Quiz-summary
0 of 1 questions completed
Questions:
- 1
Information
Presented by Dr. Pedram Argani and prepared by Dr. Sintawat Wangsiricharoen.
This is an 84 year old female with an abdominal mass and a 35 year history of “recurrent sarcoma”.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
- Not categorized 0%
- 1
- Answered
- Review
-
Question 1 of 1
1. Question
This is an 84 year old female with an abdominal mass and a 35 year history of “recurrent sarcoma”.
Correct
Answer: B. Low grade fibromyxoid sarcoma
Histologic Description: This is a bland spindle cell lesion composed of spindles fibroblastic-type cells in a variably collagenous and myxoid background. Blood vessels are arranged in thin walled arcades in the myxoid areas, but are sparse in the more collagenous areas. Mitotic activity is scant. The neoplastic cells are immunoreactive for MUC4 and focally for EMA, but not for S100 protein.
Differential Diagnosis: Myxofibrosarcoma is a more pleomorphic myxoid sarcoma which is typically superficially located on the extremities. It lacks the characteristic translocation of low grade fibromyxoid sarcoma. Myxoid liposarcoma would demonstrate uniform lipoblastic cells characterized by vacuoles with indent the nucleus, and a characteristic t(12;16) translocation resulting in a FUS-DDIT3 gene fusion. Neurofibromas feature bland spindle cells with collagen that has a “shredded-carrot” pattern, along with prominent mast cells. Neurofibroma should demonstrate some immunoreactivity for S100 protein.
Low grade fibromyxoid sarcomas are bland neoplasms that are frequently mistaken for benign processes such as perineurioma and neurofibroma. The fact that they can focally stain for EMA is a pitfall that could lead to a misdiagnosis of perineurioma. MUC4 immunoreactivity is very useful in this differential diagnosis. Low grade fibromyxoid sarcoma is typically characterized by a t(7;16) translocation resulting in a FUS-CREB3L2 gene fusion. Sclerosing epithelioid fibrosarcoma is thought to be the intermediate/higher grade form of low grade fibromyxoid sarcoma. Cases with a sclerosing epithelioid fibrosarcoma pattern more commonly demonstrate the EWSR1-CREB3L1 or CREB3L2 gene fusions.
Incorrect
Answer: B. Low grade fibromyxoid sarcoma
Histologic Description: This is a bland spindle cell lesion composed of spindles fibroblastic-type cells in a variably collagenous and myxoid background. Blood vessels are arranged in thin walled arcades in the myxoid areas, but are sparse in the more collagenous areas. Mitotic activity is scant. The neoplastic cells are immunoreactive for MUC4 and focally for EMA, but not for S100 protein.
Differential Diagnosis: Myxofibrosarcoma is a more pleomorphic myxoid sarcoma which is typically superficially located on the extremities. It lacks the characteristic translocation of low grade fibromyxoid sarcoma. Myxoid liposarcoma would demonstrate uniform lipoblastic cells characterized by vacuoles with indent the nucleus, and a characteristic t(12;16) translocation resulting in a FUS-DDIT3 gene fusion. Neurofibromas feature bland spindle cells with collagen that has a “shredded-carrot” pattern, along with prominent mast cells. Neurofibroma should demonstrate some immunoreactivity for S100 protein.
Low grade fibromyxoid sarcomas are bland neoplasms that are frequently mistaken for benign processes such as perineurioma and neurofibroma. The fact that they can focally stain for EMA is a pitfall that could lead to a misdiagnosis of perineurioma. MUC4 immunoreactivity is very useful in this differential diagnosis. Low grade fibromyxoid sarcoma is typically characterized by a t(7;16) translocation resulting in a FUS-CREB3L2 gene fusion. Sclerosing epithelioid fibrosarcoma is thought to be the intermediate/higher grade form of low grade fibromyxoid sarcoma. Cases with a sclerosing epithelioid fibrosarcoma pattern more commonly demonstrate the EWSR1-CREB3L1 or CREB3L2 gene fusions.
