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PubMedDate not supplied

[131I]metaiodobenzylguanidine therapy in paraganglioma.

Our experience with [131I]metaiodobenzylguanidine (131I-MIBG) therapy in a 10 year old boy is reported. At disease onset, in May 1988, this boy presented a large mass in the upper left abdominal quadrant, which was resected with a histopathological diagnosis of extra-adrenal malignant pheochromocytoma (paraganglioma). He subsequently underwent two further surgical resections and chemotherapy. When 131I-MIBG therapy was started, in June 1990, skeletal and abdominal metastases were present. These localizations were revealed by 131I-MIBG scans and confirmed by x-ray examination. At present 6 courses of therapy have been performed with a cumulative activity of 29.6 GBq. Side-effects have been limited to vomiting and mild thrombocytopenia, lasting 2 weeks during the second course of therapy. After 15 months of therapy, a progressive reduction of MIBG uptake, coupled with a stabilization of the lythic lesions, has been observed.

3-Iodobenzylguanidine↗
PubMed1977

Ultrastructure of a polysome-lamellae complex in a human paraganglioma.

Study of a human abdominal paraganglioma under electron microscopy revealed an unusual cytoplasmic inclusion appearing as a hollow cylinder made up of one or several concentric lamellae surrounded by single or aggregated granules. Cross sections of the lamellar element show a discontinuous juxtaposition of particles. It is moderately dense and is bordered by 140--to 250-A granules of sure ribosomal nature. The inclusion is closely connected to the rough endoplasmic reticulum. The origin and significance of this complex are discussed.

Abdominal Neoplasms↗
PubMed1991

Treatment of metastatic para-aortic paraganglioma by surgery, radiotherapy and I-131 mIBG.

A patient with a malignant, functioning, aortico-sympathetic paraganglioma and a solitary bone metastasis causing paraplegia was treated by spinal decompression, irradiation of the metastasis, surgical excision of the primary tumour and systemic I-131 meta-iodobenzyl-guanidine (mIBG). Sixteen months after treatment there was no clinical, radiological or biochemical evidence of residual disease and neurological function was restored. The case supports the use of combined treatment incorporating mIBG in patients with metastatic neuroendocrine tumours which demonstrate mIBG uptake.

3-Iodobenzylguanidine↗
PubMed1978

[Polypous nonchromaffin paraganglioma of the stomach].

It is reported on the unusual position of a nonchromaffin paraganglioma in the stomach, which in gastroscopy imposed as polyp and was removed. On the basis of the light-microscopic picture, its behaviour during staining and the electronmicroscopic investigations the tumour is classified in the group of the paragangliomata. Since there are no unequivocal histological criteria of malignity, on account of the well-known slow growth of these tumour only the polyp is removed.

Diagnosis, Differential↗
PubMed1978

Cardiac paraganglioma (chemodectoma): a case report and review of the literature.

A rare case of paraganglioma (chemodectoma) of the heart is reported. This is the fifth case reported in the world literature and only the second case discovered in a living person. A review of the historical background, embryology, anatomy, and pathology of the paraganglia is provided. The previously reported four cases are also discussed.

Female↗
PubMed1990

[Jugular paraganglioma: presentation in the form of an aural polyp].

Paraganglioma though rare in ENT-pathology are considered the most common middle ear tumors. A few of them show some particularities in its growing mood, for instance under the appearance of a banal polyp lodging in the external ear canal, perhaps the polyp represents only the outer portion of a deeper larger process encroaching the underlying bone, which threat heavy complications, specially at attempts of removal without the prior due complementary explorations. The case reported by the authors was a quickly evolutive syndrome with the polyp associated to otorrhea and facial paresia. The verification of the bone destruction make credible the actual diagnosis, malignant tumor of the temporal bone. Afterwards profuse otorragia suggested the glomic tumor. The paper emphasizes the clinic malignancy of theses growths in spite of their histologic benignity, owing both to its speed evolution and destructive power.

Aged↗
PubMed1990

Extraadrenal retroperitoneal paragangliomas: natural history and response to treatment.

Extraadrenal retroperitoneal paragangliomas (RP) are uncommon tumors. Because of their rarity, little is known of their natural history or response to treatment. We reviewed 22 patients with RP who were seen at our center between 1949 and 1990. The distribution of male and female patients was nearly equal, and the mean age was 42. Most patients were admitted with pain or a mass, and eight of 22 tumors were functional. No significant difference was noted in duration of symptoms, size of the tumor, or survival between functional and nonfunctional tumors. Eleven of 22 (50%) RP metastasized and were therefore classified as malignant. Five-year and 10-year disease free survival rates were 19% and 19% for tumors not resected and 75% and 45% for those completely resected. Once metastases occurred, the 5-year survival rate was 36%, but no patient survived beyond 76 months. Predictors of survival included complete resection of the tumor but not size or functional status. Although some patients who received chemotherapy or radiotherapy had clinical responses, a survival benefit could not be shown. RP have a high rate of malignant behavior and should be treated aggressively with operation. Late metastases are not uncommon, and prolonged follow-up is necessary. Once metastases have occurred, some patients may have prolonged survival.

Adolescent↗
PubMed1979

[Endothyroid (non-chromaffin) branchiomeric paraganglioma. Description of a clinical case].

A case of endothyroid paraganglioma in a 9-yr-old girl is described. Subtotal lobectomy was performed and the tumour was not recognised until a histological examination has been carried out on a section embedded in paraffin. Only two other cases could be found in the literature. An account is given of the diagnosis of this form, and reference is made to its prognostic aspects since there is a remote possibility that metastasis may occur many years after surgery.

Child↗
PubMedDate not supplied

[Contribution of angiography in the pre-operative evaluation of 24 jugulo-tympanic paragangliomas with otoneurologic manifestations].

The contribution of carotid and vertebral angiography for surgical planning, particularly for the infratemporal approach, in 24 jugulotympanic paragangliomas with otoneurological symptoms is discussed. These symptoms included peripheral facial palsy, sensorineural hypoacusis, labyrinthine and nervous syndrome of the jugular foramen. Angiography was essential for the diagnosis of these tumors and, above all, for the rigorous evaluation of intrapetrous and apical invasion of the carotid artery, and also of extra and intradural posterior fossa extensions. Presurgical embolization, a relevant step in treatment, was based on the criterious evaluation of the angiotopographic pattern of the tumoral hypervascularization.

Adult↗
PubMedDate not supplied

[Paraganglioma of the gallbladder].

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.

Female↗
PubMedDate not supplied

Fine needle aspiration cytology of retroperitoneal paraganglioma with lipofuscin pigmentation.

The cytologic features observed in fine needle aspiration smears and cell blocks from a retroperitoneal paraganglioma are described. The aspirate contained clusters of uniform cells of medium size, with ill-defined borders, round nuclei and evenly dispersed chromatin. An unusual feature was the presence of cytoplasmic pigment, which proved to be lipofuscin by histochemical staining and electron microscopy. The histologic and ultrastructural findings, as well as the results of histochemical and immunodiagnostic staining, are also presented.

Biopsy, Needle↗
PubMed1986

[Malignant paraganglioma of the peri-iliac artery].

A malignant nonfunctioning paraganglioma of the pelvic retroperitoneum (left external iliac artery) with metastases to the femoral lymph nodes was found in a 68-year-old woman at exploratory laparotomy. Light microscopy showed a typical alveolar pattern with fine vascular stroma. The tumor cells had finely granular eosinophilic cytoplasm and oval to round nuclei with pleomorphism and mitotic figures, Grimelius stain showed no argyrophilic granules, but S 100 protein was demonstrated in the cytoplasm by the PAP method of Sternberger. Formaling fixed tissues were examination by electron microscopy, and dense core granules consistent with neurosecretory granules were found in the cytoplasm of some tumor cells.

Aged↗
PubMed1989

Familial occurrence of paragangliomas.

The occurrence of paragangliomas in three members of the same family is reported. It concerned a left carotid body tumor in one patient, a left carotid body tumor in her older sister, and a concomitant jugulotympanic and vagal body tumor in their younger brother. The radiological diagnosis in these patients was based on the typical hypervascular character in specific locations both on angiography and computed tomography. The familial occurrence of these lesions should be kept in mind whenever a chemodectoma is found, even in the absence of clinical symptoms in the direct relatives of the patients.

Adult↗
PubMed1989

[Gangliocytic paraganglioma of the duodenum].

In a 49-year-old patient who was investigated because of abdominal complaints we found a polypoid tumor in duodenum near the papilla of Vater. The histological investigation after endoscopical polypectomy showed the rare case of a gangliocytic paraganglioma. After complete endoscopical removal no further intervention is necessary.

Duodenal Neoplasms↗
PubMed1989

[Spontaneous hemoperitoneum complicating intraperitoneal paraganglioma].

A young man of 27 years was admitted to the medical ward because of increasing ascites over 3 months. There were no signs of portal hypertension and a diagnostic tap produced hemorrhagic liquid. Ultrasound confirmed ascites associated with an abdomino-pelvic mass. Anemia progressed and right aorto-iliac axis arteriography was carried out and demonstrated a poorly vascularised mass. Emergency surgery was carried out and demonstrated an encapsulated firm and cystic abdomino-pelvic mass of 23/18 cm with a rich vascular supply from the greater omentum and a hemoperitoneum of 7 litres secondary to rupture of this pseudo-lymphangiomatous mass. Besides large vessels from the and some loose fibrous adhesions in the pouch of Douglas there was no true implantation site attaching this mass to retroperitoneal structures, gastrointestinal tract or bladder. After ligation of the vessels the entire mass was easily resected. The post-operative course was uncomplicated. Pathological examination confirmed a paraganglioma. A full clinical pathogenic, therapeutic and evolutive study is described.

Abdominal Neoplasms↗
PubMedDate not supplied

[Paraganglioma of the cauda equina with gangliocyte differentiation].

Clinicoradiologic and pathologic data of a patient with a paraganglioma of the cauda equina are described. The peculiar aspect of this rare case is the gangliocyte differentiation with mature ganglion cells scattered in the tumor. The histologic and ultrastructural features are similar to other described cases of this tumor, with Zellballen pattern and neurosecretory granules. The immunohistochemical studies are quite typical with positive staining with neuroendocrine markers (N.S.E., G.F.A.P.). An interpretation of these findings is made.

Adult↗
PubMed1987

[Values and limitations of embolization in cervical paragangliomas. Definition of a new diagnostic strategy. Apropos of 20 cases].

From 1966 to 1986, twenty cervical paragangliomas are treated by the authors: seven vagals and thirteen carotid one's. Seven patients out of these twenty underwent an embolisation during the pre-op arteriography. Studying the results, the authors show how much this embolisation increases the risk of a per-op carotid resection with by-pass, especially for the carotid localization. Dropping the embolisation, gives a chance for a new and, more acute diagnostic strategy, based on computerized tomography and digitalized arteriography. These exams should avoid the per-op discovery of these tumors in more than one half of the cases during an exploratory initial cervicotomy.

Adolescent↗