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Paraganglioma of the urinary bladder is a rare neoplasm that derives from ganglion cells located in the bladder wall and may morphologically simulate a urothelial carcinoma. The authors present the case of a vesical paraganglioma incidentally detected by ultrasound, and entirely removed by transurethral approach from detrusorial wall. Conventional approaches include surgery (partial cystectomy) or laparoscopic procedures, with inspection by transurethral endoscopy; in this case we wanted to avoid the combined approach considering the lesion diameter and the possibility of an easy removal.

Unlabelled: The Authors report a case of a voluminous abdominal paraganglioma and a review of the literature on the surgical management of paragangliomas., Case Report: A 58 years old woman was admitted to our Institution with acute endocrine impairment and simultaneous intestinal necrosis resulting from the mass effect of the tumor on the mesenteric vessels. Clinical and biochemical preoperative work up posed the suspect of paraganglioma. Previous intensive care treatment, the patient, underwent surgical resection of the tumor and left hemicolectomy, with immediate remission of both endocrine and abdominal syndromes. Histological examination confirm the preoperative clinical hypothesis. Paragangliomas are rare tumors of the extra-adrenal chromaffin tissue. A paraganglioma may be discovered in the absence of any symptom. When symptomatic, the clinical findings are related to the hypersecretion of catecholamines or to the compression of several anatomical structures from a growing mass. Combined acute syndromes of hormonal impairment and contemporaneous mass effect, as in our case, are exceptional. The diagnosis of the secreting forms may be obtained on the basis of biochemical dosage of serum and urinary catecholamines and metanephrines. The assessment of malignancy for paragangliomas is not always feasible. Several imaging, cytological and histological parameters have been proposed as predictors of malignancy, but the only element widely accepted is the existence of distant metastasis. Surgery represents the treatment of choice for paragangliomas. Benign forms, catecholamine secreting forms and tumors compressing neighbouring anatomical structures can be treated surgically with success. In general, an accurate follow-up after complete resection must be considered.

The authors describe a case of laryngeal paraganglioma (LP) occurring in a 57-year-old-woman. To date, 70 cases have been described in the literature. It is benign and recurrences are infrequent. The differential diagnosis with typical and atypical carcinoids, hemangiopericytomas, alveolar soft-part sarcomas, medullary thyroid carcinomas, malignant melanomas and metastatic renal cell carcinomas is supported by immunohistochemistry. Moreover, this tumor shows the immunohistochemical expression of galanin, a variably expressed marker of paragangliomas that it is not expressed in carcinoid tumors. Nevertheless, our observations militate against its role as a solitary marker but advocate its use in conjunction with other antibodies for the differential diagnosis of neuroendocrine neoplasms of larynx.

In this presentation the gastrointestinal pancreatic endocrine tumors (ETs) are subdivided into well differentiated ETs and poorly differentiated ETs. Well differentiated ETs are subclassified into ETs with benign behavior, ETs with uncertain behavior and well differentiated endocrine carcinomas. The criteria allowing a distinction among these main types of neoplasms are listed according to the site of origin of the ETs. In addition, the main functional types of ETs of the pancreas, stomach, duodenum, jejunum, ileum, appendix and large bowel are briefly described.

Cervical paragangliomas are uncommon benign or malignant neoplasms, originated from the stem cells of the neural crest. Up to date it is still not easy to define properly their biological behaviour, the possible multicentric location and the association with Multiple Endocrine Neoplasias. After a wide review of recent diagnostic, pathological and clinical notions, the Authors report their experience, from 1970 to 1995, of 10 patients affected by sporadic paraganglioma and 1 by familial multicentric neoplasm localized on 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 laparotomy was possible because of the visceral and vascular involvement.

Introduction: A costovertebral paraganglioma with a long history of recurrences and metastases is reported., Material and Methods: The lesion is documented on histological ground and with immunocytochemistry and electron microscopy., Results: The patient is a 42 year old man which presented with a 5 cm axillary mass and enlarged mediastinal lymph nodes. The former lesion has been interpreted as a metastasis of paraganglioma. The patient underwent surgery because of a costovertebral paraganglioma at the age of 19 and showed locoregional metastases and a recurrence over a period of 23 years. Twenty-seven years from removal of the primitive tumor, the patient is alive and well. All the neoplasms showed identical histological features as they were composed of neoplastic elements arranged in nests surrounded by dendritic cells. Neoplastic cells immunoreacted with anti-chromogranin antiserum whereas sustentacolar cells were positive with S-100 antiserum., Discussion: The differential diagnosis between a multicentric paraganglioma a metastasis has been particularly taken into consideration. In addition, axillary tumor was distinguished from a metastatic renal cell carcinoma and melanoma. The literature regarding costovertebral paraganglioma has been also revised.

We discuss in this paper the reliability of neuroimaging techniques in the diagnosis of neoplastic masses of the posterior-lateral skull base. We also discuss the role of TC and MRI in the evaluation of the extension pattern of these masses.

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.

Paragangliomas of the carotid body with an autosomal dominant genetic transmission have been described in a familial presentation in 7-10% of all the cases observed. In less than 10% malignancy is confirmed by secondary metastatic localization rather than by typical histological features of malignant diseases, such as vascular or perineural invasion, nuclear pleomorphism, mitotic activity. The purpose of the study is to present a familial group of paragangliomas of the carotid boy and a flow cytometric analysis of tumor content DNA and to discuss the prognostic value of the results. The paragangliomas of these related patients (father and daughter) are diploid, without regional lymph node or distant metastases; clinical and cytometric findings support a good prognosis even if an accurate follow up of such neuroendocrine tumors is nonetheless mandatory. Considering data in Literature, the Authors also propose a prognostic classification of paragangliomas as follows: noninvasive (capsulated, without vascular or perineural infiltration, diploid); locally invasive (histological signs such as vascular or perineural infiltration, nuclear pleomorphism, abnormal mitoses etc, diploid); potentially malignant (histological signs, non-diploid, expression of few antigens) and malignant (regional or distant metastases).

In order to evaluate 131I metaiodobenzylguanidine (MIBG) uptake in patients with benign or malignant paragangliomas, 28 patients (14 men and 14 women, mean age 37 +/- 10 years) with benign (no. = 15; group 1) or malignant (no. = 13; group 2) tumors underwent MIBG scintigraphy. A total of 110 lesions (20 benign and 90 malignant) were studied. In all patients histologic disease confirmation was obtained. MIBG uptake was quantified on 48 hours' images (Kodak NMC-1 films) using a photographic densitometer. The optical densities of tumor and adjacent or contralateral normal tissue were used to calculate the MIBG uptake intensity ratio for each lesion. In group 1, all patients exhibited 1 or 2 lesions with abnormal MIBG uptake. In group 2, all patients had 1 to 28 abnormal foci of MIBG uptake. In the patients with 2 or more lesions, the average MIBG uptake intensity ratio was calculated. MIBG uptake intensity ratio was significantly higher in malignant than in benign paragangliomas (5.2 +/- 2.4 vs 2.9 +/- 1.4, p < 0.01). Since MIBG uptake in paragangliomas reflects the intratumoral concentration of catecholamines, higher MIBG uptake in malignant lesions suggests a greater amount of stored catecholamines in these tumors. In conclusion, 131I MIBG scintigraphy may be useful to distinguish benign from malignant paragangliomas.

A case of incidental Gallbladder Paraganglioma is presented. This is the third case found until now in this organ. The parasympathetic origin of this neoplasm is thus discussed.

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.