Gangliocytic paraganglioma of the duodenum is an extremely rare disease. Few cases have been reported in the literature from 1957 to 2010. We reported a 67-year-old man with gangliocytic paraganglioma of the duodenum.
Aged ; Duodenal Neoplasms ; diagnosis ; Duodenum ; pathology ; Humans ; Male ; Paraganglioma ; diagnosis

Duodenal gangliocytic paraganglioma derived from neural crest is a peculiar neuroendocrine tumor. It is incidentally found during radiographic studies or due to gastrointestinal hemorrhage caused by frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology consisting of endocrine cells, ganglion cells and spindle-shaped Schwann cells. We experienced a duodenal gangliocytic paraganglioma in a 40-year-old woman presenting with episodes of melena. Esophagogastroduodenoscopy revealed a submucosal ulcerated tumor in the second portion of duodenum and a biopsy confirmed gangliocytic paraganglioma. The tumor was enucleated through a duodenotomy. It's size was 5.5 X 3 X 1.5 cm sized and revealed positive cellular reaction for chromogranin, synaptophysin, neuron-specific enolase, and neurofilament by immunohistochemistry. The majority of the reported duodenal gangliocytic paraganglioma were of benign nature. Therefore, radical surgery or a lymph node dissection could be avoided if that disease was confirmed. However, thorough investigation for lymph node metastasis and postoperative follow-up are needed. We report the first case of a duodenal gangliocytic paraganglioma in Korea with a review of literature.
Adult ; Duodenal Neoplasms/*diagnosis/pathology/surgery ; English Abstract ; Female ; Humans ; Paraganglioma/*diagnosis/pathology/surgery

BACKGROUND/AIMS: The incidence of duodenal tumors has increased by health surveillance. However, preoperative diagnosis of subepithelial duodenal tumors remains difficult because of the wide variety of pathologies and the location of the tumors. We analyzed endoscopic, radiological, and pathological features of subepithelial benign duodenal tumors (BDTs), which were treated by surgical resection. METHODS: Five patients with subepithelial BDTs treated by surgical resection were analyzed retrospectively. We compared the preoperative and postoperative diagnosis and evaluated the clinical presentations, endoscopic and radiological findings, surgical treatments, pathological results, and outcomes of these patients. RESULTS: All the patients underwent successful surgical resection. There were two cases of gastrointestinal stromal tumors (GISTs) treated with segmental duodenectomy, one case of carcinoid tumor treated with antrectomy, one case of gangliocytic paraganglioma treated with ampullectomy, and a lipoma removed by mass excision. The two GISTs were in the duodenal third and fourth segment close to the pancreas, and it was difficult to exclude pancreatic tumors by imaging studies. All the patients remained healthy for more than three years. CONCLUSIONS: Subepithelial BDTs are rare and difficult to diagnosis. Awareness and preoperative diagnosis of subepithelial BDTs can lead to minimally invasive treatment, including endoscopic or local surgical resection.
Carcinoid Tumor ; Diagnosis ; Duodenum ; Gastrointestinal Stromal Tumors ; Humans ; Incidence ; Lipoma ; Pancreas ; Paraganglioma ; Pathology ; Retrospective Studies

Adult ; Humans ; Male ; Paraganglioma ; diagnosis ; surgery ; Seminal Vesicles ; pathology ; surgery ; Tomography, X-Ray Computed

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.
Humans ; Male ; Middle Aged ; Pancreatic Neoplasms/*diagnosis/pathology ; Paraganglioma/*diagnosis/pathology ; *Tomography, X-Ray Computed ; *Ultrasonography, Doppler, Color

Extraadrenal paragangliomas involving the spine is less common and usually takes the form of intradural compression of the cauda equina. The authors report three cases of spinal paragangliomas resulting in extradural spinal cord compression and their MR findings. The MR imaging revealed a well-demarcated extradural mass with low to intermediate signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images compared to paravertebral muscles. After Gd-DTPA administration, heterogeneous and intense enhancement was found. Multiple punctate and serpiginous structures of signal void due to high-velocity flow were noted around and within the tumors on all sequences. In one case, the signal void structures were well corresponded with feeding arteries on angiography. These may be the characteristic findings of the extraadrenal paraganglioma involving the spine.
Adult ; Aged ; Female ; Human ; Magnetic Resonance Imaging ; Male ; Middle Age ; Paraganglioma, Extra-Adrenal/*diagnosis/pathology ; Spinal Neoplasms/*diagnosis/pathology

Paraganglioma in pregnancy is an extremely rare condition and its diagnosis is often delayed because the clinical symptoms can mimic those of preeclampsia or gestational hypertension. Here, we report the case of a 32-year-old, gravida 2, para 1 woman who presented with severe headache, palpitation, and sweating at 37 weeks' gestation. Although emergent cesarean section was performed on the assumption of severe preeclampsia, blood pressure fluctuated and heart rate remained tachycardiac. We suspected that she might have thromboembolic lesion in the chest or pheochromocytoma. Chest and abdominal computed tomography revealed a 4 cm mass in the left para-aortic space. Serum and urinary catecholamine levels were found to be significantly increased. She underwent laparoscopic mass removal and the pathology confirmed paraganglioma. When typical paroxysmal hypertension is accompanied by headache, palpitation, and sweating during pregnancy, adrenal tumors should be considered.
Adult ; Blood Pressure ; Cesarean Section ; Diagnosis ; Female ; Headache ; Heart Rate ; Humans ; Hypertension ; Hypertension, Pregnancy-Induced ; Paraganglioma ; Paraganglioma, Extra-Adrenal* ; Pathology ; Pheochromocytoma ; Pre-Eclampsia* ; Pregnancy ; Sweat ; Sweating ; Thorax

Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms: gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. About 100 cases have been reported worldwide. We report a case of Carney triad in a 42-year-old woman presented with a gastrointestinal stromal tumor in the stomach and a malignant functioning paraganglioma in the retroperitoneum that was fatal five years after diagnosis. The gastrointestinal stromal tumor was diagnosed as intermediate-risk of aggressive behavior and diffusely positive for c-kit whereas the retroperitoneal paraganglioma was negative for c-kit. Genetic analyses showed no mutations of KIT, PDGFRA, SDHB, SDHC, and SDHD genes in both tumors. To our best knowledge, this is the first case of Carney triad in Korea.
Adult ; Carney Complex/*chemically induced/complications ; Female ; Gastrointestinal Stromal Tumors/*diagnosis/pathology ; Humans ; Korea ; Paraganglioma/*diagnosis/pathology ; Retroperitoneal Neoplasms/*diagnosis/pathology ; Stomach Neoplasms/*diagnosis/pathology

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.
Aged ; Diagnosis, Differential* ; Female ; Gadolinium ; Glomus Jugulare Tumor ; Hearing Loss ; Humans ; Meningioma* ; Neurilemmoma ; Otolaryngology ; Paraganglioma ; Pathology ; Tail ; Temporal Bone ; Tinnitus

OBJECTIVE: Four cases of non-secreting paraganglioma of the cauda equina are present with an emphasis on magnetic resonance (MR) images correlated with pathological features. METHODS: From 1973 to 2001, 703 patients with spinal cord tumors had been treated with surgery at our hospital. Among them, four patients had been diagnosed as paragangliomas, all of which occurred in the cauda equina. We analyzed clinical data, including medical records, radiological, and histopathological findings for four patients. RESULTS: Some findings may help us to differentiate spinal paraganglioma from other spinal tumors. The MR images of the tumor were generally nonspecific. However, tumor margins were hypointense on T2-weighted images and serpiginous flow voids were noted in the tumor. Histopathologically paragangliomas were composed of an organoid or `zellballen' arrangement of polyhedral and argyrophilic cells, circumscribed by a richly vascular stroma. Immunohistochemical examination showed positive reaction to synaptophysin, chromogranin, vimentin, neuron specific enolase, and S100 protein. CONCLUSION: Although it is difficult to make a correct diagnosis as paraganglioma preoperatively for the intradural extramedullary tumors, especially in the cauda equina, paraganglioma should be included in the differential diagnoses.
Cauda Equina* ; Diagnosis ; Diagnosis, Differential ; Humans ; Magnetic Resonance Imaging ; Medical Records ; Organoids ; Paraganglioma* ; Pathology ; Phosphopyruvate Hydratase ; Spinal Cord Neoplasms ; Synaptophysin ; Vimentin