Atypical Spitzoid Neoplasm in a 5 year old

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1/7/2013 9:19am
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Hello, this is my first time posting on this site so please forgive me if I am in the wrong place.

My 5 year old son had what the dermatologist thought was a pyogenic granuloma removed from his chin in early December via shave.  After the pathology report was received, he was diagnosed with an atypical spitzoid neoplasm.  However, the biopsy was showing characteristics of a benign tumor and melanoma at the same time.  The tissue sample was then sent to Sloan Kettering Cancer Center for an expert opinion and we are still waiting on the results of that pathology report.

We met with a plastic surgeon because the doctors want to my son to have the remainder of the tumor removed after the 2nd biopsy comes back.

I am confused about how these spitzoid tumors can behave in two different ways?

I haven't been able to find much info on the web when it comes to children with this type of condition. 

Any advice and insight would be greatly appreciated.

Spitz nevi are found in children and are benign.  However, Spitz nevi look almost identical to melanoma under a microscope.  You need to have the pathology read by someone who really understands Spitz nevi.  I'm no longer sure where Dr. Boris Bastian practices - he was at UCSF then he moved back east but I had heard he might be back west again.  Anyway, one of his areas of research is Spitz nevi.  He and his team can review the slides.  If his team have any question about the pathology, they have developed a DNA test they can run that can be more specific in determining the pathology.  Basically, the DNA testing would show lots of DNA errors if it is melanoma, and few if it were a normal Spitz nevi.  There may be other pathologists who can read Spitz nevi, but I know this is a specialty of Dr. Bastian's and I would personally want his opinion if this was my child.  In general, because your son is so young, a Spitz nevi would be the most likely choice.  When you get a person in their late teens or twenties, this is where things become more iffy because Spitz nevi are rarely found in adults.

Best wishes,

Janner

Not sure if this is the right place to post but I need some advice. My 2 year old nephew just had something removed from his upper leg. The Dr. told us it was a pyogenic granuloma but it came back as an atypical spitzoid neoplasm on the pathology report. We are awaiting a second pathology report. Just want to know where to go from here?

Not sure if this is the right place to post but I need some advice. My 2 year old nephew just had something removed from his upper leg. The Dr. told us it was a pyogenic granuloma but it came back as an atypical spitzoid neoplasm on the pathology report. We are awaiting a second pathology report. Just want to know where to go from here?

Hi.

Our 8 year old was diagnosed with a severely atypical spitz nevus last Valentines day, later determined via FISH analysis to be melanoma.  One of our first stops was at Sloan Kettering, where we met with the pediatric oncology team, not the melanoma team.   The team dismissed the FISH results and stated that there was a "99.9% chance" the initial wide local excision (WLE) and sentinel lymph node biopsy (SLNB), both done 3/9/12, were curative. They were not willing to answer my questions.  Further, they do not treat Stage 3 melanoma but offered that they could "probably" get our son treatment on compassionate grounds if our son's disease progressed to Stage 4.   I was not willing to "wait and see" with the pathology results we had on an 8 year old child.   We ended up at St Jude's Children's Research Hospital, had the complete lymph node dissection, and more melanoma was found.  We are now on interferon, doing well. I think the clinic trial at St Jude is now closed but they may be willing to give a second opinion.

I recommend asking to have a FISH assay test done; our initial pathologists requested this after the shave biospy.  It is used to distinguish between spitz nevi and melanoma, which can be difficult to do visually.   According to the developers, it does not give false positives - so that if it finds melanoma, then it is melanoma.   But it can give false negatives - if it fails to find melanoma, melanoma may or may not be present.  It was the FISH results that clarified for us whether our son's mole was a bengin Spitzoid nevus or a malignant melanoma.  It is really really rare to have a melanoma in a child under 10 (something like 100 cases a year).  Hopefully your son just has an atypical Spitzoid, in which case you want to be sure he is monitored by a determatologist regulary.  

My best advice is to keep asking questions until you can live with the answers.  It took 6 hospitals until we found one that had the willingness and expertise to treat our son.  

Good luck.

Are you able to tell me some of the charectaristics of your sons pathology report?

I have been disecting the one we have trying to decide if its leaning towards benign or melanoma.

This is the main paragraph from my sons....again, any help is appreciated

Examination of the biopsy reveals a shave biopsy with a verrucous architecture.  There is a compound melanocytis neoplasm composed of large spindled and epithelioid melanocytes arranged in nests and as single cells at the dermoepidermal junction and in the dermis.  The majority of the lesion is within the dermis.  The melanocytes do not appear to mature with dermal descent, and scattered mitotic figures are identified in the dermal melanocytes.  A k1-67 stain reveals an increased proliferative index within the dermal component.  Overall, this is a very difficult case.  The biological potential of these lesions in this age group are largely unknown, therefore the case is bening sent to Sloan Kettering Cancer Hospital for expert opinion. 

Anonymous - (1/7/2013 - 11:05am)

I believe Dr. Bastian is a head of a department in Sloan Kettering, so, probably, the slides will go to him. I hope that it is just a Spitz nevus and not melanoma because Spitz is common in kids but melanoma is not.

Because melanoma is so rare in children, I am wondering if you could please describe how these lesions (in 5 yo and in 8 y o) looked like. Thanks.

My sons lesion appeared this past October and at first looked like a pimple.  It grew very quickly and was first misdiagnosed as a wart and that is exactly what it looked like.  I regret not taking a picture of it before it was removed but the best way I can describe it is a wart.  It was raised and had a cauliflower texture.  It almost always had a scab as it bled a lot.

Well I just found out that Sloan Kettering lost my sons slides and the original pathology department cannot duplicate them.  Now my son has to blindly have the remainder of the tumor removed and if they do not get clear margins, they will have to operate again.

This is a lot for a 5 year old!

Oh NO!!!! Did they mention the depth of the lesion in the original report? Did the offer you SNLB as a precaution? 

All of that was to be determined from this pathology report.  The plastic surgeon will have to operate blind!

I am furious!

They cannot tell the depth of the lesion when a shave biopsy was done. The information was and is not available unless the biopsy originally removed the entire tumor.  It is very unlikely they will offer a SNLD without a more complete pathological analysis.  Pay close attention to Janners postings.  She has hung around here to help others, while having had a low level melanoma inher past.  (Her Father also has Melanoma, She's very knowledgeable.

  Orlando, you mention action after the 2nd biopsy comes back.  Have they done a second biopsy?  A follow-on question is, have they done a dye test to determine which is actually correct the Sentinel Node?  The true sentinel node should be determined before too much additional tissue is removed.  Doing a search of Janner and SLN on here will provide info on the importance of this.

  Dr Bastian is the best I have heard of for providing follow up for this type case.  He is a good and knowledgeable Melanoma Specialist.  Be sure your son's case is handled by a very experienced Melanoma Oncologist.  It is not unusual for the Pediatric Department to be the ones that actually handle pediatric Melanoma cases, just make sure they work with a GOOD Melanoma specialist.

I'm me, not a statistic. Praying to not be one for years yet.

Jenny, to answer your question......first diagnosis at the pediatricians office was a wart.  Then after a few weeks and the wart only increasing in size, we went to the dermatologist.  The dermatologist diagnosed it at a pyogenic granuloma.  They performed the scrape biopsy and sent the tissue to pathology.  A week later the pathology report came back with a diagnosis of atypical spitzoid neoplasm.  The pathologist also wanted the slides sent to Sloan Kettering for an expert opinion because from what the doctors have told me, these types of lesions can be difficult to diagnose is children.  I met with a plastic surgeon last week because the majority of the lesion is still in his chin.  He will need to have an excision in a few weeks to remove the remaining tissue.  We are waiting on the pathology report from Sloan Kettering to tell the plastic surgeon how much tissue needs to me removed.  That's why this is so frustrating.....without the report from Sloan Kettering, the plastic surgeon will have to guess how much tissues needs to be taken and if he doesn't get clear margins, my son will need to have surgery again. 

I did hear back from Sloan Kettering yesterday and they "think" they have traced the slides back to a particular pathologist who has been on vacation for the past two weeks.  She returns to work today and I am praying that she has the slides.....fingers crossed!

Im hoping someone will read this and help..i was just told today that my son has compound spitz neoplasm...he is only 10 years old. Can someone share their experiences with this...and give me some good news.

Anonymous - (1/7/2013 - 2:16pm)

that's crazy, that's ridiculous. . I thought they are supposed to be a world class center of excellence, and they lose slides?

It is riduculous.  I've learned to keep my own medical file going for my husband.  I've got copies of scans, reports, anything I can get my hands on.  It's unbelivable how mixed up things were for him for about the first 4 months after diagnosis.  Doctors did not communicate with each other, information that was faxed was never received, calls were not returned,blood was lost (as least it was only blood), you name it.  One person even asked him how he was doing with his brain resection while looking at his bald head (from chemo) that has no scars!  I've had to follow through on every single thing, even making sure that the tests they said they were doing were being done.  And the mix ups happened at every hospital and every doctor's office except one.  We've been dealing with one of the best hospitals in the country. I've come to the conclusion that things like this happen everywhere.  I think the medical personnel are overworked, understaffed and have too many of us to keep track of.  At my husband's last chemo treatment, the nurse set the drip wrong so it was running way, way too slow, which was far better than running too fast.  She put in the wrong decimal on the pump.  She is normally an outstanding nurse.  She is definitely the most attentive and most personable one my husband has had. She tries very hard to go above and beyond for all of her patients. The patient or their caregiver has to stay on top of things the best they can, ask questions, follow through on everything and get as many copies as they can get.  One place even lost a copy of a scan.  It adds so much to the stress we already feel.  I've been so frustrated that I could scream.  That the only copies of slides for this child were lost is inexcusable and I feel badly for him and his parents.

Such a true response.  One needs to keep copies of all possible records (including the doctors notes - which some places eillprovide you if you ask for them).  I have many from UVA!   I wish we didn't have to keep such records, but even the good nurses and doctors seem to be human.  Wish my wife would ask for copies of her records!
(Won't talk about some of the other doctors I've had mis-diagnose me for years!) 

I'm me, not a statistic. Praying to not be one for years yet.

Hi 

UPenn "lost" our slides too - I called the director of pathology and they were found ASAP.  Don't be afraid to make a few waves.   My experience is that Sloan Kettering is going to tell you not to worry, regardless of what your path report says.  Even though the Sloan Kettering Melanoma Clinic has experts, including Dr Bastin (sp?), he may not review the slides because your son is so young.  We found that major melanoma clinics are unwilling to look at prepubescent kids because the anatomy is different than in adults. My advice is to have your doctor contact the Solid Tumor Clinic at St Jude's. (go to the St Jude website)  This may be more straightforward than requesting the FISH test I recommended earlier.  Parents don''t contact the hospital directly but rather everything goes through the referring physician.  

Our son's pathology report indicated Clark depth IV, ulcerated, and a mitosis rate of 13 mm^2.  80% of the cells in the original tumor were melanoma.  At the time we went to Sloan Kettering, microscopic melanoma had been found in his groin (primary location was shin).   It doesn't seem like you have that level of detail yet in your current report, which is leaning more towards a Spitzoid nevus.  His mole appeared as a small dark pimple in August 2011, like your son's.  In 8 months it went from non-existence to Stage 3B melanoma.  On Nov 1, 2011 our pediatrician told us not to worry and that he'd get us the first derm. appt he could.  I feel that we lost a lot of ground waiting from Nov 1, 2012 to Feb 12, 2012. 

I know this is very confusing.  It is even confusing for the experts, let alone for us parents trying to find out info for our kids.   I have also been very pro-active about having every mole on my son mapped and measured.  Four additional mole have been removed and thankfully none have been melanoma.

Hang in there...

Hi

I found this article very helpful when my son was first diagnosed. Although he was 20 when diagnosed ...which is actually the upper limit of "pediatirc" according to the doctor, he had a very unusual case ( oral, tongue) and so was sent to 3 pathologists before they decided it was melanoma for sure.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2797485/

 

It is long but informative.

Also, when Dr Bastian was at UCSF I emailed him to see if he would test C-Kit and other mutations..and he got back to me right away and was very helpful.

 

Hello,  My daughter Ella had a spitz nevus removed when she was 3 (3 years ago).  (She also had a large dysplastic nevus removed shortly after) This was quite frightning for me, as her dad passed away from melanoma about a year prior to that.  Some of the stories on here can be so scary, so I just want to let you know that my daughters stayed the same dignose of spitz nevus where as I had it stuck in my head unfortunately that they would find out it was melanoma.  There is a good chance that that is all it is!  With that said, It is still wise to keep a very close eye on this matter and follow up appointments are very important!   Be your son's advocate!  He cannot speak for himself and he is not his doctors only patient!  Ask tons of questions, keep all medical records, and research (as it appears you already are!)!  During my husbands battle with melanoma, I learned I had to "be the boss".  If you want answers, you have to ask questions!   Please feel free to get a hold of me anytime if you have any more questions, (not sure I worded this post very well!)  although it has been quite some time since I have been on MPIP, melanoma will forever be stuck in my mind!   Good luck!!

Nicole

p.s. .. my daughters spitz nevus was exactly how you described your sons! Except hers was on her back..It was initially thought to be a wart..

Good to see you dropping in, Nikki.  You must know that you and Ella was my first thought when I first read this post.   Orlando,Nikki was a great fighter and supporter for her Sean's losing battle.  She definitely knows what you are going through with worrysome spots on  your children.  She had a crash course on melanoma and Ella has had birthmarks that have to be watched closely to make sure they don't change.   And YES, Nikki, you worded the post quite well.  (And once again typed better than I usually do. - Nikki always made fun of my many typing errors when we were  in the chat room!)

I'm me, not a statistic. Praying to not be one for years yet.

Nicole, your story brings me much comfort and I thank you for that :)

I am refusing to accept the answer that my sons slides are lost.  I call the patient advocacy group at Sloan Kettering yesterday afternoon.  I almost immediately received a call from the Pathology Department Administrator regarding the missing slides.  He told me that he was working diligently to find the slides and trying to get duplicate slides from the lab in Pittsburgh.  He assured me that this would be handled appropriately and immediately.  So we will see.

I have been researching every web-site out there regarding these Spitz tumors in children.  The information is definitely scary. 

Hoping for the best!

I actually just joined the board when I saw this topic! My 3 year old son had a spot exactly like you described - started out like a pimple, then had the cauliflower look to it, but then his became dome-shaped and lost the cauliflower look. His pediatrician thought it was a wart and gave us cream to put on it 3x/week. After a month it wasn't doing anything so I took him back to the pediatrician and he gave us a referal to a dermatologist. The dermatologist gave him a shot of lidocaine, sliced it off and assured us it was "never anything in kids this age" but sent it to pathology anyway. A week later, the pathology came back as "either atypical compound spitz nevus or melanoma" - they've sent it to MD Anderson for further review.

I'm terrified! Everything I'm reading says that melanoma can be misdiagnosed as atypical spitz, and I'm so scared that we're going to under-treat something major. At the same time, I don't want to go overboard the other direction and treat him for something he doesn't have, you know? We're still waiting to hear back from MD Anderson, they told me on Tuesday they had to order more materials for further study on it. So far the only thing we know is that they're going to do surgery to make sure they have all of it gone. I've read online that sometimes a sentinel lymph node biopsy is performed, but I have no idea if they'll do that or not. Trying not to worry is hard!

Hi Steph -

I know EXACTLY what you are going thru right now!

We are also playing the waiting game right now.  Sloan Kettering lost my sons slides that were sent to them for an expert opinion.  Luckily, the slides were able to be duplicated and Sloan Kettering has them in their possession as I type.  They have already notified me today that they will have a preliminary report later this afternoon but due to the complexity of this particular case, they are performing additional testing, which could take up to 10 days.  It's some type of a DNA test called FISH.

My son is also having surgery after we receive all of the pathology results.  My sons doctor did mention the lymph node biopsy as well but we wont know for sure until all the tests are completed.

We have been dealing with this since December 4th and I feel like we are getting nowhere......ugh, I hate waiting!

All the best,

Stacy

So glad I found you guys! My son had bump removed on 12/12; we got the call exactly a week later (12/19) that it was being sent off. At first they were going to send it to the University of Florida (we live in the Florida panhandle), but the head pathologist decided to send it to MD Anderson instead. MD Anderson confirmed receipt on 12/31, and they typed an initial report on 1/2. The report is currently "on hold" because the doctor hasn't finalized it yet. On 1/7 (I'm sure I'm driving them crazy!) they ordered "more materials" so they could study it further. I'm wondering if that's the FISH analysis I've been reading about?

I feel like nobody here locally knows what's going on, and I'm reading so many conflicting things online. They haven't mentioned doing a lymph node biopsy - and everything online is 50/50, some saying it was performed & others saying it was unnecessary. The local derm told us it was atypical spitz, but when I asked her to read the report to me, the pathologist noted it was "melanoma with features of atypical spitz nevus" - to me, there's a BIG difference between "melanoma with atypical spitz features" and "atypical spitz with melanoma features"! Is it cancer or is it not cancer? There's a big difference!!

I'm also concerned because the spot is on his face, and I don't know how much tissue they'll have to remove. They did say a plastic surgeon would be there & so the scarring would hopefully be minimal. I think in my head I'm just automatically assuming the worst.

Call your dermatologists' office and ask them to fax you a copy of the original pathology report.  That's what I did and I have literally googled every word in the report!  From what I have read on this site, it's a good idea to get your hands on as many reports and results as possible.  They are after all "our" records!

My sons mole is on his chin.  We met with a plastic surgeon at Children's Hospital in Pittsburgh and he will be the one performing the surgery since the scar will be on his chin.  He told my husband and I that the pathology report from Sloan Kettering Cancer Center in New York will tell him how much tissue he needs to take in order to achieve clear margins.  He explained to us that he has rarely seen a recommendation of greater than 5mm. That will lead to a scar in the 1/2" range.  He is leaning toward taking a lymph node, which would be a separate scar most likely under his jawbone, due to the location of his mole.

It's a lot to take in and I am also assuming the worst.....why do we do that?

I called and left a message to fax the report - of *course* they're in another office today. I'm hoping the docs at MD Anderson can come up with an answer pretty quick. I'm wondering if the "materials" they had to order were for the FISH analysis? I'm really curious to find out exactly what the experts think, ya know? And we worry because we're moms ;)

Hi Steph -

I was thinking of you this morning, any news?

Thanks for checking on us! We still are waiting to hear results. I called the pathology dept at MD Anderson and they are waiting on results from the FISH analysis - which is what I was hoping they would do. The rest of the report is done and "pending final review." They said they ordered the materials on the 7th, and had the local pathologist here send them the rest of my son's slides. Not sure if that's a good thing or not? I keep telling myself no news is good news...but I'm not so sure if that's true! How are you guys doing?

We received the results from the FISH test, with 85% accuracy, negative for melanoma.  However, they noted that due to the limitations in the sensitivity of the test, a negative result is not entirely definitive.  Well, that doesn't make me feel good!

So, they have now decided to cut 5 additional slides and have them sent to a Dr. Boris Bastian at University California San Francisco for a test called comparative genomic hybridization.  I do not know anything about this test, except that it takes 28 days to receive results!

As a side note, we requested that Sloan Kettering send my sons slides back to Childrens Hospital Pittsburgh after the fish test as this is the hospital he will eventually have the remaining tissue removed.  Sloan Kettering informed me last night that the FISH test renders the slides useless.  Apparently they scrape all of the tissue from the slides and mix it with some kind of a stain to preform the test.  Just wanted to mention that to you incase your Dr. had plans for additional testing following the FISH test.

Please keep me updated on your sons progress.

You might wantt to check and see if the original operating site has a paraffin block with any remaining tumor tissue.  This is usually kept after they maake the slides from a portion of the origin tumor.

I'm me, not a statistic. Praying to not be one for years yet.

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