Background: The plexiform schwannoma, a peripheral nerve sheath tumor, is a very rare entity. But dermatologists should be familiar with since they may be the first who make diagnosis possible by taking a deep biopsy.Main observation: A 24-year-old male presented with multiple asymptomatic subcutaneous nodules of the palmar side of his right hand. Histologic investigations revealed a plexiform schwannoma with numerous Antoni-A areas. There was no evidence of neurofibromatosis type 1 or 2.Conclusions: Plexiform schwannoma of the hand is a rare nerve sheath tumor. In individual (symptomatic) cases hand surgery is an option that needs a critical indication. In every case histologic investigations are mandatory to confirm the diagnosis and not to overlook the malignant variant of this disease. Imaging diagnostics like 3-dimensional ultrasound and MRI do not allow a sufficient discrimination of schwannomas form other nerve sheath tumors. 4 The only way to confirm the diagnosis is by histopathology.
Isolated plexiform schwannoma of the handWe present an unusual case of a large unilateral plexiform schwannoma of the hand in a young male patient.
Case ReportA 24-year-old male patient experienced asymptomatic subcutaneous nodules of his right hand since 2000. There was a slow progress in size and number.On examination we found soft, yellowish subcutaneous nodules of variable size of the volar site of the fingers and the palm of his right hand ( Fig. 1A and B).Histology of two biopsies showed spindle-shaped cell proliferations in the dermis and upper subcutaneous tissue with a capsule formation. Cells were characterized by neurogenic differentiation like Antoni-A-areas (Fig. 2).Clinical examination and consiliae did not reveal any sign of neurofibromatosis type 1 or type 2.After confirmation of the diagnosis of a plexiform schwannoma of the right hand the patient was referred to the hand surgeon. He advised not to intervene by surgery because of the risk of paresis and the completely asypto- DOI: 10.3315/jdcr.2008DOI: 10.3315/jdcr. .1011 28 matic course without functional handicap in this patient.Follow-up has been recommended by us.
DiscussionSchwannomas are benign tumors of the nerve sheath occuring either peripheral, visceral, intraspinal or intracranial. Peripheral schwannomas represent as asymptomatic and painless papules or nodules. Their colour can be yellowish to light brown. Cysts and haemorrhages may occur. Schwannomas of the hands are rare. Patient's mean age at diagnosis is 38.4 years. 5 Histology is characterized by a biphasic pattern with tightly packed spindle-shaped cells (Antoni A area) and with loose, less cellular patterns with myxoid areas with thickened "hyalinized" vessel walls (Antoni B) and lipidcontaining cells. 3 Mitoses are usually absent but pleomorphism of nuclei is common. Immunohistochemistry is helpful in differential diagnosis ( Table 1). The tumor cells express S-100 protein. 1,2 Complicating in differential diagnosis is the observation of collision tumors like schwannoma and peri...