms can include poor urine flow, increased frequency of urination, and / or difficulty urinating due to an enlarged prostate and related urinary tract obstruction is rare ACC can appear in other parts of the body. Specific symptoms and clinical course may vary from person to person, depending on the location, size, nature, progression, and other factors of the primary tumor.