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In vivo growth of retinoblastoma in a newborn infant.

Shah PK, Narendran V, Kalpana N - Indian J Ophthalmol (2010 Sep-Oct)

Bottom Line: Retinoblastoma is a rare malignancy of the retina seen exclusively in children.It is known to cause rapid growth inside the eye and hence treatment should be started as soon as it is diagnosed.This case emphasizes that immediate treatment is warranted once this rare disease is diagnosed.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Retina and Ocular Oncology Department, Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India.

ABSTRACT
Retinoblastoma is a rare malignancy of the retina seen exclusively in children. It is known to cause rapid growth inside the eye and hence treatment should be started as soon as it is diagnosed. We report a case in a five-day-old infant in whom treatment (chemotherapy) was delayed by a month due to high bilirubin levels secondary to physiological jaundice, which gave us the unique opportunity to measure the growth of the tumor over a month. This case emphasizes that immediate treatment is warranted once this rare disease is diagnosed.

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B scan picture of the same eye on Day 16 showing the mass increased in size
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Figure 0004: B scan picture of the same eye on Day 16 showing the mass increased in size

Mentions: A five-day-old newborn, with a positive family history of retinoblastoma was referred to our institute with a possibility of the same in left eye (LE). On fundus examination right eye (RE) showed three small tumors (< 1 mm in size), two above the supero-temporal arcade vessels and one nasal to the disc. LE showed a solid elevated mass over the macula. The horizontal diameter of that mass was 5.8 mm; vertical diameter was 5.6 mm while the height on B scan was 3.1 mm [Figs. 1a and 1b]. The area of the tumor base was 24.5 mm2 and it was 0.24 mm from the temporal margin of the optic disc. A smaller mass with horizontal diameter of 1.7 mm and vertical diameter of 1.5 mm was also present touching the main mass in the supero-temporal quadrant. Transpupillary thermotherapy (TTT) was applied to all the three tumors in RE while systemic chemotherapy was decided for the LE. Unfortunately, on referring to our oncologist, the child was found to have physiological jaundice with the bilirubin levels at 22 mg/dl. The child was subjected to phototherapy and chemotherapy was deferred by two weeks. On Day 16 the bilirubin levels were still high at 5.7 mg/dl and the tumor size in LE had increased. Now, the horizontal diameter was 7.2 mm, vertical was 6.8 mm, height was 3.5 mm [Figs. 2a and 2b] and basal area was 39.8 mm2. The tumor was now touching the temporal disc margin. The smaller mass was almost fused with the main tumor. Another small tumor (< 1 mm in diameter) was seen nasal to disc for which TTT was done. Brachytherapy was deferred as there was more than one mass and it would take two to three weeks for the iodine 125 (I125) seeds to be prepared. Moreover our brachytherapy experience was limited, especially in newborns. To start some form of treatment immediately, we decided to give half-dose carboplatin. The normal dose is 18.6 mg/kg. We administered 9.3 mg/kg. The tumors in the RE were scarred. Two weeks after the half-dose carboplatin (Day 35 from initial diagnosis) was started the horizontal diameter had increased to 8.7 mm, vertical to 7.8 mm, height to 5.6 mm [Figs. 3a and 3b] and basal area to 55.7 mm2. The tumor was now overlapping the temporal disc margin. The nasal tumor had scarred post TTT laser. Fortunately, at that point the bilirubin levels had become normal and full-dose three-drug chemotherapy (carboplatin, etoposide, vincristine) was started immediately. The tumor responded well and after completing six cycles of chemotherapy, at six-month follow-up, it had regressed into a calcified mass (Type 1 regression). This delay in starting the treatment gave us the unique opportunity to study the growth of the tumor over a month in vivo.


In vivo growth of retinoblastoma in a newborn infant.

Shah PK, Narendran V, Kalpana N - Indian J Ophthalmol (2010 Sep-Oct)

B scan picture of the same eye on Day 16 showing the mass increased in size
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2992920&req=5

Figure 0004: B scan picture of the same eye on Day 16 showing the mass increased in size
Mentions: A five-day-old newborn, with a positive family history of retinoblastoma was referred to our institute with a possibility of the same in left eye (LE). On fundus examination right eye (RE) showed three small tumors (< 1 mm in size), two above the supero-temporal arcade vessels and one nasal to the disc. LE showed a solid elevated mass over the macula. The horizontal diameter of that mass was 5.8 mm; vertical diameter was 5.6 mm while the height on B scan was 3.1 mm [Figs. 1a and 1b]. The area of the tumor base was 24.5 mm2 and it was 0.24 mm from the temporal margin of the optic disc. A smaller mass with horizontal diameter of 1.7 mm and vertical diameter of 1.5 mm was also present touching the main mass in the supero-temporal quadrant. Transpupillary thermotherapy (TTT) was applied to all the three tumors in RE while systemic chemotherapy was decided for the LE. Unfortunately, on referring to our oncologist, the child was found to have physiological jaundice with the bilirubin levels at 22 mg/dl. The child was subjected to phototherapy and chemotherapy was deferred by two weeks. On Day 16 the bilirubin levels were still high at 5.7 mg/dl and the tumor size in LE had increased. Now, the horizontal diameter was 7.2 mm, vertical was 6.8 mm, height was 3.5 mm [Figs. 2a and 2b] and basal area was 39.8 mm2. The tumor was now touching the temporal disc margin. The smaller mass was almost fused with the main tumor. Another small tumor (< 1 mm in diameter) was seen nasal to disc for which TTT was done. Brachytherapy was deferred as there was more than one mass and it would take two to three weeks for the iodine 125 (I125) seeds to be prepared. Moreover our brachytherapy experience was limited, especially in newborns. To start some form of treatment immediately, we decided to give half-dose carboplatin. The normal dose is 18.6 mg/kg. We administered 9.3 mg/kg. The tumors in the RE were scarred. Two weeks after the half-dose carboplatin (Day 35 from initial diagnosis) was started the horizontal diameter had increased to 8.7 mm, vertical to 7.8 mm, height to 5.6 mm [Figs. 3a and 3b] and basal area to 55.7 mm2. The tumor was now overlapping the temporal disc margin. The nasal tumor had scarred post TTT laser. Fortunately, at that point the bilirubin levels had become normal and full-dose three-drug chemotherapy (carboplatin, etoposide, vincristine) was started immediately. The tumor responded well and after completing six cycles of chemotherapy, at six-month follow-up, it had regressed into a calcified mass (Type 1 regression). This delay in starting the treatment gave us the unique opportunity to study the growth of the tumor over a month in vivo.

Bottom Line: Retinoblastoma is a rare malignancy of the retina seen exclusively in children.It is known to cause rapid growth inside the eye and hence treatment should be started as soon as it is diagnosed.This case emphasizes that immediate treatment is warranted once this rare disease is diagnosed.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Retina and Ocular Oncology Department, Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India.

ABSTRACT
Retinoblastoma is a rare malignancy of the retina seen exclusively in children. It is known to cause rapid growth inside the eye and hence treatment should be started as soon as it is diagnosed. We report a case in a five-day-old infant in whom treatment (chemotherapy) was delayed by a month due to high bilirubin levels secondary to physiological jaundice, which gave us the unique opportunity to measure the growth of the tumor over a month. This case emphasizes that immediate treatment is warranted once this rare disease is diagnosed.

Show MeSH
Related in: MedlinePlus