Paraneoplastic dysmotility: loss of interstitial cells of Cajal.
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.
Department of Physiology and Biophysics, Mayo Clinic, Rochester, Minnesota 55905, USA.
Darrell S
DS
Pardi
Steven M
SM
Miller
Daniel L
DL
Miller
Lawrence J
LJ
Burgart
Joseph H
JH
Szurszewski
Vanda A
VA
Lennon
Gianrico
G
Farrugia
D000368:Aged; D018288:Carcinoma, Small Cell / Q000150:complications; D004064:Digestive System / Q000737:chemistry / Q000294:innervation / Q000473:pathology; D005767:Gastrointestinal Diseases / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology*; D005769:Gastrointestinal Motility; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications; D008297:Male; D010257:Paraneoplastic Syndromes / Q000209:etiology / Q000473:pathology*; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis
D019009:Proto-Oncogene Proteins c-kit