Witkiewicz Agnes, Galler Avi, Yeo Charles J, Gross Samuel D
Department of Pathology, Thomas Jefferson University, Philadelphia, PA, USA.
J Gastrointest Surg. 2007 Oct;11(10):1351-4. doi: 10.1007/s11605-007-0217-9. Epub 2007 Jul 25.
Gangliocytic paraganglioma is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. Only one case with distant metastasis has been reported. We present a case of duodenal gangliocytic paraganglioma treated first by local resection followed by pylorus-preserving pancreaticoduodenectomy. Examination of the first specimen revealed focal nuclear pleomorphism and mitotic activity, in addition to the presence of three characteristic histologic components: epithelioid, ganglion, and spindle cell. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor identified in the periampullary area, but metastatic gangliocytic paraganglioma was present in two of seven lymph nodes. This case report confirms the malignant potential of this tumor. We review the published literature on gangliocytic paragangliomas pursuing a malignant course. We conclude that surgical therapy of these neoplasms should not be limited to local resection, as disease recurrence, lymph node involvement, and rarely distant metastasis may occur.
神经节细胞性副神经节瘤是一种罕见肿瘤，几乎仅发生于十二指肠第二部。一般来说，该肿瘤临床过程呈良性，不过极少情况下可能复发或转移至区域淋巴结。仅报道过1例远处转移病例。我们报告1例十二指肠神经节细胞性副神经节瘤，最初行局部切除，随后行保留幽门的胰十二指肠切除术。对首个标本的检查显示，除存在上皮样、神经节和梭形细胞这三种特征性组织学成分外，还有局灶性核多形性和有丝分裂活性。在随后的胰十二指肠切除标本中，壶腹周围区域未发现残留肿瘤，但7个淋巴结中有2个存在转移性神经节细胞性副神经节瘤。本病例报告证实了该肿瘤的恶性潜能。我们回顾了已发表的关于呈恶性病程的神经节细胞性副神经节瘤的文献。我们得出结论，这些肿瘤的手术治疗不应局限于局部切除，因为可能会发生疾病复发、淋巴结受累，且极少会出现远处转移。
