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small cell osteogenic sarcoma

Description

Small cell osteosarcoma, also known as small cell osteogenic sarcoma, is a rare and aggressive subtype of osteosarcoma, which is the most common type of cancer that starts in the bones.

Characteristics:

  • Small cell osteosarcoma consists of sheets of round cells that produce an osteoid matrix [1].
  • It is characterized by a monotonous proliferation of malignant small cells that deposit osteoid matrix [8].
  • The tumor cells are relatively small to intermediate in size, with high nuclear/cytoplasmic ratios, round nuclei, and minimal anisonucleosis [7].

Distinguishing features:

  • Small cell osteosarcoma can be confused with Ewing sarcoma/PNET due to its similar histologic features [9].
  • It is also distinguished from conventional osteosarcoma by its small cell morphology and scant production of mineralized matrix [14].

Incidence and demographics:

  • Small cell osteosarcoma is a rare form of osteosarcoma, with an incidence rate of 1.3% [3].
  • It accounts for approximately 1-2% of all osteosarcomas [14].
  • The average age of diagnosis for small cell osteosarcoma is not well established, but it is most often seen in teenagers, similar to conventional osteosarcoma [4].

Clinical presentation:

  • Small cell osteosarcoma typically presents as a radiolucent lesion with preferential sites of distal femur, proximal humerus, and proximal tibia [15].
  • The tumor can grow to invade and destroy healthy body tissue, and cancer cells can break away and spread to other parts of the body [10].

Overall, small cell osteosarcoma is a rare and aggressive subtype of osteosarcoma that requires prompt diagnosis and treatment.

Additional Characteristics

  • a rare and aggressive subtype of osteosarcoma
  • characterized by sheets of round cells producing an osteoid matrix
  • consists of malignant small cells depositing osteoid matrix
  • cytoplasmic ratios
  • PNET due to similar histologic features
  • distinguished from conventional osteosarcoma by small cell morphology and scant mineralized matrix production
  • accounts for 1-2% of all osteosarcomas
  • typically presents as a radiolucent lesion in the distal femur, proximal humerus, or proximal tibia
  • can grow to invade and destroy healthy body tissue and spread to other parts of the body

Signs and Symptoms

Small cell osteosarcoma, also known as high-grade surface osteosarcoma, is a rare and aggressive type of bone cancer. The signs and symptoms of this condition are often similar to those of other types of osteosarcoma.

Common symptoms:

  • Pain in the affected bone, which may initially come and go but gradually becomes more severe [6]
  • Swelling over a bone or a bony part of the body [7]
  • Joint pain [7]
  • Difficulty walking (if a leg bone is affected) [9]

Other possible symptoms:

  • Overwhelming fatigue
  • Unintended weight loss
  • Fever
  • A low red blood cell count (anemia)
  • Swelling and tenderness in the affected area

It's worth noting that small cell osteosarcoma can be challenging to diagnose, as it often presents with non-specific symptoms. A biopsy of the tumor is usually necessary to confirm the diagnosis.

Age distribution: Small cell osteosarcoma typically affects individuals at a younger age compared to other types of osteosarcoma. The majority of cases present before the age of 25 years [4].

Location: The most common sites for small cell osteosarcoma are around the knee or in the upper arm, but it can occur in other bones as well [11].

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Small Cell Osteogenic Sarcoma

Small cell osteogenic sarcoma (SCOS) is a rare and aggressive type of bone cancer. Diagnosing SCOS can be challenging, but various tests can help confirm the presence of this disease.

  • Imaging tests: Imaging tests such as X-rays, computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans can help identify the location and size of the tumor. These tests can also show if the cancer has spread to other parts of the body [6][10].
  • Bone scan: A bone scan is a test that uses small amounts of radioactive material to detect any abnormalities in the bones. This test can help identify if the cancer has spread to other bones or if there are any metastases [3][9].
  • Biopsy: A biopsy involves taking a sample of tissue from the tumor and examining it under a microscope. This is the most definitive way to diagnose SCOS, as it allows pathologists to examine the cells and confirm the presence of cancer [2][5].
  • Molecular testing: Molecular testing can help identify specific genetic mutations that are associated with SCOS. This can be useful in confirming the diagnosis and planning treatment [14].

It's worth noting that no specific laboratory tests are available for the diagnosis of bone sarcoma, including SCOS. However, some laboratory tests may be useful in the follow-up and monitoring of chemotherapy in patients with high-grade osteogenic and Ewing's sarcoma [13].

Treatment

Differential Diagnosis

The differential diagnosis of small cell osteosarcoma (SCOS) involves considering various other bone and soft tissue tumors that can mimic its appearance under the microscope.

Key Differential Diagnoses:

  • Ewing's Sarcoma: This is a common differential diagnosis for SCOS, particularly in cases where osteoid production is not evident. Ewing's sarcoma is characterized by small, round, blue cells with a high nuclear-to-cytoplasmic ratio.
  • Synovial Sarcoma: When mainly round cells are present, synovial sarcoma can be considered as a differential diagnosis for SCOS.
  • Metastatic Small Cell Carcinoma: In older patients, metastatic small cell carcinoma can mimic the appearance of SCOS.
  • Plasmacytoma or Primary Non-Hodgkin Lymphoma of Bone: These conditions can also be considered in the differential diagnosis of SCOS, particularly in older patients.

Other Considerations:

  • Dedifferentiated Chondrosarcoma or Osteosarcoma:

Additional Information

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