Chamale Javier Biurrun, Bruno Marco, Mandojana Facundo, José Llugdar, Armando Lucas, Alejandro Doniquian
Servicio de Cirugía general, (General Surgery Services) Clínica Universitaria Reina Fabiola, Universidad Católica de Córdoba, Oncativo 1248, Córdoba Capital, Argentina.
Servicio de Cirugía general, (General Surgery Services) Clínica Universitaria Reina Fabiola, Universidad Católica de Córdoba, Oncativo 1248, Córdoba Capital, Argentina.
Int J Surg Case Rep. 2017;41:71-75. doi: 10.1016/j.ijscr.2017.09.026. Epub 2017 Oct 12.
Malignant fibrous histiocytoma is a sarcoma of uncertain origin that can be found both in soft tissues and in bones. It is currently called undifferentiated pleomorphic sarcoma not otherwise specified and it represents a final common pathway in several tumors that are subject to the progress of dedifferentiation. Local recurrence of the tumor in the same location where it was originated occurs in 20-30% of the total number of soft tissue sarcomas. It is less frequent in limbs and more likely to recur in retroperitoneal sarcomas and in head and neck. Most tumors grow during the first two years after treatment. Most sarcoma metastases, especially those in the head and neck (including malignant fibrous histiocytoma) present as a lung disease (90%). Extrapulmonary diseases are not frequent and can occur in lymph nodes (10%), bones (8%) and in the liver (1%).
61 years old woman with history of malignant fibrous histiocytoma in the right portion of the mandible, resected four years ago. Subsequent checkups did not show any disease. The patient reports a constant abdominal pain not diagnosed, related to a pancreatic nodule.
Malignant fibrous histiocytoma is a potentially curable disease. The most important part of the treatment is complete surgical removal, usually followed by adjuvant radiation therapy. Chemotherapy is a treatment usually used only on those patients with higher recurrence risk or on those showingrecurrence or who have already showed recurrence.
恶性纤维组织细胞瘤是一种起源不明的肉瘤，可发生于软组织和骨骼。目前它被称为未另行指定的未分化多形性肉瘤，它代表了几种经历去分化过程的肿瘤的最终共同途径。肿瘤在其起源部位的局部复发发生在软组织肉瘤总数的20% - 30%。在四肢较少见，而在腹膜后肉瘤以及头颈部更易复发。大多数肿瘤在治疗后的头两年内生长。大多数肉瘤转移，尤其是头颈部的转移（包括恶性纤维组织细胞瘤）表现为肺部疾病（90%）。肺外疾病不常见，可发生于淋巴结（10%）、骨骼（8%）和肝脏（1%）。
一名61岁女性，四年前切除了右下颌骨恶性纤维组织细胞瘤。随后的检查未发现任何疾病。患者自述持续腹痛，未确诊，与胰腺结节有关。
恶性纤维组织细胞瘤是一种潜在可治愈的疾病。治疗的最重要部分是完整的手术切除，通常随后进行辅助放疗。化疗通常仅用于复发风险较高的患者或已出现复发的患者。
