Pancreatic Paraganglioma Research Articles

S2700 Diagnostic Challenges in Differentiating Pancreatic Paragangliomas From Well Differentiated Neuroendocrine Tumors

S2700 Diagnostic Challenges in Differentiating Pancreatic Paragangliomas From Well Differentiated Neuroendocrine Tumors

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

Primary pancreatic paragangliomas are rare. They are mainly non-functional tumours that lack typical clinical manifestations. Definite diagnosis relies on histopathology and immunohistochemistry, and the main treatment is surgery. We report here a case of primary, non-functional, pancreatic paraganglioma in a 49-year-old woman. The tumour was approximately 5.0 × 3.2 ×4.7 cm in size and located in the pancreatic neck and body. We undertook 3D laparoscopic complete resection of the tumour. The patient developed a pancreatic fistula (biochemical leak) post-surgery, but she recovered and was discharged from hospital 11 days after surgery. We describe this case study and briefly summarize previous related reports.

Castleman disease of the pancreas mimicking pancreatic malignancy on 68Ga-DOTATATE and 18F-fluorodeoxyglucose positron emission tomography/computed tomography: A case report

BACKGROUNDCastleman disease is an uncommon nonclonal lymphoproliferative disorder, which frequently mimics both benign and malignant abnormalities in several regions. Depending on the number of lymph nodes or regions involved, Castleman disease (CD) varies in diagnosis, treatment and prognosis. It rarely occurs in the pancreas alone without any distinct clinical feature and tends to be confused with pancreatic paraganglioma (PGL), neuroendocrine tumors (NETs), and primary tumors, thus impeding proper diagnosis and treatment.CASE SUMMARYA 28-year-old woman presented with a lesion on the neck of the pancreas, detected by ultrasound during a health examination. Physical examination and laboratory findings were normal. The mass showed hypervascularity on enhanced computed tomography (CT), significantly increased 18F-fluorodeoxyglucose uptake on positron emission tomography (PET)/CT, and slightly increased somatostatin receptor (SSTR) expression on 68Ga-DOTATATE PET/CT, suggesting no distant metastases and subdiagnoses such as pancreatic PGL, NET, or primary tumor. Intraoperative pathology suggested lymphatic hyperplasia, and only simple tumor resection was performed. The patient was diagnosed with the hyaline vascular variant of CD, which was confirmed by postoperative immunohistochemistry. The patient was discharged successfully, and no recurrence was observed on regular review.CONCLUSIONHigh glucose uptake and slightly elevated SSTR expression are potentially new diagnostic features of CD of the pancreas.

Pancreatic Paraganglioma: a Case Report and Literature Review

Pancreatic Paraganglioma: a Case Report and Literature Review

Primary pancreatic paraganglioma; case report and literature review

Background: Paragangliomas represent a rare tumor comprised of neuroendocrine cells with only 21 reported cases of primary pancreatic paraganlioma found in the English language literature. We present a case of primary pancreatic paraganglioma and a literature review. Methods: Our patient is a 74 year old lady with a 2.5 x 2.2 x 1.9 cm pancreatic head mass found on computed tomography during an evaluation for frequent diarrhea. She underwent an endoscopic ultrasound with fine needle aspiration which indicated a low grade neuroendocrine tumor. Chromogranin A was 80. The day prior to her scheduled surgery, she suffered a transient ischemic attack with no residual symptoms. Surgery was delayed three months. We were then able to perform an enucleation of the tumor with immunohistochemical examination showing the tumor cells positive for synaptophysin and negative for cytokeratin AE 1/3 immunostains, confirming the diagnosis of pancreratic paraganglioma. Results: Twenty one cases found representing 16 females and 5 males, ranging in age from 19 to 74 with a median age of 55 years. Only two out of 19 cases were found to be malignant and all but one patient was treated with surgery. One patient was found to have a functional tumor and became unstable during surgery. He was subsequently treated with chemotherapy. Conclusion: Primary pancreatic paragangliomas are an extremely rare pathology, with the diagnosis confirmed with histopathological and immunohistochemical examination. Primary treatment is surgical excision. We conclude that enucleation rather than radical excision should be attempted when feasible.

Pancreatic paraganglioma: A report of case and literature review

Pancreatic paraganglioma: A report of case and literature review

Primary pancreatic paraganglioma: An extremely rare entity: Report of case and literature review

Primary pancreatic paraganglioma: An extremely rare entity: Report of case and literature review

A rare presentation of clinically intractable hypertension: Pancreatic paraganglioma

Summary Paraganglioma is a rare extra-adrenal pheochromocytoma which originates from chromaffin cells within the ganglia of the sympathetic trunk and of the celiac, renal, suprarenal, and hypogastric plexuses. Pancreatic paragangliomas are rarer still. And even then, paragangliomas are mostly reported to be nonfunctional. We report a case of a 64-year-old woman with underlying disease of hypertension who presented with biliary colic. Contrast-enhanced computer tomography showed an enhancing mass in the uncinate process of the pancreas. Pylorus-sparing Whipple procedure was performed for complete tumor excision. Hypertensive crisis developed after Whipple, which improved after continuous intravenous nicardipine infusion. Pathology revealed a paraganglioma. A 24-h catecholamine urine test showed increased norepinephrine and vanillylmandelic acid level. Functional paraganglioma was diagnosed.

English

INTRODUCTION: Paragangliomas, are rare neuroendocrine neoplasms that are derived from the paraganglia. Extra-adrenal paraganglioma arising within the pancreas is an extremely rare entity. CASE REPORT: Here we present a case of 72 year male patient with the complaints of abdominal distension and ultrasound showing a large multicystic mass measuring 12 x 8.5 cm extending from the tail of the pancreas to the pelvis. DISCUSSION: The diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immune

Primary pancreatic paraganglioma: A report of two cases and literature review

Paraganglioma is a rare tumor of paraganglia, derived from neural crest cells in sympathetic or parasympathetic ganglions. Primary paraganglioma originating from the pancreas is rare. We report two patients with paraganglioma in the head of the pancreas, in whom computed tomography showed a sharply marginated, hypervascular tumor with cystic areas. Significant intratumoral vessels and early contrast filling of the draining veins from the mass were not found. Although the pancreatic paraganglioma was located at the pancreatic head, the bile ducts often revealed no dilation, and sometimes the main pancreatic duct was mildly dilated. These findings are helpful in differentiating pancreatic paraganglioma from other pancreatic neoplasms. It is often difficult to distinguish between nonfunctional pancreatic paragangliomas and pancreatic endocrine tumors. In many reports,pancreatic paragangliomas show the retroperitoneal extension of a paraganglioma into the pancreas rather than a true pancreatic neoplasm. In surgical treatment, we could select simple excision of the tumor rather than radical surgery.

Malignant extra-adrenal pancreatic paraganglioma: case report and literature review

BackgroundPancreatic paragangliomas are rare tumors, with only 16 reported cases to date. One of these cases demonstrates metastasis to lymph node, while another case was functional, however, none of these cases showed malignant and large, pancreatic paraganglioma with marked invasion. Also another unique feature was the age of our patient compared to the average reported ages in published literature (42–85 years).Case presentationA 19-year-old woman presented with a one-year history of intermittent abdominal pain. Physical examination showed a palpable mass in the right upper abdomen, but initial laboratory results were within normal ranges; tumor markers (CEA, AFP, and CA19-9) were negative. An abdominal and pelvic computed tomography (CT) scan showed a well-defined retroperitoneal para-aortic mass. The CT scan revealed that the surrounding lymph nodes were not enlarged, but the liver showed evidence of parenchymal infiltration. Intraoperatively, a large, firm tumor originating from the head of pancreas was found pushing on the caudate hepatic lobe and the inferior vena cava (IVC). The tumor was resected through a pancreaticoduodenectomy, involving segment VI of the liver and a small segment of the IVC. The blood pressure spiked (>220 mm Hg) when the tumor was manipulated during the operation. The final pathology report showed a 9-cm tumor with lymphovascular invasions; immunohistochemistry was positive for synaptophysin and chromogranin. All resection margins were negative and 1/15 lymph nodes was positive for metastasis. Post-operative recovery was unremarkable. One month after discharge, the patient was re-admitted with abdominal pain and found to have an abdominal collection at the resection site, which was drained under CT guidance. She received a therapeutic dose of I131-metaiodobenzylguanidine (MIBG). Follow-ups showed the absence of recurrence, and she has remained disease free.ConclusionThis patient was an extraordinary example of a rare tumor. Even more remarkable was that the tumor was malignant with lymph node invasion. To our knowledge, a case similar to that presented here has not been previously reported in the literature.

Pancreatic Paraganglioma in MEN Syndrome

Context Paragangliomas are rare neuroendocrine neoplasms arising from paraganglia of sympathetic trunk. Pancreatic localization is extremely rare (only 15 cases reported worldwide). Pancreatic paraganglioma has never been described before in the context of multiple endocrine neoplasia-1 (MEN 1) syndrome. Case report A 40-year-old male, known for previous follicular adenoma and primitive hyperparathyroidism, was admitted to our hospital with high blood pressure, flushing of the face, sweating and dysaesthesia of the lower limbs. Abdominal imaging showed multifocal pancreatic masses. He underwent total pancreatectomy with histological diagnosis of multifocal paraganglioma with a proliferative index (Ki67) of 10%. Further clinical evaluation evidenced pituitary microprolactinoma and adrenal hyper­plasia. Even in the absence of genetic mutation, a diagnosis of MEN 1 syndrome was clinically established. During follow-up he developed liver metastatic disease that was treated with targeted biological therapy and multiple lines of chemotherapy. Conclusion A clinical presentation of MEN syndrome in absence of definable gene mutation is possible and becoming a clinical challenge. We discuss here a pancreatic paraganglioma presenting with rapid growth and prone to metastasis dissemination in a clinically evident MEN 1 syndrome. Further studies are needed to understand genetic causes and biology of this rare type of neuroendocrine tumor.

Epidemiological study of a von Hippel-Lindau family in northwest China

von Hippel-Lindau (VHL) disease is a rare, inherited neoplastic disease characterized by hemangioblastomas (HBL) of the central nervous system (CNS), retinal angiomas, renal cell carcinomas (RCC), pancreatic endocrine tumors (PETs), pheochromocytomas, paragangliomas, and visceral cysts. We encountered a large VHL family in northwest China and conducted a systematic screening of the family members based on their epidemiological and clinical characteristics. A self-designed questionnaire was used to collect the general sociodemographic and health information of the family members. For the preliminary family screening, physical examination and abdomen B ultrasonography were performed. The suspected patients were subjected to cranial computerized tomography and fundus examination. The clinical data of the patients with confirmed VHL disease were collected from hospital records. A total of 63 lineal descendants in six generations were observed in the family (generations O, A, B, C, D, E), including 9 dead suspected cases (6 males, 3 females) and 10 living cases (2 males, 8 females). Among the 10 living cases, 4, 2, 1, 3, 4, 8, and 2 manifested HBLs of the CNS, PETs, RCC, pancreatic cysts, renal cysts, pheochromocytomas (4 hemi and 4 bilateral), and paragangliomas, respectively. Data showed that the morbidity of VHL disease in generation C was lower than that in generation B, but the age of onset was younger. This study is the first to report VHL disease in northwest China and VHL-associated PET cases in Chinese. Therefore, follow-up checkups of the family should be focused on younger generations. Proper family screening protocols should be followed for the treatment of patients with VHL disease.

Pancreatic paraganglioma: An extremely rare entity and crucial role of immunohistochemistry for diagnosis

Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry.

Pancreatic paraganglioma: A case report of CT manifestations and literature review.

Paragangliomas are rare tumors arising from paraganglia (or chromaffin bodies) within the ganglia of the sympathetic trunk and the ganglia of the celiac, renal, suprarenal, aortic and hypogastric plexuses. These tumors can be widely distributed from skull base to the bottom of pelvis. Paraganglioma originating from pancreas is extremely rare. Here we report a 40-year-old female patient with paraganglioma located in the pancreatic head as a solid, well-demarcated mass with substantial enhancement on contrast-enhanced CT examination. Relevant literatures are also reviewed.

Patient with synchronous low grade leiomyosarcoma of the thigh, primary pancreatic neuroendocrine tumor, and lung metastases: Why biopsy of metastases should be the standard.

A group of well-defined adult neuroendocrine tumors (NETs) have variable but most often indolent biologic behavior and characteristic well-differentiated histologic features (1). The majority arise in the gastrointestinal (GI) tract (although carcinoid tumors may also arise in the lung and ovary), and collectively, they are referred to as gastroenteropancreatic NETs. They include carcinoid tumors, pancreatic islet cell tumors (gastrinoma, insulinoma, glucagonoma, VIPoma, somatostatinoma), paragangliomas, pheochromocytomas, and medullary thyroid carcinomas. Neuroendocrine tumors comprise only 0.5% of all malignancies. The incidence is approximately 2/100,000. The main primary sites are the gastrointestinal tract (62-67%) and the lung (22-27%), and 12-22% present with metastatic disease. The 5-year survival is mainly associated with stage: 93% in local disease, 74% in regional disease and 19% in metastatic disease (2). Treatment of localized disease is surgical resection if possible. In metastatic or advanced disease, locoregional treatments, as well as radionuclide therapies, may be considered. Additionally, in selected cases resection of the primary and metastatic tumors may impact outcome favorably. Although it has no significant effect on tumor growth, biotherapy with somatostatin analogs and/or interferon-α is recommended for either well-differentiated or functioning tumors for symptomatic relief. On the other hand, chemotherapy may be effective in the treatment of those tumors characterized by a poor differentiation grade and a high proliferation rate (3). Soft tissue sarcomas include a large variety of malignant neoplasms that arise in the extraskeletal mesenchymal tissues of the body. Approximately 10,390 cases are diagnosed annually in the United States, representing only 0.72 percent of all new cancers (4). Roughly 80 percent of sarcomas originate from in soft tissue, the remainder from bone (4). The histopathologic spectrum of sarcomas is broad, presumably because the embryonic mesenchymal cells from which they arise have the capacity to mature into striated skeletal and smooth muscle, adipose and fibrous tissue, bone, and cartilage. Low grade sarcomas are capable of aggressive local growth but tend not to disseminate. Overall survival of patients with sarcoma has been shown to correlate with grade in multiple studies (5),(6). The sensitivity of PET scanning for primary sarcomas ranges from 74 to 100 percent (7),(8) and is greater for high and intermediate grade sarcomas (7) than it is for low grade sarcomas (7),(9). In one report, 50 percent of low-grade sarcomas did not take up more FDG than adjacent muscle (10).

A Case of Primary Paraganglioma that Arose in the Pancreas: the Color Doppler Ultrasonography and Dynamic CT Features

Paragangliomas rarely originate from the pancreas and they are characterized on imaging studies as well-marginated, hypervascular masses with cystic areas. We herein report on a case report of pancreatic paraganglioma in a 57-year-old woman, which was confirmed on pathology. Color Doppler ultrasonography and dynamic CT demonstrated a well-demarcated, extremely hypervascular mass with prominent intratumoral vessels and early contrast filling of the draining veins from the mass. Endoscopic retrograde cholangiopancreatography showed that the main pancreatic duct was displaced and mildly dilated.