A 10-year-old male patient with no relevant past medical history.
She came to the emergency department with painless swelling of the right hemiscrotum for a week.
She had no history of previous trauma to the area or previous history of cryptorchidism.
The examination revealed a palpable mass in the right testicle of about 3-4 cm, with negative transsexuality.
Not known: inguinal or supraclavicular lymphadenopathy.
Ultrasound examination revealed a solid mass, depending on the lower pole of the right test, 4 cm in diameter.
Tumor markers were requested and α-fetoprotein and β-HCG were normal, with LDH rising to 50% of the maximum normal value.
Chest X-ray, computed tomography (CT) scan of the pelvis and bone scintigraphy were normal, with no evidence of distant metastasis or lymphadenopathy.
With the preoperative diagnosis of testicular tumor exploratory inguinotomy was performed appraising test adjacent mass of white appearance with high radical orchiectomy and hypervascularized highly suggestive of practiolic ligation neoplasia.
The macroscopic anatomopathological report reports a white mass of 4 x 2.8 cm of stony consistency, which encompasses the epididymis and rejects the testicle which measures 2 x 1.6 cm.
Microscopic examination reports a malignant tumor affecting the paratesticular area, encompassing the epididymis and consisting of a proliferation of large nuclei, myxoid cells with occasional tendency to hyperchromatism and mitotic activity.
After performing an extensive immunohistochemical study was observed isolated positivity of tumor cells by alpha-actin, desmin, myoglobin and prominent positivity for CD56.
Tumor cells were negative for cytokeratin 7, epithelial membrane antigen, alpha inhibine, C-Kit, PLAP, CD34, CD31, S-100 and synaptophysin.
Definitive pathological diagnosis was paratesticular rabies sarcoma.
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After classifying the case as stage Ia-I.R.S. of the Intergroup Rhabdomyosarcoma Study, treatment was started with adjuvant chemotherapy according to the national VAT protocol, according to the classification as follows:
- Ifosfamide: 3 g/m2 x 2 days. - Vincristine: 1.5 mg/m2 x 1 day (maximum dose: 2 mg). - Actinomycin D: 1.5 mg/m2
ESTABLISHMENT BY STAFF WITHIN THE INTERGROUP.
RHABDOMYOSARCOMA STUDY (I.R.S.)
1.
These puffs are repeated every 3 weeks and for the first 6 weeks you also receive a weekly dose of vincristine.
The patient remains disease free 6 months after treatment.
