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Differential Diagnosis of Tumors in the Adrenogenital Syndrome

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Clues in the Diagnosis of Non-tumoral Testicular Pathology

Abstract

The differential diagnosis of testicular tumors of the adrenogenital syndrome (TTAGS) with Leydig cell tumors (LCT) is not always easy. In childhood, both tumors show a pattern of hyperandrogenism, and the symptoms can be similar. The macroscopic clues in favor of TTAGS are the bilateral nature of the tumors and its location in the mediastinum testis. Among the histological data, the good delineation of the tumor nodules is outstanding: sheets of large polygonal cells with an eosinophilic cytoplasm intersected by broad fibrous bands, abundant lipofuscins, the absence of mitosis, and the presence of fat and lymphoid tissue. The peritumoral parenchyma does not show spermatogenesis. Adults often consult by infertility secondary to obstruction of the tubuli recti that are compressed by the tumor nodules. TTAGS and Leydig cell tumors share the same immunohistochemical phenotype (calretinin, inhibin, synaptophysin, and CD56) although there are differences in the intensity of positivity and the distribution of the expression – focal versus diffuse in some cases. The absence of androgen receptors and expression of INSL3 constitute the most relevant histological differential fact.

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Authors and Affiliations

  1. Universidad Autónoma de Madrid (UAM), Department of Anatomy, Histology and Neuroscience, School of Medicine, Madrid, Spain

    Manuel Nistal

  2. School of Medicine, Universidad Autónoma de Madrid (UAM), University Hospital La Paz, Department of Pathology, Madrid, Spain

    Pilar González-Peramato

  3. University Hospital Clinico San Carlos, Department of Urology, Madrid, Spain

    Álvaro Serrano

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  1. Manuel Nistal
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  2. Pilar González-Peramato
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  3. Álvaro Serrano
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Correspondence to Manuel Nistal .

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Nistal, M., González-Peramato, P., Serrano, Á. (2017). Differential Diagnosis of Tumors in the Adrenogenital Syndrome. In: Clues in the Diagnosis of Non-tumoral Testicular Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-49364-0_7

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  • DOI: https://doi.org/10.1007/978-3-319-49364-0_7

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