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Presented by Jonathan Epstein, M.D. and prepared by Jeffrey T. Schowinsky, M.D.
Case 6: An 8 year old male presented with a right testicular mass.
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1. Question
Week 293: Case 6
An 8 year old male presented with a right testicular mass. The testis was almost entirely replaced by tumor measuring 5.5 x 4 x 3.5cm.images/120406JE6a.jpg
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images/120406JE6e.jpgCorrect
Answer: Malignant sex cord stromal tumor
Histology: The tumor has a variable appearance. In its better differentiated areas the tumor forms cords of cells with focal tubular differentiation. Other areas of the tumor consist of immature cells with a rhabdoid appearance set within a slightly myxoid stroma. In some areas the tumor forms microcystic structures. The tumor shows a brisk mitotic rate with greater than 20 mitoses per ten high power fields. The tumor was positive for inhibin, CD99 and negative for calretinin, EMA, cytokeratin, CAM5.2, myogenin, actin, and desmin. The proliferation rate based on KI-67 was around 80 percent.
Discussion: Although the most common pure testicular germ cell tumor in pre-pubertal males is a yolk sac tumor, the majority of this lesion does not resemble any of the patterns of yolk sac tumor. The only histological feature that vaguely resembles a yolk sac tumor is the presence of some microcystic component. The negative keratin stain rules out yolk sac tumor as well.
Although focally the tumor shows tubular differentiation, the lesion lacks mature well formed glands typical of a teratoma. The more primitive component of this lesion does not resemble any of the elements of an immature teratoma. The only potential teratomatous lesion that this tumor could reflect would be a sarcoma arising within a teratoma such as a rhabdomyosarcoma. The lack of well formed areas of teratoma and a lack of staining for myogenin, actin and desmin rule this out. Also teratomatous tubules typically are EMA positive.
The morphology along with the positive inhibin and CD99 staining is typical of a sex cord stromal tumor. Although the staining for calretinin is negative, we have often noted this within even better differentiated sex cord stromal tumors. Pathological features that best correlate with malignant course are:
1) a tumor diameter greater than 5cm
2) necrosis
3) moderate to severe nuclear atypia
4) vascular invasion
5) a mitotic rate greater than 5 per 10 high power fieldsThe presence of multiple adverse features in the current case warrants a diagnosis of malignancy.
Incorrect
Answer: Malignant sex cord stromal tumor
Histology: The tumor has a variable appearance. In its better differentiated areas the tumor forms cords of cells with focal tubular differentiation. Other areas of the tumor consist of immature cells with a rhabdoid appearance set within a slightly myxoid stroma. In some areas the tumor forms microcystic structures. The tumor shows a brisk mitotic rate with greater than 20 mitoses per ten high power fields. The tumor was positive for inhibin, CD99 and negative for calretinin, EMA, cytokeratin, CAM5.2, myogenin, actin, and desmin. The proliferation rate based on KI-67 was around 80 percent.
Discussion: Although the most common pure testicular germ cell tumor in pre-pubertal males is a yolk sac tumor, the majority of this lesion does not resemble any of the patterns of yolk sac tumor. The only histological feature that vaguely resembles a yolk sac tumor is the presence of some microcystic component. The negative keratin stain rules out yolk sac tumor as well.
Although focally the tumor shows tubular differentiation, the lesion lacks mature well formed glands typical of a teratoma. The more primitive component of this lesion does not resemble any of the elements of an immature teratoma. The only potential teratomatous lesion that this tumor could reflect would be a sarcoma arising within a teratoma such as a rhabdomyosarcoma. The lack of well formed areas of teratoma and a lack of staining for myogenin, actin and desmin rule this out. Also teratomatous tubules typically are EMA positive.
The morphology along with the positive inhibin and CD99 staining is typical of a sex cord stromal tumor. Although the staining for calretinin is negative, we have often noted this within even better differentiated sex cord stromal tumors. Pathological features that best correlate with malignant course are:
1) a tumor diameter greater than 5cm
2) necrosis
3) moderate to severe nuclear atypia
4) vascular invasion
5) a mitotic rate greater than 5 per 10 high power fieldsThe presence of multiple adverse features in the current case warrants a diagnosis of malignancy.
