Two Week Review

Last Friday we met with Dr. Ferriera, the amazing neurosurgeon who handled my craniotomy, to follow up briefly on the post-surgery pathology report. Here’s what he said: it turns out that the tumor is a grade two myxoid chondrosarcoma. He reiterated that he was able to remove approximately 95% of it.

He said that the tumor is benign, which doesn’t make sense, because my understanding is that a chondrosarcoma is, by definition, malignant. What it tends not to be is metastatic, which is what I’m guessing he wanted to get across when he said it was benign – that it is not the sort of tumor that is inclined to spread to other body parts.

Whether it is inclined to grow, shrink, or grow back as a different variety of chondrosarcoma is less clear. He said it is possible for someone to do nothing more than we’ve done here and live to be 100 with no additional recurrence. Of course, the research makes clear that isn’t the only possibility.

Dr. Ferreira also said said not to worry about it being grade two.

He said the options now include:

  1. Leave it alone, checking back regularly to see how things are going, and wait to see whether anything else happens.
  2. Have the remainder of the tumor removed surgically, which should be a bit easier than the first surgery because the parts he left are the “low hanging fruit” – he’s already removed the most difficult stuff.
  3. Get proton radiation therapy, either instead of or in addition to additional surgical removal. This has its own risks, of course, but is a standard and well-supported treatment for chondrosarcoma.

He said we’d have another MRI in about four weeks, once the surgery healing is farther along, to see where we are and what remains of the tumor once the swelling has gone down. That will give us more information to help us decide how to proceed.

He said he has no preferences or recommendations about which of the treatment options are best, and will be equally happy to pursue any of them, depending on what we’d like to do.

I didn’t know what else to ask before processing that information (and my processing isn’t as efficient as usual at the moment), so I went home to do some research on grade two myxoid chondrosarcomas. What I discovered is that there didn’t seem to be any understandable or helpful information about skull base grade two myxoid chondrosarcomas. Nada.

So I’ve requested a copy of the pathology report that I hope will provide more detailed information. Maybe if the pathologist used other descriptive words besides “myxoid”, it will help narrow down how to understand the results and offer some additional guidance on how to proceed.

I got the impression from Dr. Ferreira that he might feel as though taking a wait-and-see approach wouldn’t be a bad idea. I’ve read research indicating that when chondroscarcoma reoccurs after having been removed once already, it’s often a far more destructive variety; but he pointed out that with all likelihood, that’s the type of variety that would probably reoccur in any case. Fair point.

Still, I can’t get Bloch’s research on recurrence out of my head. It’s compelling. Here are some of the more relevant bits:

“A total of 560 nonduplicated chondroscarcoma patients met inclusion criteria for this systematic review. The recurrence rate among all patients as 22% (93 patients) with an average disease-free interval of 32.5 months (median, 16 months)…

A total of 161 patients has surgery alone, and 325 patients had surgery in addition to postoperative adjuvant radiation therapy. Additionally, 46 patients underwent radiation therapy alone without surgical resection. The recurrence rate was higher in the group of patients that had surgery alone compared with surgery and radiation (44% versus 9%) [based on a five year recurrence rate, per later details]…

There were a total of 452 patients in our analysis who possessed chondorsarcomas of the conventional subtype, and 60 patients who had the mesechymal subtype. As expected, the recurrence rate was lower among patients with the conventional subtype (16% versus 63%) [per later notes, myxoid appears to be included as being part of the conventional subtype, so that’s nice]…

On pathological evaluation, a total of 364 patients had grade 1 disease, 80 patients had grade II disease, and eight patients had grade III chondrosarcoma. Not surprising, the lowest recurrence rate was observed among patients with grade I tumors (15%), and the highest recurrence rate was observed in the grade III group (33%), with grade II tumors in the middle (16%). There was no statistically significant difference in recurrence in patients with different grade tumors…

A large body of research has shown that many factors affect the prognosis in patients with cranial chondroscarcoma. Histological subtype, previous treatment (surgery or radiation therapy), and extent of tumor resection and the use of adjuvant postoperative radiation therapy have all been shown to influence patient outcome; however, local recurrence is considered by many to be the most significant predictor of overall mortality in these patients…

To assess whether radiation was of benefit for all patients despite the degree of resection, we divided the cohort of surgical patients into those with gross total versus subtotal resection when these data were available. Although these data were only available for a subset of the patients reviewed in our analysis, the results demonstrated a clear, statistically significant benefit in recurrence-free survival for patients receiving postoperative radiation regardless of degree of resection. Furthermore, the overall rate of tumor recurrence was comparable for patients with gross total versus subtotal resection after postoperative radiation therapy was given…

Evans et al demonstrated [the] correlation between survival and histological grade perfectly in his study of chondrosarcomas from all body sites. They reported 5-year survival rates of chondrosarcomas grade I to be 90%, grade II to be 81%, and grade III to be 43%. Bearing this in mind, it was not surprising when the recurrence rate found in our study correlated directly with the histological grade of the chondrosarcomas…” Bloch, et al, 2010

Also, from Bloch’s 2009 research on survival rates among chondrosarcoma patients, which appears to be based on the same dataset:

“The 5-year mortality among all patients was 11.5% with median survival of 24 months. Mortality at 5 years was significantly greater for patients with tumors of a higher grade or of the mesenchymal subtype, or had received surgical resection alone, when compared with their respective counterparts…

The 5-year mortality rate was higher in the group of patients that had surgery alone (25% versus  9%)…

A total of 68 patients received previous treatment, while 492 patients were being treated for the first time. Surprisingly, there was no difference in mortality between these two groups of patients (10% vs. 12%)…

The mortality rate was lower among patients with the conventional subtype (6% vs. 54%)…

Not surprising, the lowest mortality rate was among the patients with grade I (5%) and the highest mortality rate was observed in the grade III group (25%), with the grade II in the middle with a 10% mortality…

Although adjuvant radiation therapy appears to reduce the mortality rate of chondrosarcomas, further investigations into the usefulness of adjuvant radiation therapy for low-grade chondrosarcomas must be performed. It is possible that low-grade chondrosarcomas have good survival prognosis with surgery alone and be better served with resection without immediate radiotherapy or with delayed adjuvant radiation.” Bloch et al, 2009.

And for a change of pace, from Geng, et al, 2014:

“The treatment of cranial base chondromas and chondrosarcomas is a formidable challenge. Radiotherapy for these tumors remains controversial, but several studies have shown that proton-beam radiation achieves high rates of local tumor control and survival following maximal surgical resection.”

Some worrisome feedback from Iyer, et al, 2012 about stereotactic radiosurgery – proton beam radiation therapy is a newer of type of radiosurgery, so much of this research may be based on previous types, like gamma knife. Still – helpful to know:

“To assess outcomes after stereotactic radiosurgery (SRS) for chondrosarcomas of the skull base we reviewed 22 patients with cranial base chondrosarcomas who underwent SRS between 1987 and 2009… At a median follow-up of 75 months after SRS, seven patients died due to tumor progression. The actuarial overall survival after SRS for the entire group of chondrosarcoma patients was 95, 76, 70, and 56% at 1, 3, 5, and 10 years, respectively. Factors associated with longer survival after SRS included patient age >40 years, a shorter interval (<6 months) between diagnosis and SRS, and either no or a single prior resection… The ability to achieve tumor control in patients with chondrosarcoma is likely to be enhanced by earlier timing of SRS after diagnosis and multimodal management, beginning with resection when feasible followed by early SRS for progressive residual tumor.” (Iyer, et al, 2012)

More cheerful results from Jones, et al., 2014:

“Patients with skull base chondrosarcomas between 1973 and 2009 were identified from the USA Surveillance, Epidemiology, and End Results (SEER) database… We identified 226 patients with skull base chondrosarcomas. Median follow-up was 5.4 years. Median overall survival was 22 years, and 10 year survival was 68.2%. Most patients underwent surgery (92.5%). Few received radiation after diagnosis (38.1%). Ten year survival for all patients treated with surgery was significantly increased compared to those without surgery (69.3% versus 53.9%). (ibid.)

I’m waiting for the detailed notes from the University of Washington, and will let you know if they provide more helpful information. For now, I’m going to re-read the current range of journal articles to see if anything helps in ways I might have missed the first time around. (ibid.)

 

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