TY - JOUR
T1 - What Is the Value of Counting Mast Cells in Gastrointestinal Mucosal Biopsies?
AU - Panarelli, Nicole C.
AU - Hornick, Jason L.
AU - Yantiss, Rhonda K.
N1 - Publisher Copyright:
© 2022 United States & Canadian Academy of Pathology
PY - 2023/2
Y1 - 2023/2
N2 - Neoplastic and nonneoplastic mast cell disorders can cause diarrhea, nausea, and abdominal pain that result from heightened release of mast cell mediators. Systemic mastocytosis is characterized by neoplastic mast cell aggregates in the bone marrow and other sites, particularly the skin and gastrointestinal tract. In this situation, extramedullary mast cell aggregates display atypical morphology, with aberrant immunostaining for CD25 in addition to staining for other mast cell markers, such as mast cell tryptase and CD117. Morphologically normal mast cells have also been implicated in nonneoplastic conditions. For example, increased mast cell numbers have been reported in the mucosal biopsy samples from patients with irritable bowel syndrome and hereditary alpha-tryptasemia. Patients with mast cell activation syndrome presumably experience symptoms related to the aberrant elaboration of histamine and other mediators from normal-appearing mast cells present in normal numbers. Unfortunately, similarities in terminology among these biologically distinct clinical conditions have caused considerable diagnostic confusion among clinical colleagues, resulting in frequent requests for pathologists to quantify and characterize mast cells in normal gastrointestinal biopsy samples from patients with diarrheal symptoms. The purpose of this review is to summarize the available data related to mast cell assessment in the gastrointestinal tract and provide pathologists with practical information so that they can help their clinical colleagues manage patients with presumed mast cell disorders.
AB - Neoplastic and nonneoplastic mast cell disorders can cause diarrhea, nausea, and abdominal pain that result from heightened release of mast cell mediators. Systemic mastocytosis is characterized by neoplastic mast cell aggregates in the bone marrow and other sites, particularly the skin and gastrointestinal tract. In this situation, extramedullary mast cell aggregates display atypical morphology, with aberrant immunostaining for CD25 in addition to staining for other mast cell markers, such as mast cell tryptase and CD117. Morphologically normal mast cells have also been implicated in nonneoplastic conditions. For example, increased mast cell numbers have been reported in the mucosal biopsy samples from patients with irritable bowel syndrome and hereditary alpha-tryptasemia. Patients with mast cell activation syndrome presumably experience symptoms related to the aberrant elaboration of histamine and other mediators from normal-appearing mast cells present in normal numbers. Unfortunately, similarities in terminology among these biologically distinct clinical conditions have caused considerable diagnostic confusion among clinical colleagues, resulting in frequent requests for pathologists to quantify and characterize mast cells in normal gastrointestinal biopsy samples from patients with diarrheal symptoms. The purpose of this review is to summarize the available data related to mast cell assessment in the gastrointestinal tract and provide pathologists with practical information so that they can help their clinical colleagues manage patients with presumed mast cell disorders.
KW - irritable bowel syndrome
KW - mast cell activation syndrome
KW - mastocytic enterocolitis
KW - systemic mastocytosis
UR - http://www.scopus.com/inward/record.url?scp=85149153820&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85149153820&partnerID=8YFLogxK
U2 - 10.1016/j.modpat.2022.100005
DO - 10.1016/j.modpat.2022.100005
M3 - Review article
C2 - 36853780
AN - SCOPUS:85149153820
SN - 0893-3952
VL - 36
JO - Modern Pathology
JF - Modern Pathology
IS - 2
M1 - 100005
ER -