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Presented by Pedram Argani, M.D. and prepared by Lynette S. Nichols, M.D.
Case 1: The patient is a one-year old boy with a testicular mass.
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Question 1 of 1
1. Question
Week 133: Case 1
The patient is a one-year old boy with a testicular mass./images/Lyn’s/2p-1a.jpg
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/images/Lyn’s/2p-1e.jpgCorrect
Answer: Endodermal Sinus Tumor
Histology: The tumor has a microcystic and papillary growth pattern. Focally, the tumor demonstrates cells arranged radially around fibrovascular cores yielding the so-called “Schiller-Duval” body appearance. In the Schiller-Duval bodies, tumor cells are separated from the central blood vessel of the fibrovascular core by a space, similar to the appearance of an ependymoma or solid cystic papillary tumor of the pancreas. The tumor cells are polygonal with clear cytoplasm and irregular round nuclei. Hyaline intracytoplasmic and extracellular inclusions were not difficult to identify. The patient was found to have a markedly elevated serum alpha-fetoprotein level.
Discussion: In the infantile age group, endodermal sinus tumor and teratoma (immature or mature) are the main types of germ cell tumors seen; seminomas and embryonal carcinomas are virtually unheard of in this age group. Interestingly, these germ cell tumors are not associated with intratubular germ cell neoplasia in the surrounding testes, and lack the isochromosome 12p (i12p) abnormality that is characteristic of most adult germ cell tumors.
Immature teratoma is characterized by primitive neuroepithelium or highly cellular primitive stroma encircling primitive glands. Embryonal carcinoma features higher grade cytology and more gland formation. Choriocarcinoma is characterized by a biphasic appearance with both cytotrophoblast and syncytiotrophoblast.
In general, teratomas of the immature testes are benign regardless of whether or not they are mature or immature histologically. Endodermal sinus tumors (yolk sac tumors) are malignant. However, Stage I infantile yolk sac tumors are associated with a relatively good prognosis. Therefore, this patient was treated with orchiectomy alone and serum AFP levels will be closely followed.
Incorrect
Answer: Endodermal Sinus Tumor
Histology: The tumor has a microcystic and papillary growth pattern. Focally, the tumor demonstrates cells arranged radially around fibrovascular cores yielding the so-called “Schiller-Duval” body appearance. In the Schiller-Duval bodies, tumor cells are separated from the central blood vessel of the fibrovascular core by a space, similar to the appearance of an ependymoma or solid cystic papillary tumor of the pancreas. The tumor cells are polygonal with clear cytoplasm and irregular round nuclei. Hyaline intracytoplasmic and extracellular inclusions were not difficult to identify. The patient was found to have a markedly elevated serum alpha-fetoprotein level.
Discussion: In the infantile age group, endodermal sinus tumor and teratoma (immature or mature) are the main types of germ cell tumors seen; seminomas and embryonal carcinomas are virtually unheard of in this age group. Interestingly, these germ cell tumors are not associated with intratubular germ cell neoplasia in the surrounding testes, and lack the isochromosome 12p (i12p) abnormality that is characteristic of most adult germ cell tumors.
Immature teratoma is characterized by primitive neuroepithelium or highly cellular primitive stroma encircling primitive glands. Embryonal carcinoma features higher grade cytology and more gland formation. Choriocarcinoma is characterized by a biphasic appearance with both cytotrophoblast and syncytiotrophoblast.
In general, teratomas of the immature testes are benign regardless of whether or not they are mature or immature histologically. Endodermal sinus tumors (yolk sac tumors) are malignant. However, Stage I infantile yolk sac tumors are associated with a relatively good prognosis. Therefore, this patient was treated with orchiectomy alone and serum AFP levels will be closely followed.
