xRead - Nasal Obstruction (September 2024) Full Articles

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KUANetal.

Sinonasal sarcoma Management options for sinonasal rhabdomyosarcoma (RMS) have largely been dictated by research in the pedi atric population, where chemotherapy and RT remain the first-line treatment. Given the rarity of adult RMS cases, much of the evidence has been abstracted from the pediatric literature. The quality of evidence surrounding surgical treatment is low and appears to apply to sal vage cases, and thus no recommendation can be made. Most other subtypes of sinonasal sarcoma are rare and are covered in the form of literature reviews. Role of surgery in pediatric rhabdomyosarcoma

Harm

Damage to vital structures or important organs (eye, carotid artery, brain, oral cavity), postoperative complications, and cranial nerve deficits. No studies directly assessed cost. However, improved local control implies decreased future cost in terms of hospitalization, imaging, systemic therapy, etc. Endoscopic resection is associated with lower complication rates and improved QOL over the long term in select cases and is comparable to open approaches in terms of survival outcomes. Achieving negative margins will improve local control as well as improve OS. There is a high distant recurrence rate and risk of skip lesions in perineural invasion. Given the high overall local control rate, a strategy of GTR and postoperative RT while preserving function provides QOL without reduction of survival. Preponderance of benefits over harms.

Cost

Benefits–harm assessment

Value

judgments

Aggregate grade of evidence

D (Level 4: three studies)

Benefit

Possibility of additional survival benefit with upfront or salvage surgery. Risk of surgical complications including anesthetic risks, blood loss, infection, CSF leak, and orbital injury. Potential for significant morbidity and disfigurement for locally advanced tumors. Additional cost of surgery and perioperative care.

Harm

Policy level Recommendation. Intervention Surgical resection should be attempted as the

first line of treatment when feasible, with the goal to achieve GTR (with negative surgical margins whenever possible) while preserving vital structures.

Cost

Benefits–harm assessment

Balance of benefits and harms.

Value

Minimally invasive endoscopic approaches are limited by pediatric sinonasal anatomy. Studies do not differentiate between upfront and salvage surgery.

judgments

Role of adjuvant radiation therapy in sinonasal ACC

Policy level No recommendation. Intervention There is limited evidence to support routine upfront surgical intervention. May consider in salvage setting.

Aggregate grade of evidence

C (Level 2: one study; Level 3: two studies; Level 4:10 studies) Postoperative RT improves local control rates and survival outcomes. No studies directly assessed cost. However, improved local control implies decreased future cost in terms of hospitalization, imaging, systemic therapy, and so forth. Preponderance of benefits over harms. In patients with adverse features and positive surgical margins, adjuvant RT effect on local control is crucial. While RT as the primary treatment was not extensively studied and was usually reserved for unresectable cases, adjuvant RT shows clear survival benefit and a better local control trend in all patients, especially with positive surgical margins. Acute and late toxicities.

Benefit

Harm

Role of radiation therapy in pediatric rhabdomyosarcoma

Cost

Aggregate grade of evidence

B (Level 2: one study, Level 3: three studies)

Benefits–harm assessment

Benefit

Improved survival with use of RT in primary treatment modality. Acute and long-term radiation complications. Risk of secondary malignancy for pediatric patients.

Value

judgments

Harm

Cost

Additional cost of RT.

Benefits–harm assessment

Preponderance of benefits over harms.

(Continued)

Policy level Recommendation. Intervention Adjuvant postoperative RT should be

recommended in all cases, with special importance in cases of advanced-stage disease, positive margins, and PNI.