clivus chondroid chordoma

Clivus Chondroid Chordoma: A Rare Type of Tumor

A clivus chondroid chordoma is a rare type of tumor that occurs in the clivus, a portion of bone at the base of the skull. This variant of conventional chordoma contains areas in which the matrix has the appearance of hyaline cartilage [3]. It makes up 5% to 15% of all chordoma cases and often forms in the base of the skull.

Characteristics

• The chondroid variety shows areas of chondroid differentiation within an otherwise histologically indistinct conventional chordoma.
• This type of tumor is characterized by a mix of abnormal cells, making it a rare and aggressive form of cancer [2].
• Clivus chondroid chordomas are typically slow-growing but locally invasive and locally aggressive tumors [6].

Key Features

• Destructive midline mass centered in clivus with high T2 signal intensity and heterogeneous contrast enhancement.
• Irregular intratumoral calcifications may be present.

Prognosis

• The prognosis for clivus chondroid chordoma is generally better than other subtypes, but it still carries a significant risk of recurrence and metastasis [14].

References:

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Clivus Chondroid Chordoma Signs and Symptoms

Chondroid chordoma, a variant of chordoma, is a rare type of cancer that affects the base of the skull. The symptoms of clivus chondroid chordoma can vary depending on the size and location of the tumor.

• Headaches: A common symptom of clivus chondroid chordoma, headaches can range from mild to severe and may be accompanied by other symptoms such as double vision or numbness in the face.
• Double Vision (Diplopia): As the tumor grows, it can put pressure on the adjacent areas of the brain, leading to double vision. This symptom is often associated with clival chordomas.
• Numbness or Weakness: The tumor can cause numbness or weakness in the face, arms, or legs, depending on its location and size.
• Trouble Swallowing: As the tumor grows, it can put pressure on the nerves that control swallowing, leading to difficulty swallowing food or liquids.

Other Possible Symptoms

• Pain or discomfort in the neck or back
• Changes in facial sensation or movement
• Voice changes or hoarseness
• Weakness or numbness in the arms or legs

It's essential to note that these symptoms can vary depending on the individual and the specific location of the tumor. If you're experiencing any of these symptoms, it's crucial to consult with a healthcare professional for proper diagnosis and treatment.

References:

[3] Clival chordomas are generally benign and slow-growing, but as they develop they can cause significant damage to surrounding areas of the brain... (Search Result 3)

[5] For skull base chordomas, common symptoms include double vision, headaches, and neck pain. If large enough, they may affect facial sensation or movement, voice... (Search Result 5)

[15] As the chordoma grows, it puts pressure on the adjacent areas of the brain or spinal cord, leading to the signs and symptoms of the disorder... (Search Result 15)

Diagnostic Tests for Clivus Chondroid Chordoma

Clivus chondroid chordoma, a variant of chordoma, can be challenging to diagnose due to its rarity and similarity in appearance to other bone tumors. However, various diagnostic tests can help confirm the presence of this condition.

• Imaging tests: Imaging studies such as X-rays, CT scans, and MRI scans are often used to evaluate the extent of bony destruction and osseous invasion [6]. CT scans are particularly useful in assessing the degree of bone involvement and detecting patterns of calcification within the lesion [9].
• Biopsy: A biopsy is a crucial diagnostic tool for clivus chondroid chordoma. It involves removing a small sample of tissue from the tumor, which is then examined by a pathologist under a microscope to confirm the presence of cancer cells [12]. Fine-needle aspiration biopsy (FNAB) or Tru-Cut needle biopsy may be obtained for diagnostic purposes [5].
• Blood tests: Blood tests are not typically required for the evaluation of chordoma, except as needed to rule out other conditions [4].

Key Diagnostic Features

Chondroid chordomas are characterized by islands of cartilage formation, whereas classic chordomas consist of tumor cells in lacunae that are embedded in hyaline cartilage-like stroma [10]. A lobulated high-T2 enhancing clival mass originating with no calcifications is a key diagnostic feature [10].

References

[4] May 3, 2022 — Evaluation of chordoma revolves around imaging and biopsy. No laboratory studies are required for evaluation of chordoma, except as needed ...

[5] Dec 5, 2022 — A fine-needle aspiration biopsy (FNAB) or Tru-Cut needle biopsy may be obtained for diagnostic purposes. Both biopsies may require radiologic ...

[6] by E Soule · 2021 · Cited by 16 — CT is superior to MRI in evaluating bony destruction and osseous invasion, and should be utilized to delineate the extent of bony involvement.

[9] CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion.

Treatment Options for Clivus Chondroid Chordoma

Clivus chondroid chordoma, a variant of chordoma, is a rare and aggressive tumor that primarily affects the base of the skull. While surgery remains the primary treatment option, drug treatment plays an ancillary role in managing this condition.

• Chemotherapy: Conventional chemotherapy has limited efficacy in treating chordomas, including clivus chondroid chordoma [4][5]. However, some studies have reported responses to erlotinib and cetuximab in patients with refractory chordoma [3].
• Targeted Therapy: Molecular targeted drugs, such as gefitinib and gefitinib-cetuximab combination, may offer a potential therapeutic option for clivus chondroid chordoma. However, their role remains ancillary compared to surgery and radiotherapy [11].

Current Treatment Landscape

The mainstay of treatment for clivus chondroid chordoma remains surgical resection with adjuvant radiotherapy. The goal is to achieve complete tumor excision with clear margins, which is often technically challenging due to the anatomic location [9][10]. While drug treatment may be considered in certain cases, its role is limited, and further research is needed to explore its potential benefits.

References

[3] Lindén O, Stenberg L, Kjellén E: Regression of cervical spinal cord compression in a patient with chordoma following treatment with cetuximab and gefitinib. Acta Oncol 48 (1): 158-9, 2009. [4] by IS Erazo · 2018 · Cited by 25 — Chordomas are resistant to chemotherapy, and thus, the treatment of choice lies in complete surgical resection usually followed by adjuvant radiotherapy [6-8]. [5] by EI Gogo-ogute · 2022 · Cited by 2 — The mainstay of treatment remains surgical resection with adjuvant radiotherapy with a limited role of conventional chemotherapy. For locally advanced tumors, ... [9] May 3, 2022 — Optimal therapy typically involves complete surgical re

Differential Diagnosis of Clivus Chondroid Chordoma

Chondroid chordoma, a variant of conventional chordoma, is a rare bone tumor that arises in the clivus. When diagnosing this condition, it's essential to consider other entities that may present similarly. Here are some key points to consider:

• Benign lesions: Clivus chondroid chordoma can be confused with benign lesions such as ecchordosis physaliphora (EP) and neuroenteric cyst [6].
• Chondrosarcoma: This is a primary bone tumor that may be mistaken for chondroid chordoma, but will be negative for epithelial markers (cytokeratin / EMA) [4].
• Invasive pituitary macroadenoma: This condition can arise in the sella and involve the pituitary gland, extending into the sphenoid sinus but not the prepontine cistern [10].
• Skull base chondrosarcoma: This is a type of sarcoma that arises off midline at the petro-occipital fissure and can be considered in the differential diagnosis [10].

Key Diagnostic Features

To differentiate clivus chondroid chordoma from other entities, consider the following features:

• Histological subtype: Chondroid chordoma has a better prognosis than conventional chordoma, which is intermediate, while dedifferentiated chordoma has the worst prognosis [2].
• Imaging studies: Diffusion-weighted MR imaging may be useful in assessing clival tumors, particularly in differentiating chordoma from chondrosarcoma [5].

Importance of Histopathological Evaluation

While radiographic imaging can suggest a diagnosis, definitive certainty requires histopathological evaluation. CT-guided needle biopsy and tissue diagnosis are often recommended prior to definitive surgical intervention [13]. Consultation with an experienced chordoma pathologist will aid in making the correct diagnosis.

References:

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