Primary classical seminoma of testis with foci of signet-ring-cell morphology: A case report and review of the literature including ESMO guidelines on management of seminoma of testis

Abstract

Background: Signet-ring-cell carcinoma is a rare variant of mucinous adenocarcinoma. Only about 3 cases of primary testicular cancer with signet-ring-cell morphology have been reported so far in the literature. There is lack of knowledge regarding the biological behaviour of this type of tumour affecting the testis. Seminoma of the testis is quite common and its biological behaviour is well known. When a classical seminoma of the testis is found to be contemporaneously associated with signet-ring-cell morphology, in view of the absence of any detailed literature on this rare clinical entity, the biological behaviour of such a tumour cannot be predicted.

Objectives: To report a case of Classical Seminoma of the testis interspersed with seminoma cells with signet-ring morphology. To review the literature on signet-ring-cell carcinomas including carcinomas of testis with signet- ring- cell morphology. To review the ESMO guidelines on management of seminoma of testis

Results: A 32-year-old man came for urology consultation 11 months after he had noticed a lump in his left testis. He had a family history of testicular cancer. A hard lump clinically suspicious of a tumour was palpable in the lower pole of his left testis. The right testis felt normal on palpation. His serum Beta HCG level was raised. He had ultra-sound scan of testis which showed bilateral microlithiasis with a tumour in the left testis but no tumour in the right testis. He underwent left radical orchidectomy and histology of the testicular tumour revealed a classical testicular seminoma with foci of signet-ring -cell morphology based upon haematoxylin and eosin staining as well as positive immunostaining for CD117 and OCT 3 / 4. Literature review revealed paucity of literature on testicular tumours with signet-ring-cell morphology.

Conclusions: There is paucity of literature on primary testicular tumours with signet-ring-cell morphology. In order to establish the biological behaviour of such tumours we would encourage pathologists and urologists to report cases of testicular tumours associated with signet-ring-cell morphology together with the long term biological behaviour of such tumours. They should also report on the percentage of testicular tumours that have signet–ring-cell morphology and their distribution whether patchy or diffuse. Ultrasound-scan of testis was required to establish the diagnosis of testicular cancer in the patient. Ultrasound-scan of testis is useful in the investigation of a testicular lump. Ultrasound and CT scans are also useful for the follow-up of patients with testicular cancer and microlithiasis.