In spite of the name of this testicular tumor, it has a very different clinical course and prognosis than the classic seminoma. The name was proposed based on the resemblance of the chromatin in intermediate and small cells to that of the primary spermatocytes and spermatogonia respectively. The exact spermatogenic cell type, from which this tumor develops, remains unknown. It comprises about 5% of testicular seminomas arising in men with the . It has not been reported in prepubertal children or adolescents. Unlike most testicular tumors, there is no ovarian homologue and does not arise in cryptorchid testis, extra-testicular sites, or in association with other germ cell tumors. Most cases are asymptomatic and may grow to a very large size. Less than 10% of cases are bilateral with synchronous or metachronous involvement. The tumor has a multinodular cut surface with a grey-fleshy tan surface. Occasionally, the tumor is multifocal within the testis.
INCIDENCE 5% of testicular tumors AGE RANGE-MEDIAN Rare under 30 years
Mean age at diagnosis of 53.6 years (range 25 to 87 years)
SEX (M:F) M, no female counterpart
CHARACTERIZATION Laboratory Markers No known serum markers
Bilateral tumors in 9%, usually asynchronous
Well circumscribed multilobulated or multinodular
Soft, friable, and gray tan with mucoid surface
May show cystic change with hemorrhage or necrosis
HISTOLOGICAL TYPES CHARACTERIZATION General
Diffuse sheets of cells with edema filled cystic spaces.
There is little fibrous septa and lymphocytes, typical of classical seminomas.
There is considerable cellular pleomorphism with cells ranging from lymphocyte-like cells to intermediate cells to giant cells.
There is a high mitotic rate.
Polymorphous cell population with three types of neoplastic cells
VARIANTS Anaplastic variant Anaplastic variant has, in addition to the same three cell types, cells with large vesicular nuclei and macronucleoli, multinucleated tumor giant cells, a high mitotic index, abnormal mitotic figures, blood vessel invasion, invasion of tunica albuginea, and necrosis. Many of these features cause diagnostic confusion with embryonal carcinoma with solid growth pattern. A lack of diffuse immunoreactivity for PLAP and epithelial markers would support the diagnosis of anaplastic spermatocytic seminoma. These anaplastic changes probably represent dedifferentiation but do not seem to alter the biological behavior of the neoplasm. Sarcomatous change Sarcomatous change may rarely occur. The seminomatous and sarcomatous areas are intimately admixed, with no apparent morphologic transition between the two components. The sarcomatous elements have consisted of undifferentiated spindle cells or show rhabdomyosarcomatous differentiation. It is thought that it represents anaplastic transformation or dedifferentiation of a spermatocytic seminoma. The spermatocytic seminoma component has shown the usual histological features, except for an unusually high mitotic rate and frequent atypical mitotic figures in some cases. No other germ cell or teratomatous elements have been identified in any case. If present, this sarcomatous change leads to an aggressive course with extremely poor prognosis. Adjuvant chemotherapy seems to have no effect in the cases in which it has been tried. Fifty percent of patients die of metastases to lungs, liver, or retroperitoneum within in a few months.
CHARACTERIZATION Immunoperoxidase These tumors are typically negative for PLAP and HCG, unlike classic seminomas.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
CHARACTERIZATION SPERMATOCYTIC SEMINOMA TYPICAL SEMINOMA Proportion of germ cell tumors 1-2% 40-50% Sites Testis only Testis, ovary, mediastinum, pineal, retroperitoneum Bilaterality 9% 2% Association with other forms of germ cell tumor No Yes Association with IGCNU No Yes Intercellular edema Common Uncommon Composition 3 cell types, with denser cytoplasm, round nuclei 1 cell type, often clear cytoplasm, less regular nuclei Stroma Scanty Prominent Lymphoid reaction Rare to absent Prominent Granulomas Extremely rare Often prominent Sarcomatous transformation Occasional Absent Glycogen Absent to scant Abundant PLAP staining Absent to scant Prominent hCG staining Absent Present in 10% Metastases Extremely rare Common
ADDITIONAL DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Lymphoma
PROGNOSIS AND TREATMENT CHARACTERIZATION Prognostic Factors Survival Excellent prognosis Metastasis There are only four reported cases of spermatocytic seminoma with metastases and the documentation in some of these cases is questionable leading to the current viewpoint that these tumors are best regarded as potentially capable of metastases. Treatment Radical orchiectomy usually resulting in cure
Post-operative prophylactic radiation or chemotherpay do not offer any additional benefit and are not routinely recommended.
Cancer 1988; 61:409-14.
Hum Pathol 1996; 27:650-55.
Tumors of the Testis, Adnexa, Spermatic cord, and scrotum in Atlas of Tumor Pathology. Third Series. Fascicle 25. 1999.
Last Updated 4/1/2001
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