A Case of Adenocarcinoma in a Stoma Site after 27 Years of Stoma Surgery for Hirschsprung's Disease.
Adenocarcinomas originating at stoma sites are extremely rare. While many cases are associated with colorectal cancer or inflammatory bowel disease, instances without such predisposing factors are even rarer.
A 39-year-old man with a history of Hirschsprung's disease presented with tumor growth at his permanent stoma site, which had been established 27 years earlier. A biopsy confirmed adenocarcinoma. Preoperative imaging, including CT, MRI, and PET-CT, showed no evidence of lymph node or distant metastasis. Immunohistochemical staining (CK7+, CK20+, CDX2+) was consistent with a primary tumor of the small bowel. Based on the preoperative diagnosis of localized disease and the clinical goal of preserving intestinal function, local resection was performed with negative margins. Histopathological examination confirmed a primary ileal adenocarcinoma. The patient remains recurrence-free 30 months postoperatively without adjuvant chemotherapy.
This report presents a rare case of stoma-site adenocarcinoma arising 27 years after surgery for Hirschsprung's disease. In long-term survivors of pediatric stoma surgery, chronic physical and chemical irritation may contribute to malignancy even in the absence of a predisposing malignant background. Malignancy at the stoma site can be discovered by patients through self-examination; therefore, both patients and clinicians must recognize the potential risk for early detection.
A 39-year-old man with a history of Hirschsprung's disease presented with tumor growth at his permanent stoma site, which had been established 27 years earlier. A biopsy confirmed adenocarcinoma. Preoperative imaging, including CT, MRI, and PET-CT, showed no evidence of lymph node or distant metastasis. Immunohistochemical staining (CK7+, CK20+, CDX2+) was consistent with a primary tumor of the small bowel. Based on the preoperative diagnosis of localized disease and the clinical goal of preserving intestinal function, local resection was performed with negative margins. Histopathological examination confirmed a primary ileal adenocarcinoma. The patient remains recurrence-free 30 months postoperatively without adjuvant chemotherapy.
This report presents a rare case of stoma-site adenocarcinoma arising 27 years after surgery for Hirschsprung's disease. In long-term survivors of pediatric stoma surgery, chronic physical and chemical irritation may contribute to malignancy even in the absence of a predisposing malignant background. Malignancy at the stoma site can be discovered by patients through self-examination; therefore, both patients and clinicians must recognize the potential risk for early detection.
Authors
Koro Koro, Shonaka Shonaka, Tani Tani, Takeda Takeda, Otani Otani, Ohara Ohara, Hayashi Hayashi, Kamikokura Kamikokura, Aoki Aoki, Tanino Tanino, Hasegawa Hasegawa, Yokoo Yokoo
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