Large Cell Calcifying Sertoli Cell Tumor of the Testis

Abstract

We report six malignant and six benign large cell calcifying Sertoli cell tumors of the testis and compare the features of malignant and benign cases based on these cases and those in the literature. All the tumors in this report consisted of sheets, nests, solid tubules, and cords of eosinophilic cells, with focal calcifications, as well as a substantial neutrophilic infiltrate in 11 of them. Analysis of our cases and those in the literature showed that the malignant tumors were unilateral and solitary and occurred at a mean age of 39 years (range 28-51 years), whereas the benign neoplasms were bilateral and multifocal in 28% of cases and occurred at a mean age of 17 years (range 2-38 years). Only one malignant tumor occurred in a patient with evidence of a genetic syndrome (Carney syndrome), whereas 36% of benign tumors had various genetic syndromes or endocrine abnormalities. Most of the tumors in the latter cases were bilateral and multifocal. There were strong associations of malignant behavior with size >4 cm, extratesticular growth, gross or microscopic necrosis, high-grade cytologic atypia, vascular space invasion, and mitotic rate greater than three mitoses per 10 high-power fields. All malignant cases exhibited at least two of these features, whereas all benign cases lacked any of them. The presence of any one of these features in a solitary large cell calcifying Sertoli cell tumor, especially in a patient >25 years of age, should be viewed as suspicious for malignant behavior, whereas the presence of two or more of these features indicates a strong probability of a malignant course. "Low" percentages (< or =35%) of tumor cells staining for proliferating cell nuclear antigen (PCNA) also may correlate with benign behavior, but some benign tumors have high PCNA values. Ki-67 values (MIB-1 antibody) did not correlate with biologic behavior, nor did immunostains for p53 protein.

Testicular&ndash;Sparing Surgery for the Prepubertal Testicular Tumor

“…Most (80%) of these tumors are considered to be benign but 10 cases of malignant LCCSCT, either clinically or histologically have been reported. Benign LCCSCT is frequently bilateral and multifocal, especially occuring in association with complex dysplastic syndromes such as Carney’s complex, tuberous sclerosis and Peutz–Jeghers syndrome [3]. In this setting, organ–sparing surgery (enucleation or partial orchiectomy) or delayed operation (after spermatogenesis and sperm banking) should be advocated if feasible [16, 17].…”

“…They have been followed up annually with regard to the changing sexual hormonal environment and tumor recurrence with excellent outcome. Traditionally, the majority of LCCSCT cases have been treated by orchiectomy because of their malignant potential and multiplicity [3]. Subsequently, by analyzing pediatric testicular germ cell tumor in Japan and presenting these 2 cases in detail, we would like to discuss the possibility of the organ–sparing surgery for the majority of testicular tumors in prepubertal schoolboys.…”

Gynecomastia: An Uncommon but Important Clinical Manifestation for Testicular Tumors

“…Approximately 20% of this variant are bilateral, which are almost exclusively associated with Carney's syndrome [21] (a multiple endocrine neoplasia syndrome manifested as spotty skin pigmentation, myxomas of heart or other sites, endocrine neoplasms, and psammomatous melanotic schwannomas [28]). Approximately 10% of LCCSCT are associated with gynecomastia [29].…”

“…Foci of calcification are characteristic and are considered one of diagnostic criteria [30] (Figure 1(B)). LCCSCT is usually benign [29,31]. Malignant tumors are unilateral, and solitary; they occur in an older age group (mean age, 39 years), whereas the benign ones are bilateral and multifocal in more than a quarter of cases and occur in younger individuals (average age 17 years) [29].…”

“…LCCSCT is usually benign [29,31]. Malignant tumors are unilateral, and solitary; they occur in an older age group (mean age, 39 years), whereas the benign ones are bilateral and multifocal in more than a quarter of cases and occur in younger individuals (average age 17 years) [29]. The morphological features associated with aggressive behaviors include large size (>4 cm), necrosis, vascular invasion, high mitotic rate (>3/HPF) and extratesticular extension [29].…”