Perspectives in Pediatric Pathology, Chapter 11. Testicular Pathology of Hamartomatous Origin

Abstract

The term hamartoma was coined by Albrecht in 1904 from the Greek mart-ánv, to go wrong [1]. Hamartoma is currently used to name nonneoplastic lesions composed of disorganized tissue indigenous to the site where it is found. In the testis, this refers to abnormal/excessive development of structures that usually form part of the gonadal parenchyma, epididymis, or spermatic cord. We include the following as hamartomatous lesions of the testis and sperm excretory ducts: cystic dysplasia of the rete testis, rete testis hamartomas, fetal gonadoblastoid testicular dysplasia (FGTD), Sertoli cell nodules, tubular hamartomas, congenital testicular and epididymal lymphangiectasis, and smooth muscle hyperplasia of paratesticular structures. These hamartomas are always benign, but their importance resides in their association with specific disorders, or as markers of complex syndromes.

Perspectives in Pediatric Pathology, Chapter 25. Testicular and Paratesticular Tumors in the Pediatric Age Group

“…The differential diagnosis in young children should include the lesion known as fetal gonadoblastoid testicular dysplasia (described in Nistal and colleagues [148]), and, in postpubertal patients, Sertoli cell nodules colonized by intratubular germ cell neoplasia. Sertoli cell nodules are very frequent in cryptorchid testes of postpubertal patients without DSDs.…”

Androgen Insensitivity Syndrome with Bilateral Gonadal Sertoli Cell Lesions, Sertoli–Leydig Cell Tumor, and Paratesticular Leiomyoma: A Case Report and First Systematic Literature Review

“…Tubular hamartomas (SCHs, Sertoli-Leydig hamartomas) are frequently bilateral and multiple, white, well-delimited, encapsulated testicular nodules composed of small and solid seminiferous tubules/cords with immature Sertoli cells and numerous interspersed Leydig cells; the tubular wall shows hyalinization, lacking elastic fibers and spermatogonia are absent or isolated in some lesions [5].…”

“…In particular, various lesions containing Sertoli and/or Leydig cells have been described in patients with AIS, considered as hyperplastic, hamartomatous, or neoplastic, including Sertoli cell hyperplasia (SCHYP), Sertoli cell nodules (SCNs), Sertoli cell hamartomas (SCHs), Sertoli cell adenomas (SCAs), Sertoli cell tumors not otherwise specified (SCTs, NOS) or Sertoli-Leydig cell tumors (SLCTs); single or multiple, uni or bilateral, these lesions may appear as grossly detectable nodules or incidental histological findings. However, their diagnostic criteria may be subtle, challenging, and sometimes questionable in some cases, and their frequently small size and bland histology may result in a favorable prognosis; unfortunately, the rarity of these lesions and the lack of a systematic literature review on this topic have represented biases for further considerations [5][6][7].…”

Perspectives in Pediatric Pathology, Chapter 14. Natural History of Undescended Testes

“…Other abnormalities in cryptorchid adult testes include Sertoli cells nodules (Fig. 14.26) (see Nistal and colleagues [66]), seminiferous tubules with granular changes in Sertoli cells (Fig. 14.27), and Leydig cell hyperplasia (Fig.…”