Neurofibroma is also known as neuromembrane tumor, neuroma, perineural fibroma, schwannoma, or perineural fibroblastoma. The numerous names for this disease reflect differing views on its origin, which can generally be categorized into the following types: the first type is neuromembrane tumor or schwannoma; the second type is neurofibroma or perineural fibroblastoma, referring to tumor cells derived from the connective tissue of the neural mesoderm. Neurofibromas can originate from peripheral nerves, cranial nerves, or sympathetic nerves.

bubble_chart Pathological Changes

Nerve sheath tumors have a complete membrane, are solitary, occasionally with edema or cystic changes, and are generally benign, rarely malignant. Neurofibromas mostly lack a membrane, can be solitary or multiple. If accompanied by widespread subcutaneous nodules and skin pigmentation, it is called multiple neurofibromatosis, also known as Von Recklinghausen's disease, which is prone to malignant transformation. Pathologically, tumor cell arrangement has two types: ① Tumor cells are arranged in whorls or parallel to each other, forming a palisade pattern, known as Antoni type A; ② The tissue structure is loose, resembling a myxoma, with irregular cell arrangement and uneven size and shape. There is often edema fluid between tumor cells, forming microcysts or vesicles, known as Antoni type B.

bubble_chart Clinical Manifestations

There is no significant gender difference among patients, with the majority being young and middle-aged adults. The growth is slow, and the disease course can last over ten years. In the early stages, there are often no symptoms. In the late stage (third stage), symptoms depend on the tumor's location and size and may include nasal obstruction, minor nosebleeds, headaches, and varying degrees of deformity in the nasal cavity or face. If accompanied by Von Recklinghausen disease, there may also be intellectual developmental delays.

During examination, the tumor appears pink or gray-white, with a broad, flat base that is immobile or pedunculated, and has a firm texture. A pathological examination is required for definitive diagnosis.

bubble_chart Treatment Measures

Insensitive to radiation, surgical resection is required. The surgical approach can be chosen based on the situation, such as through the anterior nostril, lateral nasal incision, external nasal and ethmoid sinus approach, or Caldwell-Luc operation. The midface degloving procedure offers advantages such as a wide operative field and no postoperative facial scars, making it the optimal surgical method for resecting neurofibromas in the nasal cavity and paranasal sinus region.