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Rhabdomyosarcoma accounts for 15% of all pediatric solid tumors. Of these, 10% arise from paratesticular tissue. Review literatur: (Ahmet et al, 2010).
Pathology of Paratesticular Rhabdomyosarcoma
Paratesticular rhabdomyosarcoma arises from the distal spermatic cord and invades the testicle and surrounding tissues. The most common histologic type is the embryonic rhabdomyosarcoma, with spindle cell or botryoid histology there is a good prognosis.
Signs and Symptoms of Paratesticular Rhabdomyosarcoma
Unilateral painless scrotal mass. Palpation: paratesticular mass which is often distinct from the testis.
Diagnostic Work-Up of Paratesticular Rhabdomyosarcoma
- Scrotal ultrasound
- MRI of the abdomen and chest
- Diagnostic nerve-sparing retroperitoneal lymphadenectomy in children over 10 years (after confirmed histology of rhabdomyosarcoma)
Treatment of Paratesticular Rhabdomyosarcoma
Therapy should be carried out within clinical trials to improve treatment of this rare disease. After diagnosis, the patients are stratified to an appropriate study protocol in relation the the tumor stage and risk group. The following factors are considered: tumor location, tumor size, histology, grading, lymph node involvement, metastases, and results of surgical treatment. 60–80% of paratesticular rhabdomyosarcomas are stage I with good prognosis. Study protocolls are available at e.g. Children's Oncology Group (COG), which is the successor of the Intergroup Rhabdomyosarcoma Study Group (IRSG).
Surgery for Paratesticular Rhabdomyosarcoma
Inguinal exposure of the spermatic cord and testis. Radical orchiectomy is performed; hemiskrotectomy is necessary depending on the tumor stage or previous scrotal surgery.
Adjuvant treatment of Paratesticular Rhabdomyosarcoma
Current treatment protocol of the COG for children without visible metastases: Children under 10 years of age receive adjuvant chemotherapy without retroperitoneal lymph node dissection. In children over 10 years of age, a retroperitoneal lymph node dissection before adjuvant chemotherapy is recommended. Adjuvant radiotherapy is necessary for histologically confirmed lymph node metastases. Multimodal treatment concepts with higher toxicity are necessary in cases with visible metastases.
Prognosis of Paratesticular Rhabdomyosarcoma
Without adjuvant therapy, 50% relapse within 2 years after orchiectomy. With multimodal treatment, survival rates of 90% are possible.
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- Ahmed, H. U.; Arya, M.; Muneer, A.; Mushtaq, I. & Sebire, N. J.
- Testicular and paratesticular tumours in the prepubertal
Lancet Oncol, 2010, 11, 476-483.
Deutsche Version: Paratestikuläres Rhabdomyosarkom