Testicular Sertoli Cell Tumours and Relative Sub-Types

Abstract

Introduction: Sertoli cell tumours have a rare (0.4–1.5% of all testicular neoplasms) and heterogeneous pathology. The aim of this paper is to analyse the histological classification of Sertoli cell tumours, in order to assess if the three different histotypes – classic type, large cell calcifying Sertoli cell tumour (LCCSCT) and sclerosing Sertoli cell tumour (SSCT) – really present distinctive clinical and prognostic features. Materials and Methods: The current literature was reviewed; Sertoli cell tumour clinical series and single case reports were searched and analysed. Hence, more than 200 classic Sertoli cell tumours, 48 LCCSCTs and only 12 SSCTs were found. The thirteenth SSCT has been found by us in a 34-year-old man. Results: Every single sub-type presents clinical specific characteristics regarding age of onset, bilaterality, focality, abnormal hormone production, correlated systemic symptoms. Ultrasonographic findings, size and – above all – malignant potential. Conclusions: The precise classification of these tumours is not important only histologically: the currently recognised variants really differ in clinical presentation and course. Moreover, LCCSCTs can be further divided in two subgroups with very different clinical behaviour, those in older patients and those associated with well-known syndromes. These clinical and prognostic variables are of great importance when deciding on the therapeutical approach.

Large cell calcifying Sertoli cell tumor: a clinicopathologic study of 1 malignant and 3 benign tumors using histomorphology, immunohistochemistry, ultrastructure, comparative genomic hybridization, and polymerase chain reaction analysis of the PRKAR1A gene

“…Three of the patients have had a benign clinical course with a follow-up ranging from 2 to 6 years, whereas the patient with a malignant tumor died after 4 years despite treatment with multiagent chemotherapy. The morphologic and immunohistochemical features of LCCSCT presented in this study confirm data that have previously been published on these tumors [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27]. Moreover, the patient with a malignant LCCSCT displayed traditional morphologic features of malignancy, that is, significant cellular atypia, necrosis, vascular invasion, and high mitotic activity mirrored in a high proliferative activity (30%) in the immunohistochemical study.…”

“…Malignant behavior of LCCSCT is exceptionally rare [8][9][10][11][12]. Previous reports have presented histologic and immunohistochemical data on LCCSCT [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31], with a few studies having included their ultrastructural features [22][23][24][25][26][27]. Molecular studies dealing with genetic changes in LCCSCT have, to the best of our knowledge, not previously been published.…”

Testicular Tumors in Patients with Exstrophy-Epispadias Complex

“…6 Due to the rare occurrence of stromal tumors, the literature provides limited experience not only on associated anomalies, but also on treatment and followup recommendations. [12][13][14][15][16][17] The mostly benign Leydig cell tumors may occasionally occur in association with genetic disorders such as Klinefelter syndrome. Malignancy is reported in 10% of cases, with metastasis, as true evidence, possibly being present after a long latency period, with a limited median survival of 2 years.…”

Classification of Testicular Tumors

“…All malignant cases exhibited at least two of the above-mentioned features [22]. There are some hints that discrimination between an early and late onset type may define a different risk for metastatic disease (5.5 % compared with 23 %) [23]. In the malignant cases the prognosis is very poor and it is difficult to select the best treatment because of the limited experience with this type of tumor.…”