A rare cancer — Adenoid Cystic Carcinoma of the Tonsil

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BT is a 40-year-old female who was diagnosed with Adenoid Cystic Carcinoma of the right tonsil. She came to seek our help after undergoing surgery and radiotherapy. Barely 2 years later, the cancer recurred. BT came to see us with only a piece of hand-written note from her doctor which said:

To Those Who May Concern

Name: xxxxxxxx

The above named was diagnosed with having right adenoid cystic carcinoma of right tonsil. Operation done in 2014, and completed radiotherapy 30 #. Currently notice of having recurrent adenoid cystic carcinoma of tonsil. Plan for operation removal of tumour and radiotherapy. 

Chris: Why only this piece of paper? Can’t you get a complete set of your medical reports?

Patient: No, the doctor did not want to give the film, etc. I told him I shall return the film to him again. He said no.

C: Tell me your story from the beginning.

P: In 2014, I felt something got stuck in my throat when I was eating. Then I saw a lump in my throat. I went to see a doctor and he gave me antibiotics. Many rounds of antibiotics but it did not work. One GP told me not to worry, just go home. He said, It is not cancer. I was not happy with his advice and went to consult the doctors at the university hospital. I was given antibiotics again. It did not work. Eventually in 2014, I had an operation, followed by 30 sessions of radiation. I went back to the hospital for check-up every 3 months. Everything was okay. About 2 years later, September 2016, the cancer came back again.

A CT scan was done. The doctor was not sure about some tiny spots in my lungs. I was asked to go for a PET scan in Kuala Lumpur. I did not go for the PET scan.

After the relapse, the doctor suggested that I undergo an operation again. This time, they are going to cut open my face and remove the tumour. I have to stay in the ICU for about 2 to 3 weeks. According to the doctor, it may take 7 to 8 months for me to fully recover. Cutting up my face sounds rather scary and I did not want that. I told the doctor to give me some time to think about it. It was at this point that I decided to come and see you. If possible I don’t want to do that surgery. After the surgery, I have to go for radiotherapy again.

BT was prescribed some herbs. About a month later, I got to talk to her again.

Patient: I went back to see the doctor again last week.  The doctor asked me to get admitted right away and since he had no patient, he wanted to operate on me the next day. I told the doctor to give me more time to think about this. I also told him that I am now trying the CA Care Therapy. I showed him your name card. He said, No use, this will not work!  I told him, Never mind, give me more time to consider and also try the herbs. The doctor agreed and said, Then come back and see me again in November.

C: I really don’t know what to say. You said there was something in your lung. We don’t know what that is. Let me give you Lung Tea to help you with that. We don’t know if the cancer has already spread to the lung. Now, there is also a tumour inside your mouth. Does this give you any problem?

P: No problem.

C: So are you prepared to have your face cut up as suggested by the doctor?

P: No, I don’t want that.

C: I really don’t know what other problems may crop up after the surgery and radiation. You already had surgery and radiotherapy. These did not work. You know that. And now, they are going to do the same things again — but this time more severe procedure. It is more of the same! Did you ever ask the doctor if what he is going to do is going to cure you?

P: Yes. I asked the doctor — After the surgery will the cancer come again? He kept quiet. He did not answer my question. I asked him twice! So I dared not ask him again. In my heart, I guessed he did not know the answer either.

C: Well, try to take the herbs and see if these will help you.

Comment

This is a rare cancer. In my 20 years dealing with cancer patients, I can recall just only one case similar to this one. Anita also had cancer of her tonsil. She was asked to undergo a similar surgery — cut open her face, they call it the Commando Procedure — which she promptly declined. Anita took herbs and up to this day (14 years) she is still doing fine. So much about herbal therapy being No use, this will not work!

Listen to her testimony.

 

 

I feel real sorry for BT. While I want to believe that the doctor is trying his best to help BT, I also wonder if he has enough experience to handle this case? Why is he not forthcoming in his answer to his patient’s question? Why was the patient not told the truth about the prognosis of her cancer?

Read the information below which can easily be obtained from the Internet. We know that this type of cancer is not easy to manage.  The majority of patients will develop recurrent disease. Approximately 50% of recurrences are evident within 2 years after surgery and radiotherapy. That is what happened to BT. But was she told or forewarned?

Let me highlight more of what I have learned from my reading.

  • Little progress has been made in advancing “curative” treatment of adenoid cystic carcinoma of the head and neck. The disease is said to have a fatal outcome.
  • The current therapies available for the management of patients with adenoid cystic carcinoma is inadequate to achieve local control predictably by the aggressive strategy of surgery and irradiation therapy. It is unlikely that more aggressive surgery is beneficial to patients nor to result in a significant improvement on their quantity or quality survival.

So, after the first round of surgery and radiation had failed, what hope is there for the patient if the same procedures are repeated? Is this not more of the same? Will this not cause more misery or even kill the patient sooner? 

Information about Adenoid Cystic Carcinoma (ACC)

Sources

http://oralcancerfoundation.org/facts/rare/adenoid-cystic-carcinoma/

https://www.ncbi.nlm.nih.gov/pubmed/22134294

Patrick J. Bradley, Adenoid cystic carcinoma of the head and neck: a review.

Curr Opin Otolaryngol Head Neck Surg 12:127-132. (c) 2004 Lippincott Williams & Wilkins.

  • This is an uncommon form of cancer that arises within secretory glands, most commonly the major and minor salivary glands of the head and neck.
  • Other sites of origin include the trachea, lacrimal gland, breast, skin, and vulva.
  • ACC primary tumors occur in 38 different organs of the body, with approximately 58% occurring in the head and neck region.

Signs & Symptoms

  • Early lesions of the salivary glands present as painless masses of the mouth or face, usually growing slowly. Advanced tumors may present with pain and/or nerve paralysis, because ACC invade the peripheral nerves.
  • It is rare type of cancer and is generally advanced when diagnosed.

Clinical Course:  The clinical behavior of ACC is a paradox:

  • It is slow growing and is relatively indolent (indolent can mean a problem that causes no pain, or is slow growing and not immediately problematic). But later the cancer can be relentless and progressive.
  • Unlike most other cancers, patients with ACC survive for 5 years, only to have tumors recur and progress after that. In a recent study of a group of 160 ACC patients, survival was 89% at 5 years but only 40% at 15 years. So its 5-year survival rates are optimistically high, but 10- to 20-year survival rates are dismally low.
  • An unusual feature of ACC is that, unlike most cancers, it seldom metastasizes to regional lymph nodes.
  • But the cancer can spread to the surrounding nerves (perineural invasion) and also to distant sites.
  • The lung is by far the most common site of metastasis, with the liver being the second most common site. It also spreads to the bone.

Treatment

  • A standing medical theorem is “Remove the cancer from the patient.” Because of the high propensity for perineural spread and the difficulty in achieving clean surgical margins, many doctors recommend a combination of surgical removal followed up with radiation treatment to the tumor region.
  • There appears to be no effective chemotherapy.

Perineural invasion

 

  • ACC has a very high tendency to microscopically infiltrate the adjoining nerve tissues around the tumor site, which is called perineural invasion.
  • Nerve tissues microscopically appear like pipes with strands of wire running through them. ACC finds a “path of least resistance” by growing along the sheathing that surrounds those strands of wires inside the pipe. This creates a real challenge for both diagnosis and treatment since the cancerous cells are very small rather than a large cluster, and don’t necessarily show up on a MRI or CT scan. 

Effectiveness of treatment

  • Despite local aggressive therapy, the majority of patients (60%) will develop recurrent disease. Approximately 50% of recurrences are clinically evident within 2 years after surgery and radiotherapy.
  • Multiple locoregional recurrences are frequent and may present as early as 2 years.
  • Patients survive with recurrent and metastatic disease for several years despite not being offered any treatment.
  • Little progress has been made in advancing “curative” treatment of adenoid cystic carcinoma of the head and neck. The disease is said to have a fatal outcome.
  • ACC of the nasal cavity and paranasal sinuses has a worse prognosis than in any other area of the head and neck region.
  • The current therapies available for the management of patients with ACC is inadequate to achieve local control predictably by the aggressive strategy of surgery and irradiation therapy. It is unlikely that more aggressive surgery is beneficial to patients nor to result in a significant improvement on their quantity or quality survival.

 

 

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