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1.
Medicine (Baltimore) ; 98(44): e17463, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689750

RESUMO

RATIONALE: Malignant peripheral nerve sheath tumor (MPNST) is a very rare sarcoma of the heart, and few cases have been reported. Herein, we retrospectively reviewed clinical manifestations, imaging features and management of our patient and other reported cases. PATIENT CONCERNS: A 32-year-old woman was referred to the emergency department of our institution with expiratory dyspnea, edema of face for a month. DIAGNOSIS: The patient was initially diagnosed with asthma at a local hospital based on a history of fatigue, cough and expiratory dyspnea, as well as negative electroencephalogram (ECG) and chest radiography. Based on computed tomography (CT) and cardiac magnetic resonance imaging (CMRI) in our hospital, she was found to have a malignant tumor involving right atrium. The tumor was diagnosed as MPNST according to histopathological results. INTERVENTIONS: The tumor was deemed unresectable during the surgery. Then, the patient was referred for chemotherapy and radiotherapy. OUTCOMES: The patient deteriorated and died 4 months later. LESSONS: Cardiac MPNST is very uncommon with nonspecific clinical and imaging characteristics according to limited cased reported. CMR, due to the high tissue resolution and multiple sequence imaging advantages, is useful for the detection, location and evaluation whether there is involvement of adjacent structures, and may help better clinical decision-making.


Assuntos
Neoplasias Cardíacas/diagnóstico , Neurofibrossarcoma/diagnóstico , Adulto , Feminino , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/terapia , Humanos , Imagem por Ressonância Magnética , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/terapia , Tomografia Computadorizada por Raios X
2.
Clin Nucl Med ; 44(11): 895-897, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31274561

RESUMO

Melanotic malignant peripheral nerve sheath tumor is an extremely rare tumor, which originates from the neural crest, and more than half the cases are associated with Carney complex (myxomas, spotty pigmentation, and endocrine abnormalities). Herein, we have presented a case of a melanotic malignant peripheral nerve sheath tumor, which is not associated with Carney complex. The patient underwent preoperative nonenhanced CT, contrast-enhanced CT, and F-FDG PET/CT scans, which showed a large pelvic tumor with heterogeneous enhancement and increased F-FDG uptake. Subsequently, the patient underwent complete resection of the tumor.


Assuntos
Meios de Contraste , Fluordesoxiglucose F18 , Neurofibrossarcoma/diagnóstico por imagem , Neoplasias Pélvicas/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Adulto , Humanos , Masculino , Neurofibrossarcoma/patologia , Neoplasias Pélvicas/patologia
3.
Clin Nucl Med ; 44(8): 648-649, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31274612

RESUMO

Findings of Tc-DTPA renal scintigraphy of a retroperitoneal malignant peripheral nerve sheath tumor are reported here. The patient was a 48-year-old woman who presented discomfort and intermittent dull pain in the left upper quadrant of the abdomen for approximately 3 weeks.


Assuntos
Rim/diagnóstico por imagem , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Neoplasias Retroperitoneais/diagnóstico por imagem , Neoplasias Retroperitoneais/patologia , Pentetato de Tecnécio Tc 99m , Feminino , Humanos , Pessoa de Meia-Idade , Cintilografia
4.
Dentomaxillofac Radiol ; 48(7): 20180341, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31188646

RESUMO

Malignant peripheral nerve sheath tumour (MPNST) is a rare mesenchymal tumour which usually presents high grade malignancy. We report an atypical case of intraosseous malignant peripheral nerve sheath tumour on mandible in a 36-year-old male. Patient presented with an incidentally discovered radiolucency on the left anterior mandible and did not complain of any symptoms. Panoramic radiograph and computed tomography showed enlargement of inferior alveolar nerve canal, thinning of the cortex and calcified foci within an expansile mass. Magnetic resonance images revealed heterogeneous hyperintense signal intensity with a well-defined margin on T2 weighted images, heterogeneous enhancement on contrast-enhanced T1 weighted images and intermediate signal intensity and inferior alveolar nerve canal enlargement on T1 weighted images. In spite of benign radiologic characteristics as mentioned above, histopathologic examination of biopsied specimen otherwise revealed a low-grade malignancy. Wide excision of mandible was performed and there has been no loco-regional recurrence or distant metastasis over 1 year following surgery. This case indicates that, even when imaging modalities clearly demonstrate benign nature of intraosseous neurogenic tumour, care must be taken to establish proper treatment plan for assumed malignancy with a definitive histopathological analysis.


Assuntos
Mandíbula , Neoplasias da Bainha Neural , Neurofibrossarcoma , Adulto , Humanos , Imagem por Ressonância Magnética , Masculino , Mandíbula/diagnóstico por imagem , Mandíbula/cirurgia , Neoplasias da Bainha Neural/diagnóstico por imagem , Neoplasias da Bainha Neural/patologia , Neoplasias da Bainha Neural/cirurgia , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Neurofibrossarcoma/cirurgia , Resultado do Tratamento
5.
Int J Cardiovasc Imaging ; 35(9): 1615-1618, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31041634

RESUMO

Malignant peripheral nerve sheath tumors are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. Here we report a case of a primary cardiac malignant peripheral nerve sheath tumor without neurofibromatosis type I. A 53-year old woman presented having had cough, expectoration, and dyspnea for 20 days and was found to have a heart-involving tumor diagnosed as a malignant peripheral nerve sheath tumor, a rare cardiac sarcoma of 9 × 4.5 × 3 cm in size. The patient underwent a successful resection of the tumor but died 14 months postoperative. We report this case for its rarity and peculiar mode of morphologic and immunohistochemical presentation.


Assuntos
Neoplasias Cardíacas , Neurofibrossarcoma , Biomarcadores Tumorais/análise , Biópsia , Procedimentos Cirúrgicos Cardíacos , Ecocardiografia , Evolução Fatal , Feminino , Neoplasias Cardíacas/química , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Neurofibrossarcoma/química , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Neurofibrossarcoma/cirurgia , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Carga Tumoral
6.
World Neurosurg ; 128: 422-425, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31108251

RESUMO

BACKGROUND: Malignant melanotic schwannian tumors (MMSTs) are rare peripheral nerve sheath tumors that typically exhibit benign clinical presentation and histopathology but malignant long-term behavior. CASE DESCRIPTION: We report a case of a 22-year-old male with a T9-11 MMST who presented with acute paraplegia and complete loss of sacral function. Despite emergent decompression, he did not recover motor, sensory or bladder function, although bowel function did normalize. CONCLUSIONS: The anatomic location and rapid presentation of permanent deficits are suggestive of infarction of the spinal cord supplied by the artery of Adamkiewicz, a rare presentation of this disorder and of spinal schwannomas in general.


Assuntos
Infarto/etiologia , Neurofibrossarcoma/complicações , Paraplegia/etiologia , Isquemia do Cordão Espinal/etiologia , Neoplasias da Medula Espinal/complicações , Descompressão Cirúrgica , Humanos , Infarto/diagnóstico por imagem , Infarto/patologia , Masculino , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Neurofibrossarcoma/cirurgia , Distúrbios Somatossensoriais/etiologia , Isquemia do Cordão Espinal/diagnóstico por imagem , Isquemia do Cordão Espinal/patologia , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/patologia , Neoplasias da Medula Espinal/cirurgia , Adulto Jovem
7.
Clin Nucl Med ; 44(6): 494-495, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30889004

RESUMO

MPNSTs (malignant peripheral nerve sheath tumors) are a highly malignant group of soft tissue sarcomas and carry a very poor prognosis. Metastasis to bilateral adrenal glands is very rare in such group of neoplasms. We discuss a case of 85-year-old man who was diagnosed with MPNST from prevertebral mass with metastases to bilateral adrenal glands and bone marrow from the beginning and role of serial F-FDG PET/CT scans in response assessment first to sunitinib (tyrosine kinase inhibitor) and then to liposomal doxorubicin.


Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias da Bainha Neural/diagnóstico por imagem , Neurofibrossarcoma/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Neoplasias das Glândulas Suprarrenais/secundário , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Doxorrubicina/uso terapêutico , Fluordesoxiglucose F18 , Humanos , Masculino , Neoplasias da Bainha Neural/tratamento farmacológico , Neoplasias da Bainha Neural/patologia , Neurofibrossarcoma/tratamento farmacológico , Neurofibrossarcoma/patologia , Compostos Radiofarmacêuticos
9.
J Card Surg ; 34(4): 211-213, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30803029

RESUMO

Primary malignant schwannoma of the heart is an extremely rare disease. We, herein, report a 42-year-old female who underwent successful surgical excision of such a tumor.


Assuntos
Neoplasias Cardíacas/cirurgia , Neurofibrossarcoma/cirurgia , Adulto , Ecocardiografia , Feminino , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Humanos , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Resultado do Tratamento
10.
Neurocirugía (Soc. Luso-Esp. Neurocir.) ; 30(1): 28-32, ene.-feb. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-181458

RESUMO

Se describe el caso de una paciente de 41 años con cuadro de dorsalgia crónica. Mediante RM se diagnosticó una lesión intradural extramedular bien delimitada con morfología en reloj de arena con gran componente paravertebral izquierdo en D5-D6. El tumor fue embolizado previo a la cirugía, realizándose posteriormente laminotomía D4-D6, costotransversectomía D5-D6 izquierdas y resección de la porción intrarraquídea extradural con sección del nervio D5 izquierdo. A continuación, se realizó una resección en bloque del componente extrarraquídeo por vía posterolateral. El diagnóstico anatomopatológico fue de schwannoma maligno, precisando posteriormente tratamiento radioterápico adyuvante. El schwannoma maligno es un tumor poco frecuente perteneciente al grupo de tumores malignos de la vaina del nervio periférico (MPNST), originado en las células de Schwann. Se asocia frecuentemente con la neurofibromatosis tipo 1. Los MPNST recurren con frecuencia a nivel locorregional y pueden dar metástasis a distancia


We report a case of a 41 years old patient complaining of chronic dorsalgia. MRI showed a well defined intradural extramedular dumbbell-shaped lesion, associated to a left paravertebral tumor at D5-D6 level. The tumor was embolizated prior to surgery. Following she underwent a D4-D6 laminotomy, left D5-D6 costotransversectomy and resection of the intracanal extradural part of the lesion with section of the left D5 nerve. Posteriorly, complete resection of the extracanal portion of the tumor was performed by a postero-lateral approach. The anatomopathologic diagnosis was a malignant schwannoma. After the surgery, the patient required adyuvant radiotherapic treatment. Malignant schwannoma is a very uncommon tumor which belongs to the malignant peripheral nerve sheath tumors (MPNST). It develops from Schwann cells. It is frequently associated with type 1 neurofibromatosis. MPNST usually present local recurrence and can metastatize


Assuntos
Humanos , Feminino , Adulto , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/cirurgia , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/cirurgia , Dor nas Costas/diagnóstico por imagem , Radioterapia Adjuvante/métodos , Radiografia Torácica , Neoplasias do Mediastino/diagnóstico por imagem , Diagnóstico Diferencial
11.
Malays J Pathol ; 40(3): 355-358, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30580369

RESUMO

Malignant peripheral nerve sheath tumour (MPNST) with perineurial differentiation is a rare variant of MPNST. The pathological features and clinical significance of this variant remain to be characterised. We reported the clinicoradiological and pathological features of a case of recurrent right arm mass related to the ulnar nerve in a 42-year-old female patient. On pathological examination, the tumour showed dual features of conventional and perineurial MPNST which was proven by positive immunostaining for S-100 and EMA. The pathological diagnosis was MPNST with perineurial differentiation. In addition, a peculiar and rare finding of intracytoplasmic eosinophilic hyaline globules (thanatosomes) within tumour cells is reported. We document a rare tumour with hybrid features between conventional and perineurial MPNSTs. Further studies are needed to establish its biological behaviour.


Assuntos
Antebraço/patologia , Neurofibrossarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Adulto , Diferenciação Celular , Feminino , Antebraço/diagnóstico por imagem , Antebraço/cirurgia , Humanos , Hialina/metabolismo , Imagem por Ressonância Magnética , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/metabolismo , Neurofibrossarcoma/cirurgia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/cirurgia , Ultrassonografia
12.
Neuro Oncol ; 20(6): 818-825, 2018 05 18.
Artigo em Inglês | MEDLINE | ID: mdl-29409029

RESUMO

Background: Neurofibromatosis 1 (NF1) leads to the development of benign and malignant peripheral nerve sheath tumors (MPNST). MPNST have been described to develop in preexisting benign plexiform neurofibromas (PN) and have a poor prognosis. Atypical neurofibromas (ANF) were recently described as precursor lesions for MPNST, making early detection and management of ANF a possible strategy to prevent MPNST. We aimed to clinically characterize ANF and identify management approaches. Methods: We analyzed clinical, imaging, and pathology findings of all patients with NF1 and ANF at 3 institutions. Results: Sixty-three patients had 76 ANF (32M/31F; median age 27.1 y). On MRI, most ANF appeared as distinct nodular lesions and were 18F-fluorodeoxyglucose (FDG) avid. Forty-six ANF were associated with pain, 19 with motor weakness, 45 were palpable or visible, and 13 had no clinical signs. Completely resected ANF (N = 57) have not recurred (median follow-up, 4.1 y; range, 0-14 y). Four ANF transformed into MPNST and 17 patients had a history of MPNST in a different location than was their ANF. Conclusions: Growth of distinct nodular lesions, pain, and FDG-PET avidity should raise concern for ANF in NF1. Patients with ANF are at greater risk for development of MPNST. Complete resection of ANF may prevent development of MPNST.


Assuntos
Imagem por Ressonância Magnética/métodos , Neurofibroma/patologia , Neurofibromatose 1/patologia , Neurofibrossarcoma/patologia , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Neurofibroma/complicações , Neurofibroma/diagnóstico por imagem , Neurofibromatose 1/diagnóstico por imagem , Neurofibromatose 1/etiologia , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/etiologia , Prognóstico , Adulto Jovem
13.
Clin Neurophysiol ; 129(2): 354-359, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29288991

RESUMO

OBJECTIVE: To investigate development of sonographic abnormalities and applications of high-resolution ultrasonography (HRUS) in neurofibromatosis type 1 (NF1). METHODS: Sixteen asymptomatic or minimally symptomatic NF1 patients underwent HRUS at inclusion and 1 year follow-up. Upper and lower extremity nerves were investigated. Peripheral nerve involvement was graded. RESULTS: Plexiform neurofibromas (PNFs) were found in 7 patients (43.8%) at inclusion and 10 (62.5%) at follow-up. All initially identified PNFs were also found at follow-up; additional PNFs were found by extended longitudinal assessment at follow-up. All 3 patients with minor and 7 patients with severe peripheral nerve involvement had similar involvement at follow-up. Mean nerve size change was -0.2 mm2 (±1.6) and 0.3 mm2 (±6.2) in patients with minor and severe involvement. Mean PNF size change was -0.1 mm2 (±9.9). CONCLUSIONS: HRUS allows qualitative assessment of peripheral nerves, which makes it advantageous as initial imaging technique in suspected neuropathy. Patients with minimal nerve involvement remained so, and might therefore require less follow-up for malignant peripheral nerve sheath tumor (MPNSTs) development. Measured change in PNF size was highly variable. Repeating an extensive standardized HRUS protocol during follow-up thus seems less useful to screen for MPNSTs. SIGNIFICANCE: HRUS has potential applications as diagnostic and screening tool in NF1.


Assuntos
Neurofibroma/diagnóstico por imagem , Neurofibromatose 1/diagnóstico por imagem , Neurofibrossarcoma/diagnóstico por imagem , Nervos Periféricos/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Masculino , Ultrassonografia
14.
J Neurosurg Pediatr ; 19(1): 102-107, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27689246

RESUMO

Neurofibrosarcoma is rare in the pediatric age group. A malignant tumor of the sacrum presents significant challenges, especially if the goals are to resect with wide and clean surgical margins and to achieve acceptable functional outcomes. The authors report a case of this rare tumor affecting the sacrum and sacral nerve roots of a 7-year-old girl and review the role of total hemisacrectomy sparing the contralateral sacral nerve roots and lumbopelvic reconstruction in the treatment of this disease. This patient is, to the best of the authors' knowledge, the youngest to be treated in this manner.


Assuntos
Neurofibrossarcoma/cirurgia , Ossos Pélvicos/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Articulação Sacroilíaca/cirurgia , Sacro/cirurgia , Raízes Nervosas Espinhais/cirurgia , Criança , Feminino , Seguimentos , Humanos , Neurofibrossarcoma/diagnóstico por imagem , Ossos Pélvicos/diagnóstico por imagem , Articulação Sacroilíaca/diagnóstico por imagem , Sacro/diagnóstico por imagem , Raízes Nervosas Espinhais/diagnóstico por imagem
17.
Acta Clin Belg ; 68(4): 309-10, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24455804

RESUMO

Drug-induced pancreatitis is rare (1.4-2%). This report describes a 20-year-old female patient who developed acute pancreatitis while being treated for neurosarcoma of abdominal wall with the ifosfamide and doxorubicin regimen. Although it is unusual, it is important to consider chemotherapeutic agents as a possible etiology for acute pancreatitis in patients presenting with gastrointestinal symptoms.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neurofibrossarcoma/tratamento farmacológico , Pancreatite/induzido quimicamente , Parede Abdominal , Doxorrubicina/administração & dosagem , Feminino , Humanos , Ifosfamida/administração & dosagem , Neurofibrossarcoma/diagnóstico por imagem , Radiografia , Adulto Jovem
18.
Kyobu Geka ; 58(4): 337-40, 2005 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-15828258

RESUMO

We report a case of a 27-year-old woman with primary neurogenous sarcoma of the lung. She had no symptoms but an abnormal shadow of the right lower lung field on the chest X-ray. Chest computed tomography (CT) revealed a well defined round mass, 20 mm in maximum diameter, at the right S9. Pathological study of the specimen obtained by CT-guided percutaneous needle biopsy showed undefferentiated carcinoma. Positron emission tomography (PET) disclosed intensely increased uptake of fluoro-2-deoxy-D-glucose (FDG) at the lung lesion without other abnormal uptakes. The patient underwent right lower lobectomy of the lung and mediastinal lymph nodes dissection. Results from immunohistological study yielded a definitive diagnosis of neurogenous sarcoma. Postoperative course was uneventful, and there has been no evidence of recurrence and metastasis for more than a year after the surgery. Reported cases of primary neurogenous sarcoma of the lung are reviewed.


Assuntos
Neoplasias Pulmonares/cirurgia , Neurofibrossarcoma/cirurgia , Pneumonectomia , Adulto , Feminino , Humanos , Neoplasias Pulmonares/diagnóstico por imagem , Excisão de Linfonodo , Neurofibrossarcoma/diagnóstico por imagem , Pneumonectomia/métodos , Tomografia Computadorizada por Raios X
20.
Dtsch Med Wochenschr ; 129(8): 364-7, 2004 Feb 20.
Artigo em Alemão | MEDLINE | ID: mdl-14961444

RESUMO

HISTORY: A 65-year-old man had noted a tumor of the right lower leg that had progressively grown over the last twenty years. He had a dyspnoea on insignificant movement and was relatively immobile because of the extent of the tumor. The tumor had been diagnosed as a Klippel-Trenaunay syndrome. INVESTIGATIONS: Laboratory investigations suggested a hypochromic and microcytic anaemia and an inflammatory constellation. The tumor had no av-fistulas on ultrasonography and angiography, but there were many pathological arteries and tumor nodes. The tumor nodes had been seen also on MRI-biopsy suggested a neurogenic sarcoma. DIAGNOSIS AND TREATMENT: Amputation of the leg was necessary. The histological diagnosis was neurofibrosarcoma with extensive necrotic areas and Recklinghausen disease. Investigation did not reveal any metastasis. Postoperatively the anaemia regressed and the patient learned to walk with a prosthesis. CONCLUSIONS: This history shows the problem of separating clearly the two hereditary diseases. Often there is a different clinical picture in everyday life. One must consider the definitely higher risk of malignant tumors of patients with neurofibromatosis. Ultrasonography is a non-invasive method that can provide morphological information on soft parts and pathology of the vessels. It facilitates the classification of such.


Assuntos
Neurofibromatose 1/complicações , Neurofibrossarcoma/complicações , Idoso , Amputação , Membros Artificiais , Diagnóstico Diferencial , Humanos , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Perna (Membro)/cirurgia , Masculino , Neurofibromatose 1/diagnóstico por imagem , Neurofibromatose 1/patologia , Neurofibromatose 1/cirurgia , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/patologia , Neurofibrossarcoma/cirurgia , Resultado do Tratamento , Ultrassonografia
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