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Presented by Dr. Cimino-Mathews and prepared by Nicolas Giraldo-Castillo
This case talks about a 50 year-old female with a liver mass.
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1. Question
A 50 year-old female with a liver mass. Choose the correct diagnosis:
Correct
Answer: D. Metastatic paraganglioma
Histology: The mass has prominent vasculature and is composed of solid nests and sheets of cells with abundant amphophilic, granular cytoplasm. Many of the cells have indistinct cell borders and some contain numerous cytoplasmic vacuoles. The cells display nuclear pleomorphism with hyperchromasia and prominent nucleoli. There is no necrosis. The background liver parenchyma lacks features of cirrhosis. This patient has a history of a retroperitoneal paraganglioma, which was immunoreactive for synaptophysin and negative for cytokeratin immunostains.
Discussion: Paragangliomas arise from the extra-adrenal paraganglia and are histologically identical to the pheochromocytomas that arise from the adrenal medulla. There is accepted grading system for these tumors nor any single histologic feature that is predictive of metastatic spread. That is, the presence of features that are considered “atypical” in other tumors—such as necrosis, mitotic activity, vascular invasion, and local invasion—do not independently predict metastasis in paragangliomas or pheochromocytomas. The presence of metastatic spread is the only criteria used to diagnose malignancy in paragangliomas and pheochromocytomas, although it should be noted that the 2017 WHO Classification of Endocrine Tumours 4th Ed. specified that the term “metastatic paraganglioma/pheochromocytoma” be used rather than “malignant paraganglioma/pheochromocytoma.” A large proportion of paragangliomas arise in association with specific genetic syndromes, and it is now our practice pattern to perform immunohistochemistry for SDHB on all paragangliomas to identify patients at risk of SDH-deficiency syndromes and the development of other SDH-deficient tumors.
Reference:
1. Lam AK. Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. Endocr Pathol. 2017 Sep;28(3):213-227.
2. Gill AJ. Succinate dehydrogenase (SDH)-deficient neoplasia. Histopathology. 2018 Jan;72(1):106-116.Incorrect
Answer: D. Metastatic paraganglioma
Histology: The mass has prominent vasculature and is composed of solid nests and sheets of cells with abundant amphophilic, granular cytoplasm. Many of the cells have indistinct cell borders and some contain numerous cytoplasmic vacuoles. The cells display nuclear pleomorphism with hyperchromasia and prominent nucleoli. There is no necrosis. The background liver parenchyma lacks features of cirrhosis. This patient has a history of a retroperitoneal paraganglioma, which was immunoreactive for synaptophysin and negative for cytokeratin immunostains.
Discussion: Paragangliomas arise from the extra-adrenal paraganglia and are histologically identical to the pheochromocytomas that arise from the adrenal medulla. There is accepted grading system for these tumors nor any single histologic feature that is predictive of metastatic spread. That is, the presence of features that are considered “atypical” in other tumors—such as necrosis, mitotic activity, vascular invasion, and local invasion—do not independently predict metastasis in paragangliomas or pheochromocytomas. The presence of metastatic spread is the only criteria used to diagnose malignancy in paragangliomas and pheochromocytomas, although it should be noted that the 2017 WHO Classification of Endocrine Tumours 4th Ed. specified that the term “metastatic paraganglioma/pheochromocytoma” be used rather than “malignant paraganglioma/pheochromocytoma.” A large proportion of paragangliomas arise in association with specific genetic syndromes, and it is now our practice pattern to perform immunohistochemistry for SDHB on all paragangliomas to identify patients at risk of SDH-deficiency syndromes and the development of other SDH-deficient tumors.
Reference:
1. Lam AK. Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. Endocr Pathol. 2017 Sep;28(3):213-227.
2. Gill AJ. Succinate dehydrogenase (SDH)-deficient neoplasia. Histopathology. 2018 Jan;72(1):106-116.
