Background:
The increasing incidence of JNA has been noted lately. Reservations persist regarding treating JNA at peripheral centre owing to the potentially fatal intraoperative haemorrhage. Molecular characterization in Indian population is lacking.
Aim:
To appreciate (1)changing epidemiological trends n last 6-decades, (2) clinical presentation /treatment outcomes, (3) molecular expression (44 indian patients).
Methods:
Archival databases of 701 cases admitted at our facility in last 6 decades since 1958 were analysed and classical charts were reviewed for age, clinical presentation, surgery and outcome. A 2-year prospective series of 44 patients underwent molecular profiling with 3 markers till date viz, androgen(AR), estrogen(ER), beta-catenin(BC).
Results:
(1) Incidence rate was relatively constant in first 3 decades i.e. 50s to 70s (10 cases per year) and increased in next 2 decades i.e. 80s & 90s (15-20 per year). Subsequently thereafter marked increased incidence was noted specially in the current decade (33-37 cases per year). (2) 15% cases in last 2 years presented after adolescence (age 21-24 years). 75% presented in stage III with snoring (35%), voice change (40%), palatal buldge (45%), and cheek swelling (20%). Transpalatal approach (classical or extended) was adapted in 90% cases with piecemeal extraction in 80%. Recurrence was seen in 5% and staging showed significant effect on intraoperative hemorrhage/ completeness of resection.. (3) Mean fold expression of BC & AR was 21.2% and 49% higher than controls respectively. BC-upregulation was seen more often with recurrence while ER-alpha-expression was quite variable.
Conclusions:
A multitude of environmental changes effecting hormonal mileu interior such as use of pesticides and oxytocin-induced-cattle-lactation may play a role or it may be a specific manifestation of emerging precautious puberty in males. A comparable cure rates with minimal morbidity may be obtained in the absence of hypotensive anaesthesia,/ preoperative embolization . Further molecular characterization may facilitate better understanding for therapeutic target(s).