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acinar cell carcinoma
Description
What is Acinar Cell Carcinoma?
Acinar cell carcinoma, also known as acinic cell carcinoma or Fechner's tumor, is a rare and malignant exocrine neoplasm that can occur in various parts of the body, including the pancreas, salivary glands, and lungs.
Characteristics of Acinar Cell Carcinoma
- Pancreatic Acinar Cell Carcinoma: This type accounts for only about 1% of all pancreatic cancers. It is a rare tumor that can manifest as an exophytic, oval or round, well-marginated, and hypovascular mass on CT and MRI scans [7].
- Salivary Gland Acinar Cell Carcinoma: This type is the most common form of acinar cell carcinoma, accounting for about 5% of all salivary gland tumors. It typically affects older men and can manifest as a serous acinar differentiation with zymogen granules [4].
- Lung Acinar Cell Carcinoma: This type is an uncommon primary lung tumor that can manifest centrally or peripherally. Most descriptions of these tumors have been isolated case reports [2].
Clinical Features
Acinar cell carcinoma is often characterized by its large size, with an average diameter of 8-10 cm. The cut surface typically appears homogeneous and may be partially encapsulated [9]. This tumor type can manifest as a well-circumscribed mass on imaging studies.
Prognosis and Treatment
The prognosis for acinar cell carcinoma is generally poor due to its rarity and the lack of effective treatment options. However, surgical resection remains the primary treatment approach for this disease.
References:
[1] Acinar cell carcinoma is a malignant exocrine neoplasm that can occur in various parts of the body (Context 1). [2] Most descriptions of lung acinar cell carcinomas have been isolated case reports (Context 2). [3] Pancreatic acinar cell carcinoma accounts for only about 1% of all pancreatic cancers (Context 6). [4] Salivary gland acinar cell carcinoma is the most common form of this disease, affecting older men (Context 4). [5] Acinar cell carcinoma can manifest as a well-circumscribed mass on imaging studies (Context 9).
Additional Characteristics
- Acinar cell carcinoma
- Pancreatic acinar cell carcinoma
- Salivary Gland Acinar Cell Carcinoma
- Lung Acinar Cell Carcinoma
Signs and Symptoms
Common Signs and Symptoms
Acinar cell carcinoma, a rare type of salivary gland cancer, typically presents with non-specific symptoms. These may include:
- Abdominal pain [1]
- Weight loss [2][6]
- Nausea and vomiting [2][8]
- Diarrhea [1]
- Jaundice is often not present [5][9]
In some cases, the cancer may metastasize to regional lymph nodes, which can lead to additional symptoms.
Additional Symptoms
Depending on its location, acinar cell carcinoma may also cause:
- Swelling or pain in the cheek area [12]
- Difficulty chewing or swallowing food [12]
- Subcutaneous fat necrosis (in approximately 15% of cases) [4]
It's essential to note that these symptoms are often non-specific and can be related to other conditions. A proper diagnosis by a medical professional is necessary for an accurate assessment.
References
[1] It usually occurs during adulthood. Signs and symptoms include abdominal pain, weight loss, nausea, and diarrhea. It may metastasize to regional lymph nodes and ...
[2] by LF Calimano-Ramirez · 2022 · Cited by 29 — Most ACC s exhibit vague symptoms, which include weight loss (45%), abdominal pain (60%), back pain (50%), nausea and vomiting (20%), melena (12%), weakness, ...
[3] Jan 25, 2022 — Most acinar cell carcinomas present with non-specific symptoms which are related to the mass effect of the neoplasm but sometimes, they may be ...
[4] Most patients with acinar cell carcinomas present with nonspecific symptoms; however, approximately 15% develop subcutaneous fat necrosis, an erythema nodosum– ...
[5] by AD Toll · 2013 · Cited by 35 — The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the ...
[6] It usually occurs during adulthood. Signs and symptoms include abdominal pain, weight loss, nausea, and diarrhea. It may metastasize to regional lymph nodes and ...
[7] Oct 8, 2019 — Symptoms of Acinar Cell Carcinoma: It has a high chance of metastasis. This means that it can spread to other parts of the body very quickly.
[8] Presenting symptoms are typically non-specific and include abdominal pain, weight loss, vomiting, nausea, and/or, less commonly, jaundice. Immunohistochemical ...
[9] by AD Toll · 2013 · Cited by 35 — The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of ...
[10] The most common type of parotid gland cancer is called mucoepidermoid, followed by adenoid cystic. Acinic cell carcinoma, meanwhile, is a rare form of pancreatic cancer.
[11] What are the symptoms of acinic cell carcinoma? Most acinic cell carcinomas are slow-growing and painless. Depending on its location, it may be seen as a lump under the skin or on the inside surface of the oral cavity.
[12] The outlook for acinic cell carcinoma is generally positive but it can depend on several factors. Symptoms of acinic cell carcinoma include swelling or pain in the cheek area, and difficulty chewing or swallowing food.
Additional Symptoms
- Nausea and vomiting
- Swelling or pain in the cheek area
- Difficulty chewing or swallowing food
- weight loss
- jaundice
- diarrhea
- abdominal pain
Diagnostic Tests
Diagnostic Tests for Acinar Cell Carcinoma
Acinar cell carcinoma (ACC) is a rare type of pancreatic cancer, and its diagnosis can be challenging due to its similarity in presentation with other pancreatic tumors. However, various diagnostic tests can help confirm the presence of ACC.
- Imaging Studies: Computed Tomography (CT) scans and Magnetic Resonance Imaging (MRI) are commonly used imaging modalities for diagnosing ACC [8][9]. These studies can help identify the location and size of the tumor.
- Fine Needle Aspiration (FNA): FNA is a minimally invasive procedure that involves inserting a thin needle into the pancreas to collect cell samples. The collected cells are then examined under a microscope for abnormal cell growth [3].
- Immunostaining: Immunostaining is essential for definitive diagnosis of ACC. This test helps identify specific proteins in the tumor cells, which can confirm the presence of ACC [4].
- Serum Lipase Levels: Elevated serum lipase levels are observed in 24-58% of patients with ACC [2]. However, this test alone cannot confirm the diagnosis.
- Biopsy and Surgical Resection: A biopsy or surgical resection is considered the gold standard for diagnosing ACC. These procedures allow for direct examination of tumor tissue under a microscope [9][10].
Additional Diagnostic Tests
Other diagnostic tests that may be used to support the diagnosis of ACC include:
- PAS Stain: In well-granulated cases, a positive PAS stain can help support a diagnosis of ACC, provided that the presence of cytoplasmic zymogen granules is confirmed [7].
- Immunohistochemical Labelling: Immunohistochemical labelling for pancreatic enzyme production can be helpful in confirming the diagnosis of ACC. Antibodies against trypsin, chymotrypsin, lipase, and elastase are available for this purpose [10].
It's essential to note that a combination of these diagnostic tests may be necessary to confirm the presence of acinar cell carcinoma.
References: [2] - Context reference 2 [3] - Context reference 3 [4] - Context reference 4 [7] - Context reference 7 [8] - Context reference 8 [9] - Context reference 9 [10] - Context reference 10
Additional Diagnostic Tests
- Imaging Studies
- Fine Needle Aspiration (FNA)
- Immunostaining
- Serum Lipase Levels
- Biopsy and Surgical Resection
- PAS Stain
- Immunohistochemical Labelling
Treatment
Chemotherapy Options for Acinar Cell Carcinoma
Acinar cell carcinoma (ACC) is a rare type of pancreatic cancer that requires effective treatment strategies. While there are no clear guidelines, various chemotherapy options have been explored to manage this condition.
- Fluoropyrimidine-based combination chemotherapy: Studies have shown that fluoropyrimidine-based regimens can improve disease control rates in patients with ACC [7][24]. This type of chemotherapy is often used for patients with good fitness status.
- FOLFIRINOX regimen: For patients with metastasis, a fluorouracil-based regimen such as FOLFIRINOX may be preferred. Maintenance treatment with poly ADP-ribose polymerase (PARP) inhibitors has also been explored [8].
- Carboplatin and Paclitaxel: This combination chemotherapy has been investigated in clinical trials for patients with recurrent or metastatic pancreatic cancer, including ACC [9].
Other Treatment Options
In addition to chemotherapy, other treatment options are being researched:
- Neoadjuvant or adjuvant chemoradiotherapy: The role of these treatments remains unproven, but they may be considered in certain cases.
- Targeted therapy: Given the shared genetic alterations between ACC and pancreatic adenocarcinomas, targeted therapies that have activity against these cancers may also be effective [10].
Current Research
Researchers are actively exploring new therapeutic agents for ACC. A clinical trial led by Christine Campo Alewine is investigating a potential therapy for this rare type of pancreatic tumor [4]. Another study has found that the chemotherapy drug oxaliplatin can stop the growth of ACC [6].
It's essential to note that treatment decisions should be made on an individual basis, taking into account the patient's specific situation and medical history. Further research is needed to establish clear guidelines for treating acinar cell carcinoma.
References:
[7] F Zhao · 2023 · Cited by 1 [8] JY Xu · 2022 · Cited by 15 [9] NCT01280058 [10] Acinar cell carcinomas are chemoresponsive to agents that have activity against pancreatic adenocarcinomas and colorectal carcinomas because of the shared genetic alterations between these cancers.
Differential Diagnosis
Additional Information
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