Kyle Perry
banner
kyledperrymd.bsky.social
Kyle Perry
@kyledperrymd.bsky.social
47 followers 53 following 23 posts
Bone and soft tissue, surgical and cytopathologist at University of Michigan | @UMichPath@UMichMedicine | T/RT not medical advice
Posts Media Videos Starter Packs
Nice review article by Szczepanski, Westerhoff and Schechter on this rare entity (pubmed.ncbi.nlm.nih.gov/39743931/)
The spindled morphology and plexiform architecture can sometimes cause these tumors to be mistaken for (SDH-deficient) GIST. Unlike GIST, these tumors are negative for CD117 and DOG1.
It’s gastric and spindled, but it’s not a GIST… Plexiform fibromyxoma is a benign tumor that presents as a multinodular intramural mass in the stomach. The nodules can be fibrous or myxoid and have a delicate capillary pattern. #BSTPath, #UmichPath, #BSTpathGoBlue
Special thanks to my U of Michigan colleagues for allowing me to share this case!
Some nice papers (Fritchie and colleagues/ Chung and colleagues) further elaborating on this entity(ies)….
pubmed.ncbi.nlm.nih.gov/33097826/
pubmed.ncbi.nlm.nih.gov/33300192/
Fibromyxoid soft tissue tumors/fibroblastic lipoblastomas with a PLAG1 gene fusion often contain foci of myxoid stroma in the background of a more fibrous appearance. In addition to PLAG1 staining, these tumors usually show expression of desmin and CD34.
Dealing with an unusual fibromyxoid spindle cell neoplasm (that is MUC4 negative)? Consider PLAG1! #BSTPath, #UmichPath, #BSTpathGoBlue
I would highly recommend this course for anyone (regardless of level of expertise) looking for a comprehensive/case-based experience with bone and soft tissue. Dr. Fritchie and colleagues at Cleveland Clinic are excellent teachers.
www.clevelandclinicmeded.com/live/courses...
Nice article on ALK-positive histiocytosis of the breast: pubmed.ncbi.nlm.nih.gov/32826530/
The liberal use of ALK and histiocytic markers, with follow-up and molecular testing, can help ensure proper identification. These tumors, including the case presented here, often exhibit a KIF5B::ALK gene fusion.
Interestingly, the cells in this case also showed staining for OCT2, which has historically been associated with other histiocytic-type proliferations, such as Rosai-Dorfman disease, some Langerhans cell histiocytosis, and juvenile xanthogranulomas.
Unlike IMT, the tumor cells of interest are negative for SMA and positive for PU.1. The cells exhibit more wrinkled and irregular nuclei, which can help suggest histiocytic differentiation.
ALK-positive histiocytosis of the breast is a rare tumor that can mimic other inflammatory neoplasms or processes, such as inflammatory myofibroblastic tumor, cellular spindled histiocytic pseudotumor (among others). #BSTPath, #UmichPath, #BSTpathGoBlue
Nice article by Dermawan and colleagues on CD34-negative solitary fibrous tumors:
pubmed.ncbi.nlm.nih.gov/34152108/
Nice article by Dermawan and colleagues on CD34-negative solitary fibrous tumors:
pubmed.ncbi.nlm.nih.gov/34152108/
Although not typically associated with a “round cell sarcoma” differential, solitary fibrous tumor can sometimes mimic small round cell type sarcomas such as Ewing sarcoma. #BSTPath, #UmichPath, #BSTpathGoBlue
Nice article by Sciallis, Chen and Folpe describing this entity. pubmed.ncbi.nlm.nih.gov/22982900/
Cellular spindled histiocytic pseudotumor can sometimes be confused for inflammatory myofibroblastic tumor (or other spindled cell neoplasms). The spindled cells exhibit irregular grooved nuclei which suggest the tumor’s histiocytic nature. #BSTPath, #UmichPath, #BSTpathGoBlue
Nice article on variable morphologies in desmoid fibromatosis.
pubmed.ncbi.nlm.nih.gov/27124915/
Of note, familiarity with your lab’s beta catenin stain is important as this stain can have variable sensitivity/specificity for desmoid fibromatosis (this case was negative for nuclear staining). Sequencing for CTNNB1 can be helpful when the staining pattern is suspect.
While desmoid tumors are often deep seated, these can rarely present as more superficial/subcutaneous lesions. Areas of a more fascicular spindle cell arrangement or “perivascular edema” can help alert the pathologist to pursue additional studies.
Desmoid fibromatosis can sometimes exhibit histologic features which overlap with nodular fasciitis, including focal areas of a more haphazard spindle cell arrangement. #BSTpath, #UMichPath, #BSTpathGoBlue