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PubMed1990

Bilateral familial carotid body paragangliomas. Report of a case with DNA flow cytometric and cytogenetic analyses.

A case study of bilateral familial carotid body paragangliomas with DNA flow cytometric and cytogenetic analyses is presented. Analysis of tumor cell nuclear DNA content by flow cytometry revealed aneuploid cell populations in both tumors. Standard cytogenetic analysis (Giemsa-banding technique used) of the right carotid body paraganglioma showed no evidence of numerical or structural abnormalities. We describe parameters currently used to "predict" biological behavior in these tumors.

Adult↗

PubMedDate not supplied

[A case of chromaffin paraganglioma of the hypopharynx].

The present paper describes a case of so-called chromaffin paraganglioma of the hypopharynx. After a critical review of the international literature, the study deals with classification of paragangliomas and methods for more accurate histological identification and histochemical characterization.

Humans↗

PubMedDate not supplied

Carotid body paraganglioma (chemodectoma): cytologic remarks.

The fine needle aspiration findings of a carotid body paraganglioma observed in a female patient aged 18 showed a pattern similar to that of previously described cases, but also some other morphological features to be noticed: a continuous spectrum of nuclear volumetric variations, rosette-like structures, cell embracing phenomenon and characteristics nuclear clear areas. We believe that these findings may help in the cytological distinction of carotid body paraganglioma from other laterocervical neoplasms.

Adolescent↗

PubMed1988

Trends in neurovascular complications of surgical management for carotid body and cervical paragangliomas: a fifty-year experience with 153 tumors.

Almost 75% of carotid body and cervical paragangliomas are adherent to or surround adjacent arteries and cranial nerves. Their resection can result in neurovascular injury, stroke, and excessive blood loss. To assess trends in neurovascular complications, we reviewed 153 carotid body and cervical paragangliomas that were surgically managed between 1935 and 1985. Results of the past 10 years were compared with two previous time periods: period I (1935 to 1965), when carotid artery reconstruction was uncommon at our institution, and period II (1966 to 1975), when methods of intraoperative electroencephalographic monitoring and carotid patch angioplasty were being developed. During the past 10 years (period III), surgical approach to these tumors has included intraoperative monitoring of cerebral blood flow, selective use of shunts, vein patch or graft reconstructions after extensive tumor resections, and mobilization of the parotid gland to facilitate adequate exposure of high tumors. Although tumor resection was attempted in 80% of patients in period I, surgical resection was complete in 98% during periods II and III. Three trends were observed: (1) The perioperative stroke rate has decreased dramatically from 23% in period I to 2.7% in period III (p = 0.007); (2) the perioperative mortality rate has been reduced from 6% in period I to no deaths in the past 10 years, but (3) the rate of postoperative cranial nerve dysfunction remains unchanged over 50 years (period I, 46%; period III, 40%). The median tumor size among patients with postoperative complications was significantly larger than those without complications (median size: 17 vs. 7 cm3, p = 0.004).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1985

[Malignant paraganglioma].

A case of malignant paraganglioma of the right carotid bifurcation with metastases in the vertebral bodies in a 63-year-old man is reported. The criteria for malignancy in paragangliomas are discussed and special attention is paid to the difficult morphological differential diagnosis of these tumors and their metastases as illustrated by the present case.

Carotid Body↗

PubMed1984

High-resolution computed tomography of paragangliomas of the head and neck.

High-resolution computed tomography with intravenous contrast enhancement is the initial procedure performed on individuals who present with signs and symptoms of a paraganglioma of the head and neck. Clinical information about these entities is reviewed and technical considerations are discussed.

Adult↗

PubMed1990

Diagnosis and management of paragangliomas of the skull base.

In appropriately selected patients, glomus tumors of the head and neck are best treated surgically. Unresectability is not a factor in therapeutic planning for local disease control. Existing techniques and exposures for tumor removal can be reliably applied to these paragangliomas, with acceptable morbidity and mortality. A team approach to this problem is mandatory.

Adult↗

PubMed1984

Radiologic approach to paragangliomas of the temporal bone.

High-resolution computed tomography (HRCT) has become the primary radiologic method for evaluation of paragangliomas of the temporal bone. The HRCT evaluation of the integrity of certain bony landmarks has a profound effect on selection of surgical approach. The most important landmarks are the bony walls separating the carotid artery and the jugular bulb from the middle ear. Arteriography is not necessary if the lesion is confined to the middle ear. However, it is indicated in tumors that have eroded the above landmarks or in those patients with related neurologic symptoms.

Angiography↗

PubMed2025

The genetic changes in 11p15.5-related pheochromocytomas and paragangliomas.

Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors. The development of these tumors is associated with more than 20 genes. The aforementioned genes are subdivided into three clusters. The pseudohypoxic, kinase-signaling and Wnt clusters. The pseudohypoxic cluster is the only one that has been demonstrated to be associated with DNA methylation changes, including alterations in the 11p15.5 region. The objective of this study was to identify alterations in the 11p15.5 region, ascertain their prevalence in PPGLs, and subsequently compare them with the genomic and somatic mutations that cluster PPGLs. One hundred and fifty tumor samples were subjected to analysis. A total of 90 cases (60%) exhibited no alterations in the 11p15.5 region. The most prevalent alterations were maternal allele loss, observed in 45 cases (30%), pUPD (paternal uniparental disomy) in five cases (3.33%), and paternal allele gain in four cases (2.67%). The data presented here suggest that two mechanisms may be involved in the formation of PPGLs. These are reduced expression of CDKN1C (maternal allele deletion) and overexpression of IGF2 (pUPD, paternal allele gain). A statistically significant difference was observed in the frequency of alterations in the 11p15.5 region when comparing cluster 1 and cluster 2 (P-value <0.0001). This study is the first to describe pUPD and paternal allele gain as somatic alterations in PPGLs. In addition, our findings indicate that alterations in the 11p15.5 region are not exclusive to cluster 1. Consequently, the alterations in the 11p15.5 region cannot be regarded as a marker for cluster 1.

Humans↗

PubMed2026

Clinical relevance of HRD score in pheochromocytomas and paragangliomas: molecular cluster distribution and prognostic implications.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with variable metastatic potential across molecular clusters. While the homologous recombination deficiency (HRD) score has been established as a surrogate for HRD-related genomic instability to guide prognostic evaluation and therapeutic options in multiple solid tumors, its relevance in PPGLs remains unexplored. Here, data from 133 PPGL patients in the TCGA database were extracted to assess relationships between the HRD score and molecular clusters, metastasis, metastasis-free survival (MFS), and other clinical characteristics. Subsequently, a real-world cohort of 54 matched blood-tumor pairs was subjected to whole-exome sequencing, with HRD scores calculated to confirm the prior observations. The median HRD score was 7.0 and 9.0 in the TCGA-PPGL dataset and the clinical cohort, respectively. In the TCGA dataset, an elevated HRD score significantly correlated with metanephrine secretion, C2 cluster, and metastasis. Multivariate analysis identified a higher HRD score as an independent risk factor for shorter MFS (HR = 1.33, P = 0.008). Patients with HRD scores ≥ 7 exhibited significantly shorter MFS (P = 0.037), which was observed exclusively within the C1A cluster (P = 0.007). Analyses of our clinical validation cohort corroborated this cluster-specific distribution and further revealed higher HRD scores in three temozolomide-treated PPGLs compared with those treatment-naive C1A tumors (P = 0.043). In conclusion, despite exhibiting lower levels in C1A clusters, an elevated HRD score emerges as a promising indicator for refined metastatic risk stratification in PPGLs. Futhermore, the substantial increase in HRD score following temozolomide treatment provides a rationale for PARP inhibitor sequential or combination therapy in metastatic PPGLs.

Humans↗

PubMed2026

Molecular clusters and precision medicine in pheochromocytomas and paragangliomas.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells of the adrenal medulla and extra-adrenal paraganglia. Over the past two decades, the genomic characterization of PPGLs has profoundly transformed their diagnosis, classification, risk stratification, and therapeutic management. Up to 40% of PPGLs harbor germline pathogenic variants, the highest proportion among human neoplasms, and somatic driver events are identified in a substantial fraction of the remaining cases. Integrative multi-omic studies have established three main molecular clusters: a pseudohypoxic cluster driven by Krebs-cycle alterations (SDHx, FH, MDH2, DLST) and HIF-2α pathway alterations (VHL, EPAS1, EGLN1/2); a kinase-signaling cluster driven by activation of RAS/MAPK and PI3K/AKT pathways (RET, NF1, HRAS, TMEM127, MAX); and a Wnt-signaling cluster characterized primarily by MAML3 fusions. This review summarizes progress in PPGL genomics, highlighting geographic and sex-related particularities. Using EPAS1/HIF-2α and RET as paradigmatic examples, we illustrate how diverse germline, somatic, mosaic, and fusion events converge on common core signaling hubs that can be therapeutically exploited with FDA-approved selective inhibitors for relevant targets (e.g. belzutifan for HIF-2α; selpercatinib and pralsetinib for RET). We further review the genomic determinants of metastatic risk (SDHB, ATRX, TERT, and MAML3 fusions), the immune microenvironment of metastatic disease, and emerging radionuclide theranostics, liquid biopsy biomarkers, and integrative multi-omic approaches that are reshaping precision medicine for PPGLs.

Humans↗

PubMed1987

Catecholamine-secreting paragangliomas of the base of the skull. Report of two cases.

Two cases of catecholamine-secreting paragangliomas of the base of the skull are described. The patients presented with uncontrollable hypertension and, after investigation, tumors were discovered in the regions of the glomus jugulare and pterygopalatine ganglion, respectively. After cardiovascular stabilization and tumor embolization, the tumors were surgically removed, with subsequent resolution of hypertension. The incidence of these tumors is discussed.

Adult↗

PubMed1989

Multiple malignant paragangliomas. A case report.

The extra-adrenal paraganglia may be divided into branchiomeric (parasympathetic) and paraxial or para-aortic (sympathetic) networks. A patient is described with two synchronous branchiomeric paragangliomas--a carotid body tumour (glossopharyngeal) and a cardiac chemodectoma (vagus). Both tumours were locally invasive and therefore, by definition, malignant.

Carotid Body Tumor↗

PubMed1976

Intrathoracic paraganglioma arising from aorticosympathetic paraganglion.

A slowly growing intrathoracic paraganglion arising from an aorticosympathetic ganglion was removed from a 22-year-old woman. It was adherent to the parietal pleura and azygos vein, but not to the spinal cord. She has been well for the two years since that time. This patient represents the sixth tumor reported in this location. Most of the reported tumors are benign with occasional local invasion as seen in paragangliomas in other locations (carotid and aortic bodies). Complete excision of the tumor is the treatment of choice, but partial excision may provide long-term survival. A new classification and terminology suggested by Glenner and Grimley is emphasized. It groups logically the tumors arising from the paraganglion cells on the basis of anatomic distribution, innervation, and microscopic structure.

Adult↗

PubMed1977

Paraganglioma simulating primary rib tumor.

We report an unusual case of a nonchromaffin paraganglioma, 7 cm in diameter, presenting clinically within the thorax of a 7-year-old girl as a primary rib tumor. It is believed to be the first report of such a location for this tumor. The rib of origin and parts of the two adjacent ones were removed, along with the involved middle lobe of the lung. The possibility is proposed that the ganglion cells of origin were carried to the tumor site during development of the intercostal nerve. The defect in the patient's chest wall was repaired with Marlex mesh and there is no evidence of difficulty, nor of recurrence, four and one-half years later.

Bone Neoplasms↗

PubMed1992

Paragangliomas of the neck.

Between 1967 and 1990 inclusive, 28 patients with paragangliomas of the neck were diagnosed at the University of Alabama at Birmingham Affiliated Hospitals. There were 11 men and 17 women, whose ages ranged from 12 to 76 years (mean, 47 years). Tumor locations included the carotid bodies (19 cases), the vagus nerves (three), supraglottic larynx (two), the left lateral pharyngeal wall (one), posterior to the right jugular vein (not otherwise defined) (one), subcutaneous neck tissue (one), and a cervical lymph node with unknown primary (one). Diagnostic workup included angiography (23 cases) with preoperative embolization (three), computed tomography (one), magnetic resonance imaging (two), and urinary catecholamine assay (four). All 28 patients underwent resection of the lesions. Cranial nerve damage occurred in 11 patients (39%). There were no perioperative deaths or cerebrovascular accidents, although one of two saphenous vein grafts became thrombotic after carotid body tumor resection.

Adolescent↗

PubMed1997

Familial paragangliomas: linkage to chromosome 11q23 and clinical implications.

Familial paragangliomas (PGL), or glomus tumors, are slow-growing, highly vascular, generally benign neoplasms usually of the head and neck that arise from neural crest cells. This rare autosomal-dominant disorder is highly penetrant and influenced by genomic imprinting through paternal transmission. Timely detection of these tumors affords the affected individual the opportunity to avoid the potential morbidity associated with surgical removal, and mortality that may accompany local and distant metastases. Linkage to two distinct chromosomal loci, 11q13.1 and 11q22.3-q23, has been reported, suggesting heterogeneity. We evaluated three multigenerational families with hereditary PGL, including 19 affected, and 59 unaffected and potentially at-risk individuals. Numerous microsatellite markers corresponding to each candidate region were tested in all members of the three families. Confirmation of linkage to 11q23 was established in all three families. The inheritance pattern was consistent with genetic imprinting. Using these data, we were able to provide presymptomatic diagnosis with subsequent removal of tumor from one individual, and to start several others on an MRI surveillance protocol.

Adolescent↗

PubMed1998

Fine-needle aspiration cytology of malignant retroperitoneal paraganglioma.

The cytologic findings in a fine-needle aspiration sample are described from a large retroperitoneal mass in a 56-yr-old male. The aspiration was performed under CT guidance using a 22-gauge needle, maintaining negative pressure. For cytologic study, on-site smears were prepared and stained by the Papanicolaou method. Additionally, cytospin, filter preparations, cell blocks from the aspirate, histology, and electron microscopy of the tumor tissue were performed. Also, immunodiagnostic staining for neuron-specific enolase (NSE), chromogranin, S-100 protein, vimentin, HMB45, cytokeratin, and Grimelius preparation was performed on cytologic and histologic material. The cytologic material was characterized by cords, suggestive acinar structures, and small follicle-like clusters of cells, while cell blocks from the aspirate showed oval or spindle-shaped nuclei with a somewhat fasciculated appearance. However, sections from the tumor tissue showed an organoid "zellballen" pattern. The nuclei were round, oval, spindly, and epithelioid, with moderate to scanty cytoplasm, smooth nuclear membranes, fine, evenly dispersed chromatin, slight hyperchromasia, and mild pleomorphism. No intranuclear vacuoles were seen. Positive immunohistochemical staining for NSE, chromogranin, and Grimelius preparation was noted in the tumor cells, while staining for vimentin, HMB45, and cytokeratin was negative. Electron microscopy of the tumor tissue revealed the presence of variable numbers of round, membrane-bound, electron-dense neurosecretory granules. The cytohistologic and ultrastructural findings are presented, as well as the results of immunodiagnostic staining which helped in the diagnosis of retroperitoneal paraganglioma, an infrequently reported tumor.

Biopsy, Needle↗