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Dopamine-producing paraganglioma not visualized by iodine-123-MIBG scintigraphy.

A patient with an aorticopulmonary paraganglioma was found to have normal plasma norepinephrine and epinephrine levels and elevated dopamine levels. Iodine-123-MIBG scintigraphy did not visualize this tumor. The same finding on a negative MIBG scan in two patients with exclusively dopamine-secreting chemodectomas has been previously reported. In our patient, [111In-DTPA-D-Phe1]-octreotide scintigraphy proved to be an effective imaging technique.

3-Iodobenzylguanidine↗

[Retroperitoneal paragangliomas. Considerations on a internephro-caval case].

A case of paraganglioma situated between right renal vein, right renal artery and inferior vena cava is here with described. The report constitutes the rise to frame with precision the tumor according with WHO classification. Moreover criteria are to be discussed for nature and seat diagnosis, as well for the choice of the best way of access.

Female↗

Magnetic resonance imaging of paraganglioma of the cauda equina.

The authors describe atypical magnetic resonance imaging (MRI) findings for pathologically proven paraganglioma of the filum terminale in a 27-year-old man. MRI showed an intradural tumour, which enhanced after administration of gadolinium and which showed evidence of cystic areas. Hemosiderin rings and vessels were not observed.

Adult↗

[Retroperitoneal paraganglioma. Infrequent pathology].

Presentation of 3 cases of retroperitoneal paraganglioma non pre-operatively diagnosed: one dependent of the Zuckerkandl's organ with ultrasound examination showing a panelled cystic mass, and two located in the renal parahilar area, all accidental findings during renal surgery. Lack of appropriate pre-operative background and diagnosis produced a hypertensive crisis in one patient during removal manoeuvres which was entirely controlled. After a 4-, 2- and 1-year follow-up, respectively, patients remain asymptomatic and disease-free. An analysis of the most relevant features in these retroperitoneal tumorations was carried out from a clinical, diagnostic, therapeutic and prognostic point of view.

Adult↗

Malignant non-functioning retroperitoneal paragangliomas: a study of five cases by fine needle aspiration cytology, histopathology and autopsy.

The cytological findings, the histopathological analysis of tissues obtained by surgical exploration and autopsy of 5 cases of malignant non-functioning retroperitoneal paragangliomas are presented due to their extreme rarity in literature. Fine needle aspiration cytology of these rare retroperitoneal tumours is discussed in this study. Presence of hypertension rather than the chromaffinity denotes the functional status of these tumours. The malignant nature of these tumours is determined by metastasis rather than the histological pattern. Pain and intra-abdominal masses are the common presenting symptoms. Surgery is the only mode of treatment.

Adult↗

Flow cytometric DNA analysis for the determination of malignant potential in adrenal and extra-adrenal pheochromocytomas or paragangliomas.

DNA ploidy studies were performed by flow cytometry on extracted nuclei from 53 adrenal, 13 carotid body, 14 retroperitoneal, two intrathoracic, two urinary bladder, and one cauda equina pheochromocytomas (paragangliomas). A specific technique was used for preparation of paraffin-embedded tissue into single associated nuclei, and another specific method was used for propidium iodide staining. Twenty normal adrenal glands were also analyzed as controls. Six tumors in the adrenal medulla yielded uninterpretable histograms (coefficient of variation > 7%) because of excessive cell debris or other technical failure. All control adrenal glands were diploid. Seventy-two tumors were clinically benign and seven were malignant as evidenced by regional or distant metastases and/or extensive local invasion. Fifty-six (71%) of 79 tumors disclosed a normal DNA diploidy pattern with a benign clinical course. Abnormal DNA histograms including tetraploidy and aneuploidy patterns were observed in the remaining 23 cases, including the seven malignant tumors. Significantly more malignant tumors occurred in the DNA tetraploid group and the DNA aneuploid group than in the normal DNA group; considered together, the two groups with abnormal DNA histograms differed significantly from the normal DNA group. On the basis of these results, nondiploid tumors are considered to be more prone to aggressive behavior than diploid tumors and, therefore, should be carefully monitored.

Adolescent↗

[Morphology and clinical aspects of paragangliomas in the area of the head-neck].

Clinical data and tumor specimens from 63 patients were investigated, and included 22 glomus caroticum tumors and 41 tumors of the tympano-jugulare region. All specimens could be differentiated by their histological growth patterns into four categories. The clinical data revealed that 45% of the female patients with paragangliomas suffered from goitre, one of whom died in thyrotoxic crisis after surgery. Two patients died from sequelae of other serious diseases. In general, postoperative morbidity was solely caused by cranial nerve palsies depending on tumor extent. From the data presented, a conclusion is drawn that serious concurrent diseases are the limiting factor for surgery. Early detection is the best way to reduce postoperative morbidity if total removal of the tumor is required.

Adult↗

Neuroendocrine tumor (paraganglioma) of the cauda equina: MR and pathologic findings.

The MR and pathologic features of a case with neuroendocrine tumor (paraganglioma) of the cauda equina are presented. MR showed the tumor to be hyperintense on postcontrast examination and also showed serpiginous flow voids suggesting vessels capping the tumor. A neuroendocrine tumor should be considered in the differential diagnosis of tumors in this location.

Adult↗

Malignant retroperitoneal paraganglioma with intranuclear vacuoles in a fine needle aspirate. A case report.

The cytologic findings in fine needle aspiration smears and cell blocks from a malignant retroperitoneal paraganglioma with subsequent histologic confirmation are described. The aspirate contained clusters of cells with ill-defined borders, often varied in size, round or oval nuclei and marked anisokaryosis. An unusual feature was the presence of prominent intranuclear vacuoles in some cells. The histologic and ultrastructural findings, as well as the results of histochemical and immunodiagnostic staining, are also presented.

Adenocarcinoma↗

Peliosis of the spleen associated with a paraganglioma. Case report.

We report a case of splenic peliosis imitating metastases on CT. The patient was a 44-year-old man who previously had a retroperitoneal paraganglioma surgically removed. Peliosis must be considered a potential differential diagnosis of hypodense foci of the spleen seen on CT.

Adult↗

[Duodenal gangliocytic paraganglioma].

We report a case of duodenal Gangliocytic Paranglioma in a 73 year old man, who presented with a history of melena. An upper gastrointestinal barium study showed a polyp located in the second portion of the duodenum. This lesion was endoscopically resected. Pathological examination revealed a Gangliocytic Paraganglioma. We describe the general characteristics of this neoplasm, as well as the theories about its histogenesis.

Aged↗

Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases.

Gangliocytic paraganglioma (GPG) with local lymph node metastasis was found in the pancreas of a 74-yr-old female who presented with diarrhea, steatorrhea, vomiting, nausea, and abdominal pain. A Whipple procedure led to a complete resolution of these symptoms and a return of an elevated stomatostatin level to normal. This is the first description of GPG in this location and the first endocrinologically active GPG.

Aged↗

Human monoclonal antibody developed against a metastasizing paraganglioma: preparation and preclinical studies.

A tumor cell line, named HS, was established from a bone metastasis of a patient with metastasizing paraganglioma. In vitro immunization of normal human peripheral blood mononuclear cells by coculturing with viable HS cells, followed by fusion with mouse myeloma cells, yielded a stable human/murine heterohybridoma producing the highly specific monoclonal anti-body KM-155. This MAB KM-155 is a member of the IgG3 subclass and shows no alpha GAL glycosylation that is specific for mouse but not for human antibodies. In pilot preclinical studies it could be demonstrated that MAB KM-155 is highly efficient in targeting a KM-155 antigen-expressing human tumor developing in nu/nu mice after xenografting. Moreover, the growth of KM-155 antigen-expressing human tumors in nu/nu mice was largely inhibited when the concentration of circulating MAB KM-155 was maintained at a high enough level by serial injections.

Animals↗

[Preoperative embolization of paraganglioma in head and neck].

Preoperative embolization of 10 cases of paraganglioma in carotid artery body and jugular foramen region via endovascular approach was performed. Tumor vessels were obliterated 100% in 7 cases and 80% - 90% in 3 cases as was demonstrated by angiography. It is available for us to reduce bleeding, expose tumor, avoid the iatrogenic damage of the important vessels and nerves around tumor during operation and to total resection possible. Additionally, the manipulating points of embolization are discussed.

Adult↗

[Cervical sympathetic paraganglioma].

A 24-year-old man presented with a long-standing history of Horner's complex and a slowly growing cervical mass. Duplex-sonography, angiography, and computed tomography revealed a hypervascularized tumor with well defined borders displacing the left carotid bifurcation medially. Operative and histological findings confirmed the preoperative diagnosis of cervical sympathetic paraganglioma.

Adult↗

Fine mapping of an imprinted gene for familial nonchromaffin paragangliomas, on chromosome 11q23.

Hereditary nonchromaffin paragangliomas (PGL; glomus tumors; MIM 168000) are mostly benign, slow-growing tumors of the head and neck region, inherited from carrier fathers in an autosomal dominant fashion subject to genomic imprinting. Genetic linkage analysis in two large, unrelated Dutch families assigned PGL loci to two regions of chromosome 11, at 11q23 (PGL1) and 11q13.1 (PGL2). We ascertained a total of 11 North American PGL families and confirmed maternal imprinting (inactivation). In three of six families, linkage analysis provided evidence of linkage to the PGL1 locus at 11q23. Recombinants narrowed the critical region to an approximately 4.5-Mb interval flanked by markers D11S1647 and D11S622. Partial allelic loss of strictly maternal origin was detected in 5 of 19 tumors. The greatest degree of imbalance was detected at 11q23, distal to D11S1327 and proximal to CD3D. Age at onset of symptoms was significantly different between fathers and children (Wilcoxon rank-sum test, P < .002). Affected children had an earlier age at onset of symptoms in 39 of 57 father-child pairs (chi2 = 7.74, P < .006). However, a more conservative comparison of the number of pairs in which a child had > or = 5 years earlier age at onset (n = 33) vis-a-vis that of complementary pairs (n = 24) revealed no significant difference (chi2 = 1.42, P > .2). Whether these data represent genetic anticipation or ascertainment bias can be addressed only by analysis of a larger number of father-child pairs.

Adolescent↗