Metachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)

Table 1

Overlapping clinical presentations, morphologies, immunophenotypes, and associated endocrinopathies for LCTs and TARTs.

	Laterality	Gross appearance	Architecture	Cytology	Expected % cases with IHC reactivity	Expected serum ACTH	Expected serum testosterone
LCT	Unilateral	Intratesticular (parenchyma), solitary	Sheet-like or lobulated with fine fibrous bands	Leydig ± z. fascic. Reinke crystals; in 30–40%	I 96%; M 86%; C 93%; S < 10%	Normal	Increased
TART	Unilateral or Bilateral	Intratesticular (hilum), multifocal	Nodules with coarse fibrous bands	Leydig-like; ± z. fascic.; Reinke crystals; absent	I (+); M (+); C (+); S > 88%	Increased	Increased/normal/low

z. fascic. = similar to adrenal zona fasciculata.
IHC = immunohistochemistry; I = inhibin; M = Mart-1; C = calretinin; S = synaptophysin.