Recurrent Schwannomatosis of the Hand: A Case Report and Relevant Literature Review Rowland Pettit1, Nikhil Agrawal MD1, Razvan Nicolescu MD1 David T Netscher, MD1,2 1Baylor College of Department of Plastic , 2Houston Methodist Hospital Department of

BACKGROUND RESULTS CONCLUSIONS

Peripheral Nerve Other associated • Peripheral nerve sheath tumors (PNSTs) 1. Segmental excision of the affected radial digital nerve was performed. Sheath Tumor Diagnostic signs signs or Inheritance Differential symptoms are neoplastic soft tissue masses 2. A pulp flap based on the contralateral neurovascular resulted in a sensate, pain free digit. Diagnosis

generated from the abnormal proliferation 3. Tissue confirmed the diagnosis of multiple neurilemmomas. Intermittently painful, If malignant: Solitary peripheral solitary lesion with non- weight loss, night Sporadic of Schwann cells. nerve sheath tumor aggressive features, + sweats • Often these tumors occur in isolation, Tinel’s sign Presence of two or more “” or “neurilemmomas”, peripheral schwannomas Spares acoustic Familial (AD) Schwannomatosis with absence of clinical nerve or sporadic rarely as several “Schwannomatosis” signs of • The purpose of this report is two-fold; (1)

Neurofibromatosis Peripheral , Café-au-lait spots, to review the relevant literature and Familial (AD) describe a unique case of this rare type 1 (NF 1) plexiform Lisch nodules

condition, and (2) to emphasize salient Bilateral schwannomas Neurofibromatosis affecting the acoustic Cataracts, Familial (AD) clinical considerations in the diagnosis type 2 (NF 2) nerve (cranial nerve 8), gliomas and treatment of Schwannomatosis hearing loss Figure 1. Clinical photograph of Figure 2. MRI STIR coronal Figure 3. We excise the entire • This case illustrates an unusual CASE the volar aspect of the hand (Left) image of multiple round mass of digital schwannomas (Left) demonstrates bead-like masses. T1 sagittal post from distal to the branching of presentation of Schwannomatosis. schwannomas extending from gadolinium (Right) illustrates the main trunk of the median • We emphasize the importance of • In this report we describe the case of a the depths of the 1st webspace the beads-on-a-string sign. nerve to the radial digital fingertip  reviewing prior pathology and 52- year old Caucasian male who into the index fingertip. neurovascular structure. operative reports whenever presented with multiple recurrent soft performing repeat tissue masses of the right hand.  establishing a pre-operative • On initial presentation he described pain differential diagnosis across his right hand and index finger,  valuing preoperative imaging which persisted despite numerous prior • It was through careful consideration of operations. surgical options and specific skin flap • The index finger had a flexion contracture design, that this patient was able to around the location of the proximal Figure 4. (Left) H&E, retain a fully sensate, pain free, and interphalangeal joint and there were 75x Photomicrograph mobile index finger post operatively multiple tender masses along the length with Antoni A hyper- after definitive for of the finger and palm. and Antoni B hypo- Schwannomatosis. • There were 3 prior surgical excisions, cellularity. (Above) MacCollin et al. Diagnostic criteria for schwannomatosis. 2005. each followed by recurrence. Chick et al. Sporadic Schwannomatosis: A Systematic Review. J.N.Surg. 2018 Magnification reveals a Lai et al. Management of extremity neurilemmomas: a literature review. Ann Plast Surg 2013 typical Verocay body. Figure 5. Six-month post-operative views Nothing to disclose