Preoperative Endovascular Embolization of Glomus Jugulare Tumors: A Retrospective Case Series of 22 Embolizations in 20 Patients and Literature Review

Objective Glomus jugulare tumors, or tympanojugular paragangliomas, are rare, highly vascularized skull base tumors originated from paraganglion cells of the neural crest. With nonabsorbable embolic agents, embolization combined with surgery has become the norm. The authors assess the profile and outcomes of patients submitted to preoperative embolization in a Brazilian tertiary care hospital. Methods The present study is a single-center, retrospective analysis; between January 2008 and December 2019, 22 embolizations were performed in 20 patients in a preoperative character, and their medical records were analyzed for the present case series. Results Hearing loss was the most common symptom, present in 50% of the patients, while 40% had tinnitus, 30% had dysphagia, 25% had facial paralysis, 20% had hoarseness, and 10% had diplopia. In 7 out of 22 embolization procedures (31%) more than a single embolic agent was used; Gelfoam (Pfizer, New York, NY, USA) was used in 18 procedures (81%), in 12 of which as the single agent, followed by Embosphere (Merit Medical, South Jordan, UT, USA) (31%), Onyx (Medtronic, Minneapolis, MN, USA) (9%), and polyvynil alcohol (PVA) and Bead Block (Boston Scientific, Marlborough, MA, USA) in 4,5% each. The most common vessel involved was the ascending pharyngeal artery, involved in 90% of the patients, followed by the posterior auricular artery in 15%, the internal maxillary artery or the occipital artery in 10% each, and the superficial temporal or the lingual arteries, with 6% each. Only one patient had involvement of the internal carotid artery. No complications from embolization were recorded. Conclusions Preoperative embolization of glomus tumors is safe and reduces surgical time and complications, due to the decrease in size and bleeding.

Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity

Paragangliomas are rare, benign and slow-growing neuroendocrine tumors that can arise from the adrenal medulla (85­90%) or from the extra-adrenal paraganglia. In the central nervous system (CNS), they can be found at several sites, but more often at the cauda equina and filum terminale region, where they account for between 2.5 and 3.8% of total tumor cases of that region. There are only 8 cases described in the literature that mention the presence of the gangliocytic variant of this entity at the filum terminale. We present the case of a 41-year-old man with chronic lumbar pain refractory to medical treatment, without any associated neurological deficits. Magnetic resonance imaging (MRI) revealed an intradural, extramedullar oval lesion with regular contours and homogeneous caption of contrast at L1 level. He was submitted to surgical treatment, with complete resection of the lesion. The histological analysis revealed a gangliocytic paraganglioma of the filum terminale. At 5 years of follow-up, he remains asymptomatic and without any signs of relapse. These are lesions with an overall good prognosis with gross total resection. Although the recurrence rate is extremely low, prolonged observation is recommended due to the slow-growing nature of the tumor, being estimated that between 1 and 4% can recur even after gross total removal.

Gangliocytic paraganglioma of the spine

Paragangliomas are rare, encapsulated, benign neuroendocrine tumors that can arise from the adrenal medulla or extra-adrenal paraganglia. Extra-adrenal paragangliomas may develop a gangliocytic component with ganglion cells (Gangliocytic paragangliomas). Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. Here, we describe the case of a 35-year-old male who presented with weakness of both lower limbs over the last two months. Radiological findings were suggestive of myxopapillary ependymoma. However, the histopathological examination revealed a tumor with cells arranged in sheets, papillae, lobules, and around vessels forming pseudo rosettes. Ganglion cells were seen in small groups and, also singly. Tumor cells were immunopositive for chromogranin, synaptophysin, and S-100. Ganglion cells were immunopositive for synaptophysin, NSE, and NFP. A final histological diagnosis of Gangliocytic paraganglioma (WHO grade I) was made. To date, only nine gangliocytic paraganglioma cases have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma.

Paraganglioma yugular bilateral: reporte de un caso y revisión de la literatura / Bilateral jugular paraganglioma: case report and review of the literature

Resumen El paraganglioma (PG) es una neoplasia infrecuente originada de las células paraganglionares, embriológicamente derivadas de la cresta neural. Se localizan en la cabeza, base de cráneo, cuello, mediastino, abdomen y pelvis. La mayor parte de los PG muestran un curso clínico benigno, sin embargo, algunos casos pueden mostrar un comportamiento biológico agresivo con invasión local y metástasis a distancia. Un avance significativo en patología molecular ha sido el reconocimiento que el 30%-40% de estas neoplasias presentan alteraciones genéticas. Se han descrito más de 45 genes involucrados, incluyendo mutaciones de la línea germinal succinato deshidrogenasa. Actualmente se recomienda hacer test genético a todos los portadores de PG incluyendo los de presentación esporádica. El PG más frecuente se ubica en la glándula suprarrenal llamado feocromocitoma. El diagnóstico definitivo se realiza con histología, sin embargo, el estudio imagenológico puede entregar una aproximación diagnóstica certera. Debido a la aceptación actual que todos los PG tienen potencial metastásico, el concepto de PG benigno y maligno ha cambiado a uno de estimación de riesgo de metástasis, aunque no existe un esquema único aceptado para tal efecto. El tratamiento considera la cirugía, la radioterapia, la observación y terapias combinadas. Dado el lento crecimiento de este tipo de neoplasia y las potenciales complicaciones de la terapia quirúrgica, la observación es una opción especialmente para pacientes añosos dejando las otras opciones para pacientes más jóvenes. En este trabajo se presenta un caso de paraganglioma yugular bilateral gigante tratado con radioterapia de intensidad modulada incluyendo una revisión bibliográfica pertinente.

Abstract Paraganglioma (PG) is a rare neoplasm derived from paraganglionic cells of the neural crest. They are located in the head, skull base, neck, mediastinum, abdomen and pelvis. Most PGs show a benign clinical course, however, some cases may show aggressive biological behavior with local invasion and distant metastasis. A significant advance in molecular pathology has been the recognition that 30%-40% of these neoplasms present genetic alterations; more than 45 genes have been described, including mutations of the germline succinate dehydrogenase. Currently it is recommended to make genetic test to all patients with PG, including sporadic presentation. The most frequent PG is located in the adrenal gland called pheochromocytoma. The definitive diagnosis is made with histology; however, the imaging study can provide an accurate diagnostic approach. It is now accepted that all PG have a metastatic potential, therefore the concept of benign or malignant has been changed to a metastasis risk stratification approach however no single scheme is been widely used. The treatment considers surgery, radiotherapy, observation and combination therapies. Given the slow growth of this type of neoplasia and the potential complications of surgical therapy, observation is an option especially for elderly patients leaving the other options for younger patients. In this work we present a case of giant bilateral jugular paraganglioma treated with intensity modulated radiation therapy, including a pertinent literature review.

Non-functional paraganglioma of urinary bladder managed by transurethral resection

ABSTRACT Purpose As a rare bladder tumor, paraganglioma of the urinary bladder (PUB) is frequently misdiagnosed as bladder cancer, particularly for the non-functional type. To date, transurethral resection remains a controversial treatment for non-functional PUB. This study aimed to identify the clinical features, pathological characteristics, prognosis, and safe/effective treatment of non-functional PUB using transurethral resection of the bladder tumor (TURBT). Materials and Methods The clinical records, radiological data, pathological characteristics and follow-up times were retrospectively reviewed in 10 patients with clinically and pathologically proven non-functional PUB in our hospital from January 2008 to November 2016. All patients underwent TURBT treatment. Results The incidence of non-functional PUB in patients with bladder cancer was 0.17%. The mean age at diagnosis was 44.5 ± 13.6 years (range, 29-70 years), and the patient population had a female: male ratio of 3: 2. No patients had excess catecholamine (CA) whilst four patients had painless hematuria. All neoplasms were completely resected via TURBT. The majority of samples were positive for immunohistochemical markers including chromogranin A (CgA) and Synaptophysin (Syn), but were negative for cytokeratins (CKs). Only a single recurrence was observed from the mean follow-up period of 36.4 ± 24.8 months. Conclusion Complete TURBT is a safe and efficient treatment that serves both diagnostic and therapeutic purposes. Histopathological and immunohistochemistry examinations are mandatory for diagnostic confirmation. Long-term follow-up is recommended for patients with non-functional PUB.

Primary malignant tumors of the adrenal glands

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

A rare case of ectopic ACTH syndrome originating from malignant renal paraganglioma

SUMMARY Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of a non-pituitary ACTH-secreting tumor leading to Cushing's syndrome. Only a few cases have been reported previously as causing ectopic ACTH related to paraganglioma. Herein, we present a case of Cushing's syndrome, in who was proved to be attributable to an ACTH-secreting renal malignant paraganglioma. A 40-year-old woman presented with a five-month history of newly diagnosed hypertension and diabetes, weakness, hyperpigmentation, oligomenorrhea, hirsutism, and acneiform lesions. She showed cushingoid features, including moon face, facial hirsutism, facial and truncal acne, hyperpigmentation, and severe muscle weakness of the limbs. She did not have other findings such as striae, supraclavicular fat accumulation, and buffalo hump. Laboratory examination showed the presence of hypopotasemia, hyperglycemia, hyperthyroidism, and leukocytosis. The serum levels of ACTH, cortisol, and urine-free cortisol were markedly elevated. Results of an overnight 2-mg dexamethasone suppression test included a basal serum cortisol of 61.1 mcg/dL (normal range: 4.6-22.8 mcg/dL) and a cortisol value of 46.1 mcg/dL after dexamethasone administration. There was no suppression found after 2-day 8-mg dexamethasone administration. Magnetic resonance imaging (MRI) of the pituitary gland indicated two microadenomas. An abdominal MRI scan revealed horseshoe kidney, bilateral adrenal hyperplasia, and masses with dimensions of 35 x 31 mm in the left kidney. Inferior petrosal sinus sampling showed no evidence of a central-to-peripheral gradient of ACTH. A positron emission tomography/computed tomography scan showed intense increased activity in the lower pole of the left kidney. Left adrenalectomy and left partial nephrectomy were performed. The resected tumor was diagnosed as the ACTH-secreting paraganglioma in the pathological examination, which was confirmed by immunohistochemical studies with chromogranin A, synaptophysin, and ACTH. Only a few cases of paragangliomas as a cause of ectopic ACTH syndrome have been reported. To our knowledge, this is the first case of renal paraganglioma resulting in Cushing's syndrome due to ectopic ACTH hypersecretion.

Endoscopic Resection as a Possible Radical Treatment for Duodenal Gangliocytic Paraganglioma: A Report of Four Cases / 대한소화기학회지

Gangliocytic paraganglioma (GP) is a rare, benign tumor which is usually found in the duodenum. We here report four recent cases of GP, with successful endoscopic resection in three cases, including a lesion on the ampulla of Vater. In all cases, each lesion had a stalk that facilitated removal using an endoscopic approach. Endoscopic mucosal resection is a feasible and safe treatment if the location, depth, and lymph node status are all favorable and is also helpful for definite diagnosis of unknown duodenal mass. To avoid morbidity resulting from open surgical resection, careful inspection for the peduncle of the GP will help determine the feasibility of endoscopic resection.

Osseous metastase of occult paraganglioma: a diagnostic medical error

Diagnostic errors have a natural complexity. Medical diagnoses make up a large proportion of all medical errors and cause much suffering and harm. Compared to other types of error, diagnostic errors receive little attention-a major factor in continuity of unacceptable rates of diagnostic error. A 55-year-old woman presented to the emergency department [ED] complaining of bone pain which has been started a month ago and increased gradually in the upper right thigh. Following the emergency evaluation she was sent home with pain medication. On the second visit, a femur neck fracture was seen in the x-ray. She underwent hemiarthroplasty and was discharged. Over several weeks she was reevaluated by many Physicians, because of her worsening pain .In the third visit after the surgery, her x-ray showed bone destruction and following bone biopsy, malignant paraganglioma was diagnosed. In all cases in which patient comes to us with skeletal pain, getting a comprehensive history and a full physical examination are prior to lab tests and x-rays. Bone metastasis which can develop severe pain and pathological fractures, is common in patients with malignant paraganglioma. Effective steps for diagnostic error prevention are: Considering the diagnostic error in the normal range of quality assurance surveillance and review, identifying the elements leading to diagnostic errors and getting feedback on the diagnoses Physicians make, in order to improve their skills. It is an every health system priority to identify, analyze, and prevent diagnostic errors in order to improve patient safety.

Head and neck paragangliomas: clinical and molecular genetic classification

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

Paraganglioma of seminal vesicle and chromophobe renal cell carcinoma: a case report and literature review / Paraganglioma da vesícula seminal e carcinoma de células renais cromófobo: um relato de caso e revisão da literatura

CONTEXT: Extra-adrenal paragangliomas are rare tumors that have been reported in many locations, including the kidney, urethra, urinary bladder, prostate, spermatic cord, gallbladder, uterus and vagina. CASE REPORT: This report describes, for the first time to the best of our knowledge, a primary paraganglioma of the seminal vesicle occurring in a 61-year-old male. The patient presented persistent arterial hypertension and a previous diagnosis of chromophobe renal cell carcinoma. It was hypothesized that the seminal vesicle tumor could be a metastasis from the chromophobe renal cell carcinoma. Immunohistochemical characterization revealed expression of synaptophysin and chromogranin in tumor cell nests and peripheral S100 protein expression in sustentacular cells. Succinate dehydrogenase A and B-related (SDHA and SDHB) expression was present in both tumors. CONCLUSIONS: No genetic alterations to the VHL and SDHB genes were detected in either the tumor tissue or tissues adjacent to the tumor, which led us to rule out a hereditary syndrome that could explain the association between paraganglioma and chromophobe renal cell carcinoma in a patient with arterial hypertension.

CONTEXTO: Paragangliomas extra-adrenais são tumores raros que têm sido relatados em muitas localizações, incluindo rim, uretra, bexiga, próstata, cordão espermático, vesícula biliar, útero e vagina. RELATO DE CASO: Este relato descreve, pela primeira vez em nosso conhecimento, um paraganglioma primário da vesícula seminal ocorrendo em um paciente do sexo masculino de 61 anos de idade. O paciente apresentou hipertensão arterial persistente e um diagnóstico prévio de carcinoma de células renais cromófobo (CCRC). Foi pensado que o tumor de vesícula seminal poderia ser uma metástase do CCRC. A caracterização imunoistoquímica revelou expressão de sinaptofisina e cromogranina nos ninhos de células tumorais e expressão de proteína S100 nas células sustentaculares. Expressão de succinato de-hidrogenase A e B relacionada (SDHA e SDHB) estiveram presentes em ambos os tumores CONCLUSÕES: Nenhuma alteração genética dos genes VHL e SDHB foi detectada nos tecidos tumorais e adjacentes ao tumor, o que nos levou a afastar uma síndrome hereditária que poderia explicar a associação entre o paraganglioma e o CCRC em um paciente com hipertensão arterial.

decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: report of 67 cases and a literature review

The purpose was to highlight the diagnosis and treatment of extra-adrenal para-gangliomas, which often causes catecholamine hypersecretion and hypertension. 67 cases of extra-adrenal paraganglioma of retroperitoneum proven pathologically from 1999 to 2009 were reviewed and studied after operation. Endocrine secretion examinations, B-US, CT, MRI, 131-MIBG, octreotide and hands microcirculation inspection were used to diagnose the disease. All patients underwent successful surgical resection of the tumors, which proved to be paragangliomas. They were from 3 cm to 25 cm in size. Almost all of them were diffusely positive for cgA, syn, NSE and s-100 by immunohitochemical staining. There were nine cases assayed malignant paraganglioma by the follow-up. 131-MIBG and octreotide have high sensitivity and accuracy in diagosing extra-adrenal paraganglioma. Surgical treatment should be carried out on the basis of correct drug preparation of a-receptor blocker, such as prazosin and phenoxybenzamine. Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as recurrent or metastatic disease, which could be resected laparoscopically. Intimate lifelong follow-up is necessary and important

Relevancia de la medicina nuclear en el diagnóstico y tratamiento del paraganglioma maligno / Relevance of nuclear medicine in the diagnosis and treatment of malignant paraganglioma

Introducción: Los paragangliomas son tumores del tejido cromafín caracterizados por la síntesis o secreción de catecolaminas, en los que el tratamiento depende de su extensión y características funcionales. Dentro de la evaluación diagnóstica anatómica y funcional de este padecimiento, los métodos de imagen de medicina nuclear sobresalen por su utilidad. Caso clínico: Paciente con paraganglioma a nivel de la bifurcación de la aorta en quien el diagnóstico se realizó por estudios de laboratorio, imagenología y medicina nuclear. Se llevó a cabo centelleografía con un análogo de norepinefrina, la metayodobencilguanidina marcada radiactivamente (131I-MIBG), que demostró funcional y específicamente la presencia de tejido adrenérgico neoplásico y su extensión extratumoral. Complementariamente se efectuó tomografía por emisión de positrones fusionada con tomografía computarizada, utilizando un análogo radiactivo de la glucosa que localizó la actividad extratumoral a nivel óseo. Los estudios de medicina nuclear permitieron concluir que se trataba de un paraganglioma maligno con metástasis óseas. El tratamiento incluyó resección quirúrgica del tumor y ablación del tejido tumoral residual y de los focos metastásicos con radioterapia interna mediante 131I-MIBG. El tratamiento radioterapéutico fue posible dada la capacidad del tumor y las metástasis de concentrar el análogo hormonal radiactivo. Conclusiones: Ante casos de paragangliomas, además de la localización del tumor y la evaluación de las alteraciones bioquímicas, es indispensable la evaluación funcional que brinda la medicina nuclear para la realización de un diagnóstico y tratamiento adecuados.

BACKGROUND: Paragangliomas are neoplasms of the chromaffin tissue characterized by the synthesis and/or secretion of catecholamines. Their treatment depends on the extension and functional characteristics of the tumor. In this pathology, the anatomic and functional diagnostic evaluations provided by nuclear medicine imaging studies have significant usefulness. CLINICAL CASE: A 34-year-old male was diagnosed with a paraganglioma at the level of the aortic bifurcation by means of laboratory tests, imaging studies and nuclear medicine studies. Nuclear medicine was carried out with a scintigraphy with a norepinephrine analog, radioactive meta-iodo-benzyl-guanidine (131I-MIBG), which demonstrates functionally and specifically the presence of neoplastic adrenergic tissue and extratumoral extension. In addition, a positron emission tomography coupled with computed tomography with a radioactive analog of glucose locates the extratumoral activity at bone level. Nuclear medicine studies allow the diagnosis of a malignant paraganglioma with presence of bone metastasis. The therapy includes surgical removal of the tumor and ablation of residual malignant tissue and metastatic lesions by radiotherapy with 131I-MIBG. Radiotherapeutic treatment was possible due to the capacity of the tumor to uptake and to concentrate the radioactive hormonal analog. CONCLUSIONS: In cases of paraganglioma, in addition to the localization of the tumor and the evaluation of biochemical alterations, it is indispensable to obtain anatomic and functional evaluation provided by nuclear medicine studies in order to achieve appropriate diagnoses and treatment.

Tumor del cuerpo carotídeo: A propósito de 10 casos tratados / Carotid body tumors: Report of ten cases

Background: Carotid body tumors arise from a cellular conglomerate located at the carotid bifurcation. Progressive enlargement can involve the arterial wall and neighbor cranial nerves. Aim: To report a series of 10 patients treated of carotid body tumors and review national experience. Patients and methods: Between 1984 and 2006, we operated 8 women and 2 men, aged 19 to 75 years, with this type of tumor. Results: The most common cause for consultation was a cervical mass in 90 percent, with a mean evolution lapse of 13.2 months (range 3 to 126). In all cases, diagnosis was confirmed with angiographic imaging and histopathology. Ten tumors were surgically removed with no complications. Eighty percent of tumors were in stage II according to Shamblin classification. During long term follow up all patients have remained asymptomatic. Only 31 carotid body tumors have been reported in Chilean medical literature during a 43 year period. Conclusions: Paragangliomas of the carotid body can be diagnosed in clinical grounds, requiring vascular imaging. These infrequent lesions are generally benign, early surgical removal by surgeons with vascular expertise avoids neurological and or vascular complications.

Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of six cases.

Spinal paragangliomas are uncommon neoplasms and subject of much debate regarding the factors governing their biological behaviour. We describe the clinicopathological and immunohistochemical (IHC) features of six cases of spinal paraganglioma. The mean age of patients was 40 years (range 20-60 years) with a male to female ratio of (2:1). Majority presented with low backache, sphincter disturbances and sensory symptoms. All tumors were intradural in the cauda region one of them extending to the filum. Only one showed focal extradural extension on microscopy. Gross total resection of tumors was possible in all cases. Histologically four showed classical 'zell-ballen' pattern and two revealed an ependymal morphology. On immunohistochemistry, in all the six cases the chief cells were intensely labeled by antibody to chromogranin but not for GFAP while staining for synaptophysin was less intense and variable in five. Sustentacular cells in all cases showed strong expression for S-100 protein and chief cells were stained light in three cases. Low MIB-1 labeling index of 0.01-2% was noted in five cases and in the sixth it was 5%. None of the tumours recurred. Immunohistochemistry assisted in differentiating these relatively benign neural crest tumours from the more aggressive spinal ependymomas.

Paraganglioma de mediastino com metástases pulmonares / Pulmonary metastasis of mediastinal paraganglioma

Descrevemos uma paciente de 27 anos que se apresentou com paraganglioma de mediastino anterior e médio e nódulos pulmonares bilaterais. O tratamento consistiu na ressecção das lesões pulmonares através de toracotomia anterior bilateral transesternal e retirada do paraganglioma com auxílio de circulação extracorpórea. Como tratamento neoadjuvante foram usadas radioterapia e quimioterapia. A evolução pós-operatória foi satisfatória, e catorze meses depois a paciente encontrava-se assintomática.

Malignant nasal paraganglioma: a case report and review of the literature.

A rare case of malignant nasal paraganglioma is described. A 30 year old female patient presented with a one year history of bilateralnasal obstruction, nasal deformity and recurrent epistaxis. CT scan demonstrated an enhancing mass occupying both nasal cavities, right maxillary antrum and anterior ethmoid sinus. Histopathologic diagnosis was malignant paraganglioma. A total maxillectomy with excision of growth was performed. Post-operative radiotherapy and chemotherapy was given but patient expired before the completion of therapy. Nose being a rare site for paragangliomas, these lesions present a diagnostic challenge to histopathologists and clinicians alike. A review of the four previously described malignant nasal paragangliomas is also presented.