Orbital sarcomas in retinoblastoma patients

Baker, Meredith S.; McConnell, Lindsay K.; Kleinberg, Teri T.; Shriver, Erin M.; Bilyk, Jurij R.; Allen, Richard C.

doi:10.1097/icu.0000000000000295

Cited by 10 publications

(6 citation statements)

References 28 publications

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“…The average risk to develop a SPT after radiation therapy in hereditary retinoblastoma patients is approximately 1% per year [27]. As the majority of SPTs occur in the radiation field the physical properties of protons compared to photons with a steep dose fall-off at the end of the irradiation path and a minimal penumbra may significantly reduce the risk of radiogenic induced tumors.…”

Section: Discussionmentioning

confidence: 99%

Feasibility of Proton Beam Therapy as a Rescue Therapy in Heavily Pre-Treated Retinoblastoma Eyes

Biewald

Kiefer

Geismar³

et al. 2021

Cancers

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Despite the increased risk of subsequent primary tumors (SPTs) external beam radiation (EBRT) may be the only therapeutic option to preserve a retinoblastoma eye. Due to their physical properties, proton beam therapy (PBT) offers the possibility to use the effectiveness of EBRT in tumor treatment and to decisively reduce the treatment-related morbidity. We report our experiences of PBT as rescue therapy in a retrospectively studied cohort of 15 advanced retinoblastoma eyes as final option for eye-preserving therapy. The average age at the initiation of PBT was 35 (14–97) months, mean follow-up was 22 (2–46) months. Prior to PBT, all eyes were treated with systemic chemotherapy and a mean number of 7.1 additional treatments. Indication for PBT was non-feasibility of intra-arterial chemotherapy (IAC) in 10 eyes, tumor recurrence after IAC in another 3 eyes and diffuse infiltrating retinoblastoma in 2 eyes. Six eyes (40%) were enucleated after a mean time interval of 4.8 (1–8) months. Cataract formation was the most common complication affecting 44.4% of the preserved eyes, yet 77.8% achieved a visual acuity of >20/200. Two of the 15 children treated developed metastatic disease during follow-up, resulting in a 13.3% metastasis rate. PBT is a useful treatment modality as a rescue therapy in retinoblastoma eyes with an eye-preserving rate of 60%. As patients are at lifetime risk of SPTs consistent monitoring is mandatory.

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Section: Discussionmentioning

confidence: 99%

Feasibility of Proton Beam Therapy as a Rescue Therapy in Heavily Pre-Treated Retinoblastoma Eyes

Biewald

Kiefer

Geismar³

et al. 2021

Cancers

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“…11 Finally, the mass was within the field of his prior radiation therapy, which is a well-established risk factor for sarcoma development (most commonly leiomyosarcomas). 17 …”

Section: Discussionmentioning

confidence: 99%

Necrotizing myositis in a rectus muscle arising in the setting of long-standing Langerhans cell histiocystosis and recent dabrafenib treatment

Landingham

Puccetti

Potter

et al. 2020

American Journal of Ophthalmology Case Reports

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Purpose to describe an unusual case of necrotizing myositis in a rectus muscle, possibly related to BRAF inhibitor therapy. Observations An 18-year old man with neurodegenerative Langerhans cell histiocytosis (LCH), recently started on the BRAF inhibitor dabrafenib, presented with right eye pain. Magnetic resonance imaging (MRI) orbits revealed a rectus muscle mass concerning for LCH recurrence or malignancy. Dabrafenib was stopped, and incisional biopsy of the mass was performed. The mass was absent on post-operative MRI, so no further treatment was pursued. Histopathologic evaluation was initially concerning for sarcoma, but on further analysis, appeared more consistent with necrotizing myositis. The mass did not recur, nor did the patient develop other signs or symptoms concerning for myositis or malignancy over a 24-month follow-up period. Conclusions Necrotizing myositis has not been previously described in a rectus muscle or with BRAF inhibitor use, though myalgias and malignancies are established side effects. Necrotizing myositis may masquerade as sarcoma and should be on the differential diagnosis for a new mass in the setting of dabrafenib therapy.

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“…Increasing radiation dose and younger age at the time of radiotherapy increase a patient's risk of developing a secondary neoplasm. Patients who undergo radiotherapy during the first year of life are at increased risk for the development of a second neoplasm compared to patients who undergo radiotherapy after 1 year of age [7]. …”

Section: Discussionmentioning

confidence: 99%

“…Currently, there is no established standard of care for long-term surveillance of irradiated retinoblastoma. Early detection allows a better chance for complete tumor resection with negative surgical margins, generally considered the most important prognostic factor related to increased survival for UPS [7]. This is paramount since the leading cause of early related death in patients with hereditary retinoblastoma is due to a second malignancy.…”

Section: Discussionmentioning

confidence: 99%

Quadruple Neoplasms following Radiation Therapy for Congenital Bilateral Retinoblastoma

et al. 2017

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Purpose: The aim of this study was to describe a 34-year-old male with hereditary bilateral retinoblastoma treated with radiotherapy as a child who developed 4 distinct tumors within the radiation field. Methods: A 34-year-old male with bilateral retinoblastoma status postradiation therapy and recurrence requiring enucleation presented with left-eye visual acuity changes. Magnetic resonance imaging demonstrated a left orbital mass and a right parasellar complex lobulated mass (right sphenoid and right cavernous sinus). Two weeks later, the patient underwent excision of the orbital mass and biopsy of an upper-lid nodule. This was followed by craniotomy for removal of the complex mass. Results: Histology revealed 4 distinct tumors, including an undifferentiated pleomorphic sarcoma (left orbit), a radiation-induced meningioma (right sphenoid), a schwannoma (right cavernous sinus), and a basal-cell carcinoma (left lid). Conclusion: Although occurrence of a second neoplasm is a well-known outcome following radiation treatment in patients with hereditary retinoblastoma, the diagnosis of 4 additional neoplasms is rare. Pleomorphic sarcoma, radiation-induced meningioma, and schwannoma are uncommon tumors and not well represented in the literature describing irradiated retinoblastoma patients. Secondary malignancies are a leading cause of early death in retinoblastoma survivors, and long-term follow-up is crucial for patient care.

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Orbital sarcomas in retinoblastoma patients

Cited by 10 publications

References 28 publications

Feasibility of Proton Beam Therapy as a Rescue Therapy in Heavily Pre-Treated Retinoblastoma Eyes

Feasibility of Proton Beam Therapy as a Rescue Therapy in Heavily Pre-Treated Retinoblastoma Eyes

Necrotizing myositis in a rectus muscle arising in the setting of long-standing Langerhans cell histiocystosis and recent dabrafenib treatment

Quadruple Neoplasms following Radiation Therapy for Congenital Bilateral Retinoblastoma

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