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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.
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
5.
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
6.
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
7.
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
8.
Cir. pediátr ; 32(2): 109-112, abr. 2019. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-183474

RESUMO

Introducción: El feocromocitoma es un tumor neuroendocrino infrecuente. El 10% son extraadrenales en los ganglios simpáticos, conocidos como paragangliomas(1,2). El sitio más común es el órgano de Zuckerkandl. Son malignos en un 10 a 26%(4) con una mortalidad del 26%(5). Reporte de casos: Presentamos dos casos de paraganglioma del órgano de Zuckerkandl en adolescentes, que debutan con hipertensión arterial, diaforesis y disnea. Se realizó resección completa del tumor; durante ambas cirugías presentaron crisis hipertensiva y posterior a la ligadura del pedículo vascular del tumor presentaron hipotensión, con normalización progresiva de las cifras tensionales en el postoperatorio. Conclusiones: El paraganglioma del órgano de Zuckerkandl es una patología infrecuente en la población pediátrica, representa un importante reto diagnóstico y terapéutico. La cirugía difiere de las de otros tumores ya que la secreción de catecolaminas genera cambios hemodinámicos transoperatorios que exigen al cirujano y anestesiólogo un manejo rápido, eficaz y oportuno


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(1,2). The most common place is the Zuckerkandl organ, 10 to 26% are malign(4) and has a mortality around de 26% of the cases(5). 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
Humanos , Masculino , Adolescente , Paraganglioma/diagnóstico , Paraganglioma/cirurgia , Glomos Para-Aórticos/patologia , Tomografia Computadorizada por Raios X
10.
World Neurosurg ; 125: 32-36, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30703592

RESUMO

BACKGROUND: Differential diagnosis of sellar masses includes adenoma, meningioma, craniopharyngioma, and metastasis. Primary paraganglioma is seldom considered. We present a case of this unique pathology, review the relevant literature, and compile a compendium of immunohistochemical characteristics for use as a resource. CASE DESCRIPTION: A 73-year-old woman presented to the hospital with visual changes in her left hemifield. Noncontrast head computed tomography demonstrated a large sellar mass with suprasellar extension and displacement of the optic chiasm (diameter of 3.1 cm). Magnetic resonance imaging was unobtainable owing to an incompatible pacemaker. Computed tomography characterization was most consistent with a macroadenoma. Given the acute visual decline, surgical decompression via an endonasal transsphenoidal route was performed without complication. A diagnosis of paraganglioma was made based on histopathology. Following resection, the patient's visual field deficit improved. Computed tomography body imaging was negative for a metastatic origin. CONCLUSIONS: Paraganglioma is a rare but potential differential diagnosis to consider when evaluating sellar masses.


Assuntos
Adenoma/diagnóstico por imagem , Quiasma Óptico/diagnóstico por imagem , Paraganglioma/diagnóstico por imagem , Neoplasias Hipofisárias/diagnóstico por imagem , Sela Túrcica/diagnóstico por imagem , Adenoma/cirurgia , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Quiasma Óptico/química , Quiasma Óptico/cirurgia , Paraganglioma/cirurgia , Neoplasias Hipofisárias/cirurgia , Sela Túrcica/química , Sela Túrcica/cirurgia
11.
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
12.
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
13.
Indian Pediatr ; 56(1): 69-71, 2019 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-30806368

RESUMO

BACKGROUND: Hyponatremic-hypertensive syndrome (HHS) is characterized by combination of polyuria, polydipsia, hypertension, hyponatremia and hypokalemia in association with unilateral renal artery stenosis. CASE CHARACTERISTICS: A 10-year- old girl presented with polyuria, polydipsia, hypertension, hyponatremia, hypokalemia and proteinuria. Ultrasonography with doppler study revealed bilateral normal renal arteries. Completed tomography of abdomen detected a left adnexal mass, which was later confirmed as ovarian paraganglioma on histopathology. OUTCOME: After tumor excision, polyuria subsided and blood pressure normalized. MESSAGE: Hyponatremic-Hypertensive Syndrome does not always result from unilateral renal artery stenosis. High index of clinical suspicion with appropriate imaging technique may clinch rare endocrine causes of hypertension, like paraganglioma.


Assuntos
Hipertensão , Hiponatremia , Neoplasias Ovarianas , Paraganglioma , Criança , Feminino , Humanos , Enurese Noturna , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico por imagem , Neoplasias Ovarianas/cirurgia , Paraganglioma/complicações , Paraganglioma/diagnóstico por imagem , Paraganglioma/cirurgia , Poliúria , Síndrome , Tomografia Computadorizada por Raios X
16.
Arch Ital Urol Androl ; 90(4): 297-298, 2019 Jan 17.
Artigo em Inglês | MEDLINE | ID: mdl-30655644

RESUMO

OBJECTIVE: Report our experience of the management of a patient with undiagnosed retroperitoneal paraganglioma and the intraoperative complications that the theatre team faced. CASE REPORT: We present a case of a 36-year-old patient who during oncological follow-up for a previous diagnosis of parotid acinar cell carcinoma was incidentally identified as having an interaortocaval tumour. Following routine preoperative assessment the patient was arranged to undergo a laparoscopic retroperitoneal tumour resection. After minimal tumour manipulation the patient developed cardiac rhythm abnormalities and became hypertensive. The tumour was successfully removed laparoscopically after a cautious inter- aortocaval dissection. Abruptly, prior to extraction of the tumour containing endobag, the patient developed cardiac arrest. Following 35 minutes of life support measures there was a return of spontaneous circulation. The endobag was laparoscopically removed from the abdominal cavity 24 hours later using the initial operative port sites. The patient´s progression was satisfactory and he could be discharged six days postoperatively. CONCLUSIONS: Asymptomatic undiagnosed paragangliomas represent a real challenge during laparoscopic operations. Haemodynamic changes and life-threatening events can arise acutely intraoperatively, where an immediate and coordinated response of the whole theatre team may be required to avoid fatal outcome.


Assuntos
Laparoscopia/métodos , Paraganglioma/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Adulto , Carcinoma de Células Acinares/cirurgia , Parada Cardíaca/etiologia , Humanos , Complicações Intraoperatórias , Masculino , Paraganglioma/cirurgia , Neoplasias Parotídeas/cirurgia , Neoplasias Retroperitoneais/cirurgia
17.
World Neurosurg ; 122: e1482-e1490, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30468922

RESUMO

OBJECTIVE: Large intradural growth of tympanojugular paragangliomas (TJ-PGs) into the cerebellopontine angle is an infrequent condition that can determine an extensive involvement of vessels and brainstem, representing a surgical challenge. The current classifications lack accuracy for defining large intradural TJ-PGs and assessing their operability. This study aims to retrospectively reappraise the management of a large intradural TJ-PGs surgical series, discussing operability criteria, treatment strategies, and resection techniques. METHODS: Over a series of 63 class D TJ-PGs operated on between 1973 and 2008, patients with large intradural tumors were retrospectively reviewed. Surgical techniques and outcomes with long-term follow-up were analyzed. RESULTS: Seven patients presented with large intradural tumors, ranging from 4 cm to 5.5 cm in diameter. These cases were treated either via a petro-occipital transsigmoid approach in a single-stage resection (4 patients) or via a petro-occipital approach as a second-stage surgery after a previous infratemporal fossa approach on the extradural tumor (3 patients). Complete tumor resection was obtained in 5 cases through wide removal of the petro-occipital skull base and exposure of the dural root of the tumor, which carries its main blood supply and allows preemptive hemostasis. No new postoperative cranial nerve losses were observed. The surgical procedure was aborted in 2 cases because of cerebellar edema and carotid artery tear, respectively. CONCLUSIONS: Large intradural TJ-PGs can be effectively surgically managed with an appropriate technique combined with relevant nonsurgical strategies. Thus, operability criteria for these lesions can be extended. To share objective experience on large intradural TJ-PGs, a redefinition of Di3 tumors is recalled.


Assuntos
Paraganglioma/cirurgia , Neoplasias da Base do Crânio/cirurgia , Adulto , Idoso , Ângulo Cerebelopontino , Gerenciamento Clínico , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Paraganglioma/diagnóstico por imagem , Estudos Retrospectivos , Neoplasias da Base do Crânio/diagnóstico por imagem
18.
Chirurg ; 90(1): 29-36, 2019 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-30242437

RESUMO

The therapies available for the rare tumor entity of cervical paraganglioma (PG) are currently undergoing a paradigm shift. The treatment of choice for small carotid body tumors, malignant and active endocrine tumors is surgical resection; however, for locally advanced carotid body tumors and vagal PG, surgical therapy should be critically evaluated. Due to the immediate proximity of these hypervascularized tumors to the caudal cranial nerves, there is a risk of severe nerve damage with a significant impairment of quality of life after resection, particularly for locally advanced cervical PG, emphasizing further the importance of a restrictive surgical strategy. External radiotherapy can provide an equivalent primary therapeutic option with respect to the rate of recurrence and is accompanied by a lower morbidity. The slow rate of tumor progression and the multifocality of the familial variant of cervical PG or significant comorbidities in older, asymptomatic patients warrant a less aggressive treatment strategy for these tumors. When a wait and scan approach is implemented, a closely monitored radiological and clinical re-evaluation is of upmost importance. In a multidisciplinary approach the following critical points require consideration before a therapy is implemented,: size and location of the tumor, progression rate, genetic background, patient age and general condition, relevant comorbidities, the presence of synchronous PG and/or vasoactive catecholamine-producing tumors. Although best practice algorithms for the treatment of cervical PG have already been devised, recent innovative developments have led to more patient-tailored, individualized treatment approaches.


Assuntos
Neoplasias de Cabeça e Pescoço , Paraganglioma , Idoso , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Recidiva Local de Neoplasia , Paraganglioma/cirurgia , Qualidade de Vida
19.
Chirurg ; 90(1): 15-22, 2019 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-30306232

RESUMO

Chromaffin tumors, e.g. pheochromocytomas and paragangliomas are caused by germline mutations of several susceptibility genes in 30-40% of the patients. The corresponding syndromes are multiple endocrine neoplasia type 2 (MEN2, RET gene), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF1), paraganglioma syndrome types 1-5 (PGL1-5, SDHx gene) and familial pheochromocytoma due to mutations in the MAX and TMEM127 genes. Clinically, screening for such diseases should be carried out by clinical symptoms and mutation analyses. Important indications can be found in the history of patients and their families, young age of manifestation (<30 years), extra-adrenal localization and the presence of metastatic pheochromocytomas. Organ-preserving endoscopic adrenal operations are nowadays standard for hereditary pheochromocytomas. Previous studies have shown that the reoccurrence of tumors in residual tissue is rare and can occur many years later and that metastatic tumors arising from such recurrences are very rare. When a mutation is detected in a susceptibility gene, a multidisciplinary follow-up care tailored to each individual syndrome is essential.


Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasia Endócrina Múltipla Tipo 2a , Paraganglioma , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/cirurgia , Humanos , Neoplasia Endócrina Múltipla Tipo 2a/genética , Neoplasia Endócrina Múltipla Tipo 2a/cirurgia , Recidiva Local de Neoplasia , Paraganglioma/genética , Paraganglioma/cirurgia , Feocromocitoma/genética , Feocromocitoma/cirurgia
20.
Surgery ; 165(1): 228-231, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30340856

RESUMO

BACKGROUND: Multifocal pheochromocytoma/paraganglioma presenting at an early age is commonly associated with a hereditary syndrome. CASE REPORT: A 29-year-old woman was referred for evaluation of multifocal pheochromocytoma/paraganglioma. Interestingly, her family history did not include pheochromocytoma/paraganglioma, and comprehensive genetic testing for the well-documented pheochromocytoma/paraganglioma susceptibility genes was negative. Of note, this patient had a history of a complex cardiac defect resulting in cyanotic congenital heart disease and had never undergone operative repair. Thus she lived in a chronic hypoxic state with a baseline oxygen saturation of about 80%. Laboratory evaluation found marked increases in plasma norepinephrine and normetanephrines with normal epinephrine and metanephrines. Imaging revealed 4 aortocaval masses and a right adrenal mass. After appropriate preoperative preparation she underwent successful resection of each of the neoplasms, with pathologic testing revealing multifocal pheochromocytoma/paraganglioma. DISCUSSION: This case highlights a growing recognition of the potential development of pheochromocytoma/paraganglioma in patients with cyanotic congenital heart disease. The underlying pathophysiology and phenotypic similarities between pheochromocytoma/paraganglioma in patients with cyanotic congenital heart disease and those with mutations that lead to cellular pseudohypoxia are reviewed.


Assuntos
Neoplasias das Glândulas Suprarrenais/patologia , Cardiopatias Congênitas/complicações , Paraganglioma/patologia , Feocromocitoma/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Feminino , Humanos , Norepinefrina/sangue , Normetanefrina/sangue , Oxigênio/sangue , Paraganglioma/cirurgia , Feocromocitoma/cirurgia
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