Primary Leydig cell tumour of epididymis: a rare case report with review of literature

Abstract

Leydig cell tumour (LCT) is an uncommon tumour that typically occurs in the testis. Primary epididymal LCT is extremely rare. To the best of our knowledge, only two cases have been reported in the world literature. Herein, we report a case of primary epididymal LCT in a 41-year-old Chinese male. The patient presented with right epididymal swelling for 3 months without endocrine manifestations, including gynaecomastia and decreased libido. Scrotal ultrasound demonstrated a mass about 1.5 cm in diameter entirely in the cephalic region of right epididymis. No abnormality was found in his bilateral testes. The patient underwent total mass resection without post-operative therapy. Histological examination revealed that the well-circumscribed tumour was separated by conspicuous hyalinised fibrous stroma; the tumour cells were large and polygonal with round nuclei and abundant eosinophilic cytoplasm. Immunophenotypically, the tumour cells expressed four markers of sex cord differentiation (calretinin, melanA, CD99 and inhibin). There was no recurrence at 2-year follow-up. Our observation once again confirms that LCT could primarily occur in the epididymis, and we suppose that it probably originates from the ectopic Leydig cells. As little is known about the pathogenesis and prognosis for such a rare disease, accumulation of more pathological and clinical data can help to better interpret this tumour.

Gynecomastia and hormones

“…Gynecomastia might also be the first clinical sign of a testicular cancer [28][29][30][31], the most common neoplasm in young adults [32,33]. Germ cell tumors induce gynecomastia via a different pathway (discussed later), whereas non-germ cell tumors directly secrete estrogens or increase aromatization of T to E2 [34]. Testicular pain or enlargement should be evaluated during physical examination for all patients with gynecomastia [34].…”

Towards understanding leydigioma: do G protein-coupled estrogen receptor and peroxisome proliferator–activated receptor regulate lipid metabolism and steroidogenesis in Leydig cell tumors?

“…Gynecomastia is the main clinical manifestation in adults, but it may also be clinically significant in affected children who undergo precocious puberty (Lejeune et al 1998). Some cases of LCTs were linked with increased plasma estradiol concentrations (Huang et al 2013). Moreover, infertility and azoospermia are not an unusual finding in these patients (Bozzini et al 2017).…”

Leydig cell tumor of the testis presenting with gynaecomastia due to hyperprolactinemia after orchiectomy

“…[3] Our index patient presented with both a painless mass and endocrine features. Most of the time, LCTs are unilateral, though it is important to remember that about 3%-10% are bilateral either at presentation or latter during the course of follow up, [15,16] hence highlighting the importance of close follow up and monitoring even after orchiectomy.…”