Entropion

Disease Description (Causes and Symptoms)

• Definition: A group of malignant or benign tumors occurring in the eye and surrounding tissues, including the eyelid, conjunctiva, sclera, retina, choroid, and orbit (collectively: ocular tumors/eye cancer).
• Main pathophysiology:
  • Choroidal (choriocapillaris membrane) melanoma: Arises from melanocytes. Hematogenous metastasis to the liver is common.
  • Retinoblastoma: Usually arises from retinal precursor cells in children (associated with hereditary RB1 mutations).
  • Conjunctival and eyelid tumors: Includes skin- and mucosa-derived carcinomas such as basal cell carcinoma, squamous cell carcinoma, sebaceous gland carcinoma, and conjunctival melanoma.
• Main causes and risk factors:
  • Long-term ultraviolet exposure (particularly related to skin, conjunctival, and choroidal melanoma).
  • Age (eyelid cancer is more common in older adults, while retinoblastoma is more common in infants and young children).
  • Skin pigmentation and ethnicity: The risk of basal cell carcinoma, squamous cell carcinoma, and melanoma is higher in people with fair skin.
  • Genetic factors: The hereditary proportion of retinoblastoma has been reported to be approximately 40%.
• Main symptoms:
  • Eye redness, swelling or a palpable mass at the tumor site, localized pain (relatively uncommon), decreased vision, diplopia or proptosis, restricted eye movement, pigmented lesions, retinal folds, or retinal hemorrhage on fundus examination, and leukocoria (white pupil or white reflex) in infants.

Treatment Methods (Standard Treatment, Medications, Procedures, Surgery, Lifestyle Management, and Precautions)

• Diagnostic and therapeutic approach: Individualized treatment according to the type, location, and size of the lesion and the presence or absence of metastasis.
• Local surgery
  • Local excision (eyelid and conjunctival tumors): Secure an adequate resection margin (reconstruction or oculoplastic surgery if necessary).
  • Removal of the eye (enucleation or exenteration): Used when the tumor is large, functional or cosmetic preservation is not possible, or the risk of metastasis is high.
  • For retinoblastoma, localized removal or vitreous surgery may be used, with limitations depending on the type.
• Local and minimally invasive treatment
  • Cryotherapy: Used for superficial lesions of the conjunctiva and eyelid.
  • Topical chemotherapy (e.g., topical medication for conjunctival tumors), local radiation (plaque brachytherapy), and other treatments.
  • Laser treatment (transpupillary thermotherapy, etc.) — used for small lesions.
• Radiation therapy
  • External-beam radiation, brachytherapy (plaque), proton therapy (proton beam), and other treatments — selected in consideration of the tumor location and side effects. Proton therapy is advantageous for protecting surrounding normal tissue.
• Systemic anticancer therapy
  • Retinoblastoma: Multidrug therapy (vincristine, carmustine/carboplatin, etc.) is routinely used in children.
  • Chemotherapy, targeted therapy, or immunotherapy may be considered for metastatic lesions or some conjunctival or choroidal metastases, depending on the tumor type.
• Systemic and local examinations and metastasis evaluation: Imaging (CT/MRI), liver function tests, and liver imaging (choroidal melanoma commonly metastasizes to the liver).
• Lifestyle management and precautions
  • Regular ophthalmology follow-up (visual acuity, fundus examination, and imaging) is necessary.
  • Ultraviolet protection (sunglasses, hats, etc.) is recommended, particularly for people at risk of melanoma.
  • Report immediately if infection, bleeding, changes in vision, or eye pain occurs before or after treatment.
• Risk of complications
  • Vision loss, structural and cosmetic damage to the eye, infection, systemic side effects related to radiation or chemotherapy, and organ dysfunction caused by metastasis (e.g., liver metastasis).

Outpatient Visit Checklist (Including Items to Bring and Example Questions)

• Items to bring
  • A list of all current medications, including prescription drugs, over-the-counter medicines, and supplements
  • Past medical history and surgical records, particularly ophthalmic or cancer-related surgery records
  • Family history, particularly childhood retinoblastoma or a history of cancer
  • Copies or CDs of recent ophthalmic examination results, including fundus photographs, ultrasound, and CT/MRI
  • A record of changes in symptoms, including when symptoms began, how they have progressed, and whether one or both eyes are affected
  • A record of recent weight changes and systemic symptoms, such as fever, jaundice, and abdominal pain
• Information to tell the doctor first during the outpatient visit
  • Possibility of pregnancy (radiation and some medications are contraindicated)
  • Drug allergies
  • Whether a parent or guardian will accompany a child patient (explanation of pediatric examinations and surgery is necessary)

General Example Questions to Ask the Doctor

• "What is the exact histopathological diagnosis (type) of this disease?"
• "Which tests, such as imaging or blood tests, are recommended, and what is the purpose of each?"
• "What are the expected effects and side effects of each treatment option?"
• "Is it possible to preserve vision with treatment? What will the cosmetic outcome be?"
• "How often and for how long will follow-up examinations be needed?"

Examples of Disease-Specific Questions

• (When choroidal melanoma is suspected) "Which tests, such as liver MRI/CT or liver function tests, should be performed to check for liver metastasis?"
• (For parents or guardians of a child with retinoblastoma) "Is genetic testing for hereditary disease (RB1 testing) necessary? What are the recommendations for testing the child’s brothers and sisters?"

Additional Information

• Main diagnostic tests: Dilated indirect ophthalmoscopy (fundus examination), ocular ultrasound, fluorescein fundus angiography, fundus photography, and CT/MRI (evaluation of intraocular and extraocular metastasis).
• Prognosis: Varies according to the type of lesion, timing of diagnosis, and presence or absence of metastasis. For example, the 5-year survival rate for choroidal melanoma has been reported to be approximately 65% and the 10-year survival rate approximately 50% (affected by tumor size and the presence or absence of metastasis).
• Relevant departments: Ophthalmology (retina and ocular oncology specialists), medical oncology, radiation oncology, pediatrics (for retinoblastoma), and oculoplastics (reconstruction), among others.

If necessary, providing specific pathology results and imaging findings can allow the diagnosis and treatment options to be summarized more accurately.