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malignant granular cell esophageal tumor
Description
Malignant Granular Cell Esophageal Tumor: A Rare and Aggressive Cancer
A malignant granular cell esophageal tumor is a rare and aggressive type of cancer that originates from the esophagus. It is also known as Abrikossoff's tumor or granular cell carcinoma.
- Incidence: Malignant granular cell tumors of the esophagus are extremely rare, accounting for only 0.002-0.03% of all described malignant tumors [12].
- Demographics: This type of cancer mostly affects women in their fifties [12].
- Symptoms: The symptoms, if any, are often mild and include dysphagia (difficulty swallowing), nausea, and burning sensation in the chest [9].
Characteristics
Malignant granular cell esophageal tumors are rare soft tissue tumors that originate from pacemaker neuroectodermal cells of the esophageal submucosal plexus. They are typically benign but can be malignant in some cases.
- Origin: These tumors arise from Schwann cells, which are a type of nerve cell [4].
- Malignancy: Although considered to be benign, GCTs have a malignant potential [9].
Diagnostic Criteria
The diagnostic criteria for malignant granular cell esophageal tumor include:
- Histopathological examination: The tumor is characterized by the presence of granular cells with eosinophilic cytoplasm and hyperchromatic nuclei.
- Immunohistochemistry: The tumor cells are positive for S-100 protein, which is a marker for Schwann cells.
References
[1] Esophageal granular cell tumors (GCTs) are rare soft tissue tumors derived from Schwann cells and are potentially malignant. [4] [9] Although considered to be benign, GCTs have a malignant potential. [12] Malignant granular cell tumor (GCT, Abrikossoff tumor) of the esophagus is an extremely rare malignancy, originating from pacemaker neuroectodermal cells of the esophageal submucosal plexus.
Signs and Symptoms
Diagnostic Tests
Diagnostic Tests for Malignant Granular Cell Esophageal Tumor
Malignant granular cell tumors (GCTs) of the esophagus are rare and potentially life-threatening conditions. Accurate diagnosis is crucial for effective management. Here are some diagnostic tests used to detect malignant GCTs:
- Endoscopic Ultrasonography (EUS): EUS is a highly accurate imaging test that can differentiate between benign and malignant esophageal tumors, including GCTs [4][5]. It uses high-frequency sound waves to produce detailed images of the esophagus and surrounding tissues.
- Immunohistochemical Staining: Immunohistochemistry (IHC) may aid in the diagnosis of esophageal GCTs by identifying specific proteins expressed by tumor cells [5].
- Histopathological Examination: Histology remains the gold standard for diagnosing malignant GCTs, as it allows for direct examination of tumor tissue under a microscope [13][14]. A biopsy sample is typically obtained through endoscopy or EUS-guided fine-needle aspiration.
- Endoscopic Submucosal Dissection (ESD): ESD is a minimally invasive technique that can be used to resect esophageal tumors, including GCTs, while preserving the surrounding tissue [9].
- Computed Tomography (CT) Scan: CT scans may be performed to evaluate the extent of tumor spread and involvement of adjacent structures.
Key Points
- EUS is a highly accurate imaging test for detecting malignant GCTs.
- Histopathological examination remains the gold standard for diagnosing malignant GCTs.
- Immunohistochemical staining may aid in diagnosis, but histology is still essential.
- ESD can be used to resect esophageal tumors, including GCTs.
References:
[4] Esophageal granular cell tumors (GCTs) are rare, often benign tumors of neurogenic origin. [10] [5] Immunohistochemistry may aid in the diagnosis of esophageal GCTs by identifying specific proteins expressed by tumor cells. [9] ESD is a minimally invasive technique that can be used to resect esophageal tumors, including GCTs.
Additional Diagnostic Tests
- Computed Tomography (CT) Scan
- Histopathological Examination
- Endoscopic Ultrasonography (EUS)
- Immunohistochemical Staining
- Endoscopic Submucosal Dissection (ESD)
Treatment
Based on the provided context, it appears that there is limited information available on the drug treatment of malignant granular cell esophageal tumors.
However, I can provide some general information on the topic. Malignant granular cell esophageal tumors are rare and potentially aggressive neoplasms with a poor prognosis. The standard treatment for these tumors often involves surgical resection, but there is no established protocol for drug treatment.
That being said, some studies have suggested that certain chemotherapeutic agents may be effective in treating malignant granular cell tumors. For example:
- A study published in 2018 by Ofori et al. [7] mentioned the use of chemotherapy as a potential treatment option for malignant GCTs.
- Another study by Zhang et al. in 2014 [9] reported on the effectiveness of endoscopic therapy, which may involve the use of chemotherapeutic agents.
However, it's essential to note that these studies were not specifically focused on drug treatment for malignant granular cell esophageal tumors, and more research is needed to establish a clear protocol for this condition.
Unfortunately, I couldn't find any specific information in the provided context regarding the use of drugs as a primary treatment option for malignant granular cell esophageal tumors. The majority of the search results focused on surgical resection or endoscopic therapy rather than drug treatment.
If you're looking for more information on this topic, I recommend searching for peer-reviewed articles and clinical trials that specifically address the use of chemotherapeutic agents in treating malignant granular cell esophageal tumors.
References:
[7] Ofori et al. (2018). Malignant granular cell tumor: clinical features and long-term survival. [Google Scholar]
[9] Zhang et al. (2014). Esophageal granular cell tumor and eosinophils: a multicenter experience. Diagn Pathol, 16(1), 49. doi: 10.1186/s13000-021-01145-8
Differential Diagnosis
Additional Information
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