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Presented by Ashley Cimino-Mathews, M.D. and prepared by Sarah Karram, M.D.
10 year-old male with a suprasellar mass.
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Question 1 of 1
1. Question
Week (613): Case 1
10 year-old male with a suprasellar mass.
Correct
Answer: Germinoma
Histology: The lesion consists of sheets of loosely cohesive, round epithelioid cells with admixed chronic inflammatory cells. The individual cells have abundant, predominantly clear but variably granular cytoplasm and large round nuclei with prominent nucleoli as well as abundant mitotic figures. The morphology is identical to that of a testicular seminoma. By immunohistochemistry, this lesion is positive for ckit and OCT3/4, and negative for CD45, cytokeratin and EMA.
Discussion: Intracranial germ cell tumors are rare primary brain tumors that primarily affect children or adolescents, and most occur in the suprasellar/pineal/midline of the brain. Of the intracranial germ cell tumors, germinomas are most common and are identical to seminomas of the testis. In general germinomas, have better outcome than non-germinomatous germ cell tumors and respond well to radiation therapy, but since rarely there may be post-radiation sequela, combination lower dose radiation with chemotherapy may be used. Differential diagnosis based upon the lesional morphology and depending upon the anatomic site includes non-germinomatous germ cell tumors, glial neoplasms, lymphoma, pineal gland neoplasms, pituitary neoplasms, and metastases. Isochromosome 12p is seen in nearly all germ cell tumors and can aid in diagnosis if the histology and immunophenotype are ambiguous.
References:
1. Fujimaki T. Central nervous system germ cell tumors: classification, clinical features, and treatment with a historical overview. J Child Neurol. 2009 Nov;24(11):1439-45.
2. Packer RJ, Cohen BH, Cooney K. Intracranial germ cell tumors. Oncologist. 2000;5(4):312-20.
3. Ulbright TM. Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues. Mod Pathol. 2005 Feb;18 Suppl 2:S61-79.Incorrect
Answer: Germinoma
Histology: The lesion consists of sheets of loosely cohesive, round epithelioid cells with admixed chronic inflammatory cells. The individual cells have abundant, predominantly clear but variably granular cytoplasm and large round nuclei with prominent nucleoli as well as abundant mitotic figures. The morphology is identical to that of a testicular seminoma. By immunohistochemistry, this lesion is positive for ckit and OCT3/4, and negative for CD45, cytokeratin and EMA.
Discussion: Intracranial germ cell tumors are rare primary brain tumors that primarily affect children or adolescents, and most occur in the suprasellar/pineal/midline of the brain. Of the intracranial germ cell tumors, germinomas are most common and are identical to seminomas of the testis. In general germinomas, have better outcome than non-germinomatous germ cell tumors and respond well to radiation therapy, but since rarely there may be post-radiation sequela, combination lower dose radiation with chemotherapy may be used. Differential diagnosis based upon the lesional morphology and depending upon the anatomic site includes non-germinomatous germ cell tumors, glial neoplasms, lymphoma, pineal gland neoplasms, pituitary neoplasms, and metastases. Isochromosome 12p is seen in nearly all germ cell tumors and can aid in diagnosis if the histology and immunophenotype are ambiguous.
References:
1. Fujimaki T. Central nervous system germ cell tumors: classification, clinical features, and treatment with a historical overview. J Child Neurol. 2009 Nov;24(11):1439-45.
2. Packer RJ, Cohen BH, Cooney K. Intracranial germ cell tumors. Oncologist. 2000;5(4):312-20.
3. Ulbright TM. Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues. Mod Pathol. 2005 Feb;18 Suppl 2:S61-79.
