obsolete extraskeletal myxoid chondrosarcoma

Definition and Characteristics

Obsolete extraskeletal myxoid chondrosarcoma refers to an outdated term for a rare type of soft tissue sarcoma. According to the Disease Ontology Definition, it is characterized by the presence of chondroblast-like cells in a myxoid stroma [7][8]. This condition was previously considered a distinct entity from other types of sarcomas.

Key Features

• Located exclusively in soft tissue
• Presence of chondroblast-like cells in a myxoid stroma
• Considered an obsolete term, as it is no longer used to describe this specific type of sarcoma

It's worth noting that the term "obsolete extraskeletal myxoid chondrosarcoma" is not commonly used in modern medical literature and has been replaced by more accurate and up-to-date terminology.

Common Signs and Symptoms

Extraskeletal myxoid chondrosarcoma (EMC) is a rare type of soft tissue sarcoma that can cause various signs and symptoms. According to the search results, some common symptoms include:

• A lump or swelling in the affected area [12]
• Pain and tenderness at the site of the tumor [1][10]
• Functional impairment if the tumor is located near a joint [1][10]
• Neurologic symptoms and signs related to hypocalcemia and hyperphosphatemia [2]

Other Possible Symptoms

In some cases, EMC may also cause:

• A hematoma-like appearance of the tumor [1][10]
• Restricted movement if the lump is near a joint [12]
• Intra-abdominal or retroperitoneal primary tumors can cause advanced symptoms [6]

Important Notes

It's essential to note that these symptoms can vary depending on the size and location of the tumor. A specialist should be consulted for an accurate diagnosis, which may involve tests such as scans to take pictures of the inside of the body [12].

References: [1] - Search result 10 [2] - Search result 2 [6] - Search result 6 [10] - Search result 10 [12] - Search result 12

Diagnostic Tests for Extraskeletal Myxoid Chondrosarcoma

Extraskeletal myxoid chondrosarcoma (EMC) is a rare and aggressive type of cancer that requires prompt diagnosis. The following diagnostic tests are commonly used to diagnose EMC:

• Physical Examination: A physical examination by a specialist can help identify the lump or tumor, which is often found in the deep soft tissues of the proximal limbs [11].
• Imaging Tests: Various imaging tests such as ultrasound, x-ray, CT, MRI, and PET scans are used to take pictures of the inside of the body and confirm the presence of a tumor [11]. These tests can help determine the size, location, and extent of the tumor.
• Biopsy: A biopsy is a procedure where a sample of tissue from the tumor is taken and examined under a microscope. This test can help confirm the diagnosis of EMC by identifying the characteristic features of the tumor cells [11].
• Molecular Testing: Molecular testing, such as fluorescence in situ hybridization (FISH), can be used to identify specific genetic mutations associated with EMC [4, 9].

Additional Diagnostic Tests

In some cases, additional diagnostic tests may be necessary to confirm the diagnosis of EMC. These may include:

• Cardiac Biopsy: A cardiac biopsy may be performed if there are concerns about the tumor's impact on the heart or other organs [2].
• Echocardiography: Echocardiography can help assess the function and structure of the heart in patients with suspected EMC [2].

References

[1] Geyer HL. Complete work-up of a patient with extraskeletal myxoid chondrosarcoma. 2010.

[2] Stacchiotti S. Extraskeletal myxoid chondrosarcoma: a review of the literature. 2020.

[4] Chang KTE. Current standard molecular diagnostic methods for identifying gene fusions in use in hospital laboratories. 2018.

[9] Dejka, et al. Fluorescence in situ hybridization is a useful ancillary diagnostic tool for extraskeletal myxoid chondrosarcoma.

Treatment Options for Extraskeletal Myxoid Chondrosarcoma (EMC)

Extraskeletal myxoid chondrosarcoma (EMC) is a rare and aggressive type of cancer that can be challenging to treat. While surgery remains the primary treatment option, drug therapy has also been explored as a potential treatment approach.

Approved Drug Treatment

One approved drug treatment for EMC is pazopanib [1]. Pazopanib is an antiangiogenic agent that targets the growth of new blood vessels that feed cancer cells. It has been shown to be effective in treating EMC, particularly in patients who have not responded to other treatments [2].

Other Investigational Drugs

Several other investigational drugs have also been studied for their potential use in treating EMC. These include:

• Apatinib: This is a tyrosine kinase

The differential diagnosis for extraskeletal myxoid chondrosarcoma (EMC) involves a wide range of soft tissue tumors that can mimic its appearance. Some of the key conditions to consider in the differential diagnosis of EMC include:

• Myoepithelioma: A rare tumor that can be difficult to distinguish from EMC, particularly in cases where there is a lack of specific immunohistochemical markers [3].
• Myxomas: These are benign tumors that can have a similar appearance to EMC, but they typically do not exhibit the same level of cellular atypia or mitotic activity [2].
• Papillary fibroelastomas: A rare tumor that can be mistaken for EMC due to its myxoid matrix and papillary architecture [1].
• Hemangiomas: These are benign vascular tumors that can have a similar appearance to EMC, particularly in cases where there is a prominent myxoid stroma.
• Teratomas: A rare tumor that can contain a wide range of tissue types, including cartilage, which can make it difficult to distinguish from EMC [4].
• Rhabdomyomas: These are benign tumors that can have a similar appearance to EMC, particularly in cases where there is a prominent myxoid stroma.
• Neuroblastoma metastases: In some cases, EMC can be mistaken for neuroblastoma metastasis due to its similar appearance and location [8].
• Lymphoma: A rare tumor that can have a similar appearance to EMC, particularly in cases where there is a prominent myxoid stroma.
• Primitive neurectodermal tumor (PNET): This is a rare tumor that can be difficult to distinguish from EMC due to its similar appearance and location [8].

It's worth noting that the differential diagnosis for EMC can be challenging, and it often requires a combination of clinical, radiological, and histopathological features to make an accurate diagnosis.