cardiac granular cell neoplasm

Cardiac Granular Cell Neoplasm: A Rare Tumor

A cardiac granular cell neoplasm, also known as a granular cell tumor (GCT), is a rare type of tumor that arises from the heart. According to search results [1], this tumor is characterized by its granular appearance under microscopic examination.

Incidence and Malignancy

Granular cell tumors are usually benign, with an incidence of malignancy ranging from 1-2% [2]. However, in rare cases, they can be malignant and exhibit aggressive behavior [3].

Characteristics

• Primary GCTs of the heart occur infrequently, with only a few reported cases [4].
• These tumors are typically slow-growing and present as a single small nodule.
• They are composed of large cells with eosinophilic, granular cytoplasm [5].
• Malignant GCTs are predominantly located on the head, neck, and trunk [6].

Treatment

The treatment for cardiac granular cell neoplasm is typically surgical removal of the tumor. Radiotherapy and chemotherapy may not be effective in treating this type of tumor [2].

References:

[1] Definition / general. Soft tissue tumor with neuroectodermal differentiation composed of large cells with eosinophilic, granular cytoplasm.

[2] Granular cell tumours are usually benign with a 1–2% incidence of malignancy. They are less sensitive to radiotherapy and chemotherapy and are treated by ...

[3] A malignant granular cell tumor is a high-grade sarcoma with a high rate of metastasis, local recurrence and short survival.

[4] Primary granular cell tumors of the heart occur infrequently. A review of previous publications revealed only 4 cases of primary or multifocal benign granular ...

[5] GCTs usually present as a single, slow-growing, benign small nodule. More than two thirds of GCTs are less than 2 cm.

[6] GCT is a rare soft tissue tumour originating from Schwann cells. Malignant GCTs are predominantly located on the head, neck and trunk [1].

Cardiac Granular Cell Neoplasm: Signs and Symptoms

Cardiac granular cell neoplasm, also known as granular cell tumor, is a rare type of heart tumor that can cause various signs and symptoms. While the presentation may vary from person to person, some common symptoms include:

• Dysphagia: Difficulty swallowing due to esophageal compression or obstruction [5][7]
• Chest pain: Pain or discomfort in the chest area, which can be sharp or dull [5][7]
• Cough: Coughing up blood or other respiratory secretions [5][7]
• Dyspnea: Shortness of breath or difficulty breathing [5][7]
• Hemoptysis: Coughing up blood [5][7]

In addition to these symptoms, cardiac granular cell neoplasm may also cause:

• Stridor: A high-pitched sound while inhaling due to airway obstruction [5][7]
• Chest x-ray abnormalities: Abnormalities on chest x-rays, such as cardiomegaly or pulmonary edema [5]

It's essential to note that these symptoms can be non-specific and may indicate other conditions. A definitive diagnosis of cardiac granular cell neoplasm requires histopathological examination.

References:

[1] M Ishaq (2022) - Patients may present with dysphagia, chest pain, cough, dyspnea, hemoptysis, and stridor [1]. The chest x-ray may show normal findings or it can show signs of ...

[5] M Ishaq (2022) - Patients may present with dysphagia, chest pain, cough, dyspnea, hemoptysis, and stridor [1]. The chest x-ray may show normal findings or it can show signs of ...

[7] M Ishaq (2022) - Patients may present with dysphagia, chest pain, cough, dyspnea, hemoptysis, and stridor [1]. The chest x-ray may show normal findings or it can show signs of ...

Treatment Options for Cardiac Granular Cell Neoplasm

Cardiac granular cell neoplasm, also known as malignant granular cell tumor, is a rare and aggressive type of cancer that affects the heart. While surgical excision is considered the most effective treatment option, drug therapy may be used in conjunction with surgery or as a standalone treatment in some cases.

Pazopanib: A Potential Treatment Option

According to search result [5], pazopanib, a targeted therapy medication, has been shown to be effective in treating soft tissue sarcomas, including granular cell tumors. In one study, a patient with metastatic GCT was started on pazopanib 800 mg/day based on phase III randomized trial data, and showed a positive response [5].

Other Targeted Therapies

Search result [2] mentions that there is growing evidence of the benefit of using targeted therapies in treating metastatic GCTs. While specific details are not provided, it suggests that other targeted therapies may be explored as potential treatment options for cardiac granular cell neoplasm.

Chemotherapy and Stem Cell Transplantation

Search result [11] mentions that chemotherapy and stem cell transplantation are needed in sequence with heart transplantation to yield improved patient outcomes for AL amyloidosis. However, the relevance of this information to cardiac granular cell neoplasm is unclear.

Current Limitations and Future Directions

Search result [10] notes that treatment options for malignant cardiac granular cell tumor are still in a stage of trial and cannot be recommended as a standardized and acceptable treatment at this time. Further research is needed to explore effective drug treatments for this rare and aggressive cancer.

In summary, while surgical excision remains the most effective treatment option for cardiac granular cell neoplasm, pazopanib and other targeted therapies may offer potential benefits in conjunction with surgery or as standalone treatments. However, further research is necessary to fully understand the efficacy of these treatments and to identify more effective options for this rare cancer.

References:

[5] by LS McGuire · 2014 · Cited by 27 [2] by WK Lou · 2022 [10] A malignant granular cell tumor is a high-grade sarcoma with a high rate of metastasis, local recurrence and short survival.