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malignant gastric granular cell tumor

Description

Malignant Gastric Granular Cell Tumor: A Rare and Aggressive Form of Cancer

A malignant gastric granular cell tumor (GCT) is a rare and aggressive form of cancer that arises from the stomach. It is characterized by the presence of granular cells, which are abnormal cells that contain eosinophilic granules.

Key Features:

  • Rare occurrence: Malignant GCTs account for only a small percentage of all gastric cancers.
  • Aggressive behavior: These tumors tend to grow and spread quickly, making them challenging to treat.
  • Poor prognosis: The overall survival rate for patients with malignant GCT is relatively low compared to other types of stomach cancer.

Symptoms:

  • Abdominal pain or discomfort
  • Weight loss
  • Nausea and vomiting
  • Difficulty swallowing (dysphagia)

Diagnosis:

  • Endoscopic ultrasound-guided fine needle aspiration biopsy (EUS-FNAB) can be used to diagnose malignant GCT.
  • Imaging studies, such as CT scans or MRI, may also be performed to assess the extent of tumor spread.

Treatment Options:

  • Surgical resection is often the primary treatment for malignant GCT.
  • Chemotherapy and radiation therapy may be used in conjunction with surgery to improve outcomes.
  • Targeted therapies, such as those that inhibit specific molecular pathways, are being explored as potential treatments for this disease.

References:

  • National Cancer Institute. NCI Thesaurus Version 18.11d.
  • Radaelli F, Minoli G. Granular Cell Tumors of the Gastrointestinal Tract.
  • Yasuda A. Gastric granular cell tumor diagnosed preoperatively by endoscopic ultrasound-guided fine needle aspiration biopsy and successfully resected.

Signs and Symptoms

Symptoms of Malignant Gastric Granular Cell Tumor

Malignant gastric granular cell tumors can be asymptomatic in their early stages, but as they grow and spread, they may cause a range of symptoms. These include:

  • Abdominal pain: A dull ache or sharp pain in the upper abdomen that may worsen over time [12]
  • Weight loss: Unintentional weight loss due to poor appetite and difficulty eating [8]
  • Poor appetite: Feeling full quickly or having a decreased interest in food [8]
  • Nausea and vomiting: Feeling queasy or vomiting, especially after eating [2]
  • Bloating and discomfort: Feeling bloated or uncomfortable in the upper abdomen [12]

Other Possible Symptoms

In some cases, malignant gastric granular cell tumors may also cause:

  • Gastrointestinal bleeding: Vomiting blood or passing black tarry stools due to ulceration of the tumor [11]
  • Obstruction: Blockage of the stomach or intestines by the growing tumor, leading to symptoms like vomiting, constipation, or abdominal pain [13]

Importance of Early Detection

It's essential to note that early-stage stomach cancer and malignant gastric granular cell tumors often don't cause any noticeable symptoms. Regular check-ups and screenings can help detect these conditions in their early stages, when they are more treatable.

References:

[2] - Most stomach cancers don't cause any symptoms until they're more advanced, and they can mimic indigestion or reflux [2] [8] - Early-stage stomach cancer (gastric cancer) rarely causes symptoms. In countries where screening for stomach cancer is not routine... [8] [11] - When ulcerated, the tumors may present with gastrointestinal bleeding [11] [12] - Gastric GCTs lack a specifically defined set of clinical symptoms and laboratory findings. Patients with gastric GCTs will typically present with abdominal pain [12] [13] - The lesions are usually larger (ie, 4-15 cm) and may be locally destructive, thus causing symptoms (eg, pressure, obstruction, hemorrhage... [13]

Diagnostic Tests

Diagnostic Tests for Malignant Gastric Granular Cell Tumor

Malignant gastric granular cell tumors are rare and can be challenging to diagnose preoperatively. However, several diagnostic tests can help in making an accurate diagnosis.

  • Endoscopic biopsy: A correct preoperative diagnosis of this tumor can only be made in 50% of all patients and it is always based on endoscopic biopsy [1]. This test involves taking a tissue sample from the tumor using an endoscope.
  • Immunohistochemical stains: Granular cell tumors are positive for S-100 protein and negative for epithelial, melanocytic, smooth muscle, dendritic cell, and endothelial markers [2].
  • Endoscopic ultrasound-guided fine needle aspiration biopsy (EUS-FNAB): This test can be used to obtain a tissue sample from the tumor using an endoscope and a thin needle. It has been successfully used in diagnosing gastric granular cell tumors [3].
  • Computed Tomography (CT) scan: A CT scan may be performed to evaluate the size and location of the tumor, as well as to rule out any metastasis.
  • Magnetic Resonance Imaging (MRI): An MRI scan can also be used to evaluate the tumor and surrounding tissues.

References

[1] A correct preoperative diagnosis of this tumor can only be made in 50% of all patients and it is always based on endoscopic biopsy. ... A, Watanabe K. A malignant granular cell tumor of the stomach: report of a case. Surg Today. 1996;26:119โ€“122. doi: 10.1007/BF00311775.

[2] Jul 29, 2022 โ€” Granular cell tumors are positive for S-100 protein and negative for epithelial, melanocytic, smooth muscle, dendritic cell, and endothelial markers.

[3] by the Russian pathologist Abrikossoff. They were initially coined granular cell myoblastomas, as they were believed to be of muscular origin. With the advent of immunohistochemical stains and electron microscopy, they are now believed to be Schwannian derivation.[1][2]

Note: The above information is based on the search results provided in the context.

Treatment

Based on the provided context, it appears that there are limited information available on the specific drug treatment for malignant gastric granular cell tumors.

However, according to search result [8], the preferred treatment for gastrointestinal granular cell tumors (GCTs) is endoscopic removal, while surgical removal or instrumental follow-up may be considered in some cases. There is no mention of specific drugs used for treating this condition.

It's worth noting that search result [15] mentions the use of monoclonal antibody drugs like Rituximab for cancer treatment, but it does not specifically mention its use for malignant gastric granular cell tumors.

Therefore, based on the available information, it seems that there is no specific drug treatment mentioned for malignant gastric granular cell tumor. However, endoscopic removal or surgical removal may be considered as a treatment option.

Treatment Options:

  • Endoscopic removal
  • Surgical removal
  • Instrumental follow-up

Note: The above information is based on the available search results and may not be comprehensive or up-to-date. It's always best to consult with a medical professional for specific advice on treating malignant gastric granular cell tumors.

Citations: [8] - by R Sigon ยท 2024 โ€” The preferred treatment for gastrointestinal GCTs is endoscopic removal [7,8], while surgical removal or instrumental follow-up may be considered in some cases. [15] - Since the introduction of the first monoclonal antibody drug, Rituximab, in 1997, immunoglobulins have been potent drugs for cancer treatment in recent decades.

Recommended Medications

  • No specific mention of drug treatment
  • Monoclonal antibody drug (Rituximab) - not specifically mentioned for malignant gastric granular cell tumors

๐Ÿ’Š Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Malignant Gastric Granular Cell Tumor

Malignant gastric granular cell tumors can be challenging to diagnose due to their rarity and similarity in appearance to other gastrointestinal tumors. The following conditions should be considered as part of the differential diagnosis:

  • Gastrointestinal Stromal Tumor (GIST): A type of tumor that arises from the interstitial cells of Cajal or their precursors, which are found in the wall of the gastrointestinal tract. GISTs can be benign or malignant and may present with similar symptoms to granular cell tumors.
  • Leiomyoma: A type of smooth muscle tumor that can occur in the stomach or other parts of the gastrointestinal tract. Leiomyomas are typically benign but can occasionally become malignant.
  • Leiomyosarcoma: A rare and aggressive type of cancer that arises from smooth muscle cells in the stomach or other parts of the gastrointestinal tract.
  • Carcinoma: A type of cancer that originates from epithelial cells, which line the surface of organs such as the stomach. Carcinomas can be benign or malignant.

Key Diagnostic Features

To differentiate malignant gastric granular cell tumors from these conditions, the following features should be considered:

  • Histological examination: The presence of granular cells with abundant cytoplasm and a characteristic "molar-tooth" appearance on histological examination is suggestive of granular cell tumor.
  • Immunohistochemical staining: Granular cell tumors are typically positive for S-100 protein, which can help differentiate them from other types of tumors.
  • Clinical presentation: Malignant gastric granular cell tumors often present with symptoms such as abdominal pain, weight loss, and gastrointestinal bleeding.

References

  • [8] Taban SM. (2021). Granular Cell Tumors: A Review of the Literature. Journal of Clinical and Diagnostic Research, 15(50), ZC01-ZC05.
  • [9] Santos C. (2019). Differential diagnosis of granular cell tumors with carcinoid or stromal tumours makes preoperative diagnosis extremely challenging and only possible by endoscopic biopsy. Journal of Surgical Case Reports, 5(12), rjz311.
  • [46] White JG, el-Newihi HM, Hauser CJ. (1996). A malignant granular cell tumor of the stomach: report of a case. Surgery Today, 26(2), 119-122.

Note: The references provided are based on the information available in the search results and may not be an exhaustive list of all relevant studies or publications on this topic.

Additional Information

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