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obsolete malignant extraskeletal cartilaginous and osseous tumor

Description

Obsolescence of Extraskeletal Cartilaginous and Osseous Tumor

The term "extraskeletal cartilaginous and osseous tumor" is considered outdated, as it was previously thought to be a type of neoplasm that exhibited cartilage differentiation. However, recent classifications have redefined this entity.

Current Classification

According to the World Health Organization (WHO) classification, extraskeletal myxoid chondrosarcoma (EMC) is now classified as a mesenchymal tumor of uncertain differentiation [1]. This change reflects our improved understanding of the disease's clinico-pathological and genetic features.

Key Differences

The obsolete term "extraskeletal cartilaginous and osseous tumor" has been replaced by more specific designations, such as extraskeletal myxoid chondrosarcoma (EMC) or malignant extraskeletal cartilaginous and osseous tumor. These newer terms better reflect the tumor's true nature and characteristics.

Implications

The obsolescence of this term highlights the dynamic nature of medical knowledge and the importance of staying up-to-date with the latest classifications and terminology. This change ensures that healthcare professionals use accurate and current language when discussing and managing patients with these conditions.

References:

[1] Originally thought to be a cartilaginous neoplasm, EMC is now classified as a mesenchymal tumor of uncertain differentiation [1, 10]. Indeed, EMC exhibits distinctive clinico-pathological and genetic features while it shows no cartilage differentiation, despite the name, which has been retained only for historical reasons.

Additional Characteristics

  • Extraskeletal myxoid chondrosarcoma (EMC)
  • Malignant extraskeletal cartilaginous and osseous tumor
  • Mesenchymal tumor of uncertain differentiation

Signs and Symptoms

Based on the search results, here are the signs and symptoms associated with malignant extraskeletal cartilaginous and osseous tumors:

  • Pain: This is a common symptom, as mentioned in [result 11] (Chondrosarcomas are a group of rare bone cancer tumors that begin in your cartilage. Symptoms include bone pain...). The pain can be due to the tumor's growth or its impact on surrounding tissues.
  • Lump or swelling: A noticeable lump or swelling in an area of the body, as described in [result 11] (Chondrosarcomas are a group of rare bone cancer tumors that begin in your cartilage. Symptoms include a lump or swelling in an area of your body...).
  • Fatigue: This symptom is also mentioned in [result 11] (Chondrosarcomas are a group of rare bone cancer tumors that begin in your cartilage. Symptoms include fatigue...), indicating that the tumor's presence can lead to general feelings of tiredness or weakness.
  • Mechanical symptoms from compression: As stated in [result 10] (Most commonly a secondary low-grade chondrosarcoma develops. Clinical Presentation. Signs/Symptoms: Hard swelling for many years; Symptoms dependent on location/size; May cause mechanical symptoms from compression of adjacent structures...), the tumor can compress nearby tissues, leading to mechanical symptoms such as pain or limited mobility.
  • Hard swelling: This symptom is mentioned in [result 10] (Most commonly a secondary low-grade chondrosarcoma develops. Clinical Presentation. Signs/Symptoms: Hard swelling for many years; Symptoms dependent on location/size...), indicating that the tumor can cause a hard, persistent swelling in an area of the body.

It's essential to note that these symptoms may not be specific to malignant extraskeletal cartilaginous and osseous tumors and can be associated with other conditions. A proper medical diagnosis is necessary for accurate identification and treatment.

Additional Symptoms

  • Lump or swelling
  • Mechanical symptoms from compression
  • Hard swelling
  • pain
  • fatigue

Diagnostic Tests

Diagnostic Tests for Malignant Cartilaginous Tumors

Malignant cartilaginous tumors, also known as chondrosarcomas, are a type of cancer that affects the cartilage in bones. Diagnostic tests play a crucial role in identifying these tumors and determining their severity. Here are some diagnostic tests used to diagnose malignant cartilaginous tumors:

  • X-rays: X-rays can often provide a diagnosis for chondrosarcoma tumors, which have a distinct appearance [3]. They can also show the size of the tumor and how much bone is affected.
  • Computed Tomography (CT) scanning: CT scans are essential for the initial diagnosis of chondrosarcoma and may be used in conjunction with X-rays to provide more detailed images of the tumor [5].
  • Magnetic Resonance Imaging (MRI): MRI can help determine the extent of the tumor's spread and its relationship to surrounding tissues.
  • Bone scan: A bone scan is a diagnostic test that uses small amounts of radioactive material to detect abnormalities in the bones, including tumors.

Other Diagnostic Tests

In addition to these tests, other methods may be used to diagnose malignant cartilaginous tumors, such as:

  • Ultrasound: Ultrasound can be used to examine superficial and soft tissue tumors [4].
  • Biopsy: A biopsy involves taking a sample of the tumor tissue for examination under a microscope. This is often necessary to confirm the diagnosis.

Importance of Accurate Diagnosis

Accurate diagnosis is crucial in determining the best course of treatment for malignant cartilaginous tumors. It also helps identify any potential complications or risks associated with the tumor.

References:

[1] [3] [5] [9]

Note: The numbers in square brackets refer to the search results provided, which are used as citations for the information presented.

Treatment

Based on the provided context, it appears that there are limited treatment options available for malignant extraskeletal cartilaginous and osseous tumors (MECOT). However, some information can be gleaned from the search results.

Current Treatment Options:

  • Surgery is not always feasible due to the tumor's location and aggressive nature [7].
  • Wide or radical resection is considered the mainstay of treatment when possible [7].
  • Chemotherapy may be employed in some cases, with regimens including doxorubicin, high-dose methotrexate, cisplatin, and ifosfamide [2].

Emerging Treatment Options:

  • Trabectedin has been studied as a potential treatment for MECOT, although results are limited [10].
  • Radiation therapy may be used in some cases to alleviate symptoms or control tumor growth [8].

Prognosis: The prognosis for patients with MECOT is generally poor due to the tumor's aggressive nature and tendency for local recurrence and distant metastasis [14].

💊 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 Obsolete Malignant Extraskeletal Cartilaginous and Osseous Tumors

The differential diagnosis for obsolete malignant extraskeletal cartilaginous and osseous tumors involves considering various types of lesions that may present similarly. Based on the search results, here are some key points to consider:

  • Chondrosarcoma: This is a type of malignant bone tumor that produces cartilaginous matrix. Mutations in isocitrate dehydrogenase enzymes (IDH1/2) were recently described [5]. Chondrosarcomas can be difficult to distinguish from benign cartilage-producing lesions, such as enchondromas.
  • Myositis ossificans: This is a type of osseous lesion that can mimic malignant extraskeletal cartilaginous and osseous tumors. It is characterized by the formation of bone within muscle tissue [11].
  • Fibro-osseous pseudotumor: This is another type of osseous lesion that can be difficult to distinguish from malignant extraskeletal cartilaginous and osseous tumors. It is characterized by the presence of fibrous and osseous components [11].
  • Myoepithelioma and myoepithelial lesions: These are types of benign tumors that can mimic malignant extraskeletal cartilaginous and osseous tumors. The positivity for NR4A3 rearrangements may be particularly helpful in the differential diagnosis of these lesions [4].

Key Considerations

  • Location and extent of the tumor: The location and extent of the tumor can provide important clues for differential diagnosis.
  • Imaging studies: Imaging studies, such as CT and MR imaging, can help to differentiate between various types of lesions.
  • Histopathological examination: A thorough histopathological examination is essential for accurate diagnosis.

References

[1] by HL Geyer · 2010 · Cited by 16 — [4] by S Stacchiotti · 2020 · Cited by 50 — [5] by AE Zając · 2021 · Cited by 61 — [11] by BE Walczak · Cited by 6 —

Note: The numbers in square brackets refer to the search results provided.

Additional Differential Diagnoses

Additional Information

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