Testicular lymphoma - secondary involvement

Case contributed by Henry Knipe
Diagnosis certain

Presentation

Swollen right testis for 6 weeks.

Patient Data

Age: 75 years
Gender: Male

Right testis measures 70 mL being heterogeneous with marked hypervascularity. Focal hypoechoic region in the right upper testis. Thickened hypervascular right epididymis. Prominent vessels around the right spermatic cord.

Left testis measures 15 mL. Normal left testis and epididymis.

Small right hydrocele. No left hydrocele. No varicoceles bilaterally.

The patient's symptoms persisted with the development of a hard scrotal mass.

1 month later

ultrasound

Right testis measures 140 mL. Homogeneous echotexture, normal vascularity. Posterior wedge-shaped hypoechoic region with vascularity. Right epididymis is not well seen but the epididymal head appears enlarged and hypervascular.

Left testis measures 16 mL. Homogeneous echotexture, normal vascularity and no focal lesion. Left epididymis has a normal appearance.

Small right varicocele and small right hydrocele. No left varicocele/hydrocele.

No enlarged right inguinal lymph nodes.

Key information was missing on both referrals - a history of mantle cell lymphoma. The patient underwent an orchiectomy.

Histopathology

MACROSCOPIC:

Right testis – The specimen consists of a testis with attached spermatic cord. The spermatic cord measures up to 85 x 25mm and has a tie at its distal end. The testis measures 110 x 70 x 70mm. The testis and spermatic cord weigh 231g. The external surface of the testis is smooth and is favored to be covered with tunica vaginalis. Soft tissue adhesions are present at two points on the external surface measuring 35 x 5 x 4mm and 15 x15 x 2mm. The external surface of the spermatic cord is smooth. The specimen was serially sectioned into nine slices. The testis appears diffusely enlarged with possible tumor and measures up to 80 x 55 x 70mm. Within slices 4 through to 9 is a ill–defined cream hemorrhagic region within the testis measuring 40 x 25 x approximately 38mm. The tunica albuginea appears thickened up to 6mm and demonstrates adhesions to the overlying tunica vaginalis. A cystic area is present up to 80 x 15mm between the tunica albuginea and vaginalis. The tunica vaginalis is thickened at this site up to 5mm in thickness. This space contains hemorrhagic material. The epididymis appears infiltrated by cream tissue and has an effaced architecture. The spermatic cord appears within normal limits.

MICROSCOPIC:

Sections through the testis demonstrate involvement by Mantle cell lymphoma. The lymphoma is comprised of uniform, small to intermediate cells with angulated nuclei which display stippled chromatin. Scattered mitotic figures are present. There is diffuse involvement of the testicle with tumor cells replacing much of the testicular parenchyma. Scattered atrophic tubules are present, there is no germ cell neoplasia in situ. The lymphoma involves the tunica albuginea and tunica vaginalis and is present at the surface of the tunica vaginalis in several locations. There is also extensive involvement of the epididymis and spermatic cord. Lymphoma is present in the shave of the spermatic cord margin.

Immunohistochemistry shows positive staining for PAX 5, CD5, BCL2 and cyclin D1 within the lesional cells. CD3 stains background T cells. CD23 is negative. Ki–67 proliferative index is 90%. Despite the high Ki–67 proliferative index, there is no morphological evidence of pleomorphic or blastoid variants.

DIAGNOSIS:

Right testis: Diffuse involvement of the testis, tunica vaginalis, epididymis and spermatic cord by conventional mantle cell lymphoma exhibiting a high Ki–67 index. Lymphoma is present at the surface of the tunica vaginalis and spermatic cord shave margin.

Case Discussion

This is a case of scrotal lymphoma involving the right testis, epididymis and spermatic cord. The first ultrasound was reported as epididymo-orchitis, which based on the images seems reasonable particularly with the relevant history of known lymphoma not provided. Still, the absence of pain should raise suspicion of a malignant and not infective process.

The marked enlargement of the testis prompted re-presentation where a differential of partially treated acute epididymo-orchitis, chronic epididymo-orchitis, or an infiltrative process such as lymphoma was offered. Again, the critical history of known lymphoma was not provided.

On ultrasound, scrotal lymphoma and epididymo-orchitis can have very similar appearances.

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