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  • sayhey
    started a topic Unclassified sex cord stromal tumor

    Unclassified sex cord stromal tumor

    Long time lurker and (almost) first time poster checking in with my story, that will hopefully be helpful to others.

    I first found a lump in my left testicle sometime around May or June of 2018 after discovering an unrelated small movable marble sized lump on my ribs (dermatologist and PCP diagnosed as a lipoma). After some Dr. Googling I was pretty sure it was an epididymal cyst and since I had an appointment with my primary care physician scheduled for early August, I waited for that visit to go in for a consultation.

    He thought it was likely a cyst as well, but scheduled me for an ultrasound a couple of weeks later. I had the ultrasound, and when I received a phone call from my doctor's office that same day a few hours later I knew it was likely something more serious than a cyst.

    He referred me to the urological surgical oncology clinic, where I met with the surgeon who confirmed it was likely testicular cancer and scheduled me for a blood tests, a CT scan, chest x-ray and scheduled me for surgery.

    Blood tests came back negative for tumor markers, CT scan and chest x-ray was clean.

    I had my surgery on September 6, 2018 at 7:30 am and was feeling well enough to leave the hospital before 10:00 am the same morning. I woke up from surgery with a little nausea and pain, received a single opioid painkiller and some anti-nausea medication and was dressed and messaging my wife that I was bored and to pick me up at 9:14 am. Recovery has been uneventful and smooth, I had surgery for a right-side inguinal hernia back in the early aughts so I knew what to expect. Stayed away from opioid pain killers, and used the occasional ibuprofen and icing to manage the pain.

    After the surgery, the surgeon's assistant called me to reschedule my post-op visit because my pathology report wasn't yet prepared so I finally went in for the post-op visit a little before three weeks post-op where the surgeon said everything was healing well but that I had a very unusual tumor and that he would be referring me to a medical oncologist to handle my post-operative treatment plan.

    The pathology report is a total of five pages long, with the final diagnosis being an unclassified sex cord stromal tumor with spindle cells, 1.7cm.

    The surgeon said that there aren't well-established guidelines for active surveillance as in the case of classical seminomas or NGCTs. In the pathology report, they called out the small size (1.7 x 1.4 cm) and lack of necrosis or lymphovascular invasion as favorable features but call out “elevated mitotic activity [that] may portend a risk for recurrence.” This specific kind of tumor is malignant in approximately 10-20% of adults, but hopefully I've caught it early enough that all I will need is surveillance even though I have one of the risk factors for malignant potential.

    Since then I'm in a holding pattern waiting for my next visit and have gone through a crash course in sex cord stromal tumors, reading all of the journal entries and resources I can find on this uncommon diagnosis that aren't behind a paywall (if anybody has access to journals through their university, I would love to get a few PDFs from you). I've already contacted the International Ovarian and Testicular Stromal Tumor Registry ( and will be sending them my file as well.

  • sayhey
    They closed me up with skin glue, which was a little unexpected for me but also a positive since I won't have to have anything taken out. When I coughed for the first time I was a little terrified since it felt like I ripped myself open but all was good.

    I've been wearing the abdominal binder pretty religiously though, so that helps with the occasional cough, as well as giving me more confidence.

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  • biwi
    Good to hear it went smooth! Be-aware for bumps in the recovery road though! For me, my only annoyance was once I got my staples out, I needed to steri-strip my incision for probably 2 weeks or so as the incision was trying to open again.

    I'm sure you feel good about your choice given it has the best chance of preventing recurrence, as well as how your recovery is going.

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  • sayhey
    Just wanted to thank everybody for all of the well-wishes, and provide a quick update before a more detailed one later on once I receive my pathology and am after my follow-up visit.

    I had the open RPLND on December 26th, and walked out of the hospital on the 29th. I've been recovering at home since, making the occasional venture outside of the house whenever weather allows.

    I've been very pleasantly surprised at how easy everything has been, especially based on all of the different experiences I have read through. I am pre-chemotherapy so I am sure that's a huge part of it, but on all fronts this has been an easier experience than I anticipated.

    I had an epidural put in before the surgery, which made a huge difference in my initial recovery. It was removed on the 28th in the morning along with my Foley catheter. I received a single 10mg oxycontin before they removed the epidural, but it made me nauseous so I had them switch me to Ibuprofen. When in bed or sitting down, the pain was never more than uncomfortable. Getting up initially was painful, and can still be uncomfortable depending on my position, but nothing out of control. I stopped taking all medication (stool softeners, pain killers, and gabapentin) on the 1st.

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  • JoeTheAstronaut
    Good luck with it, sayhey! Sounds like you're in good hands, and Dr. Einhorn recommendation should help too

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  • sayhey
    Thanks to everybody for the kind words and advice.

    This is going to be a pre-chemotherapy RPLND for stage I disease, my particular pathology is unresponsive to chemotherapy or radiation as well as being very rare, so we don't have much data to work with in making the decision for treatment options. We have opted for the RPLND because it will be a useful staging tool to exclude or diagnose occult metastatic disease, and in case they do find evidence of spread into the nodes it will be a curative surgery since it'll have been caught early.

    I still don't know if it will be open or laparoscopic, but my surgeon will be consulting with Dr. Einhorn and I'll go along with whatever they decide in terms of how they conduct it and what template.

    My surgery is going to be at UCSF with Dr. Maxwell Meng, so I am confident going in.
    Last edited by sayhey; 12-15-18, 05:08 PM.

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  • biwi
    A few months down the road, you will not be any different. So I would probably lean towards full open. Mine was, and have zero long term complications and had a smooth full recovery.

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  • Trekga
    Not familiar with your type of tumor. I hope someone who knows more can suggest what might be better in your case as far as open or robotic. My personal belief is that open RPNLD is a must post chemo for TC. As 1st line treatment for TC it depends on type and stage IMHO & must be done by a surgeon who does more than 1-2 a year if done laparoscopic! My son had post chemo RPNLD, & chose not to travel to IU. Let us know what you decide.

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  • eraeparker
    My boyfriend was given the same choice after the path on his testicle came back. He had a burnt out testicle tumor so we didn't know risk factors or tumor type and were going off of one lymph node that was borderline enlarged and AFP that was normal, but had not dropped as much as it should have. He ended up choosing bilateral open since it was more aggressive treatment and would bring him more peace of mind, especially since burnt out tumors are generally an indication of more aggressive cancer. His pathology showed that he had made the right choice. With only one lymph node on the border of being enlarged (1.3 cm), no one was expecting much active cancer, but 31/47 lymph nodes had active cancer, including quite a bit of teratoma.

    The surgery itself was no walk in the park, but recovery does happen quicker than you think, assuming you can avoid major complications. We were in the hospital for a full week after, and then the aortic bleed happened less than a week after being home, so had to basically restart recovery after that. It still went fast though. He was even running 3 weeks after! Doctor experience with this is really important, as you know. We went to IU to talk to Dr. Einhorn and Dr. Cary, but ended up getting the surgery in Chicago, since that is home and we were extremely lucky to have a center of excellence in the city.

    I'm not sure if this was helpful, but while we didn't deal with a sex cord stromal tumor, we have plenty of experience with having to make decisions with very little information.
    Last edited by eraeparker; 12-14-18, 11:55 AM. Reason: Edit to add that RPLND was primary treatment. He had to do chemo as adjuvant treatment.

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  • sayhey
    I had a great meeting with the surgeon today, he agreed that in my particular circumstances it would be best to proceed with the RPLND but he gave me the unenviable position of choosing between an open procedure versus laparoscopic. He mentioned that at Indiana or MSK, there wouldn't be a choice and they would proceed with an open RPLND but he would be comfortable doing either in my case.

    My last set of scans came back negative for any metastatic activity, which is a super positive, but there is still a chance for occult metastatic disease with my unique pathology and risk factors.

    Any thoughts and personal experiences to share with either procedure?

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  • sayhey
    Thanks for the kind words, and thoughts. I'm having the normal "scanxiety" ahead of my CT scans and chest X-ray next week, before I meet with the surgeon the week after.

    I've compiled a list of relevant journal articles, along with some of my notes, that should be of relevance to anybody researching sex cord stromal tumors. To anybody with the same or similar diagnosis, if you'd like to reach out to discuss and just talk feel free to post or send me a message.

    Does retroperitoneal lymph node dissection have a curative role for patients with sex cord-stromal testicular tumors?
    Mosharafa AA, Foster RS, Bihrle R, Koch MO, Ulbright TM, Einhorn LH, Donohue JP

    Clinical Outcomes of Local and Metastatic Testicular Sex Cord-Stromal Tumors
    Silberstein JL, Bazzi WM, Vertosick E, Carver BS, Bosl GJ, Feldman DR, Bajorin DF, Motzer RJ, Al-Ahmadie H, Reuter VE, Sheinfeld J.

    Pathologic Risk Factors for Occult Metastatic Disease in Adolescent and Adult Patients with Stage I Testicular Stromal Tumors
    Rove KO, Maroni PD, Cost CR, Fairclough DL, Giannarini G, Harris A, Schultz KA, Cost NG

    Pathologic Risk Factors in Pediatric and Adolescent Patients With Clinical Stage I Testicular Stromal Tumors
    Rove KO, Maroni PD, Cost CR, Fairclough DL, Giannarini G, Harris AK, Schultz KA, Cost NG

    Sex Cord Stromal Testicular Tumors: A Clinical Series—Uniformly Stage I Disease
    J.M. Featherstone, H.S. Fernando, J.M. Theaker, P.D. Simmonds, M.C. Hayes, G.M. Mead

    Wessex regional cancer center in the UK, 38 men older than 18 years with sex cord stromal testicular tumors over a 25-year period fro 1982-2006. All patients were treated with removal of primary tumor only. No patients developed metastatic disease.

    Editorial Comment
    David A. Diamond
    Department of Urology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts

    These authors review a considerable experience with clinical stage I SCS testicular tumors, which proved to be nonmetastatic, and compared this to a literature based series of metastatic SCS testis tumors to determine predictors of malignant spread. Based on this comparison they conclude that routine prophylactic RPLND should not be performed in the absence of radiological evidence of solitary retroperitoneal disease. I would argue that they have identified histological predictors of aggressiveness and should use them to determine the applicability of RPLND whether or not retroperitoneal nodes are visualized.

    Metastatic Relapse After Initial Clinical Stage I Testicular Leydig Cell Tumor
    Nicholas G. Cost, MD, Paul D. Maroni, MD, and Thomas W. Flaig, MD

    70-year old healthy man presented with 2.1cm Leydig cell tumor with moderate (4-5 mitoses per HPF), as well as invasion of spermatic cord. No LVI, cellular atypia, or tumor necrosis. CT scans were negative. Serum testosterone and Inhibin levels were normal (can be elevated in LCT).

    Diagnosis was Stage 1B LCT.

    2 years after initial presentation CT scans revealed a 1.2-cm right inguinal lymph node and a 1-cm left para-aortic lymph node. No action was taken, and a follow up 3 months later showed an increase in size.

    Patterns of Care and Survival Outcomes for Malignant Sex Cord Stromal Testicular Cancer: Results from the National Cancer Data Base.
    Banerji, JS; Odem-Davis, K; Wolff, EM; Nichols, CR; Porter CR

    EAU Guidelines on Testicular Cancer
    P. Albers (Chair), W. Albrecht, F., Algaba, C. Bokemeyer, G. Cohn-Cedermark, K. Fizazi, A. Horwich, M.P. Laguna, N. Nicolai, J. Oldenburg

    9.4 Treatment of Leydig- and Sertoli cell tumours

    Prophylactic RPLND is unjustified for patients with clinical stage I disease without high-risk features [314].

    9.5 Follow-up of Leydig- and Sertoli cell tumours

    Without clinical signs of malignancy, an individualised surveillance strategy after orchidectomy is recommended in patients with one, or more, pathological features of malignancy. Follow-up is recommended in all high risk patients; every 3 to 6 months with physical examination, hormone assays, scrotal and abdominal ultrasonography, chest radiography, and CT [293].

    9.8 Other sex cord/gonadal stromal tumours

    Sex cord/gonadal stromal tumours may be incompletely differentiated or in mixed forms. There is limited experience with incompletely differentiated sex cord/gonadal stromal tumours and no reported cases of metastasis [32]. In mixed tumour forms, all the histological components should be reported. However, the clinical behaviour most likely reflects the predominant pattern or the most aggressive component of the tumour [320].

    [314. Featherstone, J.M., et al. Sex cord stromal testicular tumors: a clinical series--uniformly stage I disease. J Urol, 2009. 181: 2090.]

    [293. Suardi, N., et al. Leydig cell tumour of the testis: presentation, therapy, long-term follow-up and the role of organ-sparing surgery in a single-institution experience. BJU Int, 2009. 103: 197.]

    [32. Jing, B., et al. Metastases to retroperitoneal and pelvic lymph nodes: computed tomography and lymphangiography. Radiol Clin North Am, 1982. 20: 511.]

    [320. Perito, P.E., et al. Sertoli-Leydig cell testicular tumor: case report and review of sex cord/gonadal stromal tumor histogenesis. J Urol, 1992. 148: 883.]

    Adult type granulosa cell tumor of the testis with a heterologous sarcomatous component: case report and review of the literature
    Thomas EO Schubert, Robert Stoehr, Arndt Hartmann, Sabrina Schöne, Mathias Löbelenz and Gregor Mikuz

    Malignant undifferentiated sex cord-stromal testis tumor with brain metastasis: Case report
    Urologic Oncology: Seminars and Original Investigations, Volume 26, Issue 1, January–February 2008, Pages 53-55
    Priya G.Kumaravelu, M.D., Sandra Vella M.D., J. Edson Pontes, M.D., Elisabeth I.Heath M.D.

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  • Mike
    I am glad that you were able to meet with Dr. Einhorn, he is awesome isn't he? I know it is a tough choice but given that the treatments for stromal tumors is not that affective and being unclassified and high mitotic rate, there is really no data that I have seen, would lend me more toward the RPLND too. The review that I had seen from the National Cancer Database would lead me more towards using an abundance of caution and doing the RPLND but it is very much a personal decision with no perfect answer except for what is right for you.


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  • Newme2017
    Glad you were able to speak with Dr Einhorn and have a good look at the available literature. You are in my thoughts, looking forward to seeing all clear reports!


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  • sayhey
    Just posting an update to my case.

    Had my first appointment with my GU oncologist today, but before that I was able extremely lucky to be able to consult with Dr. Einhorn and even meet and discuss my case with him in person. I also was able to read most if not all of the available literature out there in the journals about testicular SCSTs.

    While saying that going on surveillance would not be incorrect per se in my case, Dr. Einhorn strongly recommended that I get an RPLND as surgery would be the only likely way to cure the disease if it were to spread if it had not already.

    I forwarded his recommendations to my GU oncologist through my hospital's new patient coordinator, and my first visit today was more of a discussion than a typical visit. She concurred with Dr. Einhorn's recommendations, and said while it was ultimately my decision to decide between surveillance or the surgery she seemed to recommend the latter. I will be able to go on a reduced surveillance schedule post-op, since with the high right of mitotic activity and undifferentiated/unclassified diagnosis she would have to treat it as potentially aggressive and malignant.

    The RPLND will also be useful as a staging tool, even if finger's crossed all of the nodes removed come back with no evidence of disease.

    I'll keep updating, and if anybody stumbles across this thread with a similar diagnosis I'd be happy to answer any questions.

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  • singa76
    I'd like to help you out with the papers. Please feel free to send me the titles of what you are looking for.

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