What question did this study set out to answer?

To characterize the clinicopathological, immunohistochemical, and molecular features of secondary rhabdomyosarcomas arising in patients previously irradiated for bilateral retinoblastoma.

November 1, 1998The American Journal of Surgical Pathology

Second Primary Rhabdomyosarcomas in Patients With Bilateral Retinoblastoma

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To characterize the clinicopathological, immunohistochemical, and molecular features of secondary rhabdomyosarcomas arising in patients previously irradiated for bilateral retinoblastoma.
Reviewed 6 pediatric cases (4 females, 2 males; age range 1.3–7.9 years) presenting with temporal muscle rhabdomyosarcomas within or adjacent to previous radiation fields.
Performed histological evaluation, RT-PCR for alveolar rhabdomyosarcoma and Ewing's sarcoma chimeric transcripts, and Western blot analysis.
Evaluated immunohistochemical staining for muscle markers, neurofilament (150 kd), and retinoblastoma-susceptibility gene protein compared to 17–20 sporadic primary rhabdomyosarcoma controls.
All 6 secondary tumors expressed desmin, muscle-specific actin, sarcomeric actin, myogenin, and neurofilament (6/6), with 4 showing rosette-like structures and 3 of 3 tested lacking alveolar or Ewing fusion transcripts.
Retinoblastoma-susceptibility gene protein was absent in all secondary tumors (0/6, 0%) compared to positive expression in 82% (14/17) of sporadic controls.
All 6 children were alive and well at 24 to 72 months of follow-up after tumor diagnosis.

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Implication

Case series reports second primary rhabdomyosarcomas lacking RB protein in children with bilateral retinoblastoma, highlighting the role of RB gene inactivation in post-radiation tumors.

Cite This Study

Hasegawa et al. (1998) studied this question.

synapsesocial.com/papers/6a898e98a4df84145ecb1621 https://doi.org/10.1097/00000478-199811000-00005

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Implication

Case series reports second primary rhabdomyosarcomas lacking RB protein in children with bilateral retinoblastoma, highlighting the role of RB gene inactivation in post-radiation tumors.

Second Primary Rhabdomyosarcomas in Patients With Bilateral Retinoblastoma

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Discussion

Implication

Cite This Study

Second Primary Rhabdomyosarcomas in Patients With Bilateral Retinoblastoma

Key Points

Discussion

Implication

Cite This Study

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