Testicular “Tumor” of Adrenogenital Syndrome

Testicular “Tumor” of Adrenogenital Syndrome

Steven S. Shen, MD, PhD

Jae Y. Ro, MD, PhD

TTAGS shows a well-circumscribed nodule composed of cells with abundant eosinophilic cytoplasm similar to that of a Leydig cell tumor. (Courtesy R. H. Young, MD.)

This TTAGS is composed of large polygonal cells with abundant eosinophilic cytoplasm. Random cells show nuclear pleomorphism image. Multifocality and bilaterality are common. (Courtesy R. H. Young, MD.)



  • Testicular “tumor” of adrenogenital syndrome (TTAGS)


  • Congenital adrenal hyperplasia


  • Benign bilateral testicular lesions in patients with congenital adrenal hyperplasia leading to growth of adrenal-like cells in testis that resemble Leydig cell tumor

  • Bilaterality, multifocality, and response to medical treatment argue against this being a neoplastic lesion


Developmental Anomaly

  • 21-hydroxylase deficiency (most common)

  • Other associated conditions

    • 11-hydroxylase deficiency

    • Cushing disease

    • Addison disease

    • Idiopathic enzyme defect

  • Cellular origin uncertain; hilar pluripotential cells, adrenal cortical rest cells, Leydig cells are possible candidates



  • Incidence

    • Rare; a few dozen cases reported

  • Age

    • Children to early adult (average: 22.5 years)


  • Bilateral orchialgia (92%)

  • Testicular masses (2/3 palpable)

  • Symptoms related to steroid hormone deficiency

    • Salt-losing form of adrenal disorder in 2/3 and non-salt-losing form in 1/3

  • Isosexual precocious puberty

Laboratory Tests

  • Steroid hormone evaluation (increased ACTH)

  • Tumor markers (AFP, hCG, LDH) to exclude possible germ cell tumor


  • Drugs

    • Exogenous high-dose corticosteroids are mainstay of medical treatment (pain control and regression of tumor)

  • Surgical approaches

    • Tumor enucleation or partial orchiectomy for persistently painful masses and steroid unresponsive lesions in setting of bilaterality

    • Radical orchiectomy is generally not indicated


  • Benign lesion, symptoms relieved by steroid therapy or surgery

  • Very rarely associated with seminoma, and 1 reported case of malignant transformation


Ultrasonographic Findings

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Testicular “Tumor” of Adrenogenital Syndrome

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