Thoracoscopically guided transaxillary resection of adjoining intercostal plexiform neurofibromas: review of mosaicism in neurofibromatosis: technical note.

Objective And Importance: The present article describes a rare presentation of Type I neurofibromatosis (NFI) involving adjoining intercostal plexiform neurofibromas, as well as the novel use of thoracoscopy to guide surgical resection. This presentation highlights the manner in which NFI may affect selective regions of the body disproportionately through genetic mosaicism.

Clinical Presentation: A 40-year-old man had intractable neuropathic anterior right chest wall pain in the distribution of T2 and T3. Magnetic resonance imaging and computed tomography of the chest revealed masses deep to the second and third ribs in the axilla. Biopsy of a subcutaneous nodule in the right chest wall revealed a small neurofibroma. An extensive workup revealed no masses outside the region of the right chest wall and no stigmata of NFI.

Intervention: The patient underwent right-sided thoracoscopy for identification of the intrathoracic neurofibromas and placement of spinal needles to localize the anterior and posterior extent of the masses. These landmarks were used to guide a transaxillary approach to third rib resection and nerve-sparing neurofibroma resection.

Conclusion: Isolated adjoining intercostal plexiform neurofibroma is a unique presentation of mosaic NFI. Because of its limited penetrance, this variant may present as a regional pain syndrome. Thoracoscopy can be used effectively to guide intercostal nerve sheath tumor resection.