Neurofibromas are a relatively rare type of tumor, accounting for 5-10% of malignant soft tissue tumors.

bubble_chart Pathological Changes

1. To the naked eye, a typical tumor is attached to a larger nerve trunk, but the fleshy tumor originates from an unnamed nerve, so no nerve can be found. The lesion is deep, and like other fleshy tumors, the cut surface is gray-white, possibly accompanied by hemorrhage and necrosis.

2. Under the microscope, malignant Schwann cells can be seen. The tumor cells are spindle-shaped, with large, deeply stained nuclei of uneven sizes, and mitotic figures can be observed. Epithelioid Schwann cells can also be seen, with the tumor cells being round and polymorphic, with varying amounts of cytoplasm, pink-stained granular, arranged in solid nests, and also in acinar or cord-like structures.

bubble_chart Clinical Manifestations

The age of onset is more common in young and middle-aged individuals. The preferred sites are often the head and neck, buttocks, limbs, and the posterior abdominal membrane, but can also occur on the back, abdominal wall, and mediastinum. Generally, it presents as a painless tumor mass that develops slowly. In some cases, there is initial pain in the affected limb, followed by the appearance of a mass and dysfunction of the involved nerve. Local tenderness is present, and 40% of cases are accompanied by neurofibromatosis. Due to mild symptoms, the tumors are often large at the time of consultation, with most having a long diameter of over 10 cm.

bubble_chart Treatment Measures

The primary treatment is wide excision. For cases where wide excision is not feasible due to recurrence, amputation may be performed. For tumors located within the pelvis or paravertebral regions that cannot be widely excised or are incompletely resected, radiotherapy may be used as an adjunct, although the efficacy is poor. Literature reports indicate that chemotherapy is effective for metastatic tumors, with commonly used drugs including doxorubicin, vincristine, and cyclophosphamide. Hematogenous and lymphatic metastases can occur, with a 5-year survival rate of approximately 30%.

It is mainly distinguished from fibrous fleshy tumors. The cells of fibrous fleshy tumors are spindle-shaped, arranged in an interwoven pattern, with significant atypia, but without palisading nuclear arrangement, and the tumor is not connected to the nerve trunk.