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.

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.

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.