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Unclassified sex cord stromal tumor

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  • 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 (https://www.otstregistry.org) and will be sending them my file as well.

  • #2
    Afraid I know absolutely nothing about sex cord stromal tumors other than what you've said. I can't recall another case here, so sorry if no one is jumping in with tons of information. However, there s no doubt in my mind that whatever information you provide may well help the next person with a similar Dx, so please, keep us posted as you find out more.

    Dave
    Jan, 1975: Right I/O, followed by RPLND
    Dec, 2009: Left I/O, followed by 3xBEP

    Comment


    • #3
      Hi Sayhey,

      Like you,I had a Stromal Tumor, (Sertoli) and am all too familiar with the 10% to 20% malignancy chance. I have been searching for papers myself for the last 18 months or so, so if I come across any,I would be more than happy to send them your way. Also I am hoping to follow Dave's suggestion, hopefully I will post my pathology info in the next day or two.

      Best,
      ​Mark
      3-21-2017 Mass found on left testicle at work-medical exam
      3-28-2017 GP confirms mass, refers to Urologist the next day
      3-29-2017 Urologist visit, US, I/O scheduled
      4-05-2017 I/O
      4-11-2017 Pathology returns Sertoli Cell Tumor, NOS
      4-12-2017 CT All Clear
      6-06-2017 Bone Scan All Clear
      9-25-2018 CT All Clear

      Comment


      • #4
        I've got a paper copy of my pathology report (it's five pages long) and I'll post it once I get it in digital form in my online patient portal.

        Elevated mitotic activity is my only risk factor (the cut off is greater than 3 or 5 depending on the paper I've read, and my tumor had up to 10 mitoses per 10 high power fields).

        Comment


        • #5
          Below is my pathology report. I've removed the clinical history since I detailed it above, and edited out the name of what was likely the supervising doctor for their privacy.

          FINAL PATHOLOGIC DIAGNOSIS


          Left testicle and spermatic cord, radical orchiectomy:
          - Testis: Unclassified sex cord stromal tumor with spindle cells, 1.7
          cm; see comment.
          - Spermatic cord: No significant pathologic abnormality.


          COMMENT:
          Sections from the testis show a well circumscribed mass that consists
          predominantly of a cellular spindle cell proliferation growing in a
          somewhat fascicular pattern. The cells contain moderate to focally
          abundant eosinophilic cytoplasm and ovoid to spindle nuclei that are
          hyperchromatic and contain prominent eosinophilic nucleoli. Brisk
          mitotic activity is present, up to 10 mitoses per 10 high power fields.
          Subtle intermixed tubular structures and Sertoliform areas are
          identified. There are scattered variably prominent foci of intermixed
          round to ovoid cells with abundant clear cytoplasm and round nuclei with
          inclusions. Also noted at the periphery are occasional seminiferous
          tubules, some with a mix of the clear round cells and the dark spindle
          cells. The surrounding testicular parenchyma shows evidence of atrophy
          in the form of tubular hyalinization and Leydig cell hyperplasia. No
          definitive germ cell neoplasia in situ is identified.
          Based on the morphology, the differential diagnosis could include a
          mixed germ cell and sex cord stromal tumor, an unusual pure germ cell
          tumor and a pure sex cord stromal tumor, amongst others.
          Immunohistochemical studies were necessary to evaluate this case and
          establish the correct diagnosis. The following immunohistochemical
          stains were performed and evaluated on A2:

          - OCT4: Negative.
          - SALL4: Negative.
          - Keratin: Patchy positive.
          - Chromogranin: Negative.
          - Synaptophysin: Negative.
          - Inhibin: Patchy positive.
          - Calretinin: Focal positive in spindle cells, positive in round cells
          with clear cytoplasm.
          - SF-1: Patchy positive.
          - FOXL2: Positive.
          - SMA: Positive.
          - Desmin: Patchy positive.
          - S-100: Patchy positive.
          - Melan-A: Negative.

          In addition a reticulin stain shows staining around nests of cells (in a
          background of staining around individual cells).

          Overall the morphology and immunoprofile is most supportive of a sex
          cord stromal tumor. We considered various possibilities including a
          Sertoli cell tumor, Sertoli-Leydig cell tumor, granulosa cell tumor and
          some unusual tumors such as myoid gonadal stromal tumor and spindle
          Leydig cell tumor but ultimately feel that this unusual tumor is best
          categorized as a sex cord stromal tumor, unclassified, with spindle
          cells.
          The prognosis of these tumors is difficult to predict and while the
          small size and lack of necrosis or lymphovascular invasion are favorable
          features, the elevated mitotic activity may portend a risk for
          recurrence. Therefore clinical follow up is recommended.
          Dr. [name removed] reviewed this case and agrees with the diagnosis.

          Laterality: Left.
          Focality: Unifocal.
          Tumor size: 1.7 x 1.4 cm.
          Tumor histologic type: Sex cord stromal tumor, unclassified, with
          spindle cells.
          Tumor extension: Tumor limited to testis.
          Spermatic cord margin: Uninvolved by tumor.
          Other margin(s): Negative.
          Lymphovascular Invasion: Not identified.
          Regional lymph nodes: No lymph nodes submitted.
          Additional pathologic findings: Atrophy.

          Pathologic stage classification (pTNM, AJCC 8th Edition): pT1NX.
          Pre-orchiectomy serum tumor markers: AFP, HCG and LDH within normal
          limits.
          Post-orchiectomy serum tumor markers: Not known.
          Serum tumor marker stage (S): Not known.
          AJCC prognostic stage group: Not known.

          Specimen(s) Received

          A:1) Left Testicle - perm.

          Gross Description

          The case is received fresh in one part and is labeled with the patient's
          name, medical record number, and additionally labeled "left testicle"
          and consists of one testicle and attached spermatic cord (43.2 g,
          spermatic cord: 9 x 2 x 1 cm, testicle: 4.1 x 2.9 x 2.7 cm, epididymis
          6.2 x 1.8 x 0.4 cm, and appendage 1.2 x 1 x 0.7 cm). The tunica
          vaginalis is intact. There is a pale yellow, firm mass in the parenchyma
          of the testicle (1.7 x 1.4 x 1.1 cm) that abuts the tunica albuginea but
          does not invade, and is 0.1 cm from the tunical vaginalis. The remainder
          of the parenchyma is pink colored and soft. The epididymis is soft and
          grey, the tunica albuginea is pale grey, smooth, with no masses or
          adhesions. The tunica vaginalis is smooth and uninterrupted.

          Comment


          • #6
            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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