A 14-year-old male patient with no personal history of interest came to the emergency room for an increase in size in the right hemiscrotum of one month of evolution; he also complained of pain in the right lumbar region.
No history of cryptorchidism or traumatisms.
The examination revealed a tumor in the right hemiscrotum of approximately 10 cm in size, which included the testicle, with negative transience adenopathies.
Urgent ultrasound is requested, which describes a right testicular tumor measuring 9 cm in size, with heterogeneous content and vascular flow, displacing the healthy testicular parenchyma.
Testicular markers (a-fetoprotein and b-HCG) are normal, and LDH is within normal limits.
Right radical orchiectomy was performed via inguinal approach.
Pathological examination revealed focal erythema with poorly differentiated embryonal episarcoma (primitive), with extensive areas of cellular spindle differentiation and tumoral necrosis, with paratesticular origin and maximum diameter of 12 cm.
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In staged non-pelvic CT scans, millimetric nodules are detected in both lower lobes adjacent to the greater fissure, adenopathy and left iliac chains with a significant sub-centimetric size.
Bone scintigraphy is negative.
Treatment with VAC (Vincristine, Dactinomycin and Cyclophoamide) was started until three cycles were completed.
Once the cycles of chemotherapy were completed, a control CT scan was performed prior to radiotherapy treatment, which showed progression in the size of the adenoids (complete iliocautery progression of the right interatrial 1.5 cm).
Paracaval and interaortocaval lymphadenectomy with extension to iliacs was performed; the pathological anatomy was reported as metastatic embryonal retroperitoneal gangliosarcoma.
The patient is classified as stage 11 b of the IRSG classification C6 (4) or stage II of the TNM (T2N1M0).
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After the surgery, new cycles of chemotherapy are started (Ifamide, Vincristine, Actinomycin D and Doxorubicin), followed by concomitant chemotherapy and radiotherapy.
At 12 months the patient is free of disease.
Two months later, the patient complained of severe pain in the left costal region. A new CAT scan was performed, showing multiple bone metastases at the level of the vertebrae, small pelvis and ribs.
The patient remains hospitalized with analgesic and chemotherapy treatment.
