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PubMed2004

Primary pulmonary paraganglioma: report of a functioning case with immunohistochemical and ultrastructural study.

We describe a case of primary pulmonary paraganglioma, a tumor that has not been reported in sufficient detail in previous literature. The patient was a 55-year-old woman with hypertension accompanied by an elevated serum norepinephrine level (2651 pg/mL; normal 100-450 pg/mL). Computed tomography revealed a well-circumscribed solid mass, 3.5 cm in diameter, located in the lower lobe of the left lung. In the lobectomy specimen, the tumor had invaded the B8 bronchus and hilar lymph nodes with microscopic metastasis to the mediastinal nodes. The tumor showed histologic, immunohistochemical, and ultrastructural features of paraganglioma: argyrophilic cells arranged in a nesting (Zellballen) or anastomosing trabecular pattern within an arcuate vascular network. Neoplastic chief cells positive for neuroendocrine markers (CD56, synaptophysin, chromogranin A) were surrounded by sustentacular cells positive for S-100 protein. Neurofilament protein was positively stained, but cytokeratins were totally negative. On electron microscopy, chief cells possessed abundant dense core granules with an eccentric halo ("norepinephrine-type" granules). The patient's blood pressure began to decline soon after the resection, and her serum norepinephrine promptly returned to almost normal. On the basis of our experience, our case is a bona fide primary pulmonary paraganglioma, a tumor heretofore subject to considerable skepticism.

CD56 Antigen↗

PubMed2006

Paraganglioma of the ovary: report of three cases of a rare ovarian neoplasm, including two exhibiting inhibin positivity.

Paraganglioma is one of the rarest neoplasms to involve the ovary, whether primary or metastatic, with only two previous reports. We describe three examples that occurred in patients 22, 58, and 68 years of age. Two patients had hypertension. Two tumors involved the left ovary and one the right ovary; they ranged from 8 to 22 cm, were solid, and were tan, brown, or yellow. One tumor was confined to the ovary; in the second case, there were tumor deposits on the posterior surface of the uterus and the contralateral ovary; in the other case, there was peri-aortic lymph node involvement and peritoneal deposits. In all cases, however, radiologic investigations did not reveal an alternative primary site. On microscopic examination, all three tumors showed a predominantly nested "zellballen" pattern with groups of cells surrounded by a vascular stroma. Tumor cells largely had abundant granular eosinophilic cytoplasm with, in 2 cases, focal clear cell areas. In 1 case, bizarre tumor giant cells were present. Immunohistochemically, all neoplasms were cytokeratin negative and diffusely positive with neuroendocrine markers. In 1 case, there was an S-100-positive population of sustentacular cells. Two cases were positive for inhibin, one focal and one diffuse, and the other was focally positive for calretinin. Electron microscopy performed in 2 cases revealed dense core neuroendocrine granules. One patient has been followed up for 15 years and is alive and well. Although metastatic spread from an undetected primary outside the ovary cannot be totally excluded for the 2 cases with extraovarian disease, we think that the neoplasms most likely represent primary ovarian paragangliomas. Because various neoplasms in the sex cord-stromal and steroid categories are likely to enter into the differential diagnosis, inhibin and calretinin positivity represents a significant potential diagnostic pitfall. The differential is broad and may include many other ovarian tumors, particularly those with an oxyphilic cell type. Possible theories of histogenesis of primary ovarian paraganglioma include an origin from extra-adrenal paraganglia in the region of the ovary or unidirectional differentiation within a teratoma.

Adenocarcinoma↗

PubMed2003

Paraganglioma of the vagina: report of a case, including immunohistochemical and ultrastructural findings.

Paragangliomas are uncommon neoplasms of the female genital tract, with fewer than 15 reported cases arising from the uterus, ovary, vagina, and cervix. Three of these tumors presented in the vagina. This report describes the authors' experience with a paraganglioma of the vagina in a 24-year-old woman that presented as an asymptomatic mass. The histologic features in the initial biopsy proved again that the paraganglioma may be confused with other vascular neoplasms. The clinical course after the biopsy implied that this neoplasm had been awakened from its functionless dormancy.

Adult↗

PubMed2006

Robot-assisted laparoscopic resection of a large paraganglioma: a case report.

A 19-year-old female patient presented with headache, nausea, hypertension, visual impairment of the left eye and exertion-related complaints of palpitations since 1 year. Fundoscopy showed severe hypertensive retinopathy grade IV. A paraganglioma in the left para-aortic region was diagnosed by urinary screening and magnetic resonance imaging scanning. The tumor was resected by a robot-assisted laparoscopic procedure. The surgical operating time was 250 minutes and the blood loss was 150 mL. After surgery the blood pressure normalized and antihypertensive drugs were no longer required. Oral intake was resumed on the first postoperative day and the hospital stay was 3 days. Final pathology examination revealed a paraganglioma with pheochromocytomal features of 7x5.1x3.5 cm. Reports on laparoscopic resection of large paragangliomas are scarce. This case has demonstrated that the procedure is feasible and associated with rapid recovery from surgery. The surgical team experienced explicit support by the robot in the dissection of this highly vascularized tumor.

Abdominal Neoplasms↗

PubMed1991

Vagal paragangliomas: a clinical, pathological, and DNA assessment.

Ten vagal paragangliomas were studied by image analysis and the results correlated with clinicopathologic features to determine if the DNA ploidy pattern could be used to separate benign from malignant paragangliomas. The tumours occurred in 8 women and 2 men ranging in age from 23 to 75 years (average 54 years). Follow-up was available in all 10 patients and ranged from 3 months to 27 years (average 7.8 years). Of the 10 tumours examined for DNA, 5 were diploid, 4 diploid-tetraploid, and 1 aneuploid. Two patients experienced local recurrences. One of these had a diploid tumour that recurred 22 years after excision and the other had an aneuploid tumour which recurred 4 years 4 months later and was associated with cervical lymph node metastasis. Two patients had malignant tumours with histologically confirmed metastases to noncontiguous cervical lymph nodes. One of the malignant tumours was diploid and the other aneuploid. This study concludes that DNA abnormalities are common in vagal paragangliomas and that tumour ploidy can not be used to assess malignant potential.

Adult↗

PubMed1985

Laryngeal neuroendocrine tumour with features of a paraganglioma, intracytoplasmic lumina and acinar formation.

A supraglottic laryngeal neuroendocrine tumour in a 71-year-old female is presented. In addition to the typical features of extra-adrenal paragangliomas it showed unusual, numerous intracytoplasmic lumina and occasional true acini with accumulation of alcian blue and PAS positive secretory product in them which led originally to an erroneous diagnosis of metastatic adenocarcinoma. Ultrastructurally, the tumour was composed of light and dark chief cells containing varying numbers of dense-core secretory granules. Intracytoplasmic lumina and true acini contained microvilli with glycocalyceal material and varying amounts of membranous and amorphous material, possibly secreted via small, smooth-surfaced cytoplasmic vesicles but typical mucin granules were not seen. These features have not been previously described in laryngeal paragangliomas but are seen in endocrine tumours of other sites and have been used as an argument in favour of an endodermal rather than ectodermal origin for endocrine cells in those sites. Laryngeal paragangliomas are often malignant and the presence of spasmodic pain appears to be the most reliable indication of possible malignancy, histological criteria being as yet poorly defined.

Aged↗

PubMed1987

Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease--a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid.

The co-existence of a gangliocytic paraganglioma and a glandular psammomatous carcinoid in the duodenum of a patient with von Recklinghausen's disease and bilateral phaeochromocytomas is reported. The two lesions were considered to be distinctive by light microscopy, electron microscopy and immunocytochemistry. The cells of the glandular carcinoid showed strong cytoplasmic immunoreactivity for somatostatin and contained only scanty intracytoplasmic microfilaments on electron microscopy. In contrast, the endocrine cells of the gangliocytic paraganglioma were positive for pancreatic polypeptide and serotonin, were negative for somatostatin, and contained conspicuous intracytoplasmic aggregates of filaments. The histogenic relationship between the two tumours is discussed. This case strengthens the known association of glandular duodenal somatostatinoma with von Recklinghausen's disease and phaeochromocytoma and, in the light of a previous case report, suggests that von Recklinghausen's disease and gangliocytic paraganglioma may co-exist more commonly than expected.

Carcinoid Tumor↗

PubMed1989

Paraganglioma presenting with an aortic aneurysm.

The concurrence of a paraganglioma of the organ of Zuckerkandl and an abdominal aortic aneurysm is reported in a 72-year-old caucasian male. Computerized tomography scan confirmed the aneurysm and noted a well-circumscribed tumour of the aneurysmal wall. At operation, the tumour was identified and excised with part of the aneurysmal wall. The tumour exhibited all the features of a paraganglioma while the vessel wall showed atherosclerosis and secondary degenerative changes. The features of the aortico-sympathetic group of paragangliomas are discussed.

Aged↗

PubMed1991

Laryngeal paragangliomas and neuroendocrine carcinomas.

Forty-eight neuroendocrine tumours of the larynx were studied, of which 41 were classified as large cell neuroendocrine carcinoma. Most of these tumours occurred in the supraglottic larynx and the patients were predominantly male. Exquisite pain was a presenting feature in one third. These carcinomas metastasized, frequently to skin, giving rise to painful secondary lesions, but long-time survival occurred. Histologically, large cell neuroendocrine carcinoma had a number of features seen in neuroendocrine tumours at other sites, including grouping into 'Zellballen' which mimics paraganglioma. Four tumours were definite paragangliomas. These tumours have behaved benignly. There were three cases of small cell neuroendocrine carcinoma, a tumour which is histologically identical to its counterpart in the bronchus and has a very aggressive course. All three types of tumour expressed general neuroendocrine markers, but only large cell neuroendocrine carcinoma marked for both cytokeratin and calcitonin. In the paraganglioma cases sustentacular cells were identified and marked for S-100 protein and glial fibrillary acidic protein. Histological examination, supplemented with immunohistochemistry, helped distinguish these tumours into those requiring different treatment regimens.

Adolescent↗

PubMed1985

Nonchromaffin paraganglioma in the nose and paranasal sinuses.

Nonchromaffin paraganglioma occurring in the nose and paranasal sinuses are extremely rare. The authors have experienced a case of nonchromaffin paraganglioma of nasal cavity which extended to the epipharynx. To our knowledge, this reported case is the fourth case that has appeared in the world literature. The patient, a 31-year-old female, had complained of a right nasal obstruction and mucopurulent rhinorrhea for two years. The right nasal cavity was filled with a polypoid mass showing no bleeding tendency or necrosis. Radiographs revealed a homogeneous shadow which occupied the entire nasal cavity and paranasal sinuses. Histopathological studies revealed that the tumor showed typical findings of nonchromaffin paraganglioma and neurosecretory granules in cytoplasm were observed by electron microscopic study. Subsequently, the tumor recurred at on the posterior epipharyngeal wall after a duration of one year and three months, and was removed completely. Since then, there was no recurrence of the tumor until the present time.

Adult↗

PubMed1995

Posterior mediastinal paraganglioma involving the spinal cord of a dog.

A posterior mediastinal paraganglioma is described in a five-year-old female collie showing paralysis of the hindlimbs. The mass was observed radiographically and a myelogram confirmed a spinal cord compression at the thoracic level. Histological examination of the mass diagnoses a paraganglioma which was confirmed by histochemical and immunohistochemical studies. This is compared with paraganglioma found in man.

Animals↗

PubMed1977

Mediastinal paraganglioma causing spinal cord compression.

An invasive paraganglioma of the posterior mediastinum caused spinal cord compression in a 31 year old women. Electron microscopic examination of the paraganglioma invading the epidural space revealed numerous dense-cored granules in the cytoplasm of the tumour cells. We are reporting this case to present the ultrastructure of mediastinal paraganglioma, and to call attention to an unusual cause of spinal cord compression.

Adult↗

PubMed1997

Duodenal gangliocytic paraganglioma: CT, MR imaging, and US findings.

PURPOSE: To determine the imaging features of duodenal gangliocytic paraganglioma that can be used to differentiate this mass from other lesions. MATERIALS AND METHODS: Imaging, histopathologic, and surgical findings in five patients with proved gangliocytic paraganglioma were reviewed. The most common symptom at presentation was abdominal pain (n = 3). All patients underwent computed tomography (CT), two underwent ultrasonography (US), and one underwent magnetic resonance (MR) imaging. Imaging findings were correlated with findings from surgical resection specimens in all cases. RESULTS: All lesions were located around the second portion of the duodenum and were 3-13 cm in diameter (mean, 6.5 cm). Two extended laterally to the duodenum, two extended medially, and one was intraluminal. All appeared solid and homogeneous on US, CT, and MR images and had homogeneous contrast material enhancement on CT and MR images. All were solid, with a prominent vascular network, but no cystic hemorrhage or necrosis was noted at pathologic examination. CONCLUSION: The imaging features of gangliocytic paraganglioma are suggestive enough for the prospective diagnosis and differentiation of this benign mass from other lesions.

Aged↗

PubMed2001

Paraganglioma and adrenal pheochromocytoma presenting simultaneously in an elderly female. Case report and review of the literature.

Paragangliomas are chromaffin tissue tumors arising in an extra-adrenal location. It is quite rare to find a paraganglioma concurrently with a pheochromocytoma. We report a patient who underwent resection of a retroperitoneal mass that was characterized pathologically as a malignant paraganglioma. An incidental finding was a microscopic pheochromocytoma in the ipsilateral adrenal gland.

Adrenal Gland Neoplasms↗

PubMed2002

Oncocytic paraganglioma of the cauda equina in a child. Case report and review of the literature.

The authors report a case of oncocytic paraganglioma of the cauda equina in a 12-year-old girl who presented with lower back and leg pain on the right side of 6 months' duration. Magnetic resonance imaging revealed an ellipsoidal, intradural, extramedullary mass causing cord compression at the level of L1. Total laminectomy was performed on T12 and L1, and the tumor was excised completely without difficulty despite adherence of the tumor to the spinal cord. Postoperatively, the leg pain and motor weakness were much improved. The use of electron microscopy, and the immunohistochemical demonstration of synaptophysin in this tumor, allowed a confident diagnosis of an oncocytic paraganglioma to be made. To the authors' knowledge, this patient represents the first definite case of an oncocytic paraganglioma of the cauda equina in a child.

Cauda Equina↗

PubMed1994

Erythrocytosis complicated by multiple paraganglioma.

We report the case of a 22-year-old woman with onset of erythrocytosis at the age of 9 years. Endocrinological and radiological examinations revealed an elevated catecholamine level and the presence of multiple abdominal tumors. After the removal of the tumors, the catecholamine level normalized, whereas erythropoietin remained at the same level and erythrocytosis persisted. The tumor lysate contained considerable amounts of catecholamine but not erythropoietin. Moreover, no erythropoietin mRNA was detected in the tumor by in situ hybridization. These data suggest that this paraganglioma did not produce erythropoietin. A review of the literature showed the existence of patients with early-onset erythrocytosis complicated with paraganglioma, whose erythrocytosis was not relieved even after the resection of paraganglioma.

Abdominal Neoplasms↗

PubMed1992

Thyroid paraganglioma.

Paraganglioma of the thyroid gland is a rare neoplasm. Only eight cases have previously been reported. The neoplasm was confined to the thyroid in all but two patients; laryngeal involvement was seen in one and tracheal involvement in another. A review of these previously reported cases is presented along with the details of an additional case with laryngeal involvement. Laryngeal and thyroid paragangliomas are compared and contrasted. The anatomic and embryologic basis of these tumors and the diagnosis and treatment are discussed. We suggest changing the nomenclature and reporting of these lesions on the basis of their apparent common origin. Surgery remains the treatment of choice in most paragangliomas, including those involving the larynx and thyroid. Although the number of reported cases is small, there have been no cases of distant metastases or local recurrence after adequate surgical removal.

Adult↗

PubMed1994

Identification, classification, treatment, and prognosis of laryngeal paraganglioma. Review of the literature and eight new cases.

This study details the clinicopathologic features of 62 cases of laryngeal paraganglioma (LP), including 54 acceptable cases identified in the literature (although clinical information is lacking on 7 of these) and 8 previously unpublished cases identified from the Registry of Otolaryngic-Endocrine Pathology at the Armed Forces Institute of Pathology. Demographic findings show that the overwhelming majority of cases affect women (41:14), mainly in the fourth to sixth decades of life (age range, 14 to 83 years; median, 44 years), with a prevalence in the supraglottic larynx. These neoplasms are treated by surgical resection and are benign. Despite the characteristic pathologic features associated with LP, it is sometimes confused with other neoplasms, particularly neuroendocrine carcinomas of the larynx, and this confusion leads to unfortunate designations such as malignant paraganglioma and metastasizing paraganglioma of the larynx. Judging from the cases reported in this study and those identified in the literature, we conclude that malignant biologic behavior associated with LP is extraordinarily rare (< 2%). Because of the misdiagnoses of LP, the prognosis associated with this entity has been skewed to suggest that LP may behave aggressively. This has led to the inappropriate classification of LP among the malignant categories of laryngeal neuroendocrine neoplasms. The goal of this study is to detail the features diagnostic of LP and to discuss the appropriate treatment, prognosis, and classification of these neoplasms.

Female↗