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Have fallopian tube ovarian cancer and genetic test showing abnormality on BRA1. What is the treatment?

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Posted on Fri, 29 Nov 2013
Question: My brother has recently been told he may have Muir–Torre syndrome. He has been treated for bladder cancer since 2004 at 54y and has recently had a skin cancer removed that has come back indicating Muir–Torre syndrome. He is waiting on genetic test results. Our mother had a very large lymphoma tumor removed from stomach in her 60s and responded well to chemo dying of heart at 84. As I have Fallopian tube Ovarian cancer I am keen to know is I should have further genetic testing for this syndrome. I already have genetic test results indicating an unusual abnormality on my BRA1. Can you give me more information on this syndrome and the use of Isotretinoin as a treatment?
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Answered by Dr. Indranil Ghosh (3 hours later)
Brief Answer: Please refer below Detailed Answer: Hi Thanks for followup. Muir-Torre syndrome (MTS) is associated with an inherited defect in one copy of a DNA (our genetic code) repair gene. This leads to defect in DNA repair and can lead to cancer. Criteria for the diagnosis of MTS include the presence of at least one skin tumor/cancer (either sebaceous adenoma, sebaceous epithelioma, or sebaceous carcinoma), and at least one visceral cancer (ie, colorectal, cancer of the uterus, small bowel, ureter, or renal pelvis). Ovarian cancer has also been reported in this. Fallopian tube cancer is very similar to ovarian cancer, hence its association can't be ruled out. But BRCA1 is a completely different entity and it leads to increased risks of breast and ovarian (or Fallopian tube) cancer. Chances of both in same patient is very unlikely. Hence, you should also get tested for MTS. Isotretinoin has been shown in small reports to be effective in prevention of skin cancers in MTS. But as this is a rare disease, no definite recommendations can be made. It has no role in BRCA mutaed patient. Hope I have answered your query. I will be available to answer further followup queries, if any.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Follow up: Dr. Indranil Ghosh (32 minutes later)
I have been told that the average time for my cancer to return is 18 months. If I have this syndrome is there any change to this information?
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Answered by Dr. Indranil Ghosh (5 hours later)
Brief Answer: No Detailed Answer: I don't think it will change. But as I said, MTS is a rare diagnosis and little is known about the individual cancers occuring in these patients.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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Dr. Indranil Ghosh

Oncologist

Practicing since :2004

Answered : 1713 Questions

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Have fallopian tube ovarian cancer and genetic test showing abnormality on BRA1. What is the treatment?

Brief Answer: Please refer below Detailed Answer: Hi Thanks for followup. Muir-Torre syndrome (MTS) is associated with an inherited defect in one copy of a DNA (our genetic code) repair gene. This leads to defect in DNA repair and can lead to cancer. Criteria for the diagnosis of MTS include the presence of at least one skin tumor/cancer (either sebaceous adenoma, sebaceous epithelioma, or sebaceous carcinoma), and at least one visceral cancer (ie, colorectal, cancer of the uterus, small bowel, ureter, or renal pelvis). Ovarian cancer has also been reported in this. Fallopian tube cancer is very similar to ovarian cancer, hence its association can't be ruled out. But BRCA1 is a completely different entity and it leads to increased risks of breast and ovarian (or Fallopian tube) cancer. Chances of both in same patient is very unlikely. Hence, you should also get tested for MTS. Isotretinoin has been shown in small reports to be effective in prevention of skin cancers in MTS. But as this is a rare disease, no definite recommendations can be made. It has no role in BRCA mutaed patient. Hope I have answered your query. I will be available to answer further followup queries, if any.