This post is going to be totally off topic from my usual posts; in this post I will be talking about the type of cancer I was diagnosed with back in late 2009 (DSRCT). What I want to do is simply talk about the nature of the cancer itself; its prognosis; its treatment; and the side-effects someone can expect as a result of said treatment (based upon my own experience). I am motivated to write this post, for one thing, because a young guy (34) I’ve been praying for, alongside multitudes of others, Nabeel Qureshi, died today; succumbing to his yearlong battle with stage 4 stomach cancer. Also, I’m motivated to write this because of some correspondence I had with another friend (and former roommate) of mine who is currently battling a terminal brain tumor. And lastly I am motivated to write this post because in the past I was part of a support group on Facebook for people who had the type of cancer I was diagnosed with—Desmoplastic Small Round Cell Tumor (DSRCT) sarcoma—or who had a family member with the diagnosis. In that group a rather outspoken person questioned whether or not I actually had DSRCT because I actually survived it (as you will see from the prognosis I share below, DSRCT is typically a terminal and incurable cancer with no known treatment). In conclusion of this post I will attempt to bring it all into perspective with a discussion about God’s providential care and purposes in the midst of human suffering.

What is Desmoplastic Small Round Cell Tumor (DSRCT)?

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Patients typically present with symptoms of abdominal sarcomatosis. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma. DSRCT is associated with a unique chromosomal translocation t(11:22) (p 13; q 12) that involves the EWSR1 and WT1 genes. The prognosis is particularly poor; median survival ranges from 17 to 25 months, largely due to the presentation of the majority of patients with metastatic disease. Management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation. Several methods are being evaluated to improve survival: addition of chemotherapy and targeted therapies to standard neoadjuvant protocol, completion of surgical resection with HIPEC, postoperative IMRT, treatment of hepatic metastases with [⁹⁰Y]Yttrium microsphere liver embolization.[1]

So, DSRCT is an aggressive and rare cancer that starts in the abdominal walls of patients and typically metastasizes quickly from there. It is classified as a pediatric cancer most often plaguing males from early adolescence up until forty years old (I was thirty-five at my diagnosis). It is hard to identify DSRCT as DSRCT given the rarity of this disease. In my case it took a full month, with two biopsies and three different labs to come to the correct and final diagnosis. They initially diagnosed me with non-Hodgkin’s Lymphoma, which would have been preferable, given that it is in the “curable category.” What this description also shows is the nature of the cancer itself, in regard to the prognosis; but let’s press into this a little further, the prognosis is quite dire. Here is study that demonstrates how dire DSRCT is; in this study several DSRCT cancer patients were evaluated based upon their response to chemotherapy treatment. What becomes apparent is how incurable this disease is; you will notice that even those who responded favorably to the treatment still only made it twenty-two months on average:

Prognosis

… The median survival time was 19 months for all patients and 22 months for the 7 achieving complete response to chemotherapy. An ongoing trial of NCI evaluates the addition of irinotecan, temozolomide, and bevacizumab to P6 protocol. It is also not clear if such high doses of chemotherapy are any more useful than standard doses of chemotherapy employed in Ewing sarcoma and similar small round cell tumors. Given the poor survival despite these high chemotherapy doses, in the adult population we generally employ lower doses than those described in the Kushner paper.[2]

In this particular study nobody survived more than twenty-two months; I think there were sixty-six patients involved in this study. And in my own experience, since my diagnosis, I have come into personal contact with others who have been diagnosed with DSRCT, and only one out of many (besides myself) have survived; so the statistics are accurate, unfortunately. What this translates to, when viewed from a five year perspective, is that there is a fifteen percent chance that someone who receives the DSRCT diagnosis will survive to the five year mark; and even if they do, they will have had been fighting it as a chronic disease up until this point. Typically the twenty-two months mark is the median survival rate for those unfortunate enough to receive the DSRCT diagnosis.

Treatment

Referring to the same study we have been engaging with, this is how it describes the treatment for desmoplastic small round cell tumor:

Therapeutic management of DSRCT remains challenging with low efficacy despite the combination of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation.

Aggressive surgical debulking is the mainstay of the therapeutic strategy. Debulking surgery is defined as definitive removal of at least 90% of the tumor burden. Two retrospective studies of prognostic factors in 32 and 66 patients with DSRCT respectively, identified gross tumor resection as a highly significant predictor of prolonged overall survival [15, 16]. Lal et al. reported a 3-years survival of 58% in patients treated with debulking compared to no survivors beyond 3 years in the nonresection cohort ().

DSRCT is known to be at least somewhat chemosensitive [17] and radiosensitive tumor. The main series evaluating the efficacy of chemotherapy was reported in 1996 by Kushner et al. [18]. Twelve patients were treated with the P6 protocol: 7 courses of chemotherapy with cyclophosphamide (4200 mg/m²), doxorubicin (75 mg/m²) and vincristine (HD-CAV) alternating with ifosfamide (9 to 12 mg/m²) and etoposide (500 to 1000 mg/m²). All tumors responded to HD-CAV, but there were no pathological complete response. Two patients died after chemotherapy (1 Budd-Chiari syndrome and 1 infectious complication). Following response to this induction regimen, tumor resection was attempted; local radiotherapy and myeloablative regimen comprising thiotepa (900 mg/m²) plus carboplatin (1500 mg/m²) with stem cell rescue were administered to 5 and 4 patients, respectively….[3]

I personally received the chemo-treatment noted in this study; mine in particular was an adaptation of the protocol used for the Ewing’s Sarcoma. My treatment took place at Oregeon Health and Sciences University (OHSU), at The Knight Cancer Institute in Portland, OR. My medical oncologist decided to compress the time between my cycles of chemo from the usual three weeks to two weeks with the hopes of maximizing the effects of the chemo on my cancer. As a result I experienced severe side effects that required I stay in the hospital, sometimes for more than a week at a time to recover from the chemo. I experienced: severe weight loss (a total of 55lbs), loss of appetite, mouth sores, rectum sores, anal fissure, neuropathy, neutropenia, edema, pulmonary edema, C-diff, dizziness, fainting, coughing fits, floaters in my eyes from broken blood vessels, ten units of blood transfused, blockages in my port, resection surgery, loss of my right kidney, and three inches of gortex holding my inferior vena cava together; among other things.

Summary

DSRCT is typically a terminal cancer in the incurable category. There is still no known treatment for the cancer, and yet they often use what is called the P6 protocol or will adapt the Ewing’s Sarcoma protocol as they did in my case. It is a cancer that is considered a pediatric cancer because the cells that turn into DSRCT are cells that normally would have died off during childhood; instead they morph in the body and transmute into DSRCT. It is a cancer that is rare, aggressive (my oncologists called it a monster), and thus, if following a purely traditional approach, requires aggressive measures with the hopes of slowing it down. Even though chemotherapy (and radiation) is used, it is only really used with the hopes of debulking the tumors enough in order to remove them via surgery; DSRCT is considered a surgical cancer. Often, because of the rarity of this cancer its diagnosis takes awhile, and can be mistaken for either some form of lymphoma, or even testicular cancer; which happened to me in my diagnosing process. Because of the aggressive nature of this cancer recurrence, even if the cancer is fully removed, is almost always going to happen.

I was diagnosed in late 2009, and went through multiple cycles of chemo. On May 6^th, 2010 my tumor had shrunk enough (in my case I had one big tumor next to my right kidney that stayed local but had involved at least one lymph node in the vicinity) that it became operable. The eight hour surgery was performed, and the surgical oncologist was able to fully remove my tumor along with twenty-five nearby lymph-nodes; also taking my right kidney and three inches of my inferior vena cava in order to get clean tissue margins (which they did). I recovered from surgery, briefly, and we did follow up cycles of chemo. The pathology returned on my tumor and lymph-nodes, and it indicated that the chemotheraphy had essentially killed any cancer that was in the nearby lymph-nodes, and had killed the cancer in the tumor itself upwards of ninety-five percent. Based upon this pathology report I was declared cancer free, or no evidence of disease (NED), which by God’s grace has been my status since that day in 2010.

Conclusion

DSRCT is typically a terminal and incurable cancer which still needs further research to be done. Because of its rarity, funding is not all that forthcoming towards this cancer, and so I hope by writing an article like this that bringing some awareness to it will help to provide more of a presence and thus exposure to it; such that funding for researching it will become more prevalent than it currently is.

Because of the length of this article, I will write a second installment to it where I talk more about the sad death of Nabeel Qureshi; and how God’s providence relates, in particular, to the form of human suffering given expression in the various diseases that make up the panoply of cancers in the world today. I will say this: I am obviously a Christian, and there is no doubt in my mind that I am alive today, not because of the medical treatment I received (which this article should, at the very least, demonstrate the impact that has with DSRCT); but because of God’s unfathomable mercy and grace towards me and my family. I obviously can’t say why God decided to heal me and let me live, but I can say that I know that it was him alone who immediately decided that I would live and remain cancer free of a cancer that in almost every case takes the life of the person who receives such a diagnosis. Soli Deo Gloria

[1] Isabelle Ray-Coquard, “Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings,” Volume 2012 (2012), Article ID 714986, 5 pages.

[2] Ibid.

[3] Ibid.