TABLE 3.
Summary for surgical management of paratesticular rhabdomyosarcoma: INSTRuCT consensus opinion document
| Item | Recommendation | Quality | Strength |
|---|---|---|---|
| Primary Inguinal Orchidectomy | Remove paratesticular RMS by radical inguinal orchidectomy | Moderate | Strong |
| Remove the tumor as a single specimen incorporating the entire cord up to the internal ring and without breaching the tunica vaginalis | High | Strong | |
| The cord should be clamped at the internal ring before mobilization of the tumor | Low | Weak | |
| The cord is ligated using a non-absorbable suture | Low | Weak | |
| When scrotal skin is fixed or grossly invaded by tumor, it should be resected en-bloc with the specimen | High | Strong | |
| Testicle preserving approaches should be avoided | High | Strong | |
|
Primary Re-Excision
|
Primary re-excision (PRE) is indicated when the initial procedure did not include en-bloc complete gross resection of the tumor, testis and spermatic cord up to the internal ring | Moderate | Strong |
| Patients with microscopic residual disease after initial resection (IRS Group II) may undergo PRE when there is microscopic disease at the cord margin | Moderate | Strong | |
|
Management of Large Tumors
|
For large tumors that are difficult to excise through a standard inguinal incision, it is better to extend the inguinal incision down to the scrotum or use a combined inguinal and scrotal approach to facilitate a complete gross total tumor resection | Moderate | Strong |
| If there is proximal extension of the tumor through the inguinal canal, or extension into the urethra and base of the penis, the tumor is primarily irresectable and should be biopsied through an inguinal approach | Low | Strong | |
| Trans-scrotal excision, scrotal violation, hemi-scrotectomy, scrotal RT | A trans-scrotal approach to tumor resection should be avoided since it makes complete resection more difficult | Moderate | Strong |
| A trans-scrotal biopsy should be avoided since it may result in a potential increased risk of tumor spill | Low | Weak | |
| Hemi-scrotectomy is not indicated if patients have a scrotal violation or microscopic residual disease (Clinical group II) present in the scrotum after gross tumor resection | Low | Weak | |
| Scrotal skin should be removed en-bloc with the tumor excision if the tumor invades or extends into the scrotal skin | High | Strong | |
| If scrotal RT is required due to Clinical group III disease, then temporary transposition of the remaining normal testis should be done to preserve function | Moderate | Strong | |
|
Retroperitoneal Lymph Node (RPLN) Assessment and Management
|
All patients ≥ 10 years old should undergo ipsilateral infrarenal surgical lymph node evaluation, regardless of imaging results, to identify patients with pathologic disease | Moderate | Strong |
| Patients <10 years old with no nodal enlargement should not undergo surgical RPLN evaluation given the low rate of pathologic nodal involvement | High | Strong | |
| The rare patients who have alveolar histopathology, irrespective of age or tumor size, should undergo surgical nodal staging procedure | Low | Weak | |
| Nodal sampling of 7-12 nodes from within the template area appears to be sufficient to identify disease presence | Moderate | Strong | |
| Sentinel nodal evaluation, may be used to help identify the positive node. The patient should still have additional nodes sampled to achieve at least 7 RPLN | Low | Weak |