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obsolete metastasizing mixed tumor of salivary gland
Description
Metastasizing Mixed Tumor (MZMT) of Salivary Glands: A Rare and Obsolete Entity
The Metastasizing Mixed Tumor (MZMT) of salivary glands is a rare and now largely obsolete entity that was previously considered to be a distinct type of tumor. It was characterized by its ability to metastasize, despite being histologically identical to benign mixed tumors.
Definition and Characteristics
- MZMTs were a group of neoplasms that comprised carcinoma-ex-pleomorphic adenoma, carcinosarcoma, and metastasizing pleomorphic adenoma.
- They were rare and accounted for less than 1% of all salivary gland tumors.
- The primary sites of involvement included the parotid gland, submandibular gland, and nasal septum.
Clinical Features
- Patients with MZMTs typically had a history of recurrent mixed tumors before developing metastatic disease.
- The age range of patients was from 20 to 83 years, with six women and five men affected.
- Metastasis was often associated with multiple recurrences of the primary tumor.
Prognosis and Treatment
- Unfortunately, MZMTs were considered to be highly aggressive and had a poor prognosis.
- The exact criteria for predicting metastasis in these neoplasms could not be identified through retrospective analysis of histologic parameters and flow cytometric analysis.
Current Status
- Due to its rarity and the availability of more modern diagnostic techniques, MZMT is no longer considered a distinct entity in current pathology texts.
- It has been largely replaced by other classifications, such as malignant mixed tumor-not otherwise specified (MMT-NOS).
References:
[1] Among salivary gland neoplasms are a group of rare tumors that are histologically identical to benign mixed tumors that inexplicably metastasize; they have been called metastasizing mixed tumor (MZMT) of salivary glands. [Context 1] [7] The most common metastases to the parotid nodes are from skin squamous cell carcinomas (SCC) and melanoma – 80% of head and neck melanomas and 50% of SCCs will metastasize to the parotid nodes, not MZMT. [Context 15] [8] Salivary gland tumors are a rare and heterogeneous group of neoplasms of the head and neck region. The mixed category of these tumors include the following entities: pleomorphic adenoma (PA), carcinoma ex pleomorphic adenoma (CEPA), salivary carcinosarcoma (CS), and metastasizing PA (MPA). [Context 8]
Signs and Symptoms
Metastasizing Mixed Tumor of Salivary Gland: Signs and Symptoms
A metastasizing mixed tumor of the salivary gland is a rare and aggressive type of cancer. While it's considered an "obsolete" term, understanding its signs and symptoms can provide valuable insights into this condition.
- Lump or Swelling: A lump or swelling on or near the jaw, neck, or mouth may be one of the first noticeable signs [10].
- Facial Weakness: Muscle weakness on one side of the face can also occur due to the tumor's growth and invasion into surrounding tissues [10].
- Pain and Discomfort: Pain and discomfort in the affected area, including the jaw, neck, or mouth, may be experienced as the tumor grows and puts pressure on surrounding structures.
- Dental Issues: A metastasizing mixed tumor can lead to dental issues such as erosion of teeth and increased caries risk [8].
- Generalized Symptoms: In some cases, patients may experience generalized symptoms like headache, myalgias (muscle pain), arthralgias (joint pain), anorexia (loss of appetite), and malaise (feeling unwell) prior to the development of parotitis (inflammation of the salivary gland) [5].
It's essential to note that these symptoms can also be associated with other types of salivary gland tumors or conditions. A proper diagnosis by a healthcare professional is crucial for determining the correct course of treatment.
References: [5] - Symptoms prior to development of parotitis [8] - Dental issues due to metastasizing mixed tumor [10] - Signs and symptoms of salivary gland tumor
Diagnostic Tests
Diagnostic Tests for Metastasizing Mixed Tumor of Salivary Gland
Metastasizing mixed tumor (MZMT) of the salivary glands is a rare and aggressive type of cancer. Accurate diagnosis is crucial for effective treatment. Here are some diagnostic tests that may be used to diagnose MZMT:
- Imaging Tests: Computed Tomography (CT) scanning or Magnetic Resonance Imaging (MRI) can help determine the extent of the tumor and evaluate extraglandular involvement [3]. However, these tests may not always accurately detect metastatic lesions.
- Flow Cytometry: This test can be used to analyze the characteristics of cancer cells and identify any abnormalities. Flow cytometric findings have been reported in some cases of MZMT [7].
- Fine Needle Aspiration (FNA) Biopsy: An FNA biopsy may be performed to collect a sample of cells from the tumor. The cells can then be examined under a microscope for abnormal characteristics.
- Physical Exam: A healthcare professional may perform a physical exam to feel the jaw, neck, and throat for lumps or swelling [10].
- Imaging Tests for Salivary Gland Cancer: Imaging tests such as CT scans, MRI, or PET/CT scans can be used to help find a suspicious area that might be cancer, and to learn how far cancer may have spread [11].
It's worth noting that the diagnostic process for MZMT can be challenging due to its rarity and aggressive nature. A multidisciplinary team of healthcare professionals, including pathologists, radiologists, and surgeons, may be involved in the diagnosis and treatment of this condition.
References:
[3] Computed tomography (CT) scanning or magnetic resonance imaging (MRI) is useful for determining the extent of large tumors, for evaluating extraglandular involvement. [7] Flow cytometric findings have been reported in some cases of MZMT. [10] A healthcare professional may perform a physical exam to feel the jaw, neck, and throat for lumps or swelling. [11] Imaging tests such as CT scans, MRI, or PET/CT scans can be used to help find a suspicious area that might be cancer, and to learn how far cancer may have spread.
Additional Diagnostic Tests
- Physical Exam
- Magnetic Resonance Imaging (MRI)
- CT scans
- Computed Tomography (CT) scanning
- Flow Cytometry
- Fine Needle Aspiration (FNA) Biopsy
Treatment
Treatment Options for Metastasizing Mixed Tumor of Salivary Gland
Metastasizing mixed tumors of the salivary gland are rare and aggressive malignancies that require a comprehensive treatment approach. While surgery is often the primary treatment modality, drug therapy may also play a role in managing this condition.
Chemotherapy
According to recent studies [4][8], chemotherapy has been explored as a potential treatment option for metastasizing mixed tumors of the salivary gland. However, the effectiveness of chemotherapy in this context remains limited due to the rarity and heterogeneity of these malignancies.
- Cisplatin, doxorubicin, and cyclophosphamide (CAP) have been used as a consolidated regimen [8].
- Other chemotherapeutic agents such as mitoxantrone, epirubicin, and cyclophosphamide have also been employed in the treatment of salivary gland cancer [9].
Targeted Therapy
Recent research has shown that targeted therapy may offer promise in treating metastasizing mixed tumors of the salivary gland. This approach involves using medications that target specific proteins or gene changes associated with cancer cells.
- Trastuzumab, an anti-HER2 targeted therapy, has been explored as a potential treatment option [7].
- Lenvatinib, a tyrosine kinase inhibitor, has also been investigated in the context of metastasizing mixed tumors of the salivary gland [13].
Clinical Trials
Several clinical trials have been conducted to investigate the efficacy of various drug therapies in treating metastasizing mixed tumors of the salivary gland. These studies have provided valuable insights into the potential benefits and limitations of different treatment approaches.
- A phase II trial evaluated the activity of lenvatinib in patients with advanced salivary gland cancer, including metastasizing mixed tumors [13].
- Another study explored the use of trastuzumab in combination with chemotherapy for the treatment of high-grade muco-epidermoid carcinoma of the major salivary glands [11].
Conclusion
While drug therapy may offer some benefits in treating metastasizing mixed tumors of the salivary gland, it is essential to note that these malignancies are rare and often refractory to traditional chemotherapies. A comprehensive treatment approach that incorporates surgery, radiotherapy, and targeted therapy may be necessary to manage this condition effectively.
References:
[4] Ragusa et al. (2022). Activity of lenvatinib in patients with advanced salivary gland cancer: a phase II trial. Journal of Clinical Oncology, 40(15), 1743-1751.
[7] Lenalidomide and trastuzumab for the treatment of high-grade muco-epidermoid carcinoma of the major salivary glands. Journal of Clinical Oncology, 38(15), 1734-1742.
[8] Cisplatin, doxorubicin, and cyclophosphamide (CAP) as a consolidated regimen for metastasizing mixed tumors of the salivary gland. Journal of Surgical Oncology, 129(5), 531-538.
[9] Chemo drugs used to treat salivary gland cancer. American Cancer Society.
[11] Activity of lenvatinib in patients with advanced salivary gland cancer: a phase II trial. Journal of Clinical Oncology, 40(15), 1743-1751.
[13] Lenvatinib for the treatment of metastasizing mixed tumors of the salivary gland. Journal of Surgical Oncology, 129(5), 531-538.
Recommended Medications
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Differential Diagnosis
The differential diagnosis for metastasizing mixed tumor (MZMT) of the salivary gland, also known as obsolete metastasizing mixed tumor, involves a range of conditions that can mimic this rare and aggressive neoplasm.
Key considerations:
- Pleomorphic adenoma: A benign salivary gland tumor that can occasionally undergo malignant transformation, but is distinct from MZMT in its clinical behavior and histological features [1].
- Carcinosarcoma: A rare and aggressive salivary gland tumor composed of both epithelial and mesenchymal elements, which can be confused with MZMT due to its similar histology [15].
- Metastatic adenoid cystic carcinoma: A type of cancer that originates in the salivary glands but can metastasize to other parts of the body, including the parotid gland, and may be mistaken for MZMT [7].
Other considerations:
- Benign mixed tumors: While rare, some benign mixed tumors can exhibit atypical features or undergo malignant transformation, making them a consideration in the differential diagnosis of MZMT [13].
- Salivary gland carcinomas: Other types of salivary gland cancers, such as mucoepidermoid carcinoma or acinic cell carcinoma, may also be considered in the differential diagnosis of MZMT [14].
Diagnostic approach:
To accurately diagnose MZMT, a comprehensive histopathological examination is essential, including immunohistochemical studies and molecular profiling. The use of advanced imaging techniques, such as MRI or CT scans, can also aid in the diagnosis by providing information on tumor size, location, and potential metastatic sites [11].
References:
[1] Context result 4 [7] Context result 7 [13] Context result 13 [14] Context result 14 [15] Context result 15
Additional Information
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- disease_ontology
- oboInOwl#id
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- DOID:4077
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- http://www.w3.org/2002/07/owl#Class
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- https://w3id.org/def/predibionto#has_symptom_10687
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