Tumor de plexo braquial con extensión cervico torácica en paciente pediátrico / Brachial plexus tumor with cervical thoracic extension in a pediatric patient

Introducción: Los tumores de plexo braquial constituyen una entidad infrecuente. Cuando la lesión cumple con criterios quirúrgicos, el tratamiento ideal es la exéresis completa. Descripción del caso: Paciente pediátrica con antecedentes de neurofibromatosis que consulta por dolor leve y parestesias episódicas. Presenta masa palpable en región supraclavicular izquierda de consistencia duro elástica con signo de Tinel negativo. Se realiza una exéresis completa del neurofibroma mediante una cervicotomía anterior. Discusión: La indicación quirúrgica fue realizada en base al crecimiento tumoral y su efecto de masa sobre estructuras contiguas, los trastornos sensitivos y su antecedente de NF1, que predispone a variantes malignas. La clavícula suele ser el elemento anatómico que define las principales vías de abordaje al plexo braquial. Conclusión: Dejamos reportado un caso de neurofibroma de plexo braquial en una paciente pediátrica con neurofibromatosis. Describimos como la cervicotomía anterior fue una excelente vía a una masa extensión cervico torácica

Introduction: Brachial plexus tumors are an infrequent entity. When the lesion meets surgical criteria, the ideal treatment is its complete removal. Case report: A pediatric patient with a history of neurofibromatosis consults for mild pain and episodic paresthesias. The patient presented to the hospital for an elastic palpable mass in the left supraclavicular region testing negative for Tinel's sign. Complete removal of the neurofibroma was performed through an anterior cervicotomy. Discussion: The surgical indication was made based on the growth of the tumor and its mass effect on contiguous structures, the patient's sensory disorders and her neurofibromatosis history, which predisposes to malignant variants. The clavicle is usually the anatomical element defining the main approaches to the brachial plexus. Conclusion: The purpose of this article was to present a case of a of brachial plexus neurofibroma in a pediatric patient with neurofibromatosis. The anterior cervicotomy it would seem to be an excellent approach to a cervical thoracic extension mass

Brachial Plexus Tumors in a Consecutive Series of Twenty One Patients

OBJECTIVE: This is a retrospective review of 22 surgically treated benign and malignant tumors of brachial plexus region to describe clinical presentation, the characteristics of brachial plexus tumor and clinical outcomes with a literature review. METHODS: Twenty-one patients with consecutive 22 surgeries for primary brachial plexus tumors were enrolled between February 2002 and November 2011 were included in this study. The medical records of all patients were reviewed. RESULTS: Eleven male and 10 female patients were enrolled. Mean age was 39 years. Three patients had brachial plexus tumor associated with neurofibromatosis (13.6%). Presenting signs and symptoms included parenthesis and numbness (54.5%), radiating pain (22.7%), direct tenderness and pain (27.2%), palpable mass (77.3%). Twelve patients presented preoperative sensory deficit (54.5%) and 9 patients presented preoperative motor deficit (40.9%). Twenty tumors (90.9%) were benign and 2 tumors (9.1%) were malignant. Benign tumors included 15 schwannomas (68.2%), 4 neurofibromas (18.2%) and 1 granular cell tumor (4.5%). There were 1 malignant peripheral nerve sheath tumor (MPNST) and 1 malignant granular cell tumor. Gross total resection was achieved in 16 patients (72.7%), including all schwannomas, 1 neurofibroma. Subtotal resection was performed in 6 tumors (27.3%), including 3 neurofibromatosis associated with brachial plexus neurofibromas, 1 MPNST and 2 granular cell tumor in one patient. CONCLUSION: Resection of tumor is the choice of tumor in the most of benign and malignant brachial plexus tumors. Postoperative outcomes are related to grade of resection at surgery and pathological features of tumor.

Neurofibroma de plexo braquial - relato de caso / Neurofibroma of Brachial plexus - case report

Objetivo: relatar um caso de uma paciente de 6 anos, portadora de neurofibromatose (NF), tipo 1, com presença de neurofibroma em plexo braquial direito. Método: descrição e análise dos dados clínico-cirúrgicos da paciente com neurofibromatose atendida na Fundação Hospital Santa Casa de Misericórdia do Pará, em abril de 2005. Relato de caso: J.L.A. do sexo feminino, 6 anos, com queixa de aumento de volume e dor na região axilar direita, referindo parestesia no membro superior, correspondente (MSD), há 1 ano e 3 meses. O exame clínico revelou a presença de volumosa nodulação localizada na região axilar direita, com extensão em trajeto dos nervos mediano e cubital até 01/3 distal do MSD. Exame de ressonância magnética da região axilar comprovou a existência de massa tumoral de características neoplásicas na região axilar D e braço D. Submetida a tratamento microcirúrgico com ressecção completa da tumoração. Exame histopatológico confirmou o diagnóstico de neurofibroma, sem sinais de malignidade. Considerações Finais: a possibilidade de transformação maligna dos neurofibromas em pacientes com NF é motivo de constante preocupação. Alterações no quadro clínico, crescimento acelerado ou surgimento de sintomatologia neural, justificam intervenção cirúrgica precoce, e, muitas das vezes, tal conduta possibilita uma cura local do processo.

Neurofibromatosis is an dominant autosomic disease with skin abnormalities and nervous system neoplasms -Café-au-lait spots and fibrous peripheral neoplasms (neurofibromas) are the main features. There is also a high incidence of central nervous system, cranial nerves and spinal cord primary neoplasms, with tendency to malignization. Objective: A six years-old female patient with type 1 neurofibromatosis, harboring a huge right braquial plexus neurofibroma. Methods: Description and analysis of main clinical-pathological features of a young female harboring a huge mass in the brachial plexus treated at the Santa Casa Hospital. Case Report: Patient J.L.A., female, six years-old, with complaints of pain and a tumor in the right axilar region, with extension to right median and cubital nerves. MR examination revealed a large mass at the brachial plexus. A microsurger was perfomzed in a two-staged fashion, with complete removal of lesion. Histology revealed a typic neuroJibrom( with no signs of malignancy.Conclusions: Malignancy of a neurofibroma is always a concerning. Thus, earl interventions and complete removal of such lesions are the goal of the treatment.