363- What are the characteristics of TART associated with CAH due to
21 α-hydroxylase defi ciency?
TART are benign unencapsulated tumors located within rete testis. There is
high variability in the reported prevalence of TART (0–94 %) in CAH, possibly
because of varying degree of disease control among the selected population and
the method used for the detection of tumor. These tumors are usually not pal-
pable as they are small and deep-seated in rete testis, but are palpable when the
size exceeds >2 cm. Ultrasonography (USG) is a good modality as it can detect
adrenal rest tumors of even 2 mm size. TART is visualized as hypoechoic lesion
on USG (stage 2 onwards) and are typically multiple, bilateral, and eccentri-
cally located within the rete testis. As ultrasound is a highly sensitive imaging
tool for the identifi cation of TART, other imaging modalities like MRI do not
provide any additional information.- How to stage TART?
The different stages of TART are summarized in the table given below (Claahsen
Vander Grinten classifi cation).Stage Histology Comments
Stage 1 Adrenal rest cell in rete testis Identifi ed only by histopathology
Stage 2 Hypertrophy and hyperplasia of adrenal
rest cellsCan be identifi ed by USG
Excellent response to glucocorticoids
Stage 3 Further cell growth and compression of rete
testisModerate response to glucocorticoids
May require surgery for fertility
Stage 4 Fibrosis and focal lymphocytic infi ltrates Require surgery for fertility
Stage 5 Parts of the tumor are replaced by adipose
tissueIrreversible testicular damage- What are the consequences of TART in a patient with CAH?
The most important long-term consequence of TART is infertility. This occurs
due to obstructive azoospermia resulting from compression of efferent ductules
from seminiferous tubule by the adrenal rest tumor located in rete testes. Long-
standing disease leads to peritubular fi brosis and irreversible testicular damage,
including impaired Leydig cell function. Intensive therapy with glucocorticoids
usually leads to the regression of TART in stage 2 and 3, whereas surgical inter-
vention may be benefi cial in stage 4 (Fig. 10.15 ).10 Congenital Adrenal Hyperplasia