Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course.
Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of "1", rather than a benign tumor of "0".
Bin Li, Yang Li, Zhi Li, Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China.
Bin
B
Li
Bin Li, Yang Li, Zhi Li, Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China.
Yang
Y
Li
Bin Li, Yang Li, Zhi Li, Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China.
Xiao-Ying
XY
Tian
Bin Li, Yang Li, Zhi Li, Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China.
Bo-Ning
BN
Luo
Bin Li, Yang Li, Zhi Li, Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China.
Zhi
Z
Li
D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D059186:Chemoradiotherapy, Adjuvant; D004379:Duodenal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D016577:Pancreaticoduodenectomy; D010235:Paraganglioma / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D010386:Pelvic Neoplasms / Q000737:chemistry / Q000556:secondary*; D012086:Reoperation; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D014408:Biomarkers, Tumor
Duodenal neoplasms; Gangliocytic neoplasms; Lymphatic metastasis; Paraganglioma; Treatment outcome