Raul,

Congratulation on this great study.

SMARCA4-deficient Thoracic Sarcomas

Clinicopathologic Study of 30 Cases With an Emphasis on Their Nosology and Differential Diagnoses

Perret, Raul, MSc, MD^*; Chalabreysse, Lara, MD^†; Watson, Sarah, MD, PhD^‡; Serre, Isabelle, MD^§; Garcia, Stephane, MD, PhD^∥; Forest, Fabien, MD, PhD^¶; Yvorel, Violaine, MD^¶; Pissaloux, Daniel, PhD^#; Thomas de Montpreville, Vincent, MD^**; Masliah-planchon, Julien, PhD, PharmD^††; Lantuejoul, Sylvie, MD, PhD^#,‡‡; Brevet, Marie, MD, PhD^†,§§; Blay, Jean-Yves, MD, PhD^§§,∥∥; Coindre, Jean-Michel, MD^*,¶¶; Tirode, Franck, PhD^##,***; Le Loarer, Francois, MD, PhD^*,¶¶

Where is this lesion? One or multiple?

Looks like a glomus tumor. SMA please.

Glomus tumor, glomangioma/mioma type

Yes, Glomus tissue...but not tumor, I was wrong. This is a normal glomus body! Nearby there is some hyperplastic change.

Think again, location is vulva

Angiomyofibroblastoma

Agree, I would go with angiomyofibroblastoma too

Alright. You guys are good. Angiomyofibroblastoma. Nice case.

Angiomyofibroblastoma could be a correct spot. Odd lobulated pattern and no alternating cellular and hypocellular areas ...why not a cellular angiofibroma?

Angiomyofibroblastoma, the ropy collagen is a good clue i think

Polypoid lesion? What's the silhouette? Also the cellular angiofibroma could be desmin+. And the fibroepithelial stroma polyp too. Cellula angiofibroma is my favorite diagnosis, because of the hyalinized vessels.

Cytologically, the tumor cells are sort of epithelioid and have a tendency to distributed around the vessels. That's quite typical for angiomyofibroblastoma. Cellular angiofibroma can be positive for desmin but the cells are spindle and do not cluster around the vessels. RB1 would help in difficult cases.

Angiomyofibroblastoma

This was a typical case of angiomyofibroblastoma. In this entity, cells are spindled to epithelioid and tend to aggregate around blood vessels and form clusters. They typically express desmin a marker that is rarely positive in spindle cell lipoma and cellular angiofibroma. However, those 3 neoplasms share genomic profiles with deletions in 13q (including RB1 locus) so they can sometimes have overlapping morphological and phenotypical features. Interestingly, superficial acral fibromyxoma has been recently found to present 13q deletions (at least in a subset of cases), hence, enlarging the Rb1 deleted family of neoplasms. Thanks for the comments

On 21/03/2019 at 02:26, anh said:

Raul,

Congratulation on this great study.

SMARCA4-deficient Thoracic Sarcomas

Clinicopathologic Study of 30 Cases With an Emphasis on Their Nosology and Differential Diagnoses

Perret, Raul, MSc, MD^*; Chalabreysse, Lara, MD^†; Watson, Sarah, MD, PhD^‡; Serre, Isabelle, MD^§; Garcia, Stephane, MD, PhD^∥; Forest, Fabien, MD, PhD^¶; Yvorel, Violaine, MD^¶; Pissaloux, Daniel, PhD^#; Thomas de Montpreville, Vincent, MD^**; Masliah-planchon, Julien, PhD, PharmD^††; Lantuejoul, Sylvie, MD, PhD^#,‡‡; Brevet, Marie, MD, PhD^†,§§; Blay, Jean-Yves, MD, PhD^§§,∥∥; Coindre, Jean-Michel, MD^*,¶¶; Tirode, Franck, PhD^##,***; Le Loarer, Francois, MD, PhD^*,¶¶

Thank you very much Anh. This was the product of teamwork as everything in life

Raul, 

You are welcome. I enjoyed reading that very much. Although I got to say I don't really know what it is. But I will keep that in the back of my mind if I see something weird in the mediastinum. My experience is sometimes you just read it, and you see one the next day. 

Anh