Thanks to Dr. Frayer and Dr. Abramson, I had the opportunity to visit the Ophthalmic Oncology Center at the Memorial Sloan-Kettering Cancer Center (MSKCC) and observe 20 Retinoblastoma cases in one day, given that the disease is extremely rare (~350 new cases of retinoblastoma diagnosed in the U.S. each calendar year). Dr. Abramson sees 15-25 retinoblastoma patients one day each week, including both established and new patients. On a given clinic day, about 2/3 of the patients are seen under general anesthesia, giving the physicians an opportunity to both observe the progression of the disease as well as treat the tumors immediately when necessary. I was introduced to a room full of people including a pediatric anesthesia team, pediatric neutrooncologists, genetic counselors, pediatric nurses (who specialize in anesthesia recovery for children), fellows, residents, and pre-med students.
Retinoblastoma is the most common primary intraocular malignancy of childhood, and the 7th most common pediatric cancer in the U.S. (Leukemia being the most common secondary intraocular cancer). The average age at diagnosis for retinoblastoma in the US is 18 month of age and the incidence of retinoblastoma is the same in boys and girls. Among RB patients, 30% of them have bilateral RB (both eyes are affected). Interestingly, only 10% of patients have a previous family history of RB. For sporadic retinoblastoma cases, leukocoria (white pupil) is the most common presenting sign, followed by esotropia and exotropia.
One of the young patients was previously diagnosed and treated in Korea. He was given three cycles of invasive chemotherapy which possibly would affect brain development later on and require bone marrow transplant after. His parents heard about Dr. Abramson and decided to travel all the way to the US, hoping to find a better (less invasive cure). Dr. Abramson detected tumor masses in both eyes and suggested intra-arterial chemotherapy (IAC), which has minimal systemic effects. IAC sends chemotherapy directly into a tumor through a catheter placed in the artery. The goal of IAC is to concentrate the drug inside the tumor and minimize the exposure to healthy tissues. I was able to observe the IAC procedure performed by Dr. Pierre Gobin the next day on this patient. I look forward to seeing this patient tomorrow for his follow-up visit.
CT scan of Retinoblastoma
Laser Therapy (Dr. Abramson)
For more information on Retinoblastoma and current treatments, you can visit Dr. Abramson’s website: www. Retinoblastoma.com.
Highlights of the rest of my week:
-Patients consultation with Dr. Nanus
-Splenectomy on a patient with Myelofibrosis
Myelofibrosis is a disorder in which bone marrow is replaced by fibrous tissue. It produces severe and painful splenomegaly. A splenectomy does not cure myelofibrosis but may be performed to relieve pain caused by the swelling of the spleen. In this case, splenectomy was performed to give the patient, a 29-year-old male a better life quality. The spleen removed by the surgeons turned out to be ~25 cm in length and ~15 cm in thickness compared to the less than 13 cm in length and less than 5cm in thickness criteria for normal spleens.
More blood spiking experiments to optimize the capture efficiency.