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1.
J Cardiothorac Surg ; 17(1): 143, 2022 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-35668526

RESUMO

Posterior mediastinal paraganglioma (PM-PGL) is a rare disease that is difficult to diagnose. If PM-PGL is misdiagnosed preoperatively, surgeons may encounter severe tachycardia and hypertension and easy bleeding from the tumor during the operation. Therefore, it is essential to include PGL as a differential diagnosis for mediastinal tumors. We herein describe a 73-year-old Japanese man with a PM-PGL that was diagnosed preoperatively and resected safely by video-assisted thoracic surgery. Preoperative management of hypertension with doxazosin mesylate, soft coagulation of the peritumor area, and careful clipping of feeding arteries were effective for hemostasis. The patient's vital signs were stable during and after the operation.


Assuntos
Hipertensão , Neoplasias do Mediastino , Paraganglioma , Idoso , Humanos , Masculino , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/cirurgia , Mediastino/patologia , Mediastino/cirurgia , Paraganglioma/diagnóstico , Paraganglioma/cirurgia , Cirurgia Torácica Vídeoassistida
2.
BMJ Case Rep ; 15(6)2022 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-35672059

RESUMO

Functional mediastinal paragangliomas (PGs) are rare, catecholamine-secreting tumours. Surgical resection is the preferred treatment, but it can be complicated by catecholamine-related symptoms, involvement of cardiac structures and/or tumour supply from major blood vessels. We report a case of a man in his 30s with a subcarinal functional PG complicated by all three factors. The PG had arterial supplies from the right coronary and bronchial arteries, with mass effect on the left atrium. Given the high risk of intraoperative bleeding, catecholamine surges and injury to right coronary artery, we attempted a minimally invasive strategy that incorporates best practices from the few published cases on functional PGs. We show that a multidisciplinary approach involving alpha/beta blockade, preoperative embolisation of tumour blood supply, robotic-assisted tumour mobilisation and, if needed, open resection with cardiopulmonary bypass can be a safe strategy in the treatment of functional mediastinal paragangliomas adherent to cardiac structures.


Assuntos
Neoplasias do Mediastino , Paraganglioma Extrassuprarrenal , Paraganglioma , Catecolaminas , Humanos , Masculino , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Mediastino , Paraganglioma/complicações , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia
3.
J Pak Med Assoc ; 72(5): 983-985, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35713072

RESUMO

Paragangliomas are rare tumours derived from the neural crest cells. Most common site for these tumours is the adrenal medulla, where they are called as pheochromocytomas. Biliary system is an extremely uncommon site for extra-adrenal paraganglioma. We report the case of a 55 year old female who presented with one-month history of jaundice and right upper quadrant discomfort. Imaging showed a well-defined rounded mass at porta hepatis with arterial enhancement causing compression over the proximal common bile duct, resulting in moderate intrahepatic biliary dilatation. The patient underwent radical excision of common bile duct and roux-en-y hepaticojejunostomy. Final histopathology confirmed paraganglioma. Approximately 10% of patients may present with extra-adrenal disease, but biliary paraganglioma are extremely rare. Only few cases have been reported in literature and the management usually involves the surgical excision. Being a rare disease, a high index of suspicion is important for diagnosis of paraganglioma at such an uncommon location.


Assuntos
Procedimentos Cirúrgicos do Sistema Biliar , Paraganglioma , Ducto Colédoco , Feminino , Humanos , Fígado , Pessoa de Meia-Idade , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia
4.
World J Surg Oncol ; 20(1): 185, 2022 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-35676716

RESUMO

BACKGROUND: Paraganglioma of the urinary bladder (Pub) is rare and presents with clinical symptoms caused by catecholamine production and release. The typical symptoms of Pub are hypertension, macroscopic hematuria, and a hypertensive crisis during micturition. The average size of detected Pubs is approximately 3 cm. Herein, we report a case of a large Pub in which the symptoms were masked by oral medication, precise preoperative diagnosis was difficult, and intraoperative confirmation of tumoral adhesion to the rectum resulted in hypertensive attacks during surgery. CASE PRESENTATION: A 64-year-old Japanese male with a history of hypertension and arrhythmia controlled with oral medication presented with a large tumor in the pelvic region, detected on examination for weight loss, with no clinical symptoms. Computed tomography and magnetic resonance imaging revealed a tumor measuring 77 mm in diameter in the posterior wall of the urinary bladder. The border with the rectum was unclear, and the tumor showed heterogeneous enhancement in the solid part with an enhancing hypodense lesion. Cystoscopy revealed compression of the bladder trigone by external masses; however, no tumor was visible in the lumen. Endoscopic ultrasonography-guided fine-needle aspiration revealed CD34-positive spindle-shaped cells in the fibrous tissue, suggestive of a mesenchymal neoplasm. The tumor was suspected to be a gastrointestinal stromal tumor, and surgery was performed. After laparotomy, we suspected that the tumor had invaded the rectum, and total cystectomy and anterior resection of the rectum were performed. Histologically, the tumor cells had granular or clear amphophilic cytoplasm with an oval nucleus and nests of cells delimited by connective tissue and vascular septations. Immunohistochemically, the tumor was positive for chromogranin A, CD56, and synaptophysin, and a diagnosis of paraganglioma of the urinary bladder was confirmed. There was no tumor recurrence at the 7-month follow-up. CONCLUSION: This case highlights the importance of careful examination of pelvic tumors, including endocrine testing, for detecting paraganglioma of the urinary bladder in patients with a history of hypertension or arrhythmia.


Assuntos
Neoplasias das Glândulas Suprarrenais , Tumores do Estroma Gastrointestinal , Hipertensão , Paraganglioma , Feocromocitoma , Neoplasias da Bexiga Urinária , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Paraganglioma/diagnóstico , Paraganglioma/patologia , Paraganglioma/cirurgia , Pelve/patologia , Reto/patologia , Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/cirurgia
6.
J Surg Res ; 277: 189-199, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35500514

RESUMO

INTRODUCTION: Surgical resection of pheochromocytoma and paraganglioma (PPGL) may be associated with excessive hemodynamic variability. Whether hemodynamic variability occurs in patients with undiagnosed PPGL undergoing unrelated, non-neuroendocrine, operations is unknown. METHODS: We identified patients who underwent non-neuroendocrine surgical procedures up to 5 y before pathologic diagnosis of PPGL. For each PPGL, two non-PPGL patients were matched based on sex, age, type, and year of operation. Electronic medical records were reviewed for intraoperative blood pressures, heart rates, and hemodynamic variability was assessed with range (maximum-minimum), standard deviation, coefficient of variation, and average real variability. RESULTS: Thirty-seven PPGL patients underwent operations preceding the diagnosis of PPGL: 25 pheochromocytomas, 11 paragangliomas, and one metastatic pheochromocytoma. Median interquartile range tumor size at diagnosis was 35 mm (23 to 60). The time from index operation to PPGL diagnosis was ≤12 mo in 21 (56.8%) patients. In 23 (62.2%) patients, the subsequently diagnosed PPGL was functional. Fifteen (40.5%) PPGL and 20 (27.0%) control patients were preoperatively treated for hypertension (P = 0.149). Maximum intraoperative systolic BP was >180 mmHg for 4 (10.8%) PPGL patients and 3 (4.1%) controls (P = 0.219). Two PPGL patients had intraoperative systolic BP >230 mmHg. No significant differences were found with all other measures of intraoperative hemodynamic variability. Similarly, in secondary analysis there was no significant difference in intraoperative hemodynamic variability between biochemically active PPGL and their respective controls. CONCLUSIONS: Patients with undiagnosed PPGL undergoing a wide variety of non-neuroendocrine operations had intraoperative hemodynamic variability comparable to non-PPGL patients undergoing the same type of procedures.


Assuntos
Neoplasias das Glândulas Suprarrenais , Paraganglioma , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/patologia , Pressão Sanguínea/fisiologia , Hemodinâmica , Humanos , Paraganglioma/diagnóstico , Paraganglioma/patologia , Paraganglioma/cirurgia , Feocromocitoma/diagnóstico , Feocromocitoma/patologia , Feocromocitoma/cirurgia
7.
Front Endocrinol (Lausanne) ; 13: 857504, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35498434

RESUMO

Head and neck paragangliomas (HNPGLs) are neuroendocrine tumors. They arise from the parasympathetic ganglia and can be either sporadic or due to hereditary syndromes (up to 40%). Most HNPGLs do not produce significant amounts of catecholamines. We report a case of a giant paraganglioma of the skull base with an unusually severe presentation secondary to excessive release of norepinephrine, with a good outcome considering the severity of disease. A 39-year-old Caucasian woman with no prior medical history was found unconscious and emaciated in her home. In the intensive care unit (ICU) the patient was treated for multi-organ failure with multiple complications and difficulties in stabilizing her blood pressure with values up to 246/146 mmHg. She was hospitalized in the ICU for 72 days and on the 31st day clinical assessment revealed jugular foramen syndrome and paralysis of the right n. facialis. A brain MRI confirmed a right-sided tumor of the skull base of 93.553 cm3. Blood tests showed high amounts of normetanephrine (35.1-45.4 nmol/L, ref <1.09 nmol/L) and a tumor biopsy confirmed the diagnosis of a paraganglioma. Phenoxybenzamine and Labetalol were used in high doses ((Dibenyline®, 90 mg x 3 daily) and labetalol (Trandate®, 200 + 300 + 300 mg daily) to stabilize blood pressure. The patient underwent two tumor embolization procedures before total tumor resection on day 243. Normetanephrine and blood pressure normalized after surgery (0.77 nmol/L, ref: < 1.09 nmol/L). The damage to the cranial nerve was permanent. Our patient was comprehensively examined for germline predisposition to PPGLs, however we did not identify any causal aberrations. A somatic deletion and loss of heterozygosity (LOH) of the short arm (p) of chromosome 1 (including SDHB) and p of chromosome 11 was found. Analysis showed an SDHB (c.565T>G, p.C189G) and PTEN (c.834C>G, p.F278L) missense mutation in tumor DNA. The patient made a remarkable recovery except for neurological deficits after intensive multidisciplinary treatment and rehabilitation. This case demonstrates the necessity for an early tertiary center approach with a multidisciplinary expert team and highlights the efficacy of the correct treatment with alpha-blockade.


Assuntos
Labetalol , Paraganglioma , Adulto , Feminino , Humanos , Mutação , Normetanefrina , PTEN Fosfo-Hidrolase , Paraganglioma/genética , Paraganglioma/cirurgia , Base do Crânio , Succinato Desidrogenase
9.
Tex Heart Inst J ; 49(2)2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-35438784

RESUMO

Carotid body tumors, rare neck paragangliomas arising from the common carotid artery bifurcation, can be classified as sporadic, hyperplastic, or familial. The familial type is often bilateral and associated with germline mutation of the mitochondrial enzyme succinate dehydrogenase. We report the rare case of a 42-year-old man who presented with bilateral giant familial carotid body tumors associated with a concomitant skull-base paraganglioma, left-sided facial nerve palsy, and an incomplete circle of Willis. We describe the excision of the tumors in 2 stages (the left mass and associated paraganglioma first and the right mass second), 6 months apart, with use of general anesthesia, and we discuss other operative considerations.


Assuntos
Tumor do Corpo Carotídeo , Neoplasias de Cabeça e Pescoço , Paraganglioma , Adulto , Tumor do Corpo Carotídeo/complicações , Tumor do Corpo Carotídeo/diagnóstico , Tumor do Corpo Carotídeo/genética , Nervo Facial/patologia , Neoplasias de Cabeça e Pescoço/complicações , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Masculino , Paraganglioma/genética , Paraganglioma/patologia , Paraganglioma/cirurgia , Paralisia , Crânio/patologia
10.
Medicine (Baltimore) ; 101(15): e29138, 2022 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-35475800

RESUMO

RATIONALE: Gangliocytic paraganglioma (GP) is a rare tumor that mostly develops in the duodenum and is composed of the following 3 cell types: epithelioid endocrine, spindle-like, and ganglion-like cells. It manifests as symptoms such as abdominal pain, gastrointestinal bleeding, and weight loss; however, occasionally, it is incidentally detected on endoscopic or radiologic examinations. Although GP is usually benign, it can metastasize to the lymph nodes, and distant metastases have been reported in some cases. PATIENT CONCERNS: A 46-year-old woman presented with anemia on health surveillance examination. She had no other specific symptoms, and her physical examination did not reveal any abnormal finding. DIAGNOSIS: Endoscopic ultrasound-guided fine-needle aspiration biopsy was performed, and the endoscopist obtained samples from the inner side of the ampullary mass. Pathological examination suggested GP or a neuroendocrine tumor. INTERVENTIONS: Initially, we planned transduodenal ampullectomy with lymph node excision. However, there was severe fibrosis around the duodenum, and an examination of a frozen biopsy sample from the periduodenal lymph node showed atypical cells in the lymph node. Therefore, we performed pylorus-preserving pancreaticoduodenectomy with lymph node dissection. OUTCOMES: The final pathological diagnosis was GP located in the ampulla of Vater. The GP showed lymphovascular and perineural invasion and invaded the duodenal wall. Furthermore, 4 out of 18 harvested lymph nodes showed metastasis. LESSONS: We described a case of GP confined to the ampulla with regional lymph node metastasis and reviewed published literature on ampullary GP with lymph node metastasis.


Assuntos
Neoplasias Duodenais , Paraganglioma , Neoplasias Duodenais/patologia , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Feminino , Humanos , Metástase Linfática , Pessoa de Meia-Idade , Pancreaticoduodenectomia , Paraganglioma/diagnóstico , Paraganglioma/patologia , Paraganglioma/cirurgia
11.
BMJ Case Rep ; 15(3)2022 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-35236704

RESUMO

Tumour to tumour metastases are uncommon, and we report a case of carotid body paraganglioma metastatic to a hepatocellular adenoma. A 54-year-old man presented after a CT chest for chronic cough that incidentally identified two liver lesions in segment 3 and caudate. The imaging findings were suspicious for atypical haemangiomas versus hepatocellular adenoma. The segment 3 lesion was biopsied, demonstrating beta-catenin activated hepatocellular adenoma. He underwent partial hepatectomy with pathology showing the beta-catenin activated hepatocellular adenoma contained a central area of paraganglioma. On closer review, the patient revealed a carotid body paraganglioma with lymph node metastases requiring resection 24 years earlier. He subsequently underwent left hepatectomy including the resection bed and caudate, which confirmed the caudate lesion as metastatic paraganglioma. This case demonstrates how paraganglioma can metastasise to liver decades after initial resection and provide insight into the diagnostic workup for hepatocellular adenoma with neuroendocrine features.


Assuntos
Adenoma de Células Hepáticas , Carcinoma Hepatocelular , Tumor do Corpo Carotídeo , Neoplasias Hepáticas , Paraganglioma , Adenoma de Células Hepáticas/cirurgia , Carcinoma Hepatocelular/cirurgia , Tumor do Corpo Carotídeo/diagnóstico por imagem , Tumor do Corpo Carotídeo/cirurgia , Hepatectomia , Humanos , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/cirurgia , Masculino , Pessoa de Meia-Idade , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia
12.
Front Endocrinol (Lausanne) ; 13: 816833, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35282440

RESUMO

Background: Despite an improved understanding of pheochromocytoma and extra-adrenal sympathetic parganglioma (PPGL), including diagnosis and management, some PPGLs are postoperatively diagnosed. Clinical characteristics and intraoperative haemodynamic instability (HI) in postoperatively diagnosed PPGL patients have been poorly defined. Thus, we investigated the clinical characteristics and HI in patients with postoperatively diagnosed PPGLs compared to patients with preoperatively diagnosed PPGLs. Methods: We obtained clinical and haemodynamic data from the electronic medical records of 256 patients with pathologically confirmed PPGLs at our institution from January 2005 to December 2019. We assessed the intraoperative HI (systolic blood pressure [SBP]>160 mmHg (min) or mean blood pressure [MBP]<60 mmHg (min)) over time. Results: Twenty-nine patients (11.3%) were diagnosed with PPGLs postoperatively. Hypertension (34.5% vs. 63.0%, P=0.006) and pheochromocytoma (17.2% vs. 81.1%, P<0.001) case rates were lower in postoperatively diagnosed patients than in preoperatively diagnosed patients. Preoperative SBP in the ward was similar between groups, but the use of α-blockers and ß-blockers was more frequent in preoperatively diagnosed patients (89.0% vs. 3.4%, P<0.001; 36.3% vs. 6.9%, P=0.003). Considering intraoperative HI, postoperatively diagnosed patients demonstrated a similar percentage of time with SBP>160 mmHg (median [IQR]; 7.9% [2.5; 11.9] % vs. 4.6% [0.0; 11.9], P=0.088) but a significantly lower percentage of time with MBP<60 mmHg (0.0% [0.0; 3.0] vs. 5.6% [0.0, 12.6], P=0.002) compared with preoperatively diagnosed patients. Conclusions: Patients diagnosed with PPGLs postoperatively may have no further higher risk of intraoperative hypertension than those diagnosed preoperatively despite insufficient preoperative management for PPGLs. Further study will be needed to ascertain intrinsic tumour characteristics, and need for universal preoperative use of α- and ß-blockers in PPGL patients postoperatively diagnosed or without typical symptoms related PPGLs.


Assuntos
Neoplasias das Glândulas Suprarrenais , Hipertensão , Paraganglioma , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Hemodinâmica/fisiologia , Humanos , Hipertensão/diagnóstico , Hipertensão/etiologia , Paraganglioma/diagnóstico , Paraganglioma/cirurgia , Feocromocitoma/diagnóstico , Feocromocitoma/cirurgia
13.
Eur J Endocrinol ; 186(5): D17-D24, 2022 Mar 18.
Artigo em Inglês | MEDLINE | ID: mdl-35230260

RESUMO

The question of systematic use of a pharmacological treatment before surgery in patients diagnosed with pheochromocytoma and paraganglioma (PPGL) remains highly controversial. While recent guidelines suggest that this should be used in all patients, some experienced teams consider it unnecessary in some cases, provided the surgery is performed in a dedicated center that has expert endocrinologists, cardiologists, surgeons, and anesthetists. This controversy is aimed at shedding light on the potential benefits and risks of such a treatment, focusing specifically on alpha blockers which are considered as the first-line medical treatments in patients with PPGL. After discussing the rationale for alpha blockers, hemodynamic instability, tolerance, and acute cardiac complications will then be discussed in the first part of the manuscript, defending a systematic use. The second section will focus on blood pressure control, tolerance of alpha blockers, and also the management of normotensive PPGL, examining the daily risks of PPGL and arguing against the systematic use of a preoperative pharmacological treatment before surgery. Finally, we will discuss the concept of expert centers and define the patients in whom the risk/benefit profile would favor the use of this preoperative treatment.


Assuntos
Neoplasias das Glândulas Suprarrenais , Paraganglioma , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/tratamento farmacológico , Neoplasias das Glândulas Suprarrenais/cirurgia , Antagonistas Adrenérgicos alfa/uso terapêutico , Pressão Sanguínea , Humanos , Paraganglioma/tratamento farmacológico , Paraganglioma/cirurgia , Feocromocitoma/tratamento farmacológico , Feocromocitoma/cirurgia
14.
BMJ Case Rep ; 15(3)2022 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-35351744

RESUMO

Pheochromocytoma and paraganglioma (PGL) are rare neuroendocrine tumours, frequently associated with genetic syndromes. We report the case of a man in his 40s with a left anterior neck mass and a history of hypertensive crisis, heavy sweating and constipation. Biochemical tests showed increased plasma and urine normetanephrines. Neck ultrasound suggested left carotid body PGL, but it was mandatory to search for other lesions. Whole-body MIBG failed to show abnormal uptake. Abdominal MRI was suggestive of another PGL, anterior to the right adrenal gland. Abdominal surgery was performed uneventfully under alpha and beta blockers. This intervention proved to be effective, as normetanephrines levels became completely normal after 1 month. Carotid body PGL was successfully excised 4-months later. Genetic study identified a large deletion in exon 1 of the SDHB gene allowing the diagnosis of paraganglioma syndrome type 4 (PGL4). After 19 months of follow-up, he is still on clinical and biochemical remission and will continue life-long surveillance.


Assuntos
Neoplasias das Glândulas Suprarrenais , Paraganglioma , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Humanos , Masculino , Paraganglioma/complicações , Paraganglioma/genética , Paraganglioma/cirurgia , Feocromocitoma/complicações , Feocromocitoma/diagnóstico , Feocromocitoma/cirurgia , Succinato Desidrogenase/genética , Síndrome
15.
Front Endocrinol (Lausanne) ; 13: 847632, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35299959

RESUMO

Gangliocytic paraganglioma (GP) is quite rare, and origin and entity remain to be elucidated. A 51-year-old man presented with GP as a sessile polyp with a smooth surface that measured about 1 cm in diameter in the descending portion of duodenum. Pathological examination displayed that a neoplasm was predominantly located in the submucosa and infiltrated mucosa focally. The tumor consisted of epithelioid, ganglion-like, and spindle cells admixing in a haphazard way. The epithelioid cells resembled paraganglioma in cytological and architectural features. The ganglion-like cells were scattered and merged with the bland spindle cells in fascicular clusters, which resembled ganglioneuroma. Synaptophysin (Syn), microtubule-associated protein-2 (MAP-2), and chromogranin A (CgA) were positive in the epithelioid and ganglion-like cells in variety, and neurofilament (NF) staining highlighted the ganglion-like cells. S-100 and SOX-10 were positive in the spindle cell proliferation and around the epithelioid cells. Progesterone receptor (PR) was positive in the epithelioid cells. The polyp was resected, and no adjuvant therapy was given. The patient remained with no recurrence in 2 years' follow-up. Origin of GP is presumed to be related to pancreas islet. GP is distinguished from neuroendocrine tumor (NET) G1 and designated as paraganglioma-ganglioneuroma, a kind of composite paragangliomas.


Assuntos
Ganglioneuroma , Tumores Neuroendócrinos , Paraganglioma , Cromogranina A , Humanos , Masculino , Pessoa de Meia-Idade , Pâncreas/patologia , Paraganglioma/patologia , Paraganglioma/cirurgia
16.
BMJ Case Rep ; 15(3)2022 Mar 23.
Artigo em Inglês | MEDLINE | ID: mdl-35321910

RESUMO

A man in his 20s presented to the neurosurgery department 2 years ago with headache and blurred vision. He was diagnosed to have a suprasellar mass on neuroimaging. Best-corrected visual acuity in the right eye was 6/36 and that in the left eye was 6/60. Automated visual fields showed a temporal hemianopia in the right eye and an advanced field defect in the left eye. His hormonal profile was normal, and he underwent partial excision of suprasellar tumour, which was a histopathologically proven paraganglioma (PGL). Subsequently, the patient underwent radiotherapy and his vision and visual fields showed improvement. Follow-up examination 3 years later showed a left retinal capillary hemangioblastoma (RCH), which was treated with green laser photocoagulation, resulting in complete sclerosis. This case is unique because of the extremely rare coexistence of a sellar PGL and RCH, which to our knowledge has not been reported so far.


Assuntos
Hemangioblastoma , Paraganglioma , Neoplasias da Retina , Doença de von Hippel-Lindau , Hemangioblastoma/diagnóstico , Humanos , Masculino , Paraganglioma/complicações , Paraganglioma/diagnóstico , Paraganglioma/cirurgia , Retina/patologia , Neoplasias da Retina/diagnóstico , Doença de von Hippel-Lindau/complicações
17.
Endocr Regul ; 56(1): 48-54, 2022 Feb 18.
Artigo em Inglês | MEDLINE | ID: mdl-35180819

RESUMO

Phaeochromocytomas are catecholamine-secreting tumors arising in the chromaffin cells of the adrenal medulla. They are a rare cause of secondary hypertension. However, catecholamine secreting tumors may also be found in the extra-adrenal sites, producing similar symptoms as the adrenal phaeochromocytoma. The extra-adrenal phaeochromocytomas, are referred to as paragangliomas (PGLs). About 75% of extra-adrenal phaeochromocytomas are intra-abdominal, mostly located in perinephric, periaortic, and bladder regions. Most phaeochromocytomas secrete excessive amount of epinephrine and norepinephrine, whereas most paragangliomas secrete only norepinephrine. The excessive secretion of these products could lead to paroxysms of symptoms that could be life threatening. Medical management is initially offered, but definitive treatment involves surgical removal of the tumor, which requires promptness on the both the clinician and the patient sides. We present a case of an extra-adrenal phaeochromocytoma in an adult male with revealing imaging of a mass surrounding the bladder. The patient was managed with both alpha- and beta-adrenergic blockers. He declined the surgery and eventually died after appearing in an acute hypertensive crisis.


Assuntos
Neoplasias das Glândulas Suprarrenais , Hipertensão , Paraganglioma , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Catecolaminas , Humanos , Hipertensão/complicações , Masculino , Paraganglioma/diagnóstico , Paraganglioma/cirurgia , Feocromocitoma/diagnóstico , Feocromocitoma/cirurgia
18.
J Med Case Rep ; 16(1): 92, 2022 Feb 25.
Artigo em Inglês | MEDLINE | ID: mdl-35216640

RESUMO

BACKGROUND: Paragangliomas are rare vascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissue. They occur most commonly at the carotid bifurcation, where they are known as carotid body tumors. Most paragangliomas are benign, locally aggressive, infiltrative tumors. Approximately 10% of patients with paragangliomas develop distant metastases, 10% present with multiple or bilateral tumors (mostly carotid body tumors), and 10% have a family history of paragangliomas. The malignant transformation of carotid body tumors has been reported in 6% of cases. CASE PRESENTATION: We present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes. CONCLUSIONS: The management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.


Assuntos
Tumor do Corpo Carotídeo , Paraganglioma Extrassuprarrenal , Paraganglioma , Tumor do Corpo Carotídeo/diagnóstico por imagem , Tumor do Corpo Carotídeo/radioterapia , Tumor do Corpo Carotídeo/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Pescoço/patologia , Paraganglioma/cirurgia , Paraganglioma Extrassuprarrenal/diagnóstico
19.
World Neurosurg ; 162: e49-e57, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35189418

RESUMO

BACKGROUND: Glomus jugulare tumors (GJTs) are benign paragangliomas of the jugular foramen. Traditional management of these tumors involves surgical resection; however, considering the proximity of these tumors to important neurovasculature, stereotactic radiosurgery (SRS) may be an appropriate noninvasive treatment to consider. The aim of this meta-analysis was to evaluate SRS as a treatment option for GJTs. METHODS: An online search using PubMed, Web of Science, Scopus, and Cochrane databases was performed in March 2019 for articles on radiosurgery treatment of GJTs. The screening process followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: The final analysis comprised 23 studies including 460 patients. Average rates of tinnitus, hearing loss, and lower cranial nerve deficit as presenting symptoms were 56% (95% confidence interval [CI], 46%-66%), 56% (95% CI, 44%-68%), and 42% (95% CI, 31%-54%), respectively. Overall clinical status improvement rate after treatment was 47% (95% CI, 37%-57%). Rates of tinnitus, hearing loss, and lower cranial nerve improvement after treatment were 54% (95% CI, 44%-63%), 28% (95% CI, 19%-40%), and 22% (95% CI, 11%-39%), respectively. The mean follow-up time across studies was 47 months (range, 4-268 months). The aggregate tumor control rate at the time of follow-up was 95% (95% CI, 93%-97%). CONCLUSIONS: The tumor control rate of 95% and 47% symptomatic improvement suggest that SRS may be a suitable treatment modality for these hypervascular skull base tumors. Future studies are warranted to further evaluate the potential role of SRS in management of GJTs.


Assuntos
Tumor do Glomo Jugular , Paraganglioma , Radiocirurgia , Zumbido , Seguimentos , Tumor do Glomo Jugular/radioterapia , Tumor do Glomo Jugular/cirurgia , Humanos , Paraganglioma/cirurgia , Estudos Retrospectivos , Zumbido/etiologia , Zumbido/cirurgia , Resultado do Tratamento
20.
World Neurosurg ; 161: 190-197.e20, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35123022

RESUMO

BACKGROUND: Primary spine paragangliomas are rare tumors. Surgical resection plays a role, but aggressive lesions are challenging. We reviewed the literature on primary spine paragangliomas. METHODS: PubMed, Scopus, Web of Science, and Cochrane were searched following the PRISMA guidelines to include studies on primary spine paragangliomas. Clinical-radiologic features, treatments, and outcomes were analyzed and compared between cauda equina versus non-cauda equina tumors. RESULTS: We included 143 studies comprising 334 patients. Median age was 46 years (range, 6-85 years). The most frequent symptoms were lower back (64.1%) and radicular (53.9%) pain, and sympathetic in 18 patients (5.4%). Cauda equina paragangliomas (84.1%) had frequently lumbar (49.1%) or lumbosacral (29%) locations. Non-cauda equina tumors were mostly in the thoracic (11.4%), thoracolumbar (5.1%), and cervical (3.6%) spine. Median tumor diameter was 2.5 cm (range, 0.5-13.0 cm). Surgical resection (98.5%) was preferred over biopsy (1.5%). Decompressive laminectomy (53%) and spine fusion (6.9%) were also performed. Adjuvant radiotherapy was delivered in 39 patients (11.7%) with aggressive tumors. Posttreatment symptomatic improvement was described in 86.2% cases. Median follow-up was 19.5 months (range, 0.1-468.0 months), and 23 patients (3.9%) had tumor recurrences. No significant differences were found between cauda equina versus non-cauda equina tumors. CONCLUSIONS: Surgical resection is effective and safe in treating primary spine paragangliomas; however, adjuvant treatments may be needed for aggressive lesions.


Assuntos
Cauda Equina , Paraganglioma Extrassuprarrenal , Paraganglioma , Neoplasias da Coluna Vertebral , Cauda Equina/diagnóstico por imagem , Cauda Equina/cirurgia , Humanos , Região Lombossacral , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Coluna Vertebral
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