Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course

Abstract

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".

Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

“…At least 20 other GPs metastatic to local LNs and a single fatal case have also been reported in the literature [31,32]. Thus, a limited, low-grade malignant potential should be attributed to such tumors, despite the usually excellent outcome of most reported cases, including 11 of our 12 cases (2 of which were locally metastatic, however).…”

Endoscopic resection of a periampullary gangliocytic paraganglioma of the duodenum: A case report

“…No local recurrence or distant metastasis were detected in 12 cases of mainly endoscopically removed duodenal GP. In contrast Li et al published a case report of a 47-year-old male patient suffering from a GP, which was removed via pancreaticoduodenectomy [3]. Four months after the removal computer tomography imaging revealed distant metastasis in the liver and pelvic cavity.…”

“…Its exact origin still remains unclear. The GP is typically characterized by consisting of three tumour components: the epithelioid, the spindle-shaped and the ganglion-like cells [7], [3]. The GP is described as being a tumour with benign clinical behaviour.…”

“…The tumour was first described by Dahl et al in 1957 [1]. Generally the GP is characterized by consisting of three tumour components: spindle-shaped cells with Schwannian differentiation, ganglion-like cells and neuroendocrine cells [2], [3]. These cells can be immunoreactive to pancreatin, synaptophysin, chromogranin and are able to express S-100, NSE, pancreatic polypeptide, somatostatin and vasoactive intestinal peptide [2].…”

Duodenal Rare Neuroendocrine Tumor: Clinicopathological Characteristics of Patients with Gangliocytic Paraganglioma

“…These studies stated that this rare tumor typically occurs in the second part of the duodenum, includes 3 characteristic components (epithelioid, spindle-shaped, and ganglion-like cells), and shows a good prognosis. On the other hand, few cases with lymph node and/or liver metastasis [11–13] and 1 fatal duodenal GP case after distant metastasis (pelvic lesion and liver mass) [11] have been reported. As most previous studies included a single patient or a small group of patients, we previously evaluated the details of this tumor in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) style [14] as much as possible and elucidated some aspects of the epidemiology and clinicopathological characteristics of GP.…”