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Presented by Jonathan Epstein, M.D. and prepared by Jospeh Kronz, M.D.
Case 5: 40-year-old male with a tumor involving the skull
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Question 1 of 1
1. Question
Week 2: Case 5
40-year-old male with a tumor involving the skull
Correct
Answer: Chordoma
Histology: Within dense fibrous tissue are myxoid nodules containing cords of tumor cells. At higher magnification, most of the tumor cells have a relatively uniform monotonous appearance with infrequent mitoses. Some of the cells have very prominent vacuolated cytoplasm resembling lipoblasts. These cells are known as “physaliferous cells”. Focally, cells show a somewhat greater degree of cytologic atypia, which may be seen in recurrent chordomas or those that have been treated with radiation.
Discussion: The location of the tumor within the skull would be helpful to know as chordoma arising in the skull classically occurs at the clivus. In some cases, it may be difficult to distinguish a myxoid chondrosarcoma from a chordoma; immunohistochemical demonstration of keratin is diagnostic of a chordoma. Chondroma has distinct cartilage formation, rather than merely a myxoid matrix. Metastatic mucinous adenocarcinomas can also bear a resemblance to chordoma. However, in this case, the presence of physaliferous cells rules out other entities.
Incorrect
Answer: Chordoma
Histology: Within dense fibrous tissue are myxoid nodules containing cords of tumor cells. At higher magnification, most of the tumor cells have a relatively uniform monotonous appearance with infrequent mitoses. Some of the cells have very prominent vacuolated cytoplasm resembling lipoblasts. These cells are known as “physaliferous cells”. Focally, cells show a somewhat greater degree of cytologic atypia, which may be seen in recurrent chordomas or those that have been treated with radiation.
Discussion: The location of the tumor within the skull would be helpful to know as chordoma arising in the skull classically occurs at the clivus. In some cases, it may be difficult to distinguish a myxoid chondrosarcoma from a chordoma; immunohistochemical demonstration of keratin is diagnostic of a chordoma. Chondroma has distinct cartilage formation, rather than merely a myxoid matrix. Metastatic mucinous adenocarcinomas can also bear a resemblance to chordoma. However, in this case, the presence of physaliferous cells rules out other entities.
