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[A case of jejunal neuroendocrine carcinoma complicated with dermatomyositis]

Natsumi Azuma1, Tatsunori Satoh1, Shinya Kawaguchi1

  • 1Department of Gastroenterology, Shizuoka General Hospital.

Nihon Shokakibyo Gakkai Zasshi = the Japanese Journal of Gastro-Enterology
|December 12, 2018
PubMed

Insights

This case report details a rare instance of jejunal neuroendocrine carcinoma (NEC) in a patient with dermatomyositis. It highlights the diagnostic journey and successful treatment of this unique gastrointestinal malignancy.

Area of Science:

  • Gastroenterology
  • Oncology
  • Dermatology

Background:

  • Dermatomyositis is an idiopathic inflammatory myopathy.
  • Gastrointestinal manifestations of dermatomyositis are rare.
  • Small bowel tumors are uncommon, with neuroendocrine carcinomas (NECs) being a distinct subtype.

Observation:

  • A 65-year-old woman with dermatomyositis presented with a jejunal mass.
  • Initial diagnosis was adenocarcinoma, later revised to large-cell NEC.
  • The tumor was staged as pT3, pN0, sM0, pStage IIA.

Findings:

  • The jejunal NEC was confirmed by histology and immunohistochemistry (positive for chromogranin A, negative for NCAM and synaptophysin).
  • The MIB-1 proliferation index was 60%.
  • The patient received cisplatin and etoposide chemotherapy post-surgery.

Implications:

  • This is the first reported case of dermatomyositis associated with primary jejunal NEC.
  • Early diagnosis and multimodal treatment are crucial for managing such rare malignancies.
  • Further research may elucidate potential links between autoimmune conditions and specific tumor types.

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