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Image of the Week
Edited and moderated by Stacey Mills, MD, Pathology Network's Image of the Week Blog is a forum for the discussion of interesting and often diagnostically challenging pathology images.
Wednesday, June 19, 2013
A 24-year-old man with occasional hemoptysis.
A 24-year-old man presented with long-standing cough and occasional hemoptysis.  Endoscopy demonstrated a 2 cm, polypoid tracheal mass.  An endoscopic excision was performed resulting in the specimen seen below. 
 

 
 
The specimen consists of nests and sheets of bland, uniform polygonal cells in a prominently vascular stroma.  Mitotic figures are extremely rare and there is no evidence of necrosis.  Clinically, and on initial pathologic examination this was thought to be a typical carcinoid tumor (well-differentiated neuroendocrine carcinoma).  However, immunohistochemical stains for neuroendocrine differentiation were all negative.
 
 
What is your diagnosis?
 
 
The case was submitted in consultation and the features above were noted by us as well.  In addition, it was noted that many of the cells had perinuclear halos creating a "fried egg" appearance and that these cells often cuffed around more dialated vascular spaces.  Stains for cytokeratin and repeat stains for neuroendocrine markers were negative.  A immunohistochemical stain for smooth muscle actin (SMA) is shown below.
 
 
 
 
This is a nice example of a glomangiopericytoma or glomus tumor.  I generally prefer the former, longer term because some clinicians still confuse "glomus tumor" with paraganglioma, particularly in the head and neck region.  However, the newer term has yet to be fully accepted, so it's your choice which term to use, and I'll use both, even in this brief text.  The skin of the extremities is by far the most common site for glomus tumors, where they represent the "G" in the "ANGEL" of painful cutaneous lesions.  However, they can occur sporadically at almost any location.  These tumors are composed of cells differentiating towards (who knows if they actually originate from) the specialized pericytes that control blood shunting for temperature regulation.  They are virtually always benign, although in some anatomic locations such as the sinonasal region they can be somewhat difficult to remove.   Glomus tumors of the trachea are quite rare but a quick search with PubMed or Google will turn up multiple examples, usually in the form of single case reports.
6/20/2013
Abdul Kadir Rifaei Abdul Rashid Khairi said:
What could it be, if almost a similar looking tumour cells are found in the the rectal wall and is positive for cytokeratin, but not for actin or neutroendocrine marker. Furthermore, the overlying rectal mucosa is normal. I have one case like this , and were not sure if it is a primary or metastatic tumour. (Dr. Mills' comment: The differential diagnosis of bland polygonal cells is broad. If it's a man, I would be concerned about high-grade prostatic adenocarcinoma, which should be easy to exclude with appropriate stains and clinical findings. If it's a woman, breast carcinoma would certainly be consideration. Good luck!)
About the Author

Stacey E. Mills, MD
Stacey E. Mills, MD, a graduate of University of Virginia (UVA) and the UVA Medical Center, has authored nearly 230 articles, 20+ books, atlases and monographs—including the renowned Sternberg's Diagnostic Surgical Pathology. He has been a practicing Professor and Staff Pathologist at UVA for 30+ years and is Director of Surgical Pathology and Cytopathology. His clinical specialty is general surgical pathology with emphasis on neoplasms and neoplasm-like lesions. Dr. Mills is also Editor-in-Chief of The American Journal of Surgical Pathology.