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PubMed1997

Combination chemotherapy for malignant paraganglioma.

Treatment with a combination chemotherapeutic regimen consisting of cyclophosphamide, vincristine, and dacarbazine for malignant paraganglioma with hepatic metastasis is reported. A 51-year-old male presented with tumors in the retroperitoneal space and liver. The patient was diagnosed as having paraganglioma based on elevated levels of serum neuron-specific enolase, urinary catecholamine and vanillylmandelic acid, and on histological findings of the liver specimen. The patient was treated with this combination chemotherapy in repeated 21-day cycles. Temporary improvement in laboratory findings and a 20% reduction in the size of the hepatic masses were observed without severe adverse effects.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

[Nasal cavity paraganglioma with intracranial extension. Case report].

A 58-year-old male was admitted following an episode of unconsciousness. Nine years prior to this, he had undergone surgery and radiation therapy for a nasal tumor. Subsequently, he had undergone surgical excision of recurrent nasal tumors and metastases to the cervical lymph nodes. The final pathological diagnosis was paraganglioma. On admission, he had no neurological deficit other than anosmia. Laboratory studies showed no abnormalities in urinary concentrations of vanillylmandelic acid or catecholamines. Computed tomography (CT) demonstrated a calcified, heterogeneously enhanced mass in the anterior ethmoid sinus and bilateral frontal fossa. Cerebral angiography revealed a mesh-like tumor stain. Through a bifrontal craniotomy the intracranial tumor was totally removed. It was hypervascular and clearly demarcated from the surrounding cerebral tissue. The pathological diagnosis was paraganglioma without malignant features. Radiation therapy was administrated postoperatively at a total dose of 5060 rads, and follow-up CT showed regression of the ethmoid sinus tumor. As of 2 years postoperatively, the tumor has not recurred.

Brain Neoplasms↗
PubMed2025

Identification of Prognostic Gene Signatures for Survival of Patients With Phaeochromocytoma, Paraganglioma, and Other Tumor Types.

BACKGROUND/AIM: Tumor treatments remain unsatisfactory, as many patients continue to die despite therapy. There is an urgent need for novel drug targets, particularly for rare tumors. In this study, we sought to identify genes with prognostic significance for survival in patients with phaeochromocytoma or paraganglioma. We also examined whether these genes are relevant in other tumor entities. PATIENTS AND METHODS: We mined the TCGA-based KM Plotter and studied 186 risk genes for phaeochromocytoma and paraganglioma. RESULTS: Using Kaplan-Meier statistics, we performed 3,163 calculations based on 7,489 tumor biopsies and identified a 2-gene signature for phaeochromocytoma/paraganglioma (AQP4, FAM84H). Since the 186 risk genes are not exclusively related to the development of phaeochromocytoma/paraganglioma alone, we also investigated their prognostic relevance in 17 other tumor types. A clustered 12-gene signature has been found common in four other tumor entities (liver hepatocellular carcinoma, renal clear cell carcinoma, renal papillary cell carcinoma, lung adenocarcinoma). This signature consisted of BUB1, BUB1B, CDK1, CENPA, CKAP2L, IQGAP3, MKI67, NDC80, PBK, RRM2, TOP2A, and TTK. CONCLUSION: Our analysis provides a basis for the development of a novel prognostic test to predict the survival time of patients.

Kaplan-Meier analysis↗
PubMedDate not supplied

Aorticopulmonary paraganglioma (aortic body tumor): report of a case.

A case of aorticopulmonary paraganglioma in a 57-year-old man is described. The tumor comprised nests of uniform cells in a fibrovascular stroma. Electron microscopy revealed abundant neurosecretory granules, and S-100 protein staining demonstrated scattered sustentacular cells at the periphery of typical zellballen. The findings in this case correlated with those of studies on the prognosis for extraadrenal paragangliomas.

Aorta↗
PubMedDate not supplied

Gangliocytic paraganglioma of the duodenum: report of a case with immunocytochemical and ultrastructural investigation.

A case of gangliocytic paraganglioma is reported in a 70-year-old female presenting as a polypoid tumor of the second portion of the duodenum. Immunohistochemical and ultrastructural features of the tumor indicate that gangliocytic paraganglioma of the duodenum (GPD) represents an unique tumor originating from the neuroectodermal derivative exhibiting dual phenotypic expression toward paraganglionic and epithelial (neuroendocrine) cells. These findings correlate well with the literature and it is further suggested that GPD belongs to the histopathologic spectrum of tumors derived from the neural crest. The findings offer a plausible explanation for histogenetic possibilities of the occurrence of pure epithelial tumors (i.e., carcinoid-like tumors) in the paraganglionic systems. The patient remains with no evidence of disease 4 years following a simple polypectomy.

Aged↗
PubMedDate not supplied

Diagnostic usefulness of sustentacular cells in paragangliomas: immunocytochemical and ultrastructural investigation.

Neuroendocrine tumors may derive either from neuroectoderm or endoderm. Both may present with a similar histologic pattern known as "Zellballen" regardless of their histogenetic origin, making it difficult to separate the two different histogenetic entities in certain cases. To evaluate the usefulness of sustentacular cells in the recognition of tumors of paraganglionic origin, the authors analyzed immunohistochemical and ultrastructural characteristics of 25 paragangliomas and 19 pulmonary and 10 small intestinal carcinoids. Sustentacular cells with characteristic dendritic features, strong immunoreactivity for S-100 protein, and agranular cytoplasm by electron microscopy were found consistently in the paragangliomas and not found in typical carcinoid tumors, except for four examples of spindle cell carcinoid of the lung. The presence of sustentacular cells in tumors with "Zellballen" pattern therefore denotes paraganglionic origin.

Adrenal Gland Neoplasms↗
PubMed1982

Paraganglioma of the cauda equina. Case report.

An unusual, well demarcated, and encapsulated neoplasm of the cauda equina is presented. At first, the tumor was considered to be a variant of myxopapillary ependymoma, but the reaction for glial fibrillary acidic protein was negative. At the ultrastructural level, the neoplastic cells contained many small dense core vesicles, and the diagnosis of paraganglioma was established. The literature and histogenesis of paraganglioma of the cauda equina is reviewed.

Aged↗
PubMed1982

Gangliocytic paraganglioma in cauda equina region, with biochemical and neuropathological studies. Case report.

Biochemical analysis of a nonfunctional paraganglioma in the cauda equina region demonstrates that its catecholamine content is predominantly dopamine with small amounts of noradrenaline and adrenaline. Scattered mature large neurons characterize the tumor as gangliocytic paraganglioma. Ultrastructural study shows intracytoplasmic neurosecretory granules in the neoplastic cells.

Adult↗
PubMed1990

Giant gangliocytic paraganglioma of the filum terminale. Case report.

The clinical and pathological features of a giant cauda equina paraganglioma arising from the intradural filum terminale is described. Scattered mature large neurons characterized the tumor as a gangliocytic paraganglioma. Histologically, these neoplasms have considerable similarity with ependymoma and the diagnosis can be easily missed unless special techniques are employed.

Adult↗
PubMed1992

DNA flow cytometry in pheochromocytoma and paraganglioma.

Flow cytometric DNA analysis was performed on 19 adrenal pheochromocytomas and 6 extra-adrenal paragangliomas in parallel with clinical and histopathological review to determine the usefulness of this technique to predict biologic behavior of these tumors. In pheochromocytomas and paragangliomas, tetraploidy or near-tetraploidy occurred in 32% and 33% and aneuploidy in 10% and none respectively. A case of malignant pheochromocytoma had diploid DNA content. Occurrence of aneuploidy or tetraploidy is frequent in clinically benign tumors in conjunction with a marked degree of nuclear atypia and cannot be a predictor of malignancy.

Adolescent↗
PubMed1999

[The benefit of head rotation on pharyngoesophageal dysphagia from three cases of paraganglioma in the parapharyngeal space].

The benefit of head rotation to the affected side is indicated during swallowing in patients complaining of dysphagia with unilateral pharyngeal palsy and/or laryngeal palsy. We experienced three cases of severe dysphagia after operations for giant paragangliomas (two vagal paraganglioma and one carotid body tumor) in the parapharyngeal space. During operation, the transmandibular transpterygoid approaches were applied to ensure better surgical views, and tracheostomy was performed to keep the airway open after operation. In each case, dysphagia during the pharyngeal stage of swallowing was significantly improved with rehabilitation using of this head rotation. We believe that rather than forbid oral intake, using an active bolus with head rotation is important for cases where dysphagia in the pharyngeal stage of swallowing is present with unilateral pharyngeal and/or laryngeal palsy. Repetitive swallowing exercises are important to reacquire the complicated movement of swallowing. Additionally, an active bolus flowing into the pyriform sinus on the healthy side will prevent a relaxation disorder of the cricopharyngeal muscle on that side. Furthermore, compensatory movement of the arytenoid on the healthy side improves dysphagia. We emphasize the usefulness of head rotation during swallowing rehabilitation for dysphagia with unilateral pharyngeal and/or laryngeal palsy in spite of its simplicity.

Aged↗
PubMed2004

Dedifferentiated liposarcoma with a paraganglioma-like histologic pattern: a case report and review of the literature.

A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 x 32 x 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.

Diagnosis, Differential↗
PubMed1999

[Retroperitoneal non-secreting paraganglioma].

Paraganglioma is a rare tumor arising from indifferentiated cells of the primitive neural crest. These tumors are most commonly found in the adrenal gland but other localisations are possible. We described a 60-year-old men with a right retro-peritoneal mass discovered on ultrasound examination. This tumor had an heterogeneous appearance on i.v. contrast injected CT (computed tomography) and MR (magnetic resonance) images. Plasma and urinary catecholamines were normal. This tumor was surgically removed. Immunohistochemical analyses revealed that the tumor cells were strongly positive for neurone specific enolase and chromogranin A. Histopathologic examination diagnosed a non secreting paraganglioma in the right retroperitoneum. After surgery, the patient remained asymptomatic without treatment.

Chromogranin A↗
PubMedDate not supplied

Paragangliomas of the carotid body and other rare localisation.

Cervical paragangliomas are uncommon benign or malignant neoplasms, originated by stem cells of neural crest. It is not easy nowadays to define properly their biological behaviour, the possible multiple location and the association with Multiple Endocrine Neoplasms. After a wide review about recent diagnostic, pathological and clinical acquisition, authors report their caseload of 10 patients affected by sporadic paragangliomas and 1 by familial multiple neoplasm localised in carotid bodies of both sides, left vagus nerve and left hypoglossus nerve. All patients but one were treated by a curative resection of the neoplasm. In one case only an explorative laparatomy was possible because of the visceral and vascular involvement.

Adult↗
PubMed1999

[A further case of tracheal paraganglioma].

Paragangliomas, or chemodectomas, are neuro-endocrine tumors of the neural type, derived from extra-adrenal paraganglia. Tracheal localizations are rare. We report the case of a patient with a paraganglioma of the upper part of the trachea. The tumor was totally removed by means of the resection of the subglottic trachea and anastomosis. Surgery is the treatment of such lesions, but can be difficult in cases of subglottic or juxta-carinal localizations.

Biopsy↗