Failure to Diagnose & Delay in Treatment of Pediatric Retinoblastoma (Retina Cancer)

Evaluate breaches in the standard of care by evaluating the questions and answers herein below to determine whether or not there was a Failure to Diagnose & Delay in Treatment of Pediatric Retinoblastoma (Retina Cancer)

Answer the following questions to find out if the standard of care was followed for your case.

  • Did the patient have a positive family history for retinoblastoma?
    Yes
    No
    Why is this important?
    Family history is an important clue when investigating an inherited cancer syndrome. It is the pediatrician's responsibility to be able to recognize the major cancer-predisposing syndromes by taking a thorough medical and family history.
    (2002) 49 EPDCNA 6 1393-1413

  • Was the child evaluated for retinoblastoma shortly after birth? Why is this important?
    Answer this question
    
All children with an immediate family history of retinoblastoma must be screened by an ophthalmologist soon after birth and then at regularly scheduled frequent intervals.

    (2005) 9 JAAPOS 4 383-385 



  • Was a red reflex examination performed? Why is this important?
    Answer this question
    
Smaller tumors produce an abnormal light reflex that is detectable by examination of the red reflex with the direct ophthalmoscope.

    (2005) 9 JAAPOS 4 383-385 



  • With which of the following symptoms did the patient present? (Choose all that apply) Why is this important?
    Answer this question
    
The most common presenting symptoms are leukocoria, (white pupil) and strabismus.

    (2005) 9 JAAPOS 4 383-385 



  • Was the patient immediately referred to an ophthalmologist? Why is this important?
    Answer this question
    
Because leukocoria, (white colored pupil) is the most common finding of retinoblastoma, emergent referral to an ophthalmologist is necessary. The child with strabismus must also be referred urgently.

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  • Was an indirect ophthalmoscopy performed? Why is this important?
    Answer this question
    
The diagnosis of retinoblastoma can usually be made during a dilated indirect ophthalmoscopic examination that is performed under anesthesia. The characteristic finding is a chalky, white-gray retinal mass with a soft, friable consistency.

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  • Which of the following diagnostic investigations were initiated? (Choose all that apply) Why is this important?
    Answer this question
    
Orbital ultrasonography, CT, or MRI are necessary to evaluate the extent of intraocular disease and extra-ocular spread. MRI allows for better evaluation of optic nerve involvement.

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  • Was molecular genetic testing recommended? Why is this important?
    Answer this question
    
Molecular genetic testing is suggested for all affected patients. Genetic testing can improve risk prediction and prevent unnecessary clinical screening of the patient’s siblings.

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  • Did the patient present with signs and symptoms of metastasis? Why is this important?
    Answer this question
    
Signs and symptoms of metastases may include anorexia or weight loss, vomiting, headache, neurologic impairment, orbital mass or soft tissue mass.

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  • What type of retinoblastoma was diagnosed? (Choose all that apply) Why is this important?
    Answer this question
    
The choice of treatment depends upon tumor type, tumor size, tumor location and the patient’s age.

    (2006) 42 ESEUJC 13 2092-2102 



  • Was enucleation followed by adjuvant chemotherapy? Why is this important?
    Answer this question
    
Enucleation usually is indicated for large tumors. In the majority of children with unilateral disease, excellent survival rates can be achieved by enucleation (removal of the eye) followed by adjuvant chemotherapy.

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  • Did the clinician advise long-term follow-up visits? Why is this important?
    Answer this question
    
In addition to the risks associated with metastatic spread from the original tumor, survivors of retinoblastoma require lifetime follow-up because of their propensity to develop (and potentially die from) secondary malignancies.

    (2006) 42 ESEUJC 13 2092-2102 



  • Which of the following advanced investigations were performed? (Choose all that apply) Why is this important?
    Answer this question
    
In the usual case of endophytic retinoblastoma (in which the tumor grows toward the vitreous cavity) where the optic nerve can be seen, the risk associated with metastatic screening tests outweighs their value.

    (2006) 42 ESEUJC 13 2092-2102 



  • Which of the following treatment measures were initiated? (Choose all that apply) Why is this important?
    Answer this question
    
For children with bilateral disease, treatment is directed at saving their vision and preserving their life.

    (2006) 42 ESEUJC 13 2092-2102 



  • Was external beam radiotherapy performed? Why is this important?
    Answer this question
    
External beam radiotherapy is reserved for children with bilateral retinoblastoma who develop recurrent disease after chemotherapy.

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