Navigation Links
Study examines risk of soft tissue sarcomas in hereditary retinoblastoma survivors

A study in the January 3 issue of the Journal of the National Cancer Institute quantifies the risk of hereditary retinoblastoma survivors developing specific subtypes of soft tissue sarcomas and finds that this risk persists for decades after the original retinoblastoma diagnosis. The findings emphasize the importance of regular medical surveillance for these patients, the authors say.

Retinoblastoma is a rare cancer of the retina that primarily affects children and is most often diagnosed before age 1. The disease can often be cured; 97 percent of retinoblastoma patients survive 5 years after diagnosis. However, patients with hereditary retinoblastoma are at a high risk of developing another cancer, including soft tissue sarcomas. The increased risk of soft tissue sarcomas is attributed both to the mutation in the RB-1 gene that causes retinoblastoma and to the radiation treatment that most retinoblastoma patients received when treated decades ago. Radiation is used less frequently today because of the risk of second cancers.

Ruth A. Kleinerman, of the National Cancer Institute in Bethesda, Md., and colleagues studied a group of 963 1-year hereditary retinoblastoma survivors to better understand the risks for specific subtypes of soft tissue sarcomas. The patients were diagnosed between 1914 and 1984 at medical centers in New York and Boston and were followed for an average of 25.2 years. More than half of the patients were younger than 1 year old when diagnosed, and most (88.2 percent) of the patients were treated with radiation.

A total of 69 soft tissue sarcomas were diagnosed in 68 hereditary retinoblastoma patients. The most common subtype was leiomyosarcoma, a cancer of smooth muscle cells that was diagnosed in 23 of the patients. Other frequently diagnosed sarcomas were fibrosarcoma (13 patients), malignant fibrous histiocytoma (12), rhabdomyosarcoma (8), and liposarcoma (3). The authors calculated that there was a 184-fold increase in soft-tissue sarcomas among hereditary retinoblastoma survivors compared with the general population and a 390-fold increase in cases of leiomyosarcomas.

Only 20 percent of the soft tissue sarcomas were diagnosed within 10 years of the retinoblastoma diagnosis; 45 percent of the sarcomas arose 30 or more years after diagnosis. In addition, 18 of the 23 (78 percent) leiomyosarcomas were diagnosed 30 or more years after the diagnosis of retinoblastoma. Almost all sarcomas were diagnosed in retinoblastoma survivors who had been treated with radiation. However, 18 of the 69 sarcomas developed outside the original radiation treatment field, and leiomyosarcoma was diagnosed more often outside the radiation field than in it.

The authors note that some of their calculations are based on few cases of these rare cancers. In addition, classification schemes for some sarcomas changed over the study period, so some cancer subtypes may have been classified differently depending on the year they were diagnosed.

"Given the excellent survival of retinoblastoma patients, it is important that survivors continue to undergo regular medical surveillance for sarcomas in their adult years," the authors write.

In an editorial, Anna Meadows, M.D., of The Children's Hospital of Philadelphia, highlights the advances in treatment for retinoblastoma in the last several decades, and she notes that some of these findings may be applicable only to patients who received treatments that are no longer used. "Improved treatment can be expected to reduce the incidence of soft tissue (and bone) sarcomas associated with radiation," Meadows writes. "The importance of this [new] report lies in its emphasis on leiomyosarcoma, a tumor that occurs in RB-1 gene carriers whether or not they were treated with radiation and that can be expected to continue to occur in older survivors, who will require more careful follow-up."


'"/>

Source:Journal of the National Cancer Institute


Related biology news :

1. Novel Asthma Study Shows Multiple Genetic Input Required; Single-gene Solution Shot Down
2. Emory Study Tests Bone Marrow Stem Cells to Improve Circulation in Legs
3. UCLA Study Shows One-Third of Drug Ads in Medical Journals Do Not Contain References Supporting Medical Claims
4. Study Demonstrates Gene Expression Microarrays are Comparable and Reproducible
5. Study Links Ebola Outbreaks To Animal Carcasses
6. Breakthrough Microarray-based Technology for the Study of Cancer
7. NYU Study Reveals How Brains Immune System Fights Viral Encephalitis
8. Study finds more than one-third of human genome regulated by RNA
9. Leukemia Drug Breakthrough Study In New England Journal Of Medicine
10. Study identifies predictors of HIV drug resistance in patients beginning triple therapy
11. New Study from Affymetrix Laboratories Points to Changing View of How Genome Works
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:4/11/2017)... Fla. , April 11, 2017 ... and secure authentication solutions, today announced that it ... Intelligence Advanced Research Projects Activity (IARPA) to develop ... Thor program. "Innovation has been a ... IARPA,s Thor program will allow us to innovate ...
(Date:4/11/2017)... 11, 2017 NXT-ID, Inc. (NASDAQ:   ... announces the appointment of independent Directors Mr. Robin D. ... Board of Directors, furthering the company,s corporate governance and expertise. ... Gino Pereira , ... forward to their guidance and benefiting from their considerable expertise ...
(Date:4/5/2017)... 5, 2017 Today HYPR Corp. , ... server component of the HYPR platform is officially ... end-to-end security architecture that empowers biometric authentication across Fortune ... already secured over 15 million users across the financial ... connected home product suites and physical access represent a ...
Breaking Biology News(10 mins):
(Date:4/24/2017)...  Dante Labs announced today the offer of whole genome ... While American individuals have been able to access WGS at ... WGS below EUR 1,000. The sequencing includes bioinformatics ... to make informed decisions about disease monitoring, prevention, nutrition, exercise, ... ...
(Date:4/21/2017)... ... April 21, 2017 , ... ... today announced first round funding to three startups through the UConn Innovation Fund. ... to new business startups affiliated with UConn. , The UConn Innovation Fund provides ...
(Date:4/21/2017)... ... 2017 , ... Frederick Innovative Technology Center, Inc. (FITCI), a ... technology start-ups, is hosting “Celebration Friday” (a festive gathering highlighting client success stories) ... and networking at 3:30 p.m. at FITCI’s 4539 Metropolitan Court location, off English ...
(Date:4/20/2017)... ... April 20, 2017 , ... Assured ... partnership to offer a full spectrum of digital security goods and services. The ... products and the ground-breaking proactive cybersecurity services and products through Assured Enterprises. The ...
Breaking Biology Technology: