Tumor maligno de la vaina del nervio periférico del ciático izquierdo / Malignant peripheral nerve sheath tumor of the left sciatic nerve

RESUMEN Fundamento: los tumores malignos de la vaina del nervio periférico, son sarcomas de partes blandas con una baja incidencia. Objetivo: presentar un caso de tumor maligno de la vaina del nervio ciático izquierdo que recibió tratamiento quirúrgico en el Hospital Universitario Manuel Ascunce Domenech de la provincia Camagüey en junio de 2018. Presentación del caso: paciente masculino de 45 años, antecedentes de neurofibromatosis tipo I. Historia de dolor ciático troncular izquierdo que se hizo constante. Refirió dificultad para la marcha y aumento de volumen a nivel del glúteo izquierdo. Se palpó tumoración de siete centímetros de diámetro de superficie lisa, firme, fija a planos profundos y dolorosos a la manipulación. La ultrasonografía de partes blandas mostró: imagen compleja de localización profunda hipoecóica ovalada de 45 X 9 X 20 milímetros, de contornos regulares, bien definidos a una profundidad de la piel de 22 mm. Se decidió tratamiento quirúrgico basado en la excéresis de la tumoración. La biopsia definitiva luego de la inmunohistoquímica informó: tumor maligno de la vaina del nervio periférico de alto grado en un neurofibroma previo. Conclusiones: los tumores malignos de la vaina del nervio ciático son neoplasias raras y aún más su localización proximal. En el caso que se presentó a pesar de su resección quirúrgica radical asociada a radioterapia, el tamaño superior a diez centímetros, la asociación a la neurofibromatosis tipo I y el alto grado histológico, ensombrecen el pronóstico. Es importante el diagnóstico precoz y tratamiento oportuno.

ABSTRACT Background: malignant peripheral nerve sheath tumors are soft tissue sarcomas with a low incidence. Objective: to present a case of malignant peripheral nerve sheath tumor of the left sciatic nerve who underwent surgical treatment at the University Hospital Manuel Ascunce Domenech in the province of Camagüey in June, 2018. Presentation of the case: a 45-year-old male patient, personal history of neurofibromatosis type 1 with a truncal left sciatic pain that turned permanent. He referred gait difficulties and increase of the volume in the left buttock. A tumor mass of seven centimeters was palpated, firm, fix to deep planes and painful at deep palpation. Soft tissue ultrasonography showed: complex oval hypoechoic image of deep location of 45 X 9 X 20 millimeters of regular and well defined contours at 22 millimeters depth from the skin. Surgical treatment was decided base on the tumor resection and radiotherapy. Definitive biopsy after immune histochemistry, informed a high degree malignant peripheral nerve sheath tumor of a previous neurofibroma. Conclusions: malignant peripheral nerve sheath tumors are weird neoplasias and even more its proximal location. The presented patient despite the radical surgical resection associated to radiotherapy, the size superior to ten centimeters, the association to neurofibromatosis type 1 and the high histological degree, worsen the forecast. The precocious diagnosis and accurate treatment are important.

Benign tumors affecting the median nerve. Case series report of diagnostic and surgical strategies / Tumores benignos que afetam o nervo mediano. Relato das estratégias cirúrgicas e diagnósticas na série de casos

ABSTRACT Objective: The aim of this study was to describe the strategies adopted in this institution to diagnose and treat patients with benign tumors affecting the median nerve. Methods: A retrospective chart review study of all patients operated on between 2010 and 2015. Histology, symptoms, complementary exams, surgical techniques performed, and demographic characteristics were analyzed. Results: Fifty-four patients were included in the study. There were three neurofibromas, six schwannomas, 15 lipofibromatous hamartomas, three hemangiomas, 12 lipomas, one benign fibrohistiocytoma, and 14 synovial cysts. Complete tumoral resection was performed in 32 cases, partial resection in five, segmented nerve resection in one, nerve decompression in eight, and amputation for macrodactyly in eight. Conclusions: The most important recommendations on treating benign tumors of the median nerve are related to the clinical symptoms, tumoral growth, and tumoral nature. The surgical approach resulted in good function for 60% of the patients. However, lipofibromatous hamartomas, hemangiomas, and neurofibromas were associated with preoperative functional deficit. It may be inferred that the diagnosis and treatment of these tumors should be performed earlier.

RESUMO Objetivo: Descrever as estratégias adotadas nesta instituição para o diagnóstico e tratamento de pacientes com tumores benignos que afetam o nervo mediano. Métodos: Um estudo de revisão retrospectivo foi feito com todos os pacientes operados entre 2010 e 2015. Foram analisados histologia, sintomas, exames complementares, técnicas cirúrgicas aplicadas e características demográficas. Resultados: O estudo incluiu 54 pacientes. Observaram-se três casos de neurofibromas, seis schwannomas, 15 hamartomas lipofibromatosos, três hemangiomas, 12 lipomas, um fibro-histiocitoma benigno e 14 cistos sinoviais. Em 33 casos, foi feita ressecção tumoral completa; em cinco, ressecção parcial; em um, ressecção segmentar de nervo; em oito, descompressão de nervo; e em oito, amputação de macrodactilia. Conclusões: As recomendações mais importantes no que diz respeito ao tratamento de tumores benignos do nervo mediano estão relacionadas aos sintomas clínicos, ao crescimento tumoral e à natureza tumoral. A abordagem cirúrgica levou a bons resultados funcionais em 60% dos pacientes. No entanto, hamartomas lipofibromatosos, hemangiomas e neurofibromas foram associados ao déficit funcional pré-operatório. Pode-se inferir que o diagnóstico e o tratamento desses tumores devem ser feitos de forma precoce.

Primary Hepatic Schwannoma / 대한소화기학회지

A primary benign schwannoma of the liver is extremely rare. Only 30 cases have been reported in the medical literature worldwide, and only one case has been reported in Korea previously. A 56-year-old man was admitted to Gil Medical Center with incidental findings of a hepatic mass by abdominal computed tomography. The computed tomography and magnetic resonance image revealed a 3×2 cm-sized solid mass in the left lobe of the liver. Histological examination confirmed the diagnosis of a benign schwannoma, proven by positive immunoreaction with the neurogenic marker S-100 protein and a negative response to CD34, CD117, and smooth muscle actin. We report a primary benign schwannoma of the liver and review the literature.

Neurofibroma Cantal Solitario: Reporte de Caso / Canthal Solitary Neurofibroma

Objetivos: presentar el caso de una paciente que desarrolló un neurofibroma solitario de localización cantal interna. Diseño de estudio: reporte de caso. Métodos: se reporta el caso de una paciente con un neurofibroma solitario localizado en el canto interno, a la vez que se presenta una breve revisión bibliográfica sobre esta patología infrecuente. Conclusiones: el neurofibroma solitario es una patología infrecuente de naturaleza benigna que puede comprometer el globo ocular, el párpado y la órbita, por lo que debe ser considerado dentro de los diagnósticos diferenciales de la patología tumoral de estas áreas anatómicas.

Objectives: to report the case of a patient with a solitary neurofibroma in the medial canthal area. Study design: case report. Methods: report the case of a patient with a solitary neurofibroma in the medial canthal area and review the pertinent literature. Conclusions: the solitary neurofibroma is a rare benign condition with the potential to compromise the eye, the eyelid and the orbit, so that should be considered in the differential diagnosis of tumoral pathology in these anatomic areas. Keywords: nerve sheath neoplasms, peripheral nervous system neoplasms, neurofibroma.

The MRI findings of meningeal peripheral-type primitive neuroectodermal tumors / 中华放射学杂志

Objective To analyze the MRI features of Ewing sarcoma/peripheral primitive neuroectodermal tumors(pPNETs) arising from the meninges.Methods The MRI imaging of 9 patients with Ewing sarcoma/pPNET were reviewed retrospectively,and imaging features and pathological characteristics were analyzed.Results The age of most patients ranged from 10 to 20 years.Magnetic resonance revealed a spindle-like lesion with a wide base in 8 cases.The lesions showed heterogenous iso-or hypo-intense signal on T1 WI in 7 cases,heterogenous hypo-iso-intense signal on T1 WI in 2 case,and iso-or mildly hypderisointense on T2WI in all cases.The solid part of the tumor was heterogeneously enhanced after injection of gadolinium with cyst degeneration or necrosis.The dural tail sign could be seen in 3 cases.The adjacent skull erosion could be seen in 6 cases.The breakthrough of the plate of cranium and soft-tissue invasion was present in 2 cases.The right eye proptosis was present in 1 case.The distant metastasis was found in 3 cases.Pathology showed that the lesions had high cell density.Hemorrhage and necrosis could be observed.The cells were like lymphocytes and spindle cells with transparent cytoplasm.CD99 and Vimentin were expressed in all tumor cells.Conclusion The imaging findings of the meningeal pPNET are different from meningiomas,which could be useful for the diagnosis and differential diagnosis.

Pathologic study on the perineural invasion in 513 cases of pancreatic cancer / 中华胰腺病杂志

Objectives To investigate the characteristics of neural invasion of pancreatic cancer as well as its relationship with other clinicopathological factors. Methods The neural invasion situation of 491 cases of ductal adenocarcinoma and other 22 pancreatic malignancies, 41 cases of benign tumor of pancreas and 21 cases of chronic pancreatitis was observed under light microscope, and its relationship with other clinicopathological factors was analyzed. Results The rate of neural invasion in ductal adenocarcinoma (74%) was much higher than in other types of pancreatic neoplasm (23% ,P ＜ 0.01). Pancreatic ductal adenocarcinoma cell often invaded through peripheral nerve membrane into inner nerve fiber bundle, sometimes even invaded the whole cross-sectional nerve fiber. But neural invasion was not associated with differentiation of the tumor. The occurrence of chronic inflammation in the para-tumoral pancreas (52%) was also higher than that in other types of malignant (14%) or benign lesions (15% ,P ＜0.01). Lymphocytes neural invasion rate in pancreatic ductal adenocarcinoma was 65%, which were significantly higher than those in other types of malignant (36%) or benign lesions (22%, P ＜ 0.01). Neural invasion rate in pancreatic ductal adenocarcinoma was associated with paratumoral chronic pancreatic inflammation and lymphocytes neural invasion, but not with lymph node metastasis. Conclusions Neural invasion was characteristic biological behavior in pancreatic ductal adenocarcinoma.

Schwannomatosis Involving Peripheral Nerves: A Case Report

Schwannomatosis or neurilemmomatosis has been used to describe patients with multiple nonvestibular schwannomas with no other stigmata of neurofibromatosis type-2 (NF-2). In our case, schwannomatosis, multiple schwannomas were present in a 21-yr-old woman with no stigmata or family history of NF-1 or NF-2. She had no evidence of vestibular schwannoma or other intracranial tumors. Multiple peripheral tumors were found in the carotid space of the neck, and soft tissue of posterior shoulder, lower back, ankle and middle mediastinum. All of those tumors were completely limited to the right side of the body. All surgically removed tumor specimens in this patient proved to be schwannomas.