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[Tympanic and jugular paragangliomas. I. Clinical symptomatology and evaluation of extension. Apropos of 25 cases].

Based on a review of 25 cases of tympanic and jugular paragangliomas treated and followed up in the Lariboisière hospital, Paris between 1978 and 1986, clinical aspects and extension of these tumors are studied. The often long delay in making the diagnosis is related to lack of knowledge of presenting symptomatology, frequent discordance between functional and physical symptomatology exists, but remarkable progress has been made in assessment of extension of tumors using new imaging techniques, particularly angiography, which can also guide the preoperative embolization and surgical procedures.

Adult↗

[Tympanic and jugular paragangliomas. II. Angiographic, surgical and irradiation treatment. Results. Indications. Apropos of 25 cases].

Results of treatment are reviewed in 25 cases of tympanic and jugular paragangliomas treated and followed up in the Lariboisière hospital, Paris between 1978 and 1986. Therapy has been transformed by the use of embolization but its non-negligible neurologic accident risks, whatever the carotid artery territory involved, justify prior weighing of indications for its application. New surgical technics allow more extensive exeresis, but intrapetrous extension to the nerve compartment of the jugular foramen and the anterior pericarotid region still raises difficult surgical problems and appears to be the principal reason for failure of treatment. Radiotherapy is very effective and applicable for inoperable forms, as a complement to incomplete surgical removal and for recurrences.

Cerebral Angiography↗

[Hormone-active paragangliomas of the base of the skull].

Biochemical examination of 15 patients with paragangliomas (PG) of the base of the skull was conducted. The content of epinephrine, and norepinephrine, dopamine, and dopa in urine and the content of serotonin and histamine in blood and tumor tissue were studied. Comparison of the biochemical findings with the vegetative symptomatology allowed 3 groups of PG to be distinguished: catecholamine-secreting, serotonin-secreting, and hormone-inactive. The group of serotonin-secreting tumors of the base of the skull has not been described in the literature. The conducted research shows that tests for biogenic amines must be performed not only for diagnostic purposes but also for prognosticating possible surgical complications.

Adolescent↗

[Angiographic and surgical control of the vertebral and carotid pedicles supplying giant paraganglioma of the skull base and neck].

Results obtained after excision of large jugular paragangliomas are largely conditioned by the possibility of control of the different vascular pedicles, and existing possibilities are discussed in relation to 6 briefly presented cases. The internal carotid artery can either be occluded radiologically by balloon catheter and then embolized if the artery is surrounded by a tumor, or controlled surgically if the artery is in contact with the tumor only and provides only a small proportion of its vascular supply. Whenever internal carotid artery occlusion is envisaged a clamping test by balloon under local anaesthesia is carried out initially. The vertebral artery pedicles are interrupted during surgical approach to the artery above the atlas. The value of a 2 or even 3 stage operation for excision of very large tumors with posterior fossa extension is discussed.

Adult↗

[Jugulotympanic paraganglioma].

A case is described of a jugulotympanic paraganglioma in a 64-year-old woman with extentions down the jugular vein to the clavicle and penetration of the cerebellar fossa. The symptoms, differential diagnosis, therapy and prognosis are discussed on the basis of this case report and compared with the literature. The importance of radiological investigation (e.g. selective angiography and computed tomography) in respect to diagnosis and determination of tumour size is emphasized. The diagnostic value of the immunohistochemical detection of neuron-specific enolase, a neuroendocrine cell marker and S-100 protein is stressed. The most favourable therapy, depending on tumour extension, seems to comprise preoperative embolisation, radical resection and postoperative radiotherapy.

Diagnosis, Differential↗

Expression of neurofilament triplet proteins in human neural tumors. An immunohistochemical study of paraganglioma, ganglioneuroma, ganglioneuroblastoma, and neuroblastoma.

Intermediate filaments which are specific to neural cells, ie, neurofilaments, consist of three subcomponents--68, 150, and 200 kd. Thirty human neural tumors were examined for the presence of these three subcomponents by means of their monospecific antisera. All 8 paragangliomas contained cells that were positive for the 68-kd component, but only 5 of them had cells positive for the 150-kd and 200-kd components. All 4 ganglioneuromas and 11 ganglioneuroblastomas contained cells that reacted with antibodies to all three components. All 7 neuroblastomas had cells reacting with antibody to 68 kd, but only 3 of them had cells that reacted with antibodies to 150 kd and 200 kd. In each case, the number of positive cells depended on the antibody used. The largest number reacting with antibody to 68 kd and the smallest with antibody to 200 kd. Furthermore, it was possible to detect tumor cells in which the 68-kd subcomponent existed by itself, but no tumor cells in which the 150-kd or 200-kd subcomponent existed alone could be detected. These results seem to indicate that antibody to the 68-kd component is sufficiently discriminating to be applied diagnostically.

Adult↗

Intravagal paraganglioma: report of a case and a discussion of vascular parapharyngeal masses.

A case of intravagal paraganglioma that appeared as a parapharyngeal mass 1 year after tonsillectomy is discussed. The diagnosis of this lesion was based on angiographic findings, and subtraction views provided the best detail of the extent and nature of the mass. Excision was made nearly bloodless by initial ligation of the external carotid artery, followed by the application of direct pressure to the mass during dissection. The diagnosis of parapharyngeal vascular masses is discussed, and the pertinent literature is reviewed.

Adult↗

[A masked paraganglioma--a rare cause of pain in lymphedema of the arm].

The history is reported of a 66 year-old patient who suffered from postmastectomy edema and continuous pain on the ulnar side of the wrist. After microsurgical lymphatic grafting the edema diminished. At the site of pain there was a resistance palpable with reduced resonance on ultrasonography. At operation the authors excised a tumor which on histological and immunohistochemical investigation was compatible with a paraganglioma.

Aged↗

[A case of anisosynchronously duplicated tumors consisting of leiomyosarcoma of the rectum and malignant paraganglioma of gluteal soft tissue].

A 62-year-old man received a Miles' operation for the recurrence of low grade rectal leiomyosarcoma which had been enucleated 8 years earlier. The tumor, measuring 11 x 12 cm, was located in the lower rectal area (Rb) and had developed into the pelvic cavity. One year after operation, after the uncovery of a malignant paraganglioma in the right gluteal soft tissue, the patient died from the metastases of the liver and the lung, and from lymph node that covered his body. This case is a rare example of anisosynchronously occurring duplicated carcinomas.

Cytoplasmic Granules↗

Paragangliomas of the head and neck.

Nonchromaffin paragangliomas of the head and neck are usually benign, slow-growing lesions arising from neural crest tissue. Although uncommon, these neoplasms should always be considered in the differential diagnosis of a neck mass. The diagnostic and therapeutic challenges posed by these lesions require continued clinical attention.

Adult↗

Gangliocytic paraganglioma associated with duodenal adenocarcinoma. Case report with immunohistochemical evaluation.

We present the first reported case (to our knowledge) of duodenal gangliocytic paraganglioma (GPG) to be associated with an underlying invasive adenocarcinoma. The patient, a 71-year-old man, presented with epigastric tenderness and was found to have metastatic adenocarcinoma in two regional lymph nodes. Immunohistochemical evaluation of the GPG demonstrated positive staining for gastrin, glial-fibrillary acidic protein, glucagon, neuron-specific enolase, pancreatic polypeptide, S100 protein, somatostatin, and substance P. The clinical, pathologic, and immunohistochemical features of GPG are discussed, with a review of the literature.

Adenocarcinoma↗

Malignant paraganglioma of the thyroid gland.

Paragangliomas of the thyroid gland are rare lesions, only four previous cases having been reported in the literature, to our knowledge. We report herein a fifth case, which, to our knowledge, is the first to exhibit malignant behavior manifested by invasion into perithyroidal soft tissue through the wall of the trachea into the tracheal mucosa.

Biopsy, Needle↗

Simultaneous local recurrence of retroperitoneal paraganglioma with liver metastases.

A 68-year-old woman with a history of a nonfunctional paraganglioma resected 23 years previously presented with an abdominal mass. Tumor metastases to the liver were seen at the second operation. The tumor had a more pleomorphic appearance, but was otherwise similar to the original tumor. We discuss the difficulty of predicting the later malignant behavior of the tumor from the original biopsy specimen.

Aged↗

[Multiple primary malignant paragangliomas of the bladder and retroperitoneal space].

An observation of the primarily multiple malignant paraganglioma of the urinary bladder and retroperitoneal space in combination with multiple uterine fibromyomas in a woman of 46 is described. Duration of the hypertension was 30 years. Hypertension crisis (up to 300/180 mm Hg) were usually associated with the urine discharge. Surgical removal of tumours was followed by a severe hypertension due to the catecholamine release into the circulation. Grimelius method confirmed the presence of endocrine granules in the cytoplasm of tumour cells. The role of chronic endocrine pathology in the evolution of extra-adrenal chromaffinomas and some questions of morphogenesis are discussed.

Female↗

[Paraganglioma of the nasal cavity].

Extraadrenal paragangliomas are very rarely located in the nose and the nasal sinus. Histological and clinical findings of this tumour are discussed.

Adult↗

Laryngeal paraganglioma. A case report.

The treatment of a 27-year-old woman with a superior paraganglioma is described. The tumour was excised with a partial supraglottic laryngectomy after repeated biopsies showed chronic inflammatory tissue. The patient has shown no evidence of malignancy during a 4-year follow-up.

Adult↗