Paraganglioma in sella.
We report a very rare case of a paraganglioma arising from sellar and suprasellar region which has been treated with radiotherapy following multiple surgeries.
Adult↗
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Nonfunctioning retroperitoneal paragangliomas.
Nonfunctioning paragangliomas of the retroperitoneum are extremely rare tumors. As of this report, 30 cases are known from published data. Characteristics of these tumors and diagnostic techniques are herein described. Approximately 20 percent of these lesions are considered malignant. Surgical resection is the treatment of choice once the diagnosis is made.
Adult↗
Mediastinal paraganglioma: a surgical experience.
Between October 1960 and January 1991, 5 male and 9 female patients (mean age, 44 +/- 19 years) were seen at the Mayo Clinic because of mediastinal paraganglioma. Nine patients presented with hypertension. In these and an additional patient, the tumors were biochemically active. In 13 patients, the mediastinal mass was localized preoperatively. All patients with functioning tumors received alpha-adrenergic and beta-adrenergic blockade preoperatively. Total resection was performed in 11 patients and partial resection in 2. In 1 patient, the tumor was found at postmortem examination. Five lesions were grossly invasive and nine were noninvasive. Blood pressure returned to normal in 6 of the 8 hypertensive patients whose tumors were totally removed. In 4, catecholamine excretion normalized as well. Tumor multicentricity occurred in 6 patients. In 7 patients, the disease took a malignant course (metastases in 5 and progression of unresectable lesions in 2). The probability of 10-year survival after resection was 50% in the group with malignant tumors, and there were no deaths in the group with benign lesions. Analysis of the DNA cellular content showed no correlation between the ploidy pattern and the behavior of the disease.
Adolescent↗
Paraganglioma of the interatrial septum.
The case of a patient undergoing successful resection of an interatrial septal paraganglioma is presented. The diagnosis of an interatrial mass was established preoperatively by echocardiography, ultrafast cine computed tomographic scan, and cardiac catheterization. The tumor was excised in total, and the interatrial septum and the roof of the left atrium were reconstructed using a bovine pericardial patch.
Animals↗
Gastric leiomyoblastoma associated with extraadrenal paraganglioma and pulmonary chondroma: a new case of Carney's triad.
Carney's triad is extremely rare. The authors report on a 12-year-old patient who had gastric epithelioid leiomyosarcoma, multiple pulmonary chondroma, and mediastinal paraganglioma. Thirty-five other cases are reviewed and analyzed.
Child↗
Paragangliomas of the spinal canal.
Paragangliomas are tumors arising in the paraganglia and rarely occur in the spinal canal. In the literature, 11 such cases have been reported. We present 7 additional cases, 3 of which were epidural in location in contrast to previously described intradural cases. The problems of histological diagnosis of these tumors are discussed. The value of determination of biogenic amines in the tumor tissue or the urine has been pointed out.
Adult↗
Paraganglioma of the cauda equina. A histologic, immunohistochemical, and ultrastructural study and review of the literature.
We report a case of paraganglioma of the cauda equina and filum terminale. Diagnosis was established by the histologic organoid pattern, immunohistochemical staining for neuron-specific enolase and ultrastructural dense-core neurosecretory granules. A survey of the literature yielded 16 previously reported cases, five of which were described in 1983. This suggests that heightened awareness of this entity will result in a corresponding increase in the reported incidence.
Cauda Equina↗
Paraganglioma of the cauda equina with associated syringomyelia: case report.
We present an association not previously described in which the rare entity of paraganglioma of the cauda equina was found in association with syringomyelia of the cervico-thoracic cord. The patient, a 50-year-old woman presented with a long history of lower back pain with recent onset of sciatic pain and altered sensation in the right leg. Surgical resection of the tumor with intraoperative syringostomy of the syrinx has led to eradication of all symptoms and at 5 months shows no evidence of residual pathology.
Cauda Equina↗
Functioning malignant nonchromaffin paraganglioma of retroperitoneum with metastases.
A case is reported of a functioning, malignant nonchromaffin paraganglioma (chemodectoma) of the retroperitoneum with metastases in a seventeen-year-old boy. The literature is reviewed and the treatment modalities are discussed.
Adolescent↗
Paraganglioma of urinary bladder in patient with neurofibromatosis.
Pheochromocytoma in patients with von Recklinghausen's neurofibromatosis is a well-known association. However, extra-adrenal pheochromocytoma with this association is rare. Herein we report a case of urinary bladder paraganglioma in a patient with neurofibromatosis.
Aged↗
Cervical paragangliomas: a report of four cases.
Four cases of cervical paragangliomas are reported. Two cases were of presumed vagal origin and two were carotid body tumours. In three of the cases the diagnosis was not made prior to surgery. The diagnosis, pathology and management of the cases are discussed.
Adult↗
Paraganglioma of the cauda equina. Report of 2 cases and review of 59 cases from the literature.
Paragangliomas of the cauda equina are not so rare as said in the literature. Two additional cases are presented with a global analysis of the 59 cases from the literature. The diagnosis of this pathology greatly benefit of the use of immunostainings as the cells are often neuron-specific enolase, neurofilament protein and somatostatin positive so that electron microscopy is thus no longer mandatory for establishing the diagnosis. In addition, we report the first magnetic resonance images of this tumor at this location.
Adult↗
Paraganglioma of the cauda equina: a case report and review of the literature.
This case report describes a paraganglioma of the cauda equina in a 57-year-old man. The clinical and pathological features of the 70 cases reported to date are reviewed. The importance of total surgical removal is stressed.
Cauda Equina↗
Paraganglioma metastatic to the cervical spine.
Paragangliomas are usually benign tumors which may be locally invasive. Only 10% of these tumors are malignant. When metastases occur the most common sites are lung, liver and bone. When bony metastases occur the spine is a common site. In the present case cervical spine metastases with resultant bony encroachment and epidural extension of tumor with cord compression was diagnosed with myelography and CT.
Cervical Vertebrae↗
Intracaval paraganglioma causing superior vena cava syndrome.
We report a 46-year old female patient with progressive symptoms and signs of superior caval syndrome. At surgery, the caval vein with a benign intravascular paraganglioma was removed, and a venous interposition using a spiral vein graft was performed.
Female↗
Paraganglioma of the sphenoid sinus: case report and review of literature.
We report a case of a 41-year-old woman who presented with occasional hemoptysis and hoarseness. Imaging showed a mass primarily in the sphenoid sinus but also extending into the right posterior ethmoid sinus and nasopharynx. Histology showed this mass to be a paraganglioma. We describe its computed tomography and magnetic resonance imaging findings, and we discuss the differential diagnosis and treatment.
Adult↗
Emergency catheter probe endoscopic sonography (CP-EUS): an effective procedure in the assessment of a bleeding gangliocytic paraganglioma.
Catheter probe endoscopic sonography is a relatively rapid and safe procedure, carried out during standard endoscopy, capable of distinguishing solid from cystic lesions and vascular from avascular masses. Herein we discuss the role of catheter probe endoscopic sonography in the emergency assessment of a patient with recent and severe bleeding from an ulcerated polyp, arising from the papilla. During the endoscopy, catheter probe endoscopic sonography showed the solid and submucosal nature of the lesion, suggesting its localised and benign nature and, most importantly, demonstrating the high risk of rebleeding from vascular structures communicating with the ulcer. Because of this finding three metallic endoclips were positioned. The lesion was not removed endoscopically because of its difficult position and the high risk of haemorrhage. The mass, removed through a duodenotomy, was shown to be a gangliocytic paraganglioma, an uncommon tumour, frequently resulting in surgery because of abdominal pain and gastrointestinal bleeding.
Adult↗
Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus.
Gangliocytic paraganglioma (GP) is a rare, typically benign tumor that shows neuroectodermal (neurosustentacular or Schwannian and neuronal) and neuroendocrine differentiation. Once thought to arise exclusively in the periampullary region as a solitary lesion, recent reports have documented both origin of GP in a variety of extra-duodenal sites as well as synchronous multifocal presentation of the tumor. Herein, we describe the first reported case of simultaneous occurrence of GP in the superior mediastinum and esophagus. A mass in the mid-distal esophagus and a separate mass in the superior mediastinum at the thoracic inlet were found in a 58-year-old woman by computed tomography scan. Subsequent biopsy of the superior mediastinal mass showed nests of epithelioid tumor cells coexisting with ganglioneuromatous elements, whereas biopsy of the esophageal mass showed nests of epithelioid cells with interspersed ganglion cells. The epithelioid tumor cells showed diffuse immunohistochemical expression of keratin (CAM 5.2), chromogranin, and synaptophysin supporting true neuroendocrine differentiation; ganglion cells expressed S-100 protein and neurofilament protein; and the spindled elements expressed S-100 protein, neurofilament protein, and glial fibrillary acidic protein indicating Schwannian differentiation. The finding of another GP occurring outside the periampullary region bolsters the argument for a stem cell origin of this unusual tumor.
Esophageal Neoplasms↗