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Presented by Dr. White and prepared by Austin McCuiston
40 year old male with a superficial inguinal mass.
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1. Question
40 year old male with a superficial inguinal mass.
Correct
Answer: D
Histology: Histologic sections show a round to ovoid, well circumscribed, variably cellular spindle cell lesion. The nuclei are ovoid to tapered, fairly uniform, and cytologically bland. The stroma in this case is dense and collagenous, and there are admixed lymphocytes, plasma cells, and mast cells. Numerous and prominent thick walled, small to medium caliber blood vessels are seen.
Discussion: Cellular angiofibromas are mesenchymal neoplasms which are typically superficial and arise in the vulvovaginal, perineal, and inguino-scrotal regions. Most lesions show the histologic features observed in this case, although some may show myxoid changes or adipocytic components.
Iwasa et al reported up to 60% of cellular angiofibromas are positive CD34, with a smaller percentage of tumors labeling positive for SMA and desmin. Positivity for ER and PR may be seen in cellular angiofibromas in both female and male patients, as demonstrated in this lesion from a male patient. S100 and cytokeratins are typically negative, and Demicco et al more recently demonstrated cellular angiofibromas are also negative for STAT6.
Cellular angiofibromas show overlapping morphologic features with spindle cell lipomas and mammary-type myofibroblastomas. More recently deletions on chromosome 13q14 have also been described in cellular angiofibromas with monoallelic deletions of RB1 detected by FISH, suggesting these entities are genetically similar.References:
Iwasa Y, Fletcher CD. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases. Am J Surg Pathol. 2004 Nov;28(11):1426-35. PubMed PMID: 15489646.Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672-82.
Flucke U, van Krieken JH, Mentzel T. Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. Mod Pathol. 2011 Jan;24(1):82-9. doi: 10.1038/modpathol.2010.170. Epub 2010 Sep 17. PubMed PMID: 20852591.
Incorrect
Answer: D
Histology: Histologic sections show a round to ovoid, well circumscribed, variably cellular spindle cell lesion. The nuclei are ovoid to tapered, fairly uniform, and cytologically bland. The stroma in this case is dense and collagenous, and there are admixed lymphocytes, plasma cells, and mast cells. Numerous and prominent thick walled, small to medium caliber blood vessels are seen.
Discussion: Cellular angiofibromas are mesenchymal neoplasms which are typically superficial and arise in the vulvovaginal, perineal, and inguino-scrotal regions. Most lesions show the histologic features observed in this case, although some may show myxoid changes or adipocytic components.
Iwasa et al reported up to 60% of cellular angiofibromas are positive CD34, with a smaller percentage of tumors labeling positive for SMA and desmin. Positivity for ER and PR may be seen in cellular angiofibromas in both female and male patients, as demonstrated in this lesion from a male patient. S100 and cytokeratins are typically negative, and Demicco et al more recently demonstrated cellular angiofibromas are also negative for STAT6.
Cellular angiofibromas show overlapping morphologic features with spindle cell lipomas and mammary-type myofibroblastomas. More recently deletions on chromosome 13q14 have also been described in cellular angiofibromas with monoallelic deletions of RB1 detected by FISH, suggesting these entities are genetically similar.References:
Iwasa Y, Fletcher CD. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases. Am J Surg Pathol. 2004 Nov;28(11):1426-35. PubMed PMID: 15489646.Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672-82.
Flucke U, van Krieken JH, Mentzel T. Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. Mod Pathol. 2011 Jan;24(1):82-9. doi: 10.1038/modpathol.2010.170. Epub 2010 Sep 17. PubMed PMID: 20852591.
