Large cell calcifying Sertoli cell tumor of the testis.

To the Editor: Large cell calcifying Sertoli cell tumor of the testis (LCCSCT) is a rare sex cord-stromal tumor that is usually benign;1,2 however, recently there have been reported cases with malignant features.3 In a literature review, there were 68 cases of LCCSCT and 10 of those showed malignant features. Patients may have associated endocrine abnormalities, and tumors tend to be bilateral and multifocal.2,3 These tumors usually occur in children and adolescents, and occur rarely in patients over 40 years of age.1–5 They are reported in patients from 2 to 73 years of age (average, 21 years).1,2,6 We report two further cases.

The first case was a 26-year-old male who presented with a painless lump in the left testicle that he felt over the past few days prior to presentation. Scrotal ultrasonography (US) revealed an irregular circular outlined hyperechoic heterogeneous solid lesion located at the lower posterior aspect of the left testicle showing features of calcified fibrosis. It measured 19 mm in maximum diameter. Hormonal assay showed normal follicle stimulating hormone/luteinizing hormone (FSH/LH), testosterone, alpha-fetoprotein and beta human chorionic gonadotropin (β-hCG) were normal in the blood. The patient was treated by lumpectomy with a surgical margin. The second case was a 4½ year-old male who presented with a 2-year history of precocious puberty, excessive growth and right testicular mass. Hormonal assay showed normal FSH/LH and β-hCG. There was a high testosterone level. US reveal a large mass of heterogeneous density. The patient was treated by orchidectomy. There was no history of trauma or inflammation in either patient. Figure 1 shows the gross picture of the first case. Microscopically, both tumors showed similar features. Sections revealed well-circumscribed and composed solid sheets, nests, trabeculae, cords, and small clusters with a rare attempt at tubular formation (Figure 2a, b, c, d). The tumor cells were polygonal, having defined outlines with abundant acidophilic cytoplasm, with occasional clear cytoplasm, a round or oval nucleus with some nuclear pleomorphism. The stroma varied from loose and myxoid to densely collagenous. Multiple areas of calcification with psammoma bodies were seen. There was no evidence of necrosis or mitosis.

Figure 1

Gross picture from the testicular tumor of case #2 showing a well-circumscribed tumor measuring 5 cm in maximum diameter, grayish yellow in appearance with no evidence of hemorrhage or necrosis.

Figure 2A

Section from the neoplasm show solid sheets, nests, trabeculae, and cords of polygonal cells (H&E, X100). .

Figure 2B

Higher power section from the neoplasm (H&E stain, X200)

Figure 2C

Immunohistochemical stain showing a strong positivity for vimentin (Immunohistochemistry, X200).

Figure 2D

Immunohistochemical stain showing a weak positivity for inhibin (Immunohistochemistry, X200).

Our two cases were microscopically misdiagnosed initially as Leydig cell tumor. Historically, the confusion between a Sertoli cell tumor in the large cell calcifying category and a Leydig cell tumor has been particularly problematic. Before the former tumor was recognized it was often misdiagnosed as a Leydig cell tumor. There were strong associations of malignant behavior with size >4 cm, extratesticular growth, gross or microscopic necrosis, high-grade cytological atypia, vascular space invasion, and a mitotic rate greater than three mitoses per 10 high-power fields.7 These reports appear to be the first cases of LCCSCT reported from Saudi Arabia.