1 型神经纤维瘤病巨大软组织神经纤维瘤的手术治疗

A. Valiev, A. Salkov, N. S. Petrochenko
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摘要

导言。神经纤维瘤病 1 型的神经纤维瘤很少会长得很大。然而,在出现大面积病变的情况下,选择最佳治疗策略可能会很复杂。临床医生通常会采用手术治疗,这是目前改善患者生活质量和达到良好美容效果的有效手段。描述一名巨大神经纤维瘤 1 型患者的成功手术治疗经验。患者现年 22 岁,在 N.N. Blokhin 国立肿瘤医学研究中心就医,诊断为 1 型神经纤维瘤病,背部下三分之一、臀部和大腿组织出现大面积病变。患者自出生起就患有此病,家族病史也很复杂:父亲死于神经源性肿瘤的恶性表现。妹妹也被诊断出患有神经纤维瘤病,脑部受到影响,身体上出现咖啡斑。由于巨大的肿瘤大大降低了患者的生活质量,为了缓解患者的病情,医生为其开具了细胞切除手术治疗处方,旨在最大限度地根治切除所有肿瘤。本临床病例展示了手术治疗巨大神经纤维瘤患者的成功经验。
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Surgical treatment of a massive soft tissue neurofibroma in neurofibromatosis type 1
Introduction. Neurofibromas developing in neurofibromatosis type 1 rarely grow to a large size. However, in cases of massive lesions, selection of optimal treatment tactics can be complicated. Clinicians usually resort to surgical treatment which currently serves as an effective instrument for improving patients’ quality of life and for achieving good cosmetic results.Aim. To describe a successful experience of surgical treatment in a patient with giant neurofibroma type 1 affecting a massive pool of soft tissues.Clinical case. Patient, 22 years, sought medical help at the N.N. Blokhin National Medical Research Center of Oncology with diagnosis of neurofibromatosis type 1 with massive lesions in the tissues of the lower third of the back, gluteal region, thighs. The disease has existed since birth, family medical history is fraught: father died of malignant manifestation of a neurogenic tumor. The younger sister is also diagnosed with neurofibromatosis affecting the brain and manifesting through cafe au lait spots on the body. Due to massive tumors significantly decreasing the patient’s quality  of life, cytoreductive surgical treatment was prescribed aimed at maximally radical resection of all available tumors to alleviate the patient’s condition.Conclusion. The presented clinical case demonstrates successful experience of surgical treatment of a patient with giant neurofibroma affecting a massive pool of soft tissues.
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