Multiple schwannomas of the upper limb related exclusively to the ulnar nerve in a patient with segmental schwannomatosis

Multiple Lower Extremity Mononeuropathies by Segmental Schwannomatosis: A Case Report

“…Although the recurrence of schwannomatosis is rare, there are several reports on recurrence [ 6 10 ] as in this case, and the probability of malignant transformation is still unknown.…”

“…However, repeated surgeries are not recommended because of the possibility of iatrogenic nerve injuries and worsening pain. Therefore, management should be focused on prevention of disease progression and strict surveillance [ 6 10 ].…”

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

“…• Confusion with NF2, presumptive schwannomatosis, 4 and familial schwannomatosis [23][24][25] • Two studies 2,26 were performed before the release of the 2005 consensus statement, 1 and other studies did not ensure the patients met the criteria for definite schwannomatosis, particularly regarding the lack of systematic brain MRI to formally eliminate NF2, [26][27][28] being < 18 years of age at diagnosis, 25,[29][30][31][32][33] and the presence of intracranial lesions 34,35 • Two studies were original articles with no individual descriptive cases 1,36 Approximately 10 to 15% of patients with schwannomatosis have a family history. 17,37 The remaining 85 to 90% have sporadic disease.…”

Case report Multiple schwannomas of the digital nerves and superficial radial nerve: two unusual cases of segmental schwannomatosis

“…They mostly occur as solitary tumours [6,7,25]. Multiple schwannomas developing in individual nerves are very rare [14,24,29,33,35]. Their presence may be one of the symptoms indic ative of neurofibromatosis type 2 or schwannoma tosis [5,13,25].…”

“…Qualification for surgical treatment of multiple schwannomas is controversial. Some authors highlight elevated risk of iatrogenic damage to the nerve during tumour excision [24]. Advocates of surgical treatment raise the possibility of development of multilevel compres sion neuropathy as a result of the growth of tumours [5].…”