Management of jugular tympanic paraganglioma: a case report.

Paragangliomas could be localized from the skull base to the pelvic floor. Tympanic localization represents the most common benign tumor of the middle ear. Diagnosis is based on clinical signs with a great contribution of radiology. A 40-year-old male presented with isolated tinnitus of the right ear evolving for 18 months. Examination revealed a red bulging right-sided tympanic membrane and a conductive hearing loss. Tomodensitometry and Magnetic resonance imagery showed findings in favor of a right jugular tympanic paraganglioma. The tumor was classified type B according to FISCH classification. The patient underwent surgery consisting in tympanotomy using a retro auricular access route. The postoperative course was uneventful. There was no recurrence during the one-year follow-up. Jugular tympanic paraganglioma diagnosis is guided by a combination of epidemiological, clinical and radiological features. Treatment is still not consensual, but surgery still have its indications in localized forms of head and neck paragangliomas (HNP´s).

Hereditary Neuroendocrine Tumors: Providing Comprehensive Care for Individuals Who Have a Germline Pathogenic Variant Associated With Paragangliomas and Pheochromocytomas.

Both paragangliomas and pheochromocytomas can be associated with germline pathogenic variants. Although these neuroendocrine tumors are relatively rare, the identification of patients and families with germline risk enables the implementation of surveillance programs to decrease the morbidity and mortality associated with these tumors. Individuals with germline risk require lifelong screening, which is implemented as early as age 5 years. In addition to ensuring that surveillance protocols are implemented, nurses provide education about symptoms that require prompt evaluation.

Development of a Function-Integrative Sleeve for Medical Applications.

Function-integrative textiles bear the potential for a variety of applications in the medical field. Recent clinical investigations suggest that the application of a function-integrative fabric could have a positive impact on currently applied diagnostic procedures of a specific type of tumour. In this context, the fabric should enable local warming of a patient's upper extremity as well as blood flow measurement. Existing solutions comprise a warming a warming system but lack a measuring apparatus for blood flow determination. With regard to the quality of results of current diagnostic procedures, the local warming of the patients' upper extremity and the simultaneous determination of the blood flow plateau are crucial. In the present paper, the development process of a function-integrative sleeve is introduced. Besides the development of an adaptable sleeve-design, the manufacturing process of an integrated warming system was also addressed. Furthermore, the identification of crucial physiological effects, using a Laser Doppler Perfusion Monitor, is introduced. During testing of the function-integrative sleeve, modulation of the desired physiological effects was observed. The results support the initial assumptions and dictate further investigations on increasing user-friendliness and cost-efficiency during adjusting and determining the physiological effects in the course of tumour diagnosis.

Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma.

PURPOSE: To evaluate the safety and efficacy of high-dose [(131)I]metaiodobenzylguanidine ([(131)I]MIBG) in the treatment of malignant pheochromocytoma (PHEO) and paraganglioma (PGL). METHODS: Fifty patients with metastatic PHEO or PGL, age 10 to 64 years, were treated with [(131)I]MIBG doses ranging from 492 to 1,160 mCi (median, 12 mCi/kg). Cumulative [(131)I]MIBG administered ranged from 492 to 3,191 mCi. Autologous hematopoietic stem cells were collected and cryopreserved before treatment with [(131)I]MIBG greater than 12 mCi/kg or with a total dose greater than 500 mCi. Sixty-nine [(131)I]MIBG infusions were given, which included infusions to 35 patients treated once and infusions to 15 patients who received two or three treatments. Response was evaluated by [(123)I]MIBG scans, computed tomography/magnetic resonance imaging, urinary catecholamines/metanephrines, and chromogranin A. RESULTS: The overall complete response (CR) plus partial response (PR) rate in 49 evaluable patients was 22%. Additionally, 35% of patients achieved a CR or PR in at least one measure of response without progressive disease, and 8% of patients maintained stable disease for greater than 12 months. Thirty-five percent of patients experienced progressive disease within 1 year after therapy. The estimated 5-year overall survival rate was 64%. Toxicities included grades 3 to 4 neutropenia (87%) and thrombocytopenia (83%). Grades 3 to 4 nonhematologic toxicity included acute respiratory distress syndrome (n = 2), bronchiolitis obliterans organizing pneumonia (n = 2), pulmonary embolism (n = 1), fever with neutropenia (n = 7), acute hypertension (n = 10), infection (n = 2), myelodysplastic syndrome (n = 2), and hypogonadism (n = 4). CONCLUSION: Although serious toxicity may occur, the survival and response rates achieved with high-dose [(131)I]MIBG suggest its utility in the management of selected patients with metastatic PHEO and PGL.

Whole-body iodine-131 metaiodobenzylguanidine imaging for detection of bone metastases in patients with paraganglioma: comparison with bone scintigraphy.

Iodine-131 metaiodobenzylguanidine ((131)I-MIBG) therapy is an effective treatment for patients with malignant paraganglioma for which surgical resection is not indicated. We performed high-dose (131)I-MIBG therapy on two patients with malignant paraganglioma and multiple bone metastases. The bone metastases were diagnosed by magnetic resonance imaging (MRI). Metastatic bone lesions were evaluated by whole-body (131)I-MIBG imaging and bone scintigraphy. Whole-body (131)I-MIBG imaging showed extensive metastatic bone lesions, whereas conventional bone scintigraphy did not. There was a remarkable discrepancy between (131)I-MIBG imaging and bone scintigraphy in the diagnosis of metastatic bone lesions of malignant paraganglioma in our two patients. High-dose (131)I-MIBG imaging may detect early stages of bone metastases, compared with bone scintigraphy, in patients with malignant paraganglioma.

Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG).

Thirty patients with malignant pheochromocytoma (PHEO) or paraganglioma (PGL) were treated with high-dose 131I-MIBG. Patients were 11-62 (mean 39) years old: 19 patients males and 11 females. Nineteen patients had PGL, three of which were multifocal. Six PGLs were nonsecretory. Eleven patients had PHEO. All 30 patients had prior surgery. Fourteen patients were refractory to prior radiation or chemotherapy before 131I-MIBG. Peripheral blood stem cells (PBSCs) were collected and cryopreserved. 131I-MIBG was synthesized on-site, by exchange-labeling 131I with 127I-MIBG in a solid-phase Cu2+-catalyzed exchange reaction. 131I-MIBG was infused over 2 h via a peripheral IV. Doses ranged from 557 mCi to 1185 mCi (7.4 mCi/kg to 18.75 mCi/kg). Median dose was 833 mCi (12.55 mCi/kg). Marrow hypoplasia commenced 3 weeks after 131I-MIBG therapy. After the first 131I-MIBG therapy, 19 patients required platelet transfusions; 19 received GCSF; 12 received epoeitin or RBCs. Four patients received a PBSC infusion. High-dose 131I-MIBG resulted in the following overall tumor responses in 30 patients: 4 sustained complete remissions (CRs); 15 sustained partial remissions (PRs); 1 sustained stable disease (SD); 5 progressive disease (PD); 5 initial PRs or SD but relapsed to PD. Twenty-three of the 30 patients remain alive; deaths were from PD (5), myelodysplasia (1), and unrelated cause (1). Overall predicted survival at 5 years is 75% (Kaplan Meier estimate). For patients with metastatic PHEO or PGL, who have good *I-MIBG uptake on diagnostic scanning, high-dose 131I-MIBG therapy was effective in producing a sustained CR, PR, or SD in 67% of patients, with tolerable toxicity.

Case report: patient with multiple paragangliomas treated with long acting somatostatin analogue.

Paragangliomas of the head and neck are uncommon neoplasms. They are usually benign, but tend to be locally invasive. Although surgical resection remains the definitive treatment, important issues about management arise when such lesions are inoperable. Beneficial effects of octreotide treatment have already been reported in a malign paraganglioma case. Here we report a 24 year old female with familial, bilateral, multiple paraganglioma in the head and neck region, who firstly presented with pulsatile tinnitus and hearing loss in her left ear. After embolization was performed, she underwent operation twice because of the gross tumor mass. No significant change in tumor size was determined after the operations, however there were no distant metastases. Although she experienced hypertension attacks, no hormonal overproduction was found in repeated measurements. As the tumor was unresectable, new alternative therapies were sought. Octreotide scintigraphy was positive in the tumoral tissue, so we began to treat her with somatostatin analogue octreotide. After a 16 month follow up period, an improvement of the performance status, the near normalisation of attacks and stabilization of tumor growth were achieved. However, in the last three visits, she began to experience symptoms more frequently and it had been necessary to increase the octreotide dose. She is now well and being followed up. In conclusion, the beneficial effects of octreotide treatment could be quantified by clinical, tumor and scintigraphic criteria. These data suggest that octreotide can be useful in the treatment of inoperable paragangliomas.

[Secreting paraganglioma of the organ of Zuckerkandl. A case report]. / Paragangliome sécrétant de l'organe de Zuckerkandl. A propos d'un cas.

We report a case of a secreting retroperitoneal paraganglioma which developed on the organ of Zuckerkandl. Paraganglioma is a rare tumor of the paraganglioma system arising from undifferentiated cells of the neural crest. The originality of our observation comes from the secreting character of the tumor and the malignity proved by the local spreading to the wall of the inferior vena cava and the metastasis. The probable existence of a double primary localisation is also exceptional.

[Secreting retroperitoneal paraganglioma. Apropos of a case]. / Paragangliome rétropéritonéal sécrétant. A propos d'un cas.

A case of secreting, asymptomatic retroperitoneal paraganglioma is presented. Paragangliomas are rare neuroendocrine tumors and the malignant behaviour, higher than pheochromocytomas, is based on metastases or local recurrence. The treatment is complete surgical excision. Radiotherapy and chemotherapy may be used for palliation of symptoms.

Tumores del corpúsculo carotídeo y otros paragangliomas cervicales / Carotid body tumors and others paragangliomas in the neck

Se presentan cinco paragangliomas en el área de cabeza y cuello, tres mujeres y dos hombres, entre la cuarta y sexta décadas de vida. Tres tumores del corpúsculo carotídeo, un glomus yugular y un quemodectoma vagal. El diagnóstico se basó en la sospecha clínica, angiografía, TAC y/o RNM y eventual punción aspirativa. Los cinco casos fueron tratados quirúrgicamente, todos los tumores fueron benignos. Se utilizó radioterapia como tratamiento complementario en un caso, (glomus yugular). Como complicación intraoperatoria se produjo una ruptura de la arteria carótida en su bifurcación, (glomus carot. tipo III), resuelto con un shunt externo, resección del área afectada y anastomosis termino terminal. Se observaron dos parálisis faringo laríngeas, luego de una resección dificultosa del X par, (quemodectoma vagal y glomus carotídeo tipo II), con episodios de microaspiración y voz áfona. La enferma con un glomus yugular con parálisis facial (secuela quirúrgica previa), presentó una paresia velofaríngea, y recuperación del 50 por ciento de su parálisis facial luego de un injerto nervioso. Los tumores glómicos siempre han despertado interés, no sólo por su rareza, sino también por el desafío que implica se resección, especialmente en los de gran tamaño. La experiencia con cinco enfermos en el transcurso de dos años, motiva esta comunicación (AU)

Tumores del corpúsculo carotídeo y otros paragangliomas cervicales / Carotid body tumors and others paragangliomas in the neck

Se presentan cinco paragangliomas en el área de cabeza y cuello, tres mujeres y dos hombres, entre la cuarta y sexta décadas de vida. Tres tumores del corpúsculo carotídeo, un glomus yugular y un quemodectoma vagal. El diagnóstico se basó en la sospecha clínica, angiografía, TAC y/o RNM y eventual punción aspirativa. Los cinco casos fueron tratados quirúrgicamente, todos los tumores fueron benignos. Se utilizó radioterapia como tratamiento complementario en un caso, (glomus yugular). Como complicación intraoperatoria se produjo una ruptura de la arteria carótida en su bifurcación, (glomus carot. tipo III), resuelto con un shunt externo, resección del área afectada y anastomosis termino terminal. Se observaron dos parálisis faringo laríngeas, luego de una resección dificultosa del X par, (quemodectoma vagal y glomus carotídeo tipo II), con episodios de microaspiración y voz áfona. La enferma con un glomus yugular con parálisis facial (secuela quirúrgica previa), presentó una paresia velofaríngea, y recuperación del 50 por ciento de su parálisis facial luego de un injerto nervioso. Los tumores glómicos siempre han despertado interés, no sólo por su rareza, sino también por el desafío que implica se resección, especialmente en los de gran tamaño. La experiencia con cinco enfermos en el transcurso de dos años, motiva esta comunicación

Iodine-131-metaiodobenzylguanidine scintigraphy in preoperative and postoperative evaluation of paragangliomas: comparison with CT and MRI.

Iodine-131-metaiodobenzylguanidine (MIBG) scintigraphy, transmission computed tomography and magnetic resonance imaging were used to evaluate 36 patients with clinically suspected functioning paragangliomas. The patients were divided into two groups. In Group 1 (n = 21), studied before surgery, patients mainly had benign adrenal disease. In Group 2 (n = 15), studied after surgery, patients frequently had malignant or extra-adrenal tumors. In Group 1, transmission computed tomography and magnetic resonance imaging were more sensitive (100% for both) than MIBG scintigraphy (82%), which, however, was the most specific (100%). In Group 2, MIBG scintigraphy and magnetic resonance imaging were more sensitive (83% for both) than transmission computed tomography (75%), but MIBG was again the most specific (100%). Thus, all three were complementary modalities for localizing paragangliomas both preoperatively and postoperatively. MIBG imaging is indicated for both groups but it is especially recommended for postsurgical patients with recurrence because the disease is often malignant or extra-adrenal.