TY - JOUR
T1 - Paraneoplastic dysmotility
T2 - Loss of interstitial cells of Cajal
AU - Pardi, Darrell S.
AU - Miller, Steven M.
AU - Miller, Daniel L.
AU - Burgart, Lawrence J.
AU - Szurszewski, Joseph H.
AU - Lennon, Vanda A.
AU - Farrugia, Gianrico
N1 - Funding Information:
Grant support: National Institutes of Health (DK52766, DK17238, and NCI CA37343) and the Mayo Clinic Cancer Center.
PY - 2002
Y1 - 2002
N2 - Autoimmune impairment and destruction of the enteric nervous plexus are thought to play a central role in the pathogenesis of paraneoplastic motility disorders. We present a case of a small-cell lung carcinoma-related paraneoplastic motility disorder associated with abnormal interstitial cells of Cajal networks. Antibodies against c-Kit and protein gene product 9.5 were used to selectively stain interstitial cells of Cajal and the enteric nervous plexus, respectively. A 68-yr-old man presented with anorexia, early satiety, nausea, and weight loss. Investigations revealed gastroparesis, delayed small intestinal transit, and mediastinal lymphadenopathy. The patient was seropositive for type 1 antineuronal nuclear autoantibody and P/Q-type calcium channel antibody. Biopsy of mediastinal lymph nodes revealed metastatic small-cell carcinoma cells that were immunoreactive for c-Kit. Immunohistochemical staining of a full-thickness small intestinal biopsy revealed a relatively intact myenteric plexus but a sparse and disorganized interstitial cells of Cajal network. The histopathology of this case suggests that interstitial cells of Cajal may be a target in the pathogenesis of paraneoplastic motility disorders.
AB - Autoimmune impairment and destruction of the enteric nervous plexus are thought to play a central role in the pathogenesis of paraneoplastic motility disorders. We present a case of a small-cell lung carcinoma-related paraneoplastic motility disorder associated with abnormal interstitial cells of Cajal networks. Antibodies against c-Kit and protein gene product 9.5 were used to selectively stain interstitial cells of Cajal and the enteric nervous plexus, respectively. A 68-yr-old man presented with anorexia, early satiety, nausea, and weight loss. Investigations revealed gastroparesis, delayed small intestinal transit, and mediastinal lymphadenopathy. The patient was seropositive for type 1 antineuronal nuclear autoantibody and P/Q-type calcium channel antibody. Biopsy of mediastinal lymph nodes revealed metastatic small-cell carcinoma cells that were immunoreactive for c-Kit. Immunohistochemical staining of a full-thickness small intestinal biopsy revealed a relatively intact myenteric plexus but a sparse and disorganized interstitial cells of Cajal network. The histopathology of this case suggests that interstitial cells of Cajal may be a target in the pathogenesis of paraneoplastic motility disorders.
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U2 - 10.1016/S0002-9270(02)04207-7
DO - 10.1016/S0002-9270(02)04207-7
M3 - Article
C2 - 12135044
AN - SCOPUS:0036314347
SN - 0002-9270
VL - 97
SP - 1828
EP - 1833
JO - American Journal of Gastroenterology
JF - American Journal of Gastroenterology
IS - 7
ER -