Non smoker with mucinous BAC - 1255065

chelle
Posts:3

This is my first time ever participating in a site like this, and unfortunately it's topic is cancer. About a month ago my 69 year old dad was diagnosed with primary pulmonary adenocarsinoma through a brochoscopy. he was originally scheduled for a left upper lobectomy. The CT and PET did not show any other cancer. However, yesterday, after a second scope of the lower left lobe, he was told that he has mucinous BAC and was not a candidate for surgery because of the likeliness of it showing up in the right lung later. The doctor provided him with information written by Dr. West explaining BAC. My dad is in very good health overall. He is a non smoker, exercises daily, and is a very positive person. The diagnosis came as a huge shock. He had been treated for pneumonia and then BOOP for nearly a year, and no one, including his doctors, suspected cancer. His BAC is the pneumatic kind. The doctors want to treat it with drugs now instead of surgery. Are there any good treatment options for him? We are meeting with his oncologist on Wednesday (3-26). Are there any studies he can participate in?
Thank you for this website, and thank you in advance for any response you can give.
Chelle Demick

Forums

Dr West
Posts: 4735

Chelle,

Unfortunately, the "pneumonic" form of BAC is named that because it looks for all the world like pneumonia, and just about everyone is treated for presumed pneumonia for at least weeks and often months before the diagnosis is made that it is BAC and not pneumonia.

If the cancer is all completely contained within one lobe, we most often favor doing the surgery even knowing that the cancer is high risk for recurring elsewhere in the lungs, often within months after the surgery. Still, that's not always the case, and if the cancer has been followed for a year now before the diagnosis was made, and the cancer hasn't spread elsewhere in that time, it suggests that surgery could still be of benefit.

In addition, many people with a pneumonic mucinous BAC are bothered by severe shortness of breath and/or a productive cough that can be relieved with surgery. We generally don't do a lobectomy as a "palliative" (non-curative) treatment if we know or suspect there is cancer elsewhere, but one exception we sometimes make is for pneumonic BAC, since that part of the lung isn't functioning the way it's supposed to, but it's having blood run through it, effectively mixing the unoxygenated blood from the BAC-affected lung with the oxygenated blood from the rest of the lung. So keeping the lung in can actually lead to worse lung function than removing it because of a so-called "shunting" effect.

As I indicated above, I'd still consider surgery to be a realistic option if there isn't evidence that the cancer is in other lobes, and if symptoms are significant enough, perhaps even if it is.

Aside from chemotherapy, which can sometimes be effective, I've seen some patients with pneumonic/mucinous BAC who have an ALK rearrangement in their tumor, so that's worth checking for. If present, that's associated with a high chance of response to oral XALKORI (crizotinib).

-Dr. West

PS: there's a ton of info about BAC on this site, more than I can summarize here.

chelle
Posts: 3

Dear Dr. West,

Thank you for your very prompt response. According to my dad's PET scan onFeb. 28, there was consolidation in the upper left lobe with SUV of up to 8.5 and two tiny nodules in the upper left lobe with SUV of 2.8. There were no other hypermetabolic foci in either lung, hila, mediastinum or abdomen. He was referred to a surgeon for the lobectomy. The surgeon reviewed the scans and saw an area of concern in the lower left lobe. The second bronchoscope of this area did not show any BAC, but the doctors reviewing the case decided that it had most likely spread out of the left upper lobe already even though they couldn't see it. His main problems are the fluid bothers him when he sleeps on his left side and some coughing when he exerts himself. Having a cold really aggravates it too. He doesn't have shortness of breath unless he exerts himself, and he still walks daily 1-2 miles for exercise to control type II diabetes. Since there is cancer in the LUL, does that mean that part of lung doesn't function at all any more? If he responds to medication, will the tissue recover and function again or will the drugs simply keep the cancer from spreading into healthy tissue? Also, will the chemo help with the fluid problem? Lastly, are ALK rearrangements common in nonsmokers and how much tissue is needed to check for ALK rearrangement? His report mentioned this; however, it said that the lobectomy would provide a greater tissue sample to check for the mutation.
Thank you so much for you time. My mom and dad both thank you as well. By reading information here, asking questions, and getting responses at least we can feel a bit more positive and in control of this situation.

Sincerely,
Chelle

Dr West
Posts: 4735

The consolidated part of the lung probably isn't functioning, but in the best case scenario, if it responds well to treatment, it could regain function. If a treatment is working, whether it's a targeted therapy or chemo, it will probably also help with the fluid collection and cough.

ALK rearrangements aren't common in lung cancer: they are seen in about 4% of people with advanced non-small cell lung cancer (NSCLC), but they're more common in never-smokers, where the probability is more like 10-15%. A core biopsy is usually enough to do testing from -- it's not necessary to do a bigger surgery for the vast majority of cases.

-Dr. West

chelle
Posts: 3

Dear Dr. West,

We met with the oncologist yesterday. My dad's tissue sample from his LUL was positive for EGFR exon deletion mutation (E746 S752>V) . KRAS was negative. He will start Tarceva. I have a few questions please:
1. If he responds to Tarceva, will it kill the cancer cells or just keep them from spreading? Is it possible to cure BAC?
2. I asked the doctor about an ALK rearrangement and he said that you never see that along with EFGR. Should it still be done anyway? I saw one report by Dr. Ross Camidge on a patient who was positive for both mutations.
3. In a second scope of his LLL 2 small biopsy fragments also showed alveoli lined by atypical alveolar cells. It was TTF 1 positive and Napsin A positive just like the tissue from the upper lobe. I think finding cancer here was why the doctors decided not to do the lobectomy as originally planned. If he responds to Tarceva, would it still be good to take out the areas of tissue with known cancer but not take the whole lung?

Thank you so much for all of the links and information you have made available on BAC. Thank you in advance for any response or advice you can give. My mom was actually ready to jump on a plane and fly from St. Louis to Seattle to have you treat my dad!

Sincerely,
Chelle

Dr West
Posts: 4735

I'm glad he has an EGFR mutation, which suggests he has a high probability of responding well to an EGFR inhibitor like Tarceva, but no, these agents don't cure BAC. They can lead to a dramatic response, even one so good that you can't see any evidence of the cancer, but it is pretty close to inevitable that the cancer will return if the cancer has spread outside of a single lobe.

I think the best we can say about an EGFR mutation and ALK rearrangement co-existing is that it's possible, though it's extremely unlikely. Almost all experts would consider it not indicated to do testing for an ALK rearrangement at this point, if an EGFR mutation has been seen. I don't think it's really an appropriate test to do, if the best we can say is that it's not impossible to have an ALK rearrangement. The presence of an EGFR mutation clearly guides the decision of the best systemic treatment to pursue.

I can't answer your last question. It's too specific for me to make a recommendation based on such a limited amount of information, and it pretty clearly veers into the range of giving a very specific medical recommendation on a specific case of someone who isn't my patient.

-Dr. West