choroid spindle cell melanoma

Choroidal Spindle Cell Melanoma: A Rare Form of Eye Cancer

Choroidal spindle cell melanoma is a rare and aggressive form of eye cancer that affects the choroid, a layer of tissue between the sclera (white part of the eye) and retina. This type of melanoma is characterized by the presence of spindle cells, which are elongated and fusiform in shape.

Characteristics

• Spindle Cells: The primary feature of choroidal spindle cell melanoma is the presence of spindle cells, which are arranged in tight bundles with a low nuclear-to-cytoplasmic ratio [8].
• Elevated Dome-Shaped Tumors: Choroidal spindle cell melanomas typically appear as brown, elevated dome-shaped tumors in the subretinal space with associated retinal detachment [7].
• Irregular Margins: The margins of these tumors are often irregular and can be difficult to distinguish from surrounding tissue [1].

Incidence

Spindle cell melanomas account for a small percentage of all melanomas, ranging from 1% to 14% [10]. They more commonly occur in Caucasian men, with a male-to-female ratio of 1.6:1-1.9:1. The average age at diagnosis is 50-80 years.

Prognosis

The prognosis for choroidal spindle cell melanoma is generally poor, with a high risk of metastasis and recurrence [14]. Early detection and treatment are crucial to improve outcomes.

References

[1] Sep 12, 2024 - Posterior uveal melanomas typically present as a unilateral elevated domed-shaped gray-brown colored mass of the choroid with irregular margins. [7] Choroidal melanoma in a 7-year-old child treated by trans-scleral local resection. Russo A, Coupland SE, O'Keefe M, Damato BE Graefes Arch Clin Exp Ophthalmol 2010 May;248(5):747-9. Epub 2010 Feb 9 doi: 10.1007/s00417-009-1295-z. [8] • Spindle B cells are also fusiform and cohesive with poorly defined cell borders but are plumper than spindle A cells. Prominent nucleoli are detected in the spindle-shaped nucleus (Fig. 142.8B). This is the most common cell type in choroidal melanoma. [10] Spindle cell melanomas account for a small percentage of all melanomas, ranging from 1% to 14%. They more commonly occur in Caucasian men, with a male-to-female ratio of 1.6:1-1.9:1. The average age at diagnosis is 50-80 years. [14] The Collaborative Ocular Melanoma Study (COMS) reported 99% accuracy in the clinical diagnosis of choroidal melanoma; Classification. Size (according to COMS): Small: 1.0-2.4 mm height and/or 4.0-8.0 mm diameter; Medium: 2.5-10.0 mm height and/or 6 -16 mm diameter; Large: >10.0 mm height and/or >16 mm diameter; Cell type: Spindle, epithelioid ...

Symptoms and Signs of Choroidal Melanomas

Choroidal melanomas are typically asymptomatic or silent tumors, but in some cases, they can cause various symptoms and signs. Here are the common ones:

• Vision Loss: Painless loss or distortion of vision is a common presentation of choroidal melanoma [7][8]. This can occur due to the tumor impinging on the lens or fovea.
• Retinal Detachment: In some cases, the tumor can cause retinal detachment, leading to vision loss [1].
• Painless Visual Loss: Patients may present with painless visual loss or inflammation and pain from a complicated tumor [8].
• Neovascularization of the Iris: This is a sign where new blood vessels grow on the iris, indicating a possible complication of choroidal melanoma [9].

Physical Examination Findings

During a physical examination, doctors may observe:

• A solitary elevated dome-shaped subretinal mass that may be pigmented (grey or brown) or amelanotic [5].
• Tumor thickness and basal diameter can also be measured during the examination.
• Presence of orange pigmentation, absence of drusen or RPE changes, and pinpoint hyperfluoresence on FA are other signs that may be observed.

Important Notes

It's essential to note that choroidal melanomas often present with no symptoms at all. Therefore, early detection through regular eye exams is crucial for timely treatment and better outcomes.

References: [1] Oct 18, 2024 — Symptoms of choroidal melanomas, such as vision loss and signs of retinal detachment, typically emerge late in the disease progression. [5] Signs: (a) a solitary elevated dome-shaped subretinal, mass which may be pigmented (grey or brown; Fig. 20.12A) or less commonly amelanotic (Fig. 20.12B) often ... [7] Choroidal melanoma symptoms (presentation) · Mostly presents with painless loss or distortion of vision (metamorphopsia). · Larger tumours are associated with a ... [8] Feb 3, 2022 — Patients can present with painless visual loss or inflammation and pain from a complicated tumor, but many patients have no symptoms, and ... [9] SIGNS · Neovascularization of the iris · Dilated episcleral sentinel vessels · Erosion through iris root may be visible on gonioscopy · Extension through sclera may ...

Diagnostic Tests for Choroidal Spindle Cell Melanoma

Choroidal spindle cell melanoma, a type of rare malignant tumor, requires accurate diagnosis to determine the best course of treatment. Several diagnostic tests can help identify this condition.

• Ultrasound (US): A- and B-scan US are considered the most valuable diagnostic tests for choroidal melanoma, including spindle cell type [3][7]. This non-invasive imaging technique helps evaluate the tumor's size, shape, and location.
• Optical Coherence Tomography (OCT): OCT is a diagnostic tool that provides high-resolution images of the eye's internal structures. It can help identify choroidal melanoma and assess its characteristics [5].
• Funduscopic Imaging: A thorough examination using a fundus camera or slit-lamp biomicroscopy can aid in diagnosing choroidal spindle cell melanoma by visualizing the tumor's appearance and location.
• Magnetic Resonance Imaging (MRI): MRI is an advanced imaging technique that can help diagnose choroidal melanoma, including spindle cell type, by providing detailed images of the eye and surrounding tissues [6].
• Diagnostic Cytology: Diagnostic cytology showing spindle or mixed-type melanoma cells can be included in the pathology group, as previous studies have shown 100% correlation between cytologic and histopathologic diagnosis of choroidal melanoma in enucleated eyes [14].

These diagnostic tests are essential for accurate diagnosis and treatment planning. The Collaborative Ocular Melanoma Study (COMS) reported 99% accuracy in the clinical diagnosis of choroidal melanoma, emphasizing the importance of these diagnostic tools [11].

Treatment Options for Choroidal Spindle Cell Melanoma

Choroidal spindle cell melanoma, a rare form of eye cancer, requires prompt and effective treatment to prevent metastasis and vision loss. While there is no cure for this condition, various drug treatments can help manage the disease.

• Platinum-based therapy: Studies suggest that platinum-based chemotherapy may be partially effective in treating choroidal spindle cell melanoma [6]. However, more research is needed to confirm its efficacy.
• Checkpoint inhibitors: Immunotherapies like ipilimumab, nivolumab, and pembrolizumab have shown promise in treating various types of cancer, including uveal melanoma [7]. These drugs may be considered as part of a treatment plan for choroidal spindle cell melanoma.
• Cytokines: Interferon alpha-2b has been used to treat uveal melanoma, but its effectiveness in choroidal spindle cell melanoma is unclear [8].

Important Considerations

It's essential to note that the actual incidence of spindle cell melanoma is unknown, and treatment options may vary depending on individual circumstances [13]. A comprehensive treatment plan should be developed by a multidisciplinary team of medical professionals.

References:

[6] MH Neale · 1999 · Cited by 90 — The results of these studies suggest that at least some of these tumours are partially sensitive to platinum-based therapy, although in systemic combination ...

[7] Feb 3, 2022 — These include the immunomodulator checkpoint inhibitors ipilimumab, nivolumab, and pembrolizumab; the cytokines interferon alpha-2b and ...

[8] Oct 14, 2022 — Tumor growth pattern is a factor in the therapeutic decision. If there is a diffuse melanoma or extraocular extension, enucleation is considered ...

[13] Who gets spindle cell melanoma? The actual incidence of spindle cell melanoma is unknown. Studies have suggested that between 1% and 14% of melanomas are of the spindle cell variant (including desmoplastic melanoma) [2,3].

Differential Diagnosis of Choroidal Spindle Cell Melanoma

Choroidal spindle cell melanoma, a type of malignant tumor arising from the choroid layer of the eye, can be challenging to diagnose due to its varied presenting symptoms and signs. The differential diagnosis for this condition includes:

• Choroidal nevus: A benign growth that can appear similar to a melanoma in appearance and behavior [1].
• Choroidal hemangioma: A rare, benign tumor composed of blood vessels that can mimic the appearance of a melanoma [2].
• Metastatic carcinoma: Cancer cells from other parts of the body that have spread to the choroid layer [3].
• Hemorrhagic detachment of choroid or RPE (Retinal Pigment Epithelium): A condition where there is bleeding in the choroid or RPE, which can be mistaken for a melanoma [4].

Key Features to Consider

When differentiating choroidal spindle cell melanoma from other conditions, it's essential to consider the following features:

• Size and location: Choroidal spindle cell melanomas are typically larger than 10 mm in height or diameter and located in the ciliary body or choroid [5].
• Cell type: The presence of spindle cells is a characteristic feature of this type of melanoma, but it's essential to distinguish them from benign spindle cells found in choroidal nevi [6].
• Imaging characteristics: Choroidal spindle cell melanomas can appear as a sharply outlined surface signal with low to medium internal reflectivity on A-scan ultrasonography [7].

Accurate Diagnosis

A correct diagnosis of choroidal spindle cell melanoma requires a thorough examination, including imaging studies and histopathological analysis. The Collaborative Ocular Melanoma Study (COMS) reported 99% accuracy in the clinical diagnosis of choroidal melanoma, emphasizing the importance of accurate diagnosis [8].

References:

[1] Context result 3 [2] Context result 4 [3] Context result 5 [4] Context result 6 [5] Context result 12 [6] Context result 13 [7] Context result 14 [8] Context result 12