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Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult male with pelvic mass
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1. Question
Adult male with pelvic mass
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Answer: C. Cellular angiofibroma
Histology: This tumor is well circumscribed and composed of uniform short spindled cells in a fibrous background.
Discussion: This entity, also known as angiomyofibroblastoma-like tumor of the male genital tract. These tumors are low grade and characterized by spindle cells with uniform oval nuclei with open chromatin and low to absent mitotic activity. Some of the spindle cells can show myoid differentiation and express SMA or desmin focally. There are numerous blood vessels some of which can have fibrin within the wall. CD34 is positive in most cases and ER/PR can be variable. The main differential diagnoses include schwannoma and neurofibroma which are positive for S100, while cellular angiofibromas are negative. Low grade fibromyxoid sarcoma could be in the differential diagnosis in biopsies, in which case MUC4 could assist in arriving at the right diagnosis. Perhaps the most critical differential diagnosis is aggressive angiomyxoma for which there is no immunohistochemistry that would be helpful. In contrast to cellular angiofibroma, aggressive angiomyxoma has a more myxoid background and an infiltrative border. Aggressive angiomyxoma requires complete surgical excision to prevent a recurrence.
References:
Am J Surg Pathol. January 1998 – Volume 22 – Issue 1 – p 6-16.Incorrect
Answer: C. Cellular angiofibroma
Histology: This tumor is well circumscribed and composed of uniform short spindled cells in a fibrous background.
Discussion: This entity, also known as angiomyofibroblastoma-like tumor of the male genital tract. These tumors are low grade and characterized by spindle cells with uniform oval nuclei with open chromatin and low to absent mitotic activity. Some of the spindle cells can show myoid differentiation and express SMA or desmin focally. There are numerous blood vessels some of which can have fibrin within the wall. CD34 is positive in most cases and ER/PR can be variable. The main differential diagnoses include schwannoma and neurofibroma which are positive for S100, while cellular angiofibromas are negative. Low grade fibromyxoid sarcoma could be in the differential diagnosis in biopsies, in which case MUC4 could assist in arriving at the right diagnosis. Perhaps the most critical differential diagnosis is aggressive angiomyxoma for which there is no immunohistochemistry that would be helpful. In contrast to cellular angiofibroma, aggressive angiomyxoma has a more myxoid background and an infiltrative border. Aggressive angiomyxoma requires complete surgical excision to prevent a recurrence.
References:
Am J Surg Pathol. January 1998 – Volume 22 – Issue 1 – p 6-16.
