Q&A: Diagnosis

Earlier this week, I emailed 45 questions to my oral & maxillofacial surgeon, Dr P.

He responded in a little under 2,000 words.  By the next morning.  While on holiday.

I read his email more than half a dozen times over the next day or so.  His answers reassured me, as much by their existence as their content.  Yes, they gave me a clearer picture in my head about how things would likely pan out but, more than that, they satisfied me he has this well in hand.

So here begins the three part Q&A series.  Today diagnosis, tomorrow surgery, finally recovery.  Enjoy.

Q&A: DIAGNOSIS*

How large is my tumour?

Your tumour is in the retromolar region of the mandible, extending forward to the body of the mandible and posteriorly to the ramus. The dimensions are approximately 4-7cms antero posteriorly, 3-4cms vertically and 2-4 cms transversely.

What has been destroyed other than the bone?  Do you often see ameloblastomas this destructive of mandible etc?

The tooth roots have been eroded and this is indicative of the tumour process. The destruction of the jaw is similar in other people with this tumour. As a sub group the “Unicystic Ameloblastoma” is the only ameloblastoma that has a more innocuous and predictable outcome.

Do you often see ameloblastomas that have infiltrated the soft tissue?  What does the infiltration of the soft tissue mean?  Will it affect the surgery or prognosis?

Ameloblastomas erode mandible (bone), breaching the periosteum surrounding the mandible and may invade soft tissue. Although relatively unusual it depends primarily on the length of time you have had the tumour and also the aggressive local expansion of the tumour itself.

Infiltration of the soft tissue means we have to involve this in the resection (removal) as we want to minimise the chance of recurrence (Ameloblastomas have a high rate of recurrence). This will involve removal of adjacent muscle, fat, attached and free gingiva (gum) and associated nerves and blood vessels.

I do not believe we will need to remove external skin but this is predicated on soft tissue Frozen Sections (we cannot do bone frozen sections). Invasion of the soft tissue will mean more tissue is required to be removed, more tissue is required to replace it (Fibula Flap).

What does the pathologist in the second biopsy mean by: “a suggestion of basal palisading certainly prompts consideration of a residuum of an ameloblastomatous process”?

The description is that the 2nd biopsies showed a lot of reactive scar tissue and fibrosis from the first biopsy. The residuum confirms that Ameloblastoma was indeed found and concurs with the first biopsy and diagnosis.

The pathologist in the second biopsy wrote that the tumour has “a somewhat basaloid appearance, apoptotic cells are seen and scattered mitoses”.  What does this mean?  Is this the same as the pathologist in the first biopsy saying my tumour “exhibits basaloid features with evidence of apoptosis and occasional mitoses”?

Apoptotic cells are cells that are dead and dying, programmed as part of the tumour or living process. Mitoses indicate the tumour  cells are dividing (ie. tumour is getting bigger)

The pathologist in the second biopsy wrote, “Overall the radiology had a somewhat aggressive appearance which could fit for a large ameloblastoma.”  However, they also said, that the tumour’s appearance is an “unusual finding in a straightforward benign ameloblastoma”.   What other diagnoses could the tumour’s appearance fit with?

The aggressive appearance is a term that denotes the expression of rapid growth, unusual features or a tendency towards malignancy. It is used by surgeons to convey to patients that a more aggressive approach to surgery is required In your case this essentially means mandibulectomy, resection of associated soft tissue and replacement with an autogenous flap (Fibula). This is aggressive surgery versus less aggressive surgery (enucleation or scooping out the tumour which we do for unicystic ameloblastoma).

Some other diagnoses that could be entertained include Pindborg Tumour (Calcifying Epithelial Odontogenic Tumour) but I rely on the pathologist for clarification, in your case they confirm it is an ameoblastoma.

The pathologist in the first biopsy said “unequivocal malignant features are not observed”.  The pathologist in the second biopsy did not see “frank malignant features”.  What would these features have been?  How would they have differed from what the pathologists did see?

The pathologists confirm that in the biopsies noted they are clear and un-ambiguous that there is no process suggesting cancer (malignancy). Cancerous features may involve cellular atypia, bizarre or frequent mitoses, the absence of an ordered structure or other aspects histologically that do not resemble normal tissue.

The pathologist who undertook the first biopsy said had “done progesterone receptor on the tissue and found it to be strongly positive, raising the possibility of some degree of hormonal effect on this tumour”.  What does this mean?  Does the possible hormone effect suggest another or concurrent diagnosis?

He’ll get back to me on this.

In my notes, I wrote “if the biopsy results do not come back as clearly benign, I’ll likely have a CT PET scan”.  Will I still have a CT PET scan?

You do not need a CT PET scan

*Disclaimer: He did not have access to my files and his answers are indicative only.

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Details

Now of course, nowhere does the report say it’s not cancer.

Let me see what sense I can make of it.

The radiologists and the pathologists were concerned about the way the tumour had grown.

For the radiologist, it was the tumour’s proliferative activity, the buccal and lingual expansion and the perforation of the lingual cortex and the focal buccal perforation.  That, I think, just means the way the cheek and tongue had expanded and the way the tumour had penetrated their outer layers.

The pathologists were concerned about the way the tumour’s cells looked: somewhat basaloid appearance, apoptotic cells and scattered mitoses.  From what I took in speaking to Dr P this morning, this means the way the tumour had infiltrated surrounding tissue.

The two properties that separate cancer from your garden variety benign tumour are metastasis and invasiveness.  Metastasis is the ability of the tumour to spread from one organ to another and to start the growth of a secondary tumour.  This is the aspect of cancer that really scared me.  Invasiveness is the ability to infiltrate and destroy surrounding tissue.  Benign tumours are more visually defined than malignant tumours.

We already knew my tumour is aggressive and destructive.  It has eaten away at my jaw bone (mandible) until paper thin in sections and it has eaten into the surrounding tissue.

It seems the degree of invasiveness was an ‘unusual finding in a straightforward benign ameloblastoma’.  But it lacks ‘frank malignant features’.

So yes, they will have to remove a great deal of my jaw.  Yes, they will have to take a large margin around it to be sure they get all the cells from the tumour that have invaded the surrounding tissue.  But this aggressive resection will mean it is really unlikely that the tumour will grow back in my jaw.  Possible, but very unlikely.  And because it is not malignant, a related tumour is not going to pop up elsewhere unannounced.

Back to The Plan – Part 2.  Never did I think I would be so thankful.

If you haven’t had your fill of big medical words, feel free to read on…

Macroscopic

1.  ‘Left lateral mandible’.  The specimen consists of portion of grey soft tissue 11mm in maximum dimension.

2.  ‘Periosteum left mandible’.  The specimen consists of a portion of cream soft tissue 14mm in maximum dimension.

3.  ‘Deep mandible’.  The specimen consists of a portion of firm cream soft tissue 10mm in maximum dimension.

Microscopic

I’ll spare you this bit.

Comment

The histologic appearances in all of the three biopsies predominantly reflect organising fibrosis and granulation tissue with occasional strips of reactive bone all of which could reflect a reaction to prior biopsy.  In specimens 1 and 2, rare islands of odontogenic epithelial cells persist and although a suggestion of basal palisading certainly prompts consideration of a residuum of an ameloblastomatous process.

I have taken this case in conjunction with the imaging to the Royal Prince Alfred bone and soft tissue tumour meeting which is attended by Professor H who reported the original biopsy.  He has sent the initial material to Dr C in the USA in consultation as he had some concerns regarding its proliferative activity.

Professor H kindly brought recuts of the histology to the meeting,  The images were reviewed in which the presence of a destructive radiolucent lesion of the left posterior mandible with perforation of the lingual cortex and focal buccal perforation.  The lesion had focally multiloculated appearance and it extended into the ramus of the mandible.  On MRI it had a heterogenous appearance with soft tissue extension on the buccal and lingual aspect.  Overall the radiology had a somewhat aggressive appearance which could fit for a large ameloblastoma.

I have reviewed the sections originally reported by him in which the presence of an ameloblastoma with a partly cystic and partly solid infiltrative appearance is confirmed.  As noted by Dr H, much of the ameloblastomatous component has a somewhat basaloid appearance, apoptotic cells are seen and scattered mitoses are noted, an unusual finding in a straightforward benign ameloblastoma.  While these findings are of some concern, frank malignant features are not seen.

Professor H did inform me that he had done progesterone receptor on the tissue and found it to be strongly positive, raising the possibility of some degree of hormonal effect on this tumour.  Within the current material as receive by me. scattered islands of residual epithelium consistent with residual ameloblastoma are noted in speciment 1 and 2.  Residual tumour is not seen in specimen 3.

It’s not cancer

I am numb with relief.

In the end, after all the testing, after all the discussing, they do not think it is cancer.

The tumour is benign but aggressive.  More aggressive than they expect to see in a benign tumour.

But they think it is benign nonetheless.

It has penetrated the soft tissue and has areas of fast growth.  If untreated, there are indications it could turn malignant.

But they are treating it aggressively with resection.

I have time.

I have so much time.

It is an amazing thing.

The details will come later.  For the moment, I want to drink in the time, let the relief wash over me as the numbness abates.

Tomorrow is the day

J at Dr P’s office will receive the biospy results from the pathology lab tomorrow.

She will fax them to Dr P in Wollongong.

He will call me.

And then I will know.

Either way.

Homeward bound

I will be discharged from hospital by lunch time.  Dr P just popped in to see his patients in the ward.

He took three samples, just like in my first biopsy.  The worrisome extension of the tumour into the soft tissue was glistening and white, he said. In the soft tissue, there were signs of infection and efforts to protect against the expanding tumour.  Of course, as I have heard many times now, only the pathologists will be able to determine if the tumour is cancerous by examining the samples under a microscope.

The samples have been whisked away to a pathologist at the Royal Prince Alfred.  She will likely consult with the pathologist from the first biopsy.  If necessary, the radiation oncologist from Macquarie University Hospital will also have a look.  Dr P now seems to think he may hear by the end of the week.

I will have a consultation with him next week to discuss the pathology results.  Unless the results are nasty.  Nasty means clearing their schedules.  Nasty means resection and reconstruction surgery either this coming Saturday or the next.

Time to root for a late July surgery date.

Biopsy two down

I have oxygen pumping gently in my nose.  An IV drip in my arm. No feeling on the outside of my left jaw. Raw throat from breathing tubes through my nose down my throat during surgery. The facial swelling has started.  And the pain inside my mouth at the biopsy site is beginning to stir.

But I am awake.  I even feel in much better shape this time.  I asked for and ate my dinner: mashed potato, soft pizza, steamed veg, bread and butter pudding, custard and apple juice. Not too shabby in this hotel, as Darren kept accidentally calling it as we filled out the admission forms.

Do you mind hanging on there a tick while I find Kevin, my nurse tonight?

They are on their pain meds round, the little assistant tells me.  Pain out of ten?  Oh five, maybe six.

I can wait. I’ve had practice at that today.  I waited about four hours before I heard the dulcet tones of Dr P strolling along the hallway.  Relief.  I was overwhelmed with relief.  He will take it all in hand now.

Ruth and the porter took me down to theatre.  Dr P wandered by with coffees for the operating team.  Good to know they are taking a break and will be ready to go again.  The anaesthetist Kirsten (good sign) and operating nurse Bev were lovely about my needle phobia and followed the worst bit quickly with a dose of the good stuff.

Helen, the nurse in the intensive care unit, called Darren for me as soon as I asked and I could speak to him.  By about 4pm, maybe a bit later, I was back in my room and on the cockpit phone to Mum when they waltzed in chirpily reading her new book from today’s mothers group combined first birthday party.

Alannah wasn’t super keen to get close and burst into distressed tears.  Then moments later she was enthusiastically shoving in one mandarin, then a second, and high-fiving me from the armchair. Tiredness overtook her after not too long. She is a trooper, my little girl.  Virus, long day, much change already from the rhythm she knew and loved.

Time for pain meds. I can hear Kevin now.

Waiting

I have showered with chlorhexidine antiseptic wash. Ruth, my nurse, has dressed me in my hospital gown.  She has put on my funky leg stockings and red surgical hat.  She got me emla patches to desensitise my elbow crease and hand.  I cry like a baby from fear of needles. I am labelled all over.  I checked, right name, right doctor.

There is a cockpit, as they call it, on a large mechanical arm over my bed.  Internet, telephone, games, television, the works.  Touch screen and keyboard.

I have read some of your emails and comments this morning.  I have played sudoku.  I have watched some bad television.  I called Dad to try out the phone (and say hi, of course).

I am nervous.  Scared perhaps.  Yes, scared.  It seems a much bigger deal this time.  This hospital is modern, imposing, serious.

Darren has gone home.  Alannah has a last-minute doctor’s appointment and a party today.  The combined first birthday party of our mothers group boys and girls.  No one is actually one yet, not until 29 June, but they will all come in a rush soon.  I am happy that Alannah isn’t missing out.  It will all be over by the time I wake up.  I wonder how much I will miss out on as this runs its course.

Lalalalalala.

Sometimes I can distract myself.  More often than not.  Other times it feels like time stands still. I was in the bath for half an hour on Thursday night.  Lying still and staring at my orange toe nails.  Seeing them and not seeing them.

I am feeling calmer now.  It comes in waves.

Three hours of waiting will do that.

Ruth knows I am waiting and nervous.  Bless her cottons socks.  She just called to say they are caught up in surgery and she will be in soon.  I shall seek out some more distraction until then.

Second biopsy -18 June 2011

Ready

Tomorrow is my second biopsy.  We found out yesterday, filled out the paperwork today, and need to be at the hospital by 8.30am tomorrow.

I didn’t expect to be here again.  I do know what to expect.  From the surgery and the recovery.  But am I ready?  It’s not the same thing.

I’ll be in overnight and home on Sunday. For the first few days, it will be sleep, antibiotics, pain meds, more sleep.  The general anaesthetic and panadeine forte knock me about a bit.  It hurt to talk for a few days last time.

In all this sudden activity, I know I haven’t replied to all your emails.  I will.  I’ve had a few things on, ya know?  Don’t think I don’t love hearing from you.  And please don’t think you need to say something profound.  I know it’s hard to know what to say.  I totally get it.  Normal is good.  Chit chat is good.

There have been a few lovely food deliveries this week.  Thank you.  I feel very cared for.

If I’m up to it, I’ll be online here Saturday night or Sunday morning for a quick hello.  I’ll have to tell you about the facilities in my hospital room sometime, quite incredible.  Darren might be on as well to say a few words.

Until then.

Is this happening?

It is all quiet here now.

There’s the soft sound of watery white noise through the monitor and Alannah coughing intermittently.  My parents have gone home.  Darren has gone to bed.  He isn’t sleeping much or well.

I have a cup of tea and my notes from our telephone call with Dr P this evening.  He listened.  He answered our questions.  He reassured us.  Perhaps it was my initial judgment that was post-haste.

He ran us through the basics to begin.  Ameloblastomas are locally aggressive, destroying much around them but not damaging further afield.  My ameloblastoma is the multi-cystic or multi-locular variety, meaning there is more than one area of erosion of the bone.  Only the unicystic variety of ameloblastoma is treated conservatively.  The other types, including mine, are treated aggressively with resection with a wide clearance.  That’s roughly a 2cm to each side of my 10cm damage zone.

The incidence of malignancy in ameloblastomas is fairly small, he said.  They like to check.  The recurrence rate for benign ameloblastomas is high.  With the aggressive resection he undertakes, however, he thinks the recurrence rate is less than 15%.  I don’t believe we touched on the recurrence rate for malignant ameloblastomas.

The MRI scan enables them to see the difference between the spongy and hard bone.  It showed the tumour had breached through the cortex into the soft tissue.  That, if I understood correctly, is the area of concern on which they will focus their attention in the second biopsy this Saturday.

They will send the biopsy samples to a pathologist for examination under a microscope.  They will look for signs of ameloblastic carcinoma or sarcoma.  They’ll look to see if there is infiltration of the lymph nodes or blood vessels, if there is potential to metastasize (spread to other parts of the body), and how the cells look.

Can the pathologist say for certain?  Will you need a second option?  Malignant ameloblastoma can be difficult to diagnose, he agreed.  The pathologist at Westmead Hospital for my original biopsy is a world leading figure.  He will ask a pathologist at Royal Prince Alfred Hospital to take a look.   The pathologists in this field all know each other and usually like to pass it around to get others’ opinions.  A group of them get together formally every Friday fortnight and may look at it together.

All in all, it’s a lengthy process.  It may take one to two weeks for the results of this second biopsy.  The results of the first biopsy took nearly two weeks.  I won’t know until right towards the end of June.

If the biopsy results do not come back as clearly benign, I’ll likely have a CT PET scan.  Cancer cells, among others, take up glucose and the radio-isotope scan will show if this is happening in my jaw.

Whether they think the tumour is benign or malignant, the treatment is aggressive: resection with a wide margin and reconstruction.  If they still think the tumour is benign but locally aggressive, they will only take our my level 1 and 2 lymph nodes.  This will allow them the access they need to get the fibula flap in through my neck to reconstruct my jaw.  If they think the tumour is cancerous, they will take out the level 3 lymph nodes as well.  These are located below the jaw in the glands.

Waiting on the biopsy results alone and leaving the CT PET scan aside, this brings us right up to school holidays.  Can he be sure that the tumour won’t cause more destruction in that time?  Even if the tumour is benign, could the joint at the top of my jaw not be compromised in that time?

In his 25 years experience in this field, he has only once or twice seen the jaw joint removed.  The tumour has probably been growing for years and he would prefer to wait to have all the information available.  If he is allocated an operating room by the hospital, he assured us, he can operate the day he is back.

That’s mid July.  A month away.

In March 2007, they found a cyst on my right ovary when it hemorrhaged.  It had grown to 10 by 15 cm when they operated to remove the cyst in June or July that year and decided to remove the ovary and appendix as well because the cyst looked pre-cancerous.   It wasn’t, but the danger of delay is on my mind.

It occurred to me that, in preparation for that surgery, I had the blood test for the cancer marker CA125.  While I didn’t have the marker, that wouldn’t exclude cancer here, he said.  (Indeed, a quick google indicates that’s only helpful for ovarian or breast cancer.)

However, he mused, did I know the ovaries were the most common location for tumours?  And did I know the second most common?  Yes, the jaw. Lucky me.

He is happy to see us all before the surgery.  He’ll show us the scans and answer any more questions.  He can also arrange for me to meet the young man on whose malignant ameloblastoma he recently operated.  Yes, I said, I’d appreciate that.

Then somehow, almost as we hung up, the kicker.  Even this biopsy coming back as benign will not exclude malignancy.  Only examination of the full tumour under a microscope can do that.

And on that bright note, we said our goodbyes.

This is not good

The CT and MRI results from Tuesday are in.

They are not good.

We have a telephone conference tonight with Dr P and they are scheduling me for a second biopsy on Saturday afternoon.

They need to find out more than the MRI can show them.  They need to exclude malignancy.

Malignancy.

The next step might be a CT PET scan to determine whether my ameloblastoma is in fact cancer.

Cancer.

Read on below if you can.  We hope Dr P can explain it tonight.

CT scan

Report: There is an expansile unilocular lesion involving the posterior left mandible and ramus. The second and third left mandibular molars are absent. There is no evidence of an unerupted tooth. The mandibular canal is displaced inferiorly and its roof is dehiscent. There is thin remodelled bone peripherally with areas of focal dehiscence through the buccal and lingual cortex and inferior border of the mandible. There is periosteal reaction seen anteroinferiorly over the buccal cortex. No osteoid matrix is present. Extraosseous extension is better demonstrated on the MRI performed today. No other bone lesions are seen.

Conclusion: There is an expansile lesion within the posterior left mandibular body and ramus, consistent with the histological diagnosis. There is extension through the cortex into the soft tissues. The periosteal reaction is atypical and may represent secondary infection. A malignant ameloblastoma should also be considered.

MRI scan

Report: There is a expansile mass involving the posterior left mandible body and ramus. The mass is largely isointense to muscle on T1 and slightly hyperintense on T2. Centrally there is an area measuring of T11T2 hyperintensity.The mass demonstrates avid enhancement, with a small central non enhancing component measuring 8mm.

There is dehiscence of the buccal conex medially and the lesion extends into the submandibular space abutting the mylohyoid muscle. There is periosteal reacti0n anteriorly over the lingual cortex and the mass extends through the cortex and perlosteum, buccal gingival sulcus and buccinator msucle. The mass surrounds the facial artery and is limited faterally by the facial muscles. There is edema and enhancement seen around the anteror border of the masseter muscle.

Posteriorly there is involvement of the mandibular ramus and the lesion abuts the anterior border of the medial pterygoid muscle. Inferiorly the inferior areolar canal is dehiscent and displaced. The tumor breaches the lower border of the mandible to project into the submandibular space.

There is normal fat within the pterygoid palatine fossa. There is no evidence of perineural tumour spread along the trigeminal nerve. There is no denervation of the muscles of mastication. There is a left retropharyngeal lymph node measuring 6mm. There are non enlarged lymph nodes in level 1B.

Conclusion: There is an enhancing mass within molar ramus region of the left hemi-mandible, corresponding to the the histologically diagnosed ameloblastoma. There is extraosseus extension into the soft tissues as described and inferior displacment and dehiscence of the inferior alveolar canal. Periosteal reaction is an atypical finding and may indicate secondary infection. Infection could account for some of the soft tissue changes laterally. A malignant ameloblastoma should also be considered.