BASAL CELL NEVUS SYNDROME (1) Evaluation (2) Treatment with Efudex and Retin-A --------------------------------------------------------- ---------- EVALUATION ---------- I have a 12 year old young man with BCNS. He is third generation with manifestations in his mother and maternal grandmother. It seems as though each generation has noticed lesions earlier than the last. He has palmar pits and has had three basal cells removed. My question is: Which radiographic studies are felt to be most indicated- not necessarily to confirm the diagnosis but as far as to screen for any prognostically significant lesions. D. O'Connell, M.D. ------------------ I am not sure what you mean by prognostically significant lesions. However, I do a chest x-ray to look for bifid ribs when I have a question about diagnosis. A panoramic film of the jaw, available from some dentists, can find jaw cysts, which may become problems for a few patients. I recall that the falx cerebri can be calcified and found on a PA view of the skull, but I don't think this is clinically a problem. Also, the cysts and calcifications are more likely to found as the patient gets older. Jerry Eisner MD --------------- --------------------------------- TREATMENT WITH EFUDEX AND RETIN-A --------------------------------- I have had patients with 20+ biopsy proven BCE's for various reasons and all of them heve done extremely well using a mixture of Retin A cream 0.025% with equal parts of Efudex cream 5%, bid until a brisk reaction supervenes...several patients (without the BCN syndrome) have never had another skin cancer, even those with non-sun causes (radiation therapy for keloids etc). Steven D. Emmet, M.D. --------------------- A patient with BCNSyndrome, 38 year old male, characteristic lesions, and many of them. Are there any pharmacologic agents that may be of benefit? Any of the retinoids help? It would be an intimidating chore to remove all of his lesions. Current plan is to do Mohs on the more threatening ones, and cryotherapy on many of the others in the interest of comsmesis and cost. Lesions are too deep for 5FU, and would doubt that Retin-A would be of benefit. I thought about the Ultrapulse, but the lesions are too scattered, and who knows what would go on in the "new" epiedermis. Suggestions are appreciated. Elliot Puritz Diane - I was the one who made the statement at the meting in San Diego. I have seen individuals with multifocal superficial BCC develop new clinical lesions within months after intermediate depth (Jessners -35% TCA) peels; this occurs far less frequently in individuals who go through a full court press course of 5FU. Specifically, for the face, it is Efudex 5% cream b.i.d. for at least 30 days. If you miss a dose, you add a day. When they come into the office complaining at 14 days, they get OTC benadryl 25-50 qhs to sleep; if this doesn't work, they get Doxepin 10-20mg qhs or Ambien 5-10mg qhs. If they are really hurtung they get narcs, but above all else, they are encourages to continue the complete course. To modify this for faces with lots of thick keratoses, especially in Xpalnt patients, have them treat until they heal despite continues continued Rx, a course of 2-3 months in some. Daniel M. Siegel --------------- Dan, do you have data on 2, 3, or 4 week treatment periods? Or have you found new lesions which start responding after 2 or 3 weeks and might have been missed with shorter treatment periods? Does what you have observed with BCEs serve as well for AK's? And what do you feel about the hair follicle involvement? Thanks, Diane Thaler --------------- Does what you have observed with BCEs serve as well for AK's? BCCs track down follicles but AKs generally do not. I have only seen folicular involvement with full thickness atypia, but then again,,are the ones that go down follicles not truly SCCs based on behavior. Remember, 5-FU will keep "eating away" at a thick AK, whereas a peel is a one shot deal. Daniel M. Siegel --------------- I basically do the same as you with my one patient. I see him every two months, treat all red spots (incipient BCCs in these folks often look like small AKs) with cryo, attempt to treat small obvious BCCs with a saucerization type shave, and refer for MOHs any that threaten eyes, ears, nose, mouth etc. I have read about but not tried the use of Etretinate. Early lesions reportedly recede to point of disappearance, but recur with withdrawal of the drug. Jerry Eisner --------------- Luckily the patient I had with BCNS got most of her lesions as multifocal superficial BCCs on her back. We sent the first 5 or 10 for histology, "for the record", and then just treated new ones prn. She found the LN2 to be more painful than superficial ED&Cs. We would use a little anesthesia and a light ED&C once. We would 10 or 15 of the lesions at a time. Minimal scar and rare recurrances remained superficial and were treated with light ED&C again. If she was still in my area I might try to electrodisiccate the smaller ones without anesthesia, low voltage desiccation like I do for DPNs. There were some reports of using Etretinate for patients with xeroderma pigmentosa with some positive results. At the time my patient wasn't a candidate and I didn't pursue it. If no one has newer data you might do a search for that. NIH was doing some trials and as I recall they showed a significant decrease in numbers of BCCs in there patients with XP. Mark Crowe, MD --------------- Again, the best reference would be the journal Dermatology Today and Tomorrow. Telephone: +44 81-446 8898; Fax: +44 81-446 9194 . London. The journal is basically "a forum for the exchange of information on the use of retinoids in dermatology." Everything you ever wanted to know about Retinoids, Cancer, and related subjects are detailed. If you are thinking of chronic low dose Accutane, beware of the skeletal changes -though I would still use it. The lag time is an interesting point-so be patient as well. Speaking of sebaceous things, has anyone else noticed that older patients with sebaceous hyperplasia clear on Accutane? Diane Thaler --------------- I have read about but not tried the use of Etretinate. Early lesions reportedly recede to point of disappearance, but recur with withdrawal of the drug. >> I've used isotretinoin with great results in one BCCN patient, who I lost to f/u and in one Muir-Torre who I didn't lose. In the latter he had several new Seb Cas /month which stopped following a couple months of 40/day. Finally I tapered him down to 10/d and he had one new Seb Ca in 7 months. There was a delay in the suppression of carcinomas, which I interpret to mean that accutane will suppress the formation of new Ca's but not ones that've already started. I chose accutane over etretinate because of its greater effect on sebaceous glands and because the sparse literature on retinoid suppression of skin ca usually uses it. Guy Webster --------------- I have seen some improvement with Accutane and also with Vit.A--200,000u/day (I didn't Rx the vitamin, but was surprized at shrinkage and disappearance of many,but not all lesions) gary salenger ------------- You may want to explore retinoids more thouroughly depending on what dose and course the patient has had. I have heard that high doses may be needed and long courses which may need to be repeated intermittently or chronically. Whether this is tolerated by the patient (balancing side effects vs repeated cancers) will determine if the tx is possible long term. Etretinate may be better tolerated from TG, side effect view, than Accutane. Also consider the trials by Dr. Allan Oseroff at the Dept. of Dermatology, Roswell Park Cancer Institute, Buffalo, NY, using photodynamic therapy for BCNS. David J. Altman, MD, PhD -------------