Rectal Paraganglioma

Abstract

A 57-year-old woman was admitted with lower abdominal pain and bloody bowel discharge. She was diagnosed with rectal tumor by colonoscopy, and a biopsy was performed. Surgery was performed, resulting in a diagnosis of rectal paraganglioma. Since recurrence was confirmed three years later, reoperation was done, and chemotherapy with cyclophosphamide, vincristine and dacarbazine (CVD) was subsequently carried out for further recurrence. After the administration of up to 15 courses of CVD, we delivered best supportive care due to disease progression. She died a year and a half after starting chemotherapy. We herein report this rare disease with a review of the relevant literature.

Keywords: CVD; cyclophosphamide; dacarbazine; paraganglioma; pheochromocytoma; vincristine.

Figure 1.

Colonoscopy revealed an elevated lesion in the rectum that was a non-epithelial, soft tumor that easily bled.

Figure 2.

It shows macro specimen (A), low-power field (B), and high-power field (C-F). The pathological findings showed a Zellballen pattern (C), pseudorosette formation (D), positivity for vascular invasion, lymph node metastasis and more than 50% of the cells were Ki-67 positive at a 200-fold high-power field. The tumor cells were chromogranin A- (E), tyrosine hydroxylase- (F) and dopamine β-hydroxylase-positive (G) and PNMT-negative (H), findings that were consistent with norepinephrine-producing paraganglioma.