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1.
Isr Med Assoc J ; 22(1): 53-59, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31927807

RESUMO

BACKGROUND: Primary retroperitoneal neoplasms (PRN) arise from diverse retroperitoneal tissues. Soft tissue sarcomas (STS) comprise the majority and are well studied. Other non-sarcomatous PRN are very rare and less familiar. OBJECTIVES: To evaluate the clinicopathologic and radiologic features of non-sarcomatous PRN, as well as the outcome of complete tumor resection (TR). METHODS: Retrospective data were collected on consecutive patients (June 2006 to January 2015) who underwent resection of retroperitoneal lesions at our department. Final pathology of non-sarcomatous PRN was included. RESULTS: The study population included 36 patients (26% with PRN). PRN were neurogenic (17%), fat-containing (3%), and cystic (6%). The preoperative diagnosis was correct in only 28%. All patients underwent TR via laparotomy (72%) or laparoscopy (28%), for mean operative time of 120 ± 46 minutes. En bloc organ resection was performed in 11%. Complete TR was achieved in 97%. Intra-operative spillage occurred in 8%. Intra-operative, 90-day postoperative complications, and mortality rates were 11%, 36%, and 0%, respectively. The mean length of stay was 6.5 ± 5.5 days. The median overall survival was 53 ± 4.9 months. CONCLUSIONS: Familiarity with radiologic characteristics of PRN is important for appropriate management. Counter to STS, other PRN are mostly benign and have an indolent course. Radical surgery is not required, as complete TR confers good prognosis. Expectant management is reserved for small, asymptomatic, benign neoplasms.


Assuntos
Neoplasias Retroperitoneais/diagnóstico , Idoso , Feminino , Ganglioneuroma/diagnóstico , Ganglioneuroma/diagnóstico por imagem , Ganglioneuroma/patologia , Ganglioneuroma/cirurgia , Humanos , Lipoma/diagnóstico , Lipoma/diagnóstico por imagem , Lipoma/patologia , Lipoma/cirurgia , Masculino , Pessoa de Meia-Idade , Neurilemoma/diagnóstico , Neurilemoma/diagnóstico por imagem , Neurilemoma/patologia , Neurilemoma/cirurgia , Neurofibroma/diagnóstico , Neurofibroma/diagnóstico por imagem , Neurofibroma/patologia , Neurofibroma/cirurgia , Paraganglioma/diagnóstico , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Paraganglioma/cirurgia , Neoplasias Retroperitoneais/diagnóstico por imagem , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
2.
Medicine (Baltimore) ; 98(39): e17145, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31574816

RESUMO

RATIONALE: Paragangliomas are rare neuroendocrine tumors that originate in specialized cells derived from the neural crest with metastasis to the thoracic spine being among the rarest forms. Here, we are presenting a detailed analysis of a case of malignant paraganglioma in the thoracic spinal region in a 14-year-old boy. Our focus is to emphasize the importance of considering malignant paraganglioma as a diagnosis and guiding the perioperative management upon surgical treatment. The management of these unique cases has yet to be well-documented. PATIENT CONCERNS: A 14-year-old boy presented with a 5-month history of continuous and progressive elevated blood pressure and back pain. The patient, who had been diagnosed of malignant paraganglioma in the left posterior mediastinum for 3 months, received surgical resection of paraganglioma in the left posterior mediastinum, which had involved the left intervertebral foramen of T4. However, the tumor was not completely resected during the first operation. DIAGNOSES: Magnetic resonance imaging of spine and positron emission tomography-computed tomography showed spinal cord compression secondary to the epidural component of the T4 mass, with increased marrow infiltration of the left T4 intervertebral foramen, which was difficult to be removed. Postoperative pathology confirmed the diagnosis of spinal involvement of malignant paraganglioma. INTERVENTIONS: The patient underwent biopsy and percutaneous vertebroplasty of T4 and paravertebral lesions, and needle-track cement augmentation via a posterior approach. OUTCOMES: The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 10-month follow-up visit. There were no other complications associated with the operation during the follow-up period. LESSONS: Combined efforts of specialists from orthopedics, neurosurgery, thoracic surgery, and medical oncology led to the successful diagnosis and management of this patient. Malignant paraganglioma of thoracic spine, although rare, should be part of the differential diagnosis when the patient has a history of paraganglioma and presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression of the malignant paraganglioma when the tumor has caused neurological deficits. Osteoplasty by cement augmentation is also a good choice for surgical treatment. However, we need to take the potential risk of complications in bone cement applications into full consideration.


Assuntos
Neoplasias do Mediastino/cirurgia , Procedimentos Neurocirúrgicos/métodos , Procedimentos Ortopédicos/métodos , Paraganglioma/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Adolescente , Descompressão Cirúrgica , Humanos , Masculino , Neoplasias do Mediastino/patologia , Paraganglioma/patologia , Neoplasias da Coluna Vertebral/patologia , Vértebras Torácicas/patologia , Vértebras Torácicas/cirurgia , Vertebroplastia/métodos
4.
Mol Biol (Mosk) ; 53(4): 613-626, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31397435

RESUMO

Carotid paragangliomas (CPGLs) are rare neuroendocrine tumors of the head and neck. "Germline" and somatic mutations in a number of genes were shown to be associated with the development of CPGLs; however, molecular mechanisms of the tumor pathogenesis have not been fully understood. In the work, we have used whole exome sequencing data of 52 CPGLs obtained earlier. Using MutSigCV, the search for genes with high mutation rate was performed. Thirty four genes (MADCAM1, SARM1, ZFPM1, CTDSP2, DSPP, POTED, ANP32B, FRG2B, BAGE3, CCDC89, ACOT2, KRTAP10-1, ATXN1, GXYLT1, MUC2, AQP7, TMPRSS13, KRTAP4-3, PRR21, PSPH, PLBD1, ZNF595, IGSF3, PRR16, FAM157A, KCNJ12, HYDIN, IGFBP2, KIAA1671, DISC1, MUC6, XKR3, HRNR, and MUC4) potentially associated with the CPGL initiation and progression were revealed. The involvement of these genes in the pathogenesis of CPGLs was first shown, and possible mechanisms of their participation in that were discussed.


Assuntos
Carcinogênese/genética , Neoplasias de Cabeça e Pescoço/genética , Paraganglioma/genética , Progressão da Doença , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Paraganglioma/patologia , Sequenciamento Completo do Exoma
5.
Medicine (Baltimore) ; 98(28): e16383, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31305440

RESUMO

RATIONALE: Paraganglioma refers to a set of neuroendocrine tumors derived from the chromaffin cells of the adrenal diplomatic ganglion. Paragangliomas can be classified as functional or nonfunctional based on the ability to synthesize and release catecholamines. PATIENT CONCERNS: We report a 47-year-old man with a functional paraganglioma in the left posterior mediastinum and highlight the key elements of management of mediastinal paragangliomas. DIAGNOSES: A left posterior mediastinal mass was found by computed tomography (CT) scan and Chest-enhanced CT. Preoperative ultrasound-guided biopsy suggested the possibility of a paraganglioma. A diagnosis of paraganglioma was established by immunohistochemistry. INTERVENTIONS: The patient underwent single-stage resection of the lesion via left thoracotomy after preoperative oral α-adrenoceptor (phenoxybenzamine) therapy and intravenous fluid resuscitation for two weeks. OUTCOMES: The postoperative period was uneventful. The patient exhibited no abnormal blood pressure or recurrence during the 12-month follow-up period. LESSONS SUBSECTIONS AS PER STYLE: Pathological examination alone cannot determine whether it was a benign or malignant paraganglioma, which can be determined by pathological examination combined with distant metastasis. Long-term follow-up is required to assess the treatment effect.


Assuntos
Neoplasias do Mediastino/diagnóstico , Paraganglioma/diagnóstico , Diagnóstico Diferencial , Humanos , Masculino , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/terapia , Pessoa de Meia-Idade , Paraganglioma/patologia , Paraganglioma/terapia
6.
Clin Nucl Med ; 44(10): e583-e585, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31348087

RESUMO

Carotid body paragangliomas are mostly benign tumors with very rare propensity to metastasize to lymph nodes, bones, liver, and lungs. Leptomeningeal metastasis from a carotid body paraganglioma is a very rare phenomenon. We describe Ga-DOTANOC PET-CT findings of a 33-year-old man, a known case of metastatic paraganglioma who underwent scan for response assessment to everolimus therapy.


Assuntos
Tumor do Corpo Carotídeo/patologia , Carcinomatose Meníngea/diagnóstico por imagem , Carcinomatose Meníngea/secundário , Compostos Organometálicos , Paraganglioma/patologia , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Adulto , Humanos , Masculino
7.
Clin Nucl Med ; 44(9): e517-e518, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31348093

RESUMO

Paratesticular paragangliomas are a rare occurrence. We present the case of a 43-year-old man who presented with paresthesia and paraparesis and was found to have pathologic fracture involving D1 vertebra as a manifestation of metastasis from a nonsecretory right paratesticular paraganglioma.


Assuntos
Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Neoplasias Testiculares/diagnóstico por imagem , Neoplasias Testiculares/patologia , Adulto , Humanos , Imagem por Ressonância Magnética , Masculino , Metástase Neoplásica
8.
Horm Metab Res ; 51(7): 437-442, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31174229

RESUMO

Carney-Stratakis Syndrome (CSS) comprises of paragangliomas (PGLs) and gastrointestinal stromal tumors (GISTs). Several of its features overlap with Carney Triad (CT) - PGLs, GISTs, and pulmonary chondromas. CSS has autosomal dominant inheritance, incomplete penetrance, and greater relative frequency of PGL over GISTs. The PGLs in CSS are multicentric and GISTs are multifocal in all the patients, suggesting an inherited susceptibility and associating the two manifestations. In this review, we highlight the clinical, pathological, and molecular characteristics of CSS, along with its diagnostic and therapeutic implications.


Assuntos
Condroma , Tumores do Estroma Gastrointestinal , Mutação em Linhagem Germinativa , Leiomiossarcoma , Neoplasias Pulmonares , Paraganglioma Extrassuprarrenal , Paraganglioma , Neoplasias Gástricas , Adulto , Condroma/diagnóstico , Condroma/genética , Condroma/metabolismo , Condroma/patologia , Feminino , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/metabolismo , Tumores do Estroma Gastrointestinal/patologia , Humanos , Leiomiossarcoma/diagnóstico , Leiomiossarcoma/genética , Leiomiossarcoma/metabolismo , Leiomiossarcoma/patologia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/metabolismo , Neoplasias Pulmonares/patologia , Masculino , Paraganglioma/diagnóstico , Paraganglioma/genética , Paraganglioma/metabolismo , Paraganglioma/patologia , Paraganglioma Extrassuprarrenal/diagnóstico , Paraganglioma Extrassuprarrenal/genética , Paraganglioma Extrassuprarrenal/metabolismo , Paraganglioma Extrassuprarrenal/patologia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/genética , Neoplasias Gástricas/metabolismo , Neoplasias Gástricas/patologia
9.
Int J Mol Sci ; 20(11)2019 Jun 10.
Artigo em Inglês | MEDLINE | ID: mdl-31185588

RESUMO

A syndrome of multiple paragangliomas/pheochromocytomas, somatostatinoma, and polycythemia due to somatic mosaic gain-of-function mutation of EPAS1, encoding HIF-2α, was previously described. HIF-2α has been implicated in endochondral and intramembranous ossification. Abnormal bone growth of the skull base may lead to Chiari malformation type I. We report two cases of EPAS1 gain-of-function mutation syndrome with Chiari malformation and developmental skull base anomalies. Patients were referred to the Section on Medical Endocrinology, Eunice Kennedy Shriver NICHD, NIH for evaluation of recurrent and metastatic paragangliomas or pheochromocytoma. The syndrome was confirmed genetically by identification of the functional EPAS1 gain-of-function mutation in the resected tumors and circulating leukocytes. Both patients were confirmed for characteristics of EPAS1 gain-of-function mutation syndrome by complete blood count (CBC), plasma biochemistry, and computed tomography (CT) of the abdomen and pelvis. Chiari malformation type I and abnormal bony development of the posterior fossa was found on MRI and CT of the head. The present study implicates EPAS1 mutations in abnormal posterior fossa development resulting in Chiari malformation type I.


Assuntos
Malformação de Arnold-Chiari/genética , Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética , Anormalidades Craniofaciais/genética , Paraganglioma/genética , Adulto , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/patologia , Anormalidades Craniofaciais/diagnóstico por imagem , Anormalidades Craniofaciais/patologia , Feminino , Mutação com Ganho de Função , Humanos , Masculino , Pessoa de Meia-Idade , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Síndrome
10.
BMJ Case Rep ; 12(6)2019 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-31229970

RESUMO

We describe the case of a 35-year-old man with focal epilepsy since age 16. Due to a refractory course, several treatments were tried over the years, including insertion of a deep brain stimulator. At the time of his first assessment at our unit, he had recently been diagnosed with hypertension. An MR scan of brain revealed multiple T2 hyperintense white matter lesions, and evidence of previous haemorrhage in the left basal ganglia and pons. On follow-up imaging, the changes were considered to be in keeping with hypertensive arteriopathy. He was referred for further assessment of his hypertension and was found to have a para-aortic paraganglioma. This was excised 16 months after his initial presentation to us. The surgery was associated with an improvement in his seizure control. This case serves as a reminder of the need to be vigilant about the possibility of coexisting conditions in people with epilepsy.


Assuntos
Neoplasias Encefálicas/patologia , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Hipertensão/diagnóstico por imagem , Paraganglioma/patologia , Substância Branca/patologia , Adulto , Anticonvulsivantes , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Epilepsia Resistente a Medicamentos/fisiopatologia , Epilepsia Resistente a Medicamentos/cirurgia , Humanos , Hipertensão/etiologia , Hipertensão/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia , Resultado do Tratamento , Substância Branca/diagnóstico por imagem , Substância Branca/cirurgia
11.
Int Braz J Urol ; 45(5): 910-915, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31038858

RESUMO

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 effi cient treatment that serves both diagnostic and therapeutic purposes. Histopathological and immunohistochemistry examinations are mandatory for diagnostic confi rmation. Long-term follow-up is recommended for patients with non-functional PUB.


Assuntos
Paraganglioma/cirurgia , Neoplasias da Bexiga Urinária/cirurgia , Adulto , Idoso , Cromogranina A/análise , Cistoscopia/métodos , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Paraganglioma/patologia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sinaptofisina/análise , Resultado do Tratamento , Uretra/cirurgia , Neoplasias da Bexiga Urinária/patologia
12.
Cir Pediatr ; 32(2): 109-112, 2019 Apr 22.
Artigo em Espanhol | MEDLINE | ID: mdl-31056873

RESUMO

INTRODUCTION: Pheochromocytoma is an infrequent neuroendocrine tumor, originated from neural crest cells. 10% of them are extra adrenal, located at sympathetic nodules and are known as paragangliomas. The most common place is the Zuckerkandl organ, 10 to 26% are malign and has a mortality around de 26% of the cases. CASE REPORT: We present two cases of teenagers with Zuckerkandl's organ paraganglioma, who debut with high blood pressure, diaphoresis and dyspnea. Complete tumor resection was performed in both cases, during which they presented hipertensive crisis and, after vascular pedicle ligation, hypotension. CONCLUSIONS: Zuckerkandl's organ paraganglioma is a rare pathology in pediatric population, therefore represents an important diagnostic and therapeutic challenge. Surgery differs from other tumors because of catecholamine secretion that produces hemodynamic changes and demands prompt and accurate management from surgeon and anesthetist.


Assuntos
Glomos Para-Aórticos , Paraganglioma , Adolescente , Humanos , Hipertensão/etiologia , Masculino , Paraganglioma/complicações , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Paraganglioma/cirurgia
13.
Br J Neurosurg ; 33(5): 500-503, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31130023

RESUMO

Purpose: The carotid body functions as a chemoreceptor and receives richer blood supply, by weight, than any other organ in the body. We review the literature regarding the anatomy, histology, and function of the carotid body and the incidence, functionality, and clinical relevance of carotid body tumors and paragangliomas. These lesions are often nonfunctional but can be associated with catecholamine secretion. Most patients are asymptomatic or present initially with a cervical mass. As the tumors grow, they can impinge on nearby cranial nerves. Although there is some debate, the dominant clinical strategy is to surgically resect these tumors as early as possible. If they are resected early, the risk of postoperative neurovascular injury is minimized. Methods: Literature search was performed using the PubMed database with focus on articles including descriptions of the carotid body and associated tumors. Results: We reviewed recent literature that related to the anatomy of the carotid body while also including carotid pargangliomas and associated diagnosis with treatment interventions. Conclusion: As the carotid body serves as a vital modulator of cardiovascular and respiratory functions, illustrates the importance of identifying potential carotid paragangliomas due its ability to impede function of the carotid body. By understanding carotid paraganglioma's distinct etiologies while also understanding proper diagnosis of tumors allows for early detection and appropriate treatment options.


Assuntos
Tumor do Corpo Carotídeo/cirurgia , Corpo Carotídeo/cirurgia , Paraganglioma/cirurgia , Corpo Carotídeo/anatomia & histologia , Corpo Carotídeo/fisiopatologia , Tumor do Corpo Carotídeo/patologia , Tumor do Corpo Carotídeo/fisiopatologia , Humanos , Paraganglioma/patologia , Paraganglioma/fisiopatologia
14.
BMJ Case Rep ; 12(5)2019 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-31079039

RESUMO

Vernet syndrome, often referred to as jugular foramen syndrome, is a rare clinical entity characterised by a set of signs and symptoms caused by dysfunction of IX, X and XI cranial nerves. Although paraganglioma of the head and neck is the most frequent aetiology, it may also be caused by meningioma, VIII cranial nerve schwannoma, pontocerebellar cistern metastases, head and neck trauma, infections and very rarely by cholesteatoma which extends to the petrous apex. The authors describe a case of a patient with a jugulotympanic paraganglioma in which evolution ends up in Vernet syndrome. The patient preferred a 'wait and scan' strategy. With the lack of data available to develop an unequivocal algorithm for paraganglioma management, we always consider not only age but also comorbidities, prior treatment and progression of the lesion. Each case has to be addressed individually and treatment should be discussed in detail with every patient.


Assuntos
Doenças dos Nervos Cranianos/patologia , Paraganglioma/patologia , Membrana Timpânica/patologia , Idoso , Tratamento Conservador , Doenças dos Nervos Cranianos/diagnóstico , Feminino , Forame Magno/patologia , Humanos , Veias Jugulares/patologia , Síndrome , Zumbido/etiologia , Paralisia das Pregas Vocais/etiologia
15.
Endocr Pathol ; 30(2): 90-95, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31001800

RESUMO

We describe a consistently present, previously unrecognized, population of monocytes in pheochromocytomas and paragangliomas. Although sustentacular cells are generally recognized as a common component of these tumors, differential immunohistochemical staining for CD163 and S100 shows that monocytes can in fact be more numerous. These cells frequently resemble sustentacular cells topographically and cytologically, possibly explaining why they have not been previously noticed. They contribute to the tumor proteome and may have implications for tumor biology. No correlations were identifiable between the presence of these cells and any clinical characteristics of the tumors in the present study. A possible association with genotype is suggested by immunoblot showing high expression of CD163 protein in tumors with succinate dehydrogenase mutations.


Assuntos
Neoplasias das Glândulas Suprarrenais/patologia , Monócitos/patologia , Paraganglioma/patologia , Feocromocitoma/patologia , Neoplasias das Glândulas Suprarrenais/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígenos CD/análise , Antígenos de Diferenciação Mielomonocítica/análise , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Mutação/genética , Paraganglioma/genética , Feocromocitoma/genética , Receptores de Superfície Celular/análise , Proteínas S100/análise , Succinato Desidrogenase/genética
17.
World Neurosurg ; 126: 399-404, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30831293

RESUMO

BACKGROUND: Paraganglioma in cavernous sinus is a rare entity often misdiagnosed as meningioma or schwannoma on radiology. The embryological origin, from cells of neural crest, does not explain the location per se. Because of its highly vascular nature and close proximity to the carotid artery, surgical excision is a challenge. We herein report the first case of cavernous sinus paraganglioma, without the characteristic "salt and pepper appearance," excised near completely by the subtemporal approach. CASE DESCRIPTION: A 30-year-old woman without a history of any comorbid conditions, particularly hypertension, presented with a 6-month history of progressive headache and double vision. She exhibited upward gaze palsy and hypoesthesia over the right half of her face. There was no papilledema and proptosis. Radiologic imaging revealed an extra-axial T1-weighted isointense, T2-weighted hyperintense lesion with homogenous intense contrast uptake in right parasellar region encasing cavernous internal carotid artery. Furthermore, the tumor was extending along the superior orbital fissure. Suspecting a cavernous sinus meningioma, we performed near-total excision via a subtemporal approach. Postoperative recovery was uneventful, and patient was discharged on fifth postoperative day. The histopathology and immunohistochemistry were consistent with paraganglioma. The patient underwent adjuvant radiotherapy and is under our outpatient follow-up. CONCLUSIONS: Paraganglioma must be considered in the differential diagnosis of parasellar tumors, even when secretory symptoms are absent. The radiologic findings are obscure, and it is difficult to differentiate it from more common tumors of this region such as meningioma, schwannoma, and pituitary adenoma. Surgical excision followed by radiotherapy is the mainstay of management.


Assuntos
Seio Cavernoso/diagnóstico por imagem , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Paraganglioma/diagnóstico por imagem , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Seio Cavernoso/patologia , Diagnóstico Diferencial , Feminino , Humanos , Meningioma/patologia , Paraganglioma/patologia
18.
Gynecol Endocrinol ; 35(7): 567-570, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30786782

RESUMO

Paragangliomas are tumors that originate from the extra-adrenal neural crest, the incidence of which during pregnancy is not more than two to eight cases per million people per year and are known to be highly morbid. The purpose of this report is to describe the experience and results obtained during management of a primigravida diagnosed with paraganglioma on week 21.2 and received both medical and surgical management with good maternal and perinatal outcomes. This case report evidences the importance of practicing interdisciplinary management of patients with clinical suspicion of paragangliomas or pheochromocytomas during pregnancy at high-complexity centers even in a medium-income country.


Assuntos
Neoplasias das Glândulas Suprarrenais/cirurgia , Paraganglioma/cirurgia , Complicações Neoplásicas na Gravidez/cirurgia , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/patologia , Feminino , Humanos , Imagem por Ressonância Magnética , Paraganglioma/diagnóstico por imagem , Paraganglioma/patologia , Gravidez , Complicações Neoplásicas na Gravidez/diagnóstico por imagem , Complicações Neoplásicas na Gravidez/patologia , Resultado do Tratamento , Adulto Jovem
19.
Kyobu Geka ; 72(2): 108-111, 2019 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-30772875

RESUMO

Pigmented paraganglioma of the anterior mediastinum is extremely rare. We report a 72-year-old female patient who was pointed out an anterior mediastinum tumor showed by chest computed tomography(CT) scan during a medical check up. The tumor was 50×35 mm showing gradual enlargement and was slightly homogenously contrasted by CT scan and magnetic resonance imaging(MRI). During the resection of the tumor, sudden elevations of both blood pressure up to 205 mmHg and tachycardia up to 131 beat per minate(bpm)was noted on tumor manipulation. Pathologically, the tumor was diagnosed as pigmented paraganglioma with adjacent lymph node metastasis and microscopic extracapsular invasion. The patient is well 5 years post-surgery without recurrence.


Assuntos
Neoplasias do Mediastino/patologia , Paraganglioma/patologia , Idoso , Feminino , Humanos , Hipertensão/etiologia , Complicações Intraoperatórias/etiologia , Metástase Linfática , Imagem por Ressonância Magnética , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Mediastino/diagnóstico por imagem , Invasividade Neoplásica , Recidiva Local de Neoplasia , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia , Taquicardia/etiologia , Tomografia Computadorizada por Raios X
20.
Hum Pathol ; 86: 66-75, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30529752

RESUMO

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are neuroendocrine tumors that express somatostatin receptors (SSTRs), a phenomenon that constitutes a basis for tumor imaging and treatment with somatostatin analogues and peptide receptor radionuclide therapy. We studied the immunohistochemical expression of SSTR1-5 in 151 primary tumors, including 14 metastasized and 16 SDHB-deficient tumors. SSTR2 and SSTR3 were most abundantly present in these tumors, whereas the tumors were mostly negative for SSTR1, SSTR4, and SSTR5. All metastasized PGLs (9/9), but only one metastasized PHEO (1/5), were strongly SSTR2 positive. SSTR3 expression was lower in metastatic tumors and tumors with a high proliferation rate (MIB1 ≥ 5%), but tumors had variable individual SSTR profiles. No correlation was found between SDHB status and SSTR expression. Our results suggest that new SSTR analogues with affinity for several SSTRs could be relevant for a subgroup of patients with these tumors. Better knowledge of tumor SSTR profiles could open the door for personalized imaging and treatment in the future. Because SSTR profiles vary in PHEOs and PGLs, individual analysis is required for each tumor.


Assuntos
Neoplasias das Glândulas Suprarrenais/metabolismo , Paraganglioma/metabolismo , Feocromocitoma/metabolismo , Receptores de Somatostatina/metabolismo , Neoplasias Retroperitoneais/metabolismo , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paraganglioma/patologia , Feocromocitoma/patologia , Neoplasias Retroperitoneais/patologia , Adulto Jovem
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