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Paraganglioma is an uncommon tumor of the larynx. The diagnosis and management of laryngeal paragangliomas remain problematic for the clinician. A recent experience with this uncommon tumor provides a forum for a discussion of laryngeal paraganglioma.
Paragangliomas of the duodenum are rare. Only 40 cases, most of them corresponding to benign tumours, have been published. In this study, we report a malignant paraganglioma of the duodenum with lymph node metastasis occurring in a 73 year old woman. The essential clinical manifestations which brought the patient to surgery were abdominal pain, melaena and anemia. The surgical specimen was investigated histologically, immunohistochemically and ultrastructurally. The tumour was composed of three types of cells: epithelial, gangliocytic and spindle shaped mesenchymal cells. The first contained ultrastructurally intracytoplasmic granules bound with double membranes; they stained with monoclonal antibodies against chromogranin. Gangliocytic cells stained with monoclonal N.S.E. antibodies and the spindle cells stained with S 100 protein. The spindle cells displayed ultrastructurally glial cell characteristics, containing micro-filaments of glial type. These immunological and ultrastructural features argued in favor of the neuroendocrine cell origin of this paraganglioma.
Cardiac paragangliomas are extremely rare neoplasms. Four surgically resected tumors were examined by immunohistochemistry and electron microscopy. The patients ranged in age from 18 to 36 years. All patients had hypertension and elevated urine catecholamine levels. Three tumors were located on the posterior left atrium, and one tumor was located in the interventricular groove at the aortic root. The tumors ranged in size from 5 to 7 cm, and they displayed a prominent Zellballen pattern without significant necrosis or mitosis. The tumors were mostly unencapsulated and infiltrated adjacent cardiac tissue in two cases. Immunoperoxidase staining showed that all tumors were positive for chromogranin and neuron-specific enolase. Three tumors were positive for methionine enkephalin. Positive staining for S-100 protein was seen in the sustentacular cells of all tumors but was negative in chromaffin cells. All tumors were negative for insulin, glucagon, gastrin, vasoactive intestinal polypeptide, somatostatin, adrenocorticotropic hormone, calcitonin, serotonin, pancreatic polypeptide, and rat atrial peptide. Ultrastructural studies of all four tumors showed moderate numbers of predominantly norepinephrine-type granules and a few epinephrine-type granules. These results show that cardiac paragangliomas are commonly found in close proximity to the left atrium and have immunohistochemical and ultrastructural features similar to other paragangliomas.
A 10-cm tumor of the mesoappendix was removed from an asymptomatic 81-year-old woman, who is alive and healthy 5 years later. Histologically, the tumor was a paraganglioma. Five other case reports of paraganglioma involving the gastrointestinal tract distal to the duodenum are reviewed. The authors believe these tumors should be regarded as aberrant retroperitoneal paragangliomas.
Paragangliomas are tumors arising in the paraganglia and rarely occurring in the cauda equina. We report two cases of paraganglioma arising in this region. The tumors exhibited a typical "Zellballen" pattern, and immunohistochemical studies were positive for neuron-specific enolase, but negative for S-100 protein and glial fibrillary acidic protein. Electron microscopic studies confirmed electron-dense neurosecretory types of granules in the tumor cells. Only 23 cases of paragangliomas in this region have been previously reported. A brief review of the literature is included.
The triad of pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma has been reported in five patients, all young women. The association of tumors apparently constitutes a syndrome. This paper describes the sixth affected patient, also a young woman, draws attention to the frequency of thoracic paraganglioma in the syndrome, and notes that the pulmonary cartilaginous lesion has frequently been misinterpreted as pulmonary hamartoma. Early exploration of the chest is indicated not only to rule out the possibility of metastatic gastric tumor but also to search for paragangliomas.
The natural behaviour, clinical diagnosis and treatment of paragangliomas of the petrous temporal bone are reviewed. Nine patients with this tumour treated at Groote Schuur Hospital are presented; 2 patients had tympanic paragangliomas and 7 jugular paragangliomas. Surgical excision is recommended as the primary treatment. However, in poor-risk patients or when tumour growth is extensive, irradiation may be of benefit. Although rare, this eventually life-threatening lesion can be successfully removed if diagnosed early enough.
Between 1971 and 1982, pheochromocytomas and sympathetic paragangliomas from 48 patients were examined at the Institute of Pathology of the University of Zurich. Thirty-four specimens had been resected surgically while 14 were discovered at autopsy, i.e. in 0.072% of the 19610 autopsies performed during that period. There were 42 cases (87.5%) of pheochromocytoma, of which 14.6% were bilateral, and 6 cases (12.5%) of sympathetic paragangliomas. The average age of our patients was 37.8% years, but the first clinical signs of the sympathetic paragangliomas preceded those of the pheochromocytomas by 20 years. Of all chromaffin tumors, 12.5% were obviously malignant and were metastasizing. In the assessment of biologic behavior it is noted that a histologically malignant tumor need not follow a malignant course and that, conversely, a tumor of benign appearance may develop metastases. Hence, long-term clinical follow-up is indicated as well as careful histologic diagnosis. The second part of this study will deal with associated diseases such as Sipple's disease and various neurocutaneous syndromes, which were present in 20.9% of our cases, and also with familial chromaffin tumors without associated diseases.
Paragangliomas of the head and neck are characterized by their profuse vascularity and persistent tumour blush during angiography. Routine bilateral carotid angiograms are needed to exclude bilateral localization. Subtraction angiography and computed tomography (CT) are of limited value in the diagnosis of paragangliomas of the neck, but play an important part in the diagnosis and management of jugulotympanic paragangliomas related to the base of the skull. CT scans demonstrate a mass of soft-tissue density which enhances on administration of contrast medium. CT is important in the evaluation of intracranial extension as well as in follow-up after operation and irradiation.
Pulmonary metastases from a carotid body paraganglioma developed in a patient with von Hippel-Lindau disease. On electron microscopic examination, these metastases consisted solely of chief cells that contained membrane-bound, dense-core granules and formed numerous cytoplasmic processes. These data support the hypothesis that the chief cell is the sole neoplastic cell in paragangliomas. Finally, to our knowledge, this clinical association of a malignant carotid body paraganglioma and pheochromocytoma in von Hippel-Lindau disease is unique.
The authors describe one case of paraganglioma of the posterior mediastinum successfully removed after being discovered accidentally in a young male patient who showed no symptoms attributable to the tumor. The latter was a round mass, about 6 cm in diameter, richly vascular, located in the right costomediastinal gutter. Histologically it was a functionally silent paraganglioma originating from the intrathoracic aortosympathetic paraganglia, these being part of the very numerous groups of the paragangliar system distributed segmentally into metamers, particularly in the adrenal medulla. According to the more recent views, chemodectoma or paraganglioma (chromaffine or non-chromaffine, functionally active or silent) and pheochromocytoma (functionally active or silent, adrenal or extra-adrenal) constitute a group of tumors sharing the same tissue matrix, namely paragangliar cells.
Paragangliomas are exceptional in the orbit. Among the 47 cases reported in the world literature more than 1/3 has been classified as alveolar soft part sarcoma. Diagnosis of paraganglioma was confirmed on the basis of pathology reports in only 10. We observed a case in a 46-year-old woman who presented with a tumour of the orbit. The histology examination and the ultrastructure determined by electron microscopy confirmed the diagnosis of paraganglioma. Characteristic histologic features are discussed together with a review of the literature.
From the study of one case of non secreting paraganglioma of the organ of Zuckerkandl, the authors have surveyed the existing literature gathering 95 cases from 1902 to 1992. It is an exceptional tumor of the paraganglioma and it often a secreting one. When it is not it looks like a malignant retroperitoneal tumor. The surgical resection is often performed without the operating diagnosis. Asserting the malignancy of these tumors only depends on the presents of metastasis appearing lately. There fore a long continued control of the patients who have been operated upon is a must all the more as 30% of the paraganglioma of Zuckerkandl prove to be malignants.
Retroperitoneal paragangliomas are uncommon neoplasms that arise from neural crest cells. Typical retroperitoneal sites of origin include adrenal medulla, organs of Zuckerkandl, and along sympathetic and parasympathetic nerves. We present a case of a woman diagnosed with retroperitoneal paraganglioma treated with debulking, intraoperative radiotherapy and external beam radiation therapy. She experienced a 7-year disease-free interval prior to symptomatic metastatic recurrence. Such remissions are consistent with the natural history for paragangliomas. The purpose of this report and literature review is to summarize the pertinent biologic and therapeutic options for these uncommon tumors.
OBJECTIVE: To present our experience of 10 patients with extra-adrenal retroperitoneal paragangliomas, and assess prognostic tests. DESIGN: Retrospective study of casenotes. SETTING: University hospital, Italy. SUBJECTS: 10 Patients who presented with paragangliomas between 1970 and 1991. MAIN OUTCOME MEASURES: Histological and immunohistochemical results, and outcome. RESULTS: All tumours were completely resected and there was no operative mortality. Of the 8 patients who had no metastases at presentation 3 died of recurrence 3, 5, and 10 years later, respectively; 4 were alive and free of disease 2-7 years after diagnosis. The 2 patients with synchronous bone metastases at presentation died 1 and 4 years later. Immunohistochemical analysis of type I cells (chromogranin A and neurone-specific enolase) showed little correlation with progression of disease, but there was a correlation between the presence of type II cells (S100 protein) and good prognosis. CONCLUSIONS: Excision is the treatment of choice for paraganglioma. Immunohistochemical techniques may provide useful information about prognosis, in particular about those patients who are at increased risk of recurrence. Long term follow up is essential, because successful management of recurrence is dependent on early recognition.
This is a revision of our experience between 1975 and 1992 over a total of 35 patients who underwent surgery in 40 occasions for pheochromocytoma. If we dismiss one case of pheochromocytoma with early metastatic malignant presentation and which was not removed, a total of 6 patients with abdominal paraganglioma (17%) have undergone surgery in 10 occasions. Three of them (50%) were multiple and recurrent, an one (16%) become malignant after multiple recurrences. Average age was 34.6 years with balanced sex distribution. No patient had neurofibromatosis, Von Hippel Lindau disease, MEN syndromes or Carney's triad and only one case, which corresponded to the malignant pheochromocytoma, showed direct familial background. All patients were hypertensive. Four cases (66%) presented with catecholamine crisis, one case was an incidentaloma that at the 24-hour monitoring presented hypertensive crisis during the sleep, and the last one was diagnosed while investigating a case of sustained HTA in a young female. All patients had high urinary catecholamine excretion. To establish location of the tumour MIBG scanning (90% sensitivity) was used in first place followed by guided CT (80% sensitivity) since both techniques have a good correlation in order to design the surgical approach. Angiography was reserved for cases where the other two techniques were inconclusive or when it was necessary to obtain a better profile of the surgical anatomy. A total of 15 paraganglioma were removed, the most frequent location being renal parahilar (26%) followed by preaortic in Zuckerkandl's organ (20%) and vesical (20%). The most relevant intraoperative complications were HTA crisis related to anaesthetics manoeuvres and tumoral handling (90%), and hypotension following tumour exercises (10%). During the postoperative period, three patients required blood transfusion and one of them had to be re-intervened for bleeding caused by unnoticed damage to the right renal vein. Currently, three patients (50%) are disease free, one with residual mild HTA controlled with diuretics. Two patients (33%) are awaiting for re-intervention due to recurrence (one with multiple extra-abdominal recurrence) after one and two prior interventions respectively. The last one (16%) is the malignant pheochromocytoma, operated four times for recurrence which currently shows lung metastasis with adequate drug control of clinical manifestations, after polychemotherapy failure and 27-months follow-up since metastasis has been detected. Revision of existing literature and discussion of issues related to signs and symptoms, diagnosis, surgical preparation and approach, as well as prognostic implications related to paraganglioma as compared with adrenal-located pheochromocytoma.
Paragangliomas are uncommon tumours arising from the neural crest and are widely distributed throughout the body. Because of the morphologic diversity of these tumours fine needle aspirate (FNA) diagnosis is not very accurate in identifying these neoplasms. Review of FNA from seven paragangliomas (three extra-adrenal, three carotid body and one multicentric in location) was conducted to find any diagnostic criteria. On initial morphologic diagnosis five of the seven paragangliomas were considered malignant (four undifferentiated and one adenocarcinoma). Review of these cases in our series made us wiser. Pleomorphic nuclei, prominent nucleoli, clumped chromatin with finely granular cytoplasm and occasional intranuclear cytoplasmic inclusions were characteristically seen and these were probably the cause for misdiagnosis. This lesion must be entertained in an aspirate from the neck or retroperitoneum which contains cells showing marked nuclear pleomorphism with naked nuclei and have a tendency to spindle or from acini. To confirm these cases argyrophilic granules were demonstrated in two of the four cases studied. However, neuron specific enolase and calcitonin were found to be non-contributory. Ultrastructural examination was helpful and done in one case showed abundant neurosecretory granules.
By definition, the term paraganglioma includes all the tumours deriving from the body's paraganglion system, which are therefore ranked as intra-adrenal (pheochromocytoma) and extra-adrenal or paraganglion. Vesical paraganglion represents about 10% of all extra-adrenal paraganglioma and can be functional and non-functional. When functional, the symptoms are those originated from the products synthesised in excess, usually occurring in a paroxismical fashion, in relation with urination, ejaculation and defecation. Contribution of one vesical paraganglioma accidentally found in a 71 year-old female patients undergoing tests to study the extent of a right kidney carcinosarcoma.