Primary pancreatic paraganglioma harboring lymph node metastasis: A case report

Abstract

BACKGROUND Primary pancreatic paragangliomas are extremely rare tumors. Limited by the diagnostic efficacy of histopathological examination, their malignant behavior is thought to be associated with local invasion or metastasis, with only four malignant cases reported in the literature to date. As pancreatic paragangliomas share similar imaging features with other types of pancreatic neuroendocrine neoplasms, they are difficult to diagnose accurately without the support of pathological evidence. As primary pancreatic paragangliomas are rare, especially those accompanied by lymph node metastasis, there is currently no consensus on treatment. Herein, we report a case of primary pancreatic paraganglioma with lymph node metastasis. CASE SUMMARY A mass located in the pancreatic body was incidentally discovered on computed tomography in a 41-year-old Tibetan man. Distal pancreatectomy was subsequently performed and a 4.1 cm × 4.2 cm tumor was found embedded in the body of the pancreas during surgery. Histological examination confirmed the characteristics of paraganglioma in which the neoplastic chief cells were arranged in a classic Zellballen pattern under hematoxylin-eosin staining. Further, immunohistochemistry demonstrated that the sustentacular cells in the tumor tissue were positive for S-100 protein, and neoplastic cells and pancreatic draining lymph nodes were positive for chromogranin A and synaptophysin; thus, the presence of lymph node metastasis (two of the eight resected pancreatic draining lymph nodes) was also confirmed. A diagnosis of primary pancreatic paraganglioma with lymph node metastasis was finally established. The patient remained disease-free for 1 year after the surgery. CONCLUSION A definite diagnosis of pancreatic paraganglioma mainly depends on postoperative histopathological and immunohistochemical examinations. Surgical resection may be the first treatment of choice for patients with primary pancreatic paraganglioma that has metastasized to the lymph nodes.

Pancreatic paraganglioma with multiple lymph node metastases found by spectral computed tomography: A case report and review of the literature

“…This tumor usually occurs in the head, neck, and retroperitoneum, and paragangliomas in the pancreas are rare[ 4 ]. Malignancy is even rarer, with only four cases reported to date (Table 1 )[ 5 - 8 ], including three patients who showed lymph node metastases and one patient with multiple liver metastases. Currently, the presence of invasion into vascular and peripheral structures or metastasis is a reliable basis for diagnosing malignant paraganglioma[ 9 ].…”

Primary non-functional pancreatic paraganglioma: A case report and review of the literature

“…Six cases were diagnosed as PGL following examination of fineneedle aspiration (FNA) or frozen section (FS) 20,27,48 Of the 53 cases, eight had been diagnosed as pancreatic PGL before surgery, 13,20,24,27,47,48 twenty-five had been diagnosed as pancreatic neuroendocrine neoplasm (pNEN), 4,10-12,15,20,23,29-38, 40,41,44-46 and five had been diagnosed as other diseases (i.e., pancreatic cystadenoma, 9 insulinoma, 14 pancreatic cancer, 16 pancreatic pseudocyst, 20 and gastrointestinal stromal tumour (GIST). 42 Forty-seven cases underwent surgery (15 tumour local resection (TLR), 9,11,13,15,20,21,25,27,28,38-40, 42,47 one case of TLR þ splenectomy, 30 10 cases of pancreaticoduodenectomy (PD), 10,19,20,22,23,29,31,45,46 one case of PD and hepatectomy, 24 three cases of pylorus preserving pancreaticoduodenectomy (PPPD), 14,26,34 one case of PPPD þ distal pancreatectomy (DP) þ left adrenalectomy (L-ADX), 37 seven cases of DP, 4,20,41,43 one case of DP þ L-ADX, 16 one case of DP þ splenectomy, 33 three cases of resection of the pancreas head (RPH), 12,18,44 three cases of central pancreatectomy (CP) 32,35,…”

“…Tumour markers did not show any significant abnormalities (data not shown). Six cases were diagnosed as PGL following examination of fine-needle aspiration (FNA) or frozen section (FS) 20,27,48 Of the 53 cases, eight had been diagnosed as pancreatic PGL before surgery, 13,20,24,27,47,48 twenty-five had been diagnosed as pancreatic neuroendocrine neoplasm (pNEN), 4,10–12,15,20,23,29–38,40,41,44–46 and five had been diagnosed as other diseases (i.e., pancreatic cystadenoma, 9 insulinoma, 14 pancreatic cancer, 16 pancreatic pseudocyst, 20 and gastrointestinal stromal tumour (GIST). 42 Forty-seven cases underwent surgery (15 tumour local resection (TLR), 9,11,13,15,20,21,25,27,28,38–40,42,47 one case of TLR + splenectomy, 30 10 cases of pancreaticoduodenectomy (PD), 10,19,20,22,23,29,3...…”

“…3 The incidence of PGLs is low and estimated to occur in approximately 2-8 cases/ million people, and the tumours can arise in sympathetic and parasympathetic nervous systems. 4 The parasympathetic PGLs are often located in the head and neck, and are mostly non-functional. 3,4 The sympathetic PGLs are often located in the abdomen, followed by the chest and pelvis.…”

“… 4 The parasympathetic PGLs are often located in the head and neck, and are mostly non-functional. 3 , 4 The sympathetic PGLs are often located in the abdomen, followed by the chest and pelvis. 5 Abdominal PGLs can produce, store, and secrete catecholamines; and they can produce typical signs and symptoms such as hypertension, palpitations, dizziness, anxiety, blushing, headaches, and sweating.…”