Acinar cell carcinoma is the neoplasm of the epithelial cells, and it is called because it resembles acinar cells and due to the production of pancreatic exocrine enzymes. It’s ratio of occurrence is mostly high in the male gender, especially with above age 60. Females are less prone to it in comparison with the male, and the ratio is 2:1. Rarely found in the pediatric age but among the most common ones. Mostly they are solid and malignant. Rarely benign cystic, malignant cystic, and mixed carcinoma is found. Classification of acinar cell carcinoma are the following:
- Malignant solid neoplasm
- Benign cystic neoplasm
- Malignant cystic neoplasm
- Mixed carcinoma
- Mixed acinar–neuroendocrine carcinoma
- Mixed acinar–neuroendocrine–ductal carcinoma
- Mixed acinar, ductal carcinoma
What is Acinar cell carcinoma?
Before knowing more about acinar cell carcinoma, first, let’s understand what acinar cells are? The acinar cell found in the pancreas, responsible for producing all the essential digestive enzymes in the small intestine, plays a vital role in the digestion of essential nutrients. Acinar cell carcinoma is also known as acinic cell carcinoma (ACC). It is malignant type of salivary gland tumor of low-grade. This tumor is found in older people with an age range from 40 to 81 years old, but it is also found in the pediatric age group’s, and it is the second most common type in them. ACC is a tumor of slow growth, with pain or without pain. ACC is a rare tumor for which surgical resection increases the survival rate.
Acinar cell carcinoma Symptoms
The majority of the symptoms of acinar cell carcinoma are not specific. Usually, many patients with ACC complain about abdominal pain, weight loss, nausea, vomiting, and melena. But symptoms of weakness, anorexia, or diarrhea are also found in lesser percentages. It rarely results in bile duct obstruction and jaundice, by comparison with ductal adenocarcinoma. Some patients also show lipase hypersecretion as a clinical symptom. It is considered the first presenting sign of the tumor, in its blood serum found with the massive lipase quantities. It is characterized by subcutaneous fat necrosis in the bone, polyarthralgia, eosinophilia, and thrombotic endocarditis. Patients with acinar cell carcinoma have metastases in the early stages; lymph nodes and the liver are the most common regions of the spread. Distant metastases are also developed in some patients.
Acinar cell carcinoma Survival Rate
Patients’ survival rate with acinar cell carcinoma depends on the tumor’s size, the patient’s age, clinical features, and the follow-up method for treating cancer. Long-term survival is insufficient. Only 6 to 10% of patients live more than five years. The mean survival for patients is 18 months but can be improved to 23 months by surgical resection, in contrast to those patients who go for the bypass surgery survive only 18.5 months. Mean survival decreases up to 6 months only in the patients who do not receive any surgical treatment. The survival rate varies with the neoplasm size; patients with smaller neoplasms have more survival rates than those having more extensive or large size tumors.
Acinar cell carcinoma Causes
As there is no good record of the patients with acinar cell carcinoma existing, all information is collected from small case series and case reports. So concluded that the exact cause of ACC is unknown. But some researches help figure out that any history of radiation exposure, inhalation of wood dust, and a family history of acinar cell carcinoma is the possible cause of the carcinoma.
Acinar cell carcinoma Treatment
Complete surgical abscission is considered the treatment of choice in the early stages of ACC, no matter the size. Guidelines are not cleared for these patients who go for complete surgical resection as it is not curative in most of the patients. Still, it seems to be the only available option; abscission is only recommended on feasible conditions. However, the chances of the recurrence rate are very high. Rarely metastasis occurs; for the treatment of locally spread disease, radiotherapy is used. Go for nodal dissection, only in the case of nodal metastasis.
Chemotherapy is the option for those patients in which a tumor is unresectable, either with or without radiation. Due to ACC’s shared genetic alterations with pancreatic adenocarcinoma and colorectal carcinoma, these cancers show response to similar agents. 5-fluorouracil with neoadjuvant therapy is used most commonly with the patients of an unresectable tumor. Some other drugs that have been used as the chemotherapeutic agent are cisplatin, oxaliplatin, doxorubicin, docetaxel, capecitabine, and irinotecan. The outcome of tumor staging decides the method of treatment; usually, histologic features are not considered.