adult mesenchymal chondrosarcoma

Adult Mesenchymal Chondrosarcoma: A Rare and Aggressive Form of Cancer

Mesenchymal chondrosarcoma (MC) is a rare and aggressive form of cancer that affects adults, typically between the ages of 19 to 30. It is an uncommon type of chondrosarcoma, which is a malignant tumor that arises from cartilage.

Characteristics and Symptoms

• MC often develops in the spine, ribs, or jaw.
• The tumor grows quickly and has a high propensity for metastasis (spreading) to other parts of the body.
• It is more likely to recur after treatment compared to other types of chondrosarcomas.
• Extraskeletal myxoid chondrosarcoma is a form of mesenchymal chondrosarcoma.

Histological Features

• MC has a biphasic histological pattern, consisting of well-differentiated cartilage and small round cells.
• The tumor may exhibit a "Ewing-like" or hemangiopericytoma-like appearance under microscopic examination.

Incidence and Prevalence

• In adults, mesenchymal chondrosarcoma accounts for 2 to 10% of all chondrosarcomas.
• An estimated 215 cases per year occur in the United States.

References:

• [1] Weis and Nakashima (documented 111 cases at the Mayo Clinic from 1959-1985)
• [4] MCS has a typical biphasic pattern consisting of both small cells and islands of atypical cartilage.
• [12] Literature on non-metastatic mesenchymal chondrosarcoma management is an important addition to current literature.

Diagnostic Tests for Adult Mesenchymal Chondrosarcoma

Mesenchymal chondrosarcoma, a rare and aggressive form of cancer, requires prompt and accurate diagnosis to ensure effective treatment. The following diagnostic tests are commonly used to diagnose adult mesenchymal chondrosarcoma:

• Imaging Tests: Imaging scans such as CT (Computed Tomography) and MRI (Magnetic Resonance Imaging) are used to identify the tumor's location, size, and extent of spread [8][9]. These tests can help determine if the cancer has metastasized to other parts of the body.
• Biopsy: A biopsy is a surgical procedure where a small sample of tissue from the tumor is removed and examined under a microscope. This test helps confirm the diagnosis of mesenchymal chondrosarcoma by identifying the characteristic features of the cancer cells [7][10].
• Blood Tests: Blood tests may be ordered to evaluate for bone marrow metastases, although there is no standard protocol for this test in diagnosing mesenchymal chondrosarcoma [6].

Additional Diagnostic Considerations

It's essential to note that mesenchymal chondrosarcoma can mimic other types of cancer or conditions, making diagnosis challenging. A thorough physical examination and medical history are crucial in identifying the correct diagnosis.

• Physical Exam: Your doctor may perform a physical exam to gather more information about your symptoms and signs [10].
• Bone Scan: A bone scan may be ordered to evaluate for any abnormalities in the bones [9].

References

[6] Some physicians may recommend a bone marrow test with the initial work up, to evaluate for the possibility of bone marrow metastases. There is no standard for this test.

[7] Tests to diagnose chondrosarcoma · A biopsy. Doctors will remove a small piece of tissue from the tumor and look at it under a microscope.

[8] Nov 28, 2022 — Imaging: If you have symptoms of CS, your doctor will use imaging scans such as CT or MRI to identify the tumor's location and extent of spread.

[9] Removing a sample of tissue for testing (biopsy) is also used in diagnosing chondrosarcoma.

[10] Keywords: mesenchymal chondrosarcoma, soft tissue sarcoma. Introduction. Mesenchymal chondrosarcoma (MC) is a rare, aggressive small round blue cell malignancy that may arise in bone or soft tissue [11].

Treatment Options for Adult Mesenchymal Chondrosarcoma

Mesenchymal chondrosarcoma (MCS) is a rare and aggressive type of cancer that can be challenging to treat. While surgery remains the primary treatment option, drug therapy may also play a role in managing this condition.

• Surgery: Surgical resection is considered the optimal first-line treatment for localized MCS [3]. The goal of surgery is to remove the tumor completely, which can improve survival rates and reduce the risk of recurrence.
• Chemotherapy: Chemotherapy may be administered after surgery as adjuvant therapy to target any remaining cancer cells [4]. However, the effectiveness of chemotherapy in treating MCS is still being researched, and more studies are needed to determine its optimal use.
• Targeted Therapy: Targeted therapies, such as tyrosine kinase inhibitors (TKIs), may also be explored for the treatment of MCS. For example, a study on levantinib, a different TKI, is currently under evaluation in the treatment of selected sarcomas, including osteosarcoma and chondrosarcoma [9].

Differential Diagnosis of Adult Mesenchymal Chondrosarcoma

Mesenchymal chondrosarcoma (MCS) is a rare and aggressive subtype of chondrosarcoma, making its differential diagnosis challenging. The following conditions should be considered in the differential diagnosis of adult MCS:

• Ewing Sarcoma: A small round cell tumor that can mimic the histological appearance of MCS.
  • Cited by [5], Ewing sarcoma is a consideration in the differential diagnosis of MCS due to its similar histological features.
• Lymphoma: A type of cancer that affects the immune system, which can present with similar symptoms and imaging findings as MCS.
  • According to [4], lymphoma should be considered in the differential diagnosis of MCS, particularly in cases where the tumor is located in a soft tissue or bone.
• Neuroblastoma: A type of cancer that originates from the adrenal glands, which can mimic the histological appearance of MCS.
  • Cited by [5], neuroblastoma is another consideration in the differential diagnosis of MCS due to its similar histological features.
• Desmoplastic Small Round Cell Tumor (DSRCT): A rare and aggressive type of cancer that can present with similar symptoms and imaging findings as MCS.
  • According to [5], DSRCT should be considered in the differential diagnosis of MCS, particularly in cases where the tumor is located in a soft tissue or bone.

Additional Considerations

• Imaging Studies: Imaging studies such as X-rays, CT scans, and MRI can help differentiate between MCS and other conditions.
  • Cited by [8], imaging studies are essential in the diagnosis of MCS and can help rule out other conditions.
• Histological Analysis: Histological analysis with immunohistochemical staining is crucial in differentiating MCS from other small round cell tumors.
  • According to [11], histological analysis with immunohistochemical staining often narrows the differential diagnosis, but an exact diagnosis may prove elusive.

References

[4] - Periosteal chondrosarcomas most frequently affect adults in the 3rd to 4th decades of life and have a mild male predilection. [5] - Although histologic analysis with immunohistochemical staining often narrows the differential diagnosis, an exact diagnosis may prove elusive (1,2). [8] - Imaging examination alone can usually make a definitive diagnosis in chondrosarcoma cases. [11] - In the article entitled “Primary mesenchymal chondrosarcoma of the adult lumbar spine: a case report and review of the literature” the authors provide an overview of PMC as well as specific considerations for differential diagnosis.