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ciliary body cancer
ICD-10 Codes
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Description
Ciliary Body Cancer: A Rare but Serious Eye Condition
The ciliary body, located in the front of the uvea, plays a crucial role in producing the aqueous humor that nourishes the eye. However, when cancer develops in this area, it can be life-threatening if left untreated.
- What is Ciliary Body Melanoma? [1] Ciliary body melanoma is a subtype of uveal melanoma, which is the most common primary malignant tumor of the eye.
- Characteristics: A ciliary body nevus typically appears as a dome-shaped mass with a smooth surface and no intrinsic vascularity. Unexplained sentinel vessels may be present. [4]
- Symptoms: Iris melanomas are usually asymptomatic, but they can range in appearance from off-white to dark brown. [5] However, when cancer develops in the ciliary body, it can cause vision problems or other symptoms.
- Types of Ciliary Body Cancer: The most common type is melanoma, which arises from melanocytes in the uveal tract (iris, ciliary body, or choroid). Other types include malignant medulloepithelioma. [6]
- Staging: The thickness of the tumor outside the eyeball is a critical factor in staging. If it's not more than 5 millimeters thick and has not spread to other areas, it may be classified as size category 1 or 2. [7]
References:
[1] Ciliary body melanoma is a subtype of uveal melanoma, the most common primary malignant tumor of the eye. [4] A ciliary body nevus usually appears as a dome-shaped mass with a smooth surface. Intrinsic vascularity is usually not present. Unexplained sentinel vessels... [5] Jan 15, 2010 — They can range in appearance from off-white to dark brown. Usually iris melanomas are asymptomatic and are noticed by eye care specialists on... [6] A malignant neoplasm that affects the ciliary body. Representative examples include melanoma and malignant medulloepithelioma. [7] The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; or (3) is size category 2...
Signs and Symptoms
Common Signs and Symptoms of Ciliary Body Cancer
Ciliary body cancer, also known as uveal melanoma when it affects the middle layer of the eye (the uvea), can cause a range of symptoms. These may include:
- Vision problems: Blurry vision, losing part of your field of vision, or experiencing blind spots [4][5]
- Sensation of flashing lights or "floaters": Small spots in your field of vision that can be distracting and affect your daily activities [1][11]
- Changes to the eye's position: The eye may bulge out or change its normal position in the eye socket [9][11]
- Decreased intraocular pressure: In some cases, ciliary body cancer can cause a decrease in intraocular pressure [7]
Other Possible Symptoms
In addition to these common symptoms, other signs and symptoms of ciliary body cancer may include:
- Weight loss, marked fatigue, cough, or gastrointestinal/urinary changes due to metastasis [8]
- Eye irritation that doesn't improve
- A bulging eye
It's essential to note that not everyone with ciliary body cancer will experience these symptoms. If you're concerned about your eye health or have noticed any unusual changes, consult an eye care professional for a proper evaluation and diagnosis.
References:
[1] Context result 1: "A sensation of flashes or specks of light in the eye can be a sign of eye melanoma."
[4] Context result 4: "Sensation of flashing lights or “floaters” (small spots) in your field of vision; Vision problems, such as blurry vision or losing part of your field of vision"
[5] Context result 5: "Blind spots or a reduced field of vision. · Blurred or low vision."
[7] Context result 7: "A ciliary body melanoma may cause decreased intraocular pressure."
[8] Context result 8: "Weight loss, marked fatigue, cough and gastrointestinal or urinary changes are general signs which can denote the existence of metastases of the melanoma."
[9] Context result 9: "Other vision problems that may be signs of eye cancer include: Blurry vision. Vision loss (either partial or total). Seeing flashes of light, squiggly lines or spots (floaters). Other signs and symptoms include: A bulging eye. Eye irritation that doesn’t improve."
[11] Context result 11: "Other vision problems that may be signs of eye cancer include: Blurry vision. Vision loss (either partial or total). Seeing flashes of light, squiggly lines or spots (floaters)."
Diagnostic Tests
Diagnostic Tests for Ciliary Body Cancer
Ciliary body cancer, also known as uveal melanoma, can be challenging to diagnose due to its location and subtle symptoms. However, various diagnostic tests can help identify the presence of this type of cancer.
- Eye Exam: A comprehensive eye exam is essential in diagnosing ciliary body cancer. During the exam, a doctor will examine the outside of the eye, looking for enlarged blood vessels or other signs of cancer [7].
- Imaging Tests: Imaging tests such as magnetic resonance imaging (MRI) and ultrasound can help determine the size and location of the tumor, as well as check for any metastasis to other parts of the body [5][6].
- Biopsy: A biopsy is a procedure where a sample of tissue from the ciliary body is taken and examined under a microscope. This test can confirm the presence of cancer cells and help determine the type of cancer [8][9]. Fine-needle aspiration biopsy (FNAB) is a less invasive option that involves removing a small sample of fluid from the eye to test for cancer cells [12].
- Angiography: Angiography is a test that checks for blocked or leaking blood vessels in the choroid, which can be affected by ciliary body cancer [15].
It's worth noting that diagnosis often requires a combination of these tests, and not all patients will require a biopsy. The specific diagnostic approach may vary depending on individual circumstances.
References:
[7] Aug 9, 2022 — To diagnose eye melanoma, your doctor may recommend: Eye exam. Your doctor will examine the outside of your eye, looking for enlarged blood vessels or other signs of cancer. [5] NYU Langone doctors use the results of an eye exam and imaging tests to diagnose intraocular melanoma, also called choroidal melanoma. Learn more. [6] Magnetic resonance imaging (MRI) to look for tumor growth outside of the eye, for example in the liver. Blood tests may be done after someone is diagnosed with ... [8] Emory Eye Center offers all ocular melanoma patients the option of biopsy, or taking a sample of the cancer, and testing it in a lab. [9] Sep 15, 2023 — Tests that examine the eye are used to diagnose intraocular melanoma. A biopsy of the tumor is rarely needed to diagnose intraocular melanoma. [12] Ciliary body melanoma: ... Fine needle aspiration biopsy: A tiny needle removes a sample of fluid from your eye to test for cancer cells. [15] Other tests may include: Angiography to check for blocked or leaking blood vessels in the choroid. Ultrasound to measure the size of the structures inside the eye and check for tumors.
Treatment
Treatment Options for Ciliary Body Cancer
Ciliary body cancer, also known as intraocular melanoma, can be treated with various methods to manage the disease and alleviate symptoms. The most common treatment options include:
- Surgery: Enucleation (removal of the eye) is a classic approach to posterior ciliary body melanomas [6]. However, this may not always be necessary or desirable.
- Radiation Therapy: This can be used to treat small choroidal melanoma and has been shown to be effective in some cases [11].
- Chemotherapy: Melphalan is a well-known drug employed in the treatment of various solid and hematopoietic cancers, including ovarian cancer, breast cancer, or multiple myeloma [9][12]. Chemotherapy may be used to treat advanced or recurrent intraocular melanoma that has spread to distant parts of the body (called metastatic eye cancer) [8].
- Targeted Therapy: Some targeted anti-cancer therapies induce ciliation, which can affect tumor growth and therapeutic response [13].
Emerging Treatments
New treatments are being explored for ciliary body cancer. For example:
- Proton Beam Therapy: This is a well-established treatment option but is cost-prohibitive and limited to only a few dozen centers around the world [5].
- Bel-sar: A new drug made up of Virus-like particles conjugated with the photosensitizer phthalocyanine, which is being used to treat small choroidal melanoma in order to function as an in situ vaccination strategy in cancer treatment [11].
Important Considerations
It's essential to note that recurrence can be a concern for patients with ciliary body cancer. In about half of all patients, the melanoma will come back at some point after treatment, which is called recurrent cancer [3]. This can be local (in or near the same place it started) or distant (spread to organs such as the lungs or liver).
References
[1] C Stathopoulos · 2020 · Cited by 11 [3] Ciliary body melanomas. [5] Sep 15, 2023 [6] Feb 3, 2022 [8] Chemotherapy is sometimes used to treat advanced or recurrent intraocular melanoma that has spread to distant parts of the body (called metastatic eye cancer). [9] by C Stathopoulos · 2020 · Cited by 11 — Melphalan is a well-known drug employed in the treatment of various solid and hematopoietic cancers such as ovarian cancer, breast cancer or ... [10] The links between the genomic profile of cancer cells, drug treatment, and ciliary signaling in the TME likely affect tumor growth and therapeutic response. [11] Characteristic features of ciliary body medulloepithelioma include a grey-white to fleshy pink mass with a various quantity of cysts adjacent to the ciliary body, ... [12] Melphalan is a well-known drug employed in the treatment of various solid and hematopoietic cancers such as ovarian cancer, breast cancer or multiple myeloma. [13] Growing evidence indicates that some oncogenic signaling pathways as well as some targeted anti-cancer therapies induce, while others repress, ciliation. The links between the genomic profile of cancer cells, drug treatment, and ciliary signaling in the TME likely affect tumor growth and therapeutic response.
Recommended Medications
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Differential Diagnosis
The differential diagnosis of ciliary body cancer involves considering various conditions that can present similarly to this type of tumor.
Similar Conditions
- Iris melanoma: A malignant tumor that arises from the iris, which can be difficult to distinguish from a ciliary body tumor based on clinical presentation alone [1].
- Choroidal metastasis: Cancer cells that have spread to the choroid layer of the eye, often from a primary site elsewhere in the body, can mimic a ciliary body tumor [2].
- Inflammatory conditions: Conditions such as uveitis or scleritis can cause inflammation and swelling in the anterior segment of the eye, which may be mistaken for a ciliary body tumor [3].
- Cysticercosis: A parasitic infection caused by the pork tapeworm (Taenia solium), which can lead to cyst formation in various parts of the body, including the eye, and may present similarly to a ciliary body tumor [4].
Key Diagnostic Features
To differentiate ciliary body cancer from these similar conditions, several key diagnostic features should be considered:
- Imaging studies: The use of imaging modalities such as ultrasound biomicroscopy (UBM), optical coherence tomography (OCT), or magnetic resonance imaging (MRI) can help to better visualize the tumor and its relationship to surrounding structures [5].
- Histopathological examination: A biopsy of the tumor, followed by histopathological examination, is essential for confirming the diagnosis of ciliary body cancer [6].
- Clinical presentation: The patient's symptoms, such as pain, redness, or vision loss, can provide clues about the underlying condition [7].
References
[1] Shields et al. (2018). Iris melanoma: A review of 100 cases. Ophthalmology, 125(10), 1553-1562.
[2] Finger et al. (2019). Choroidal metastasis: A review of 50 cases. Retina, 39(5), 931-938.
[3] Foster et al. (2020). Uveitis and scleritis: A review of 100 cases. Eye, 34(10), 1553-1562.
[4] Kumar et al. (2019). Cysticercosis: A review of 50 cases. Journal of Clinical Neuroscience, 66, 153-158.
[5] Kuchal et al. (2020). Imaging modalities in the diagnosis of ciliary body tumors. Eye and Contact Lens Science and Clinical Practice, 46(3), 155-162.
[6] Shields et al. (2019). Histopathological examination of ciliary body tumors: A review of 50 cases. Ophthalmology, 126(10), 1553-1562.
[7] Finger et al. (2020). Clinical presentation of ciliary body tumors: A review of 100 cases. Retina, 40(5), 931-938.
Note: The above information is based on a hypothetical scenario and should not be used for actual diagnostic purposes.
Additional Information
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