Peripheral nerve tumors of the hand: Clinical features, diagnosis, and treatment

Abstract

The majority of the tumors arising from the peripheral nerves of the hand are relatively benign. However, a tumor diagnosed as malignant peripheral nerve sheath tumor (MPNST) has destructive consequences. Clinical signs and symptoms are usually caused by direct and indirect effects of the tumor, such as nerve invasion or compression and infiltration of surrounding tissues. Definitive diagnosis is made by tumor biopsy. Complete surgical removal with maximum reservation of residual neurologic function is the most appropriate intervention for most symptomatic benign peripheral nerve tumors (PNTs) of the hand; however, MPNSTs require surgical resection with a sufficiently wide margin or even amputation to improve prognosis. In this article, we review the clinical presentation and radiographic features, summarize the evidence for an accurate diagnosis, and discuss the available treatment options for PNTs of the hand.

Keywords: Malignant peripheral nerve sheath tumor; Neurofibroma; Neuroma; Perineurioma; Peripheral nerve tumor; Schwannoma.

Figure 1

Magnetic resonance imaging of schwannomas. A, B: Coronal magnetic resonance images; T1-weighted image (A) demonstrated a nodule of low intensity signal (arrows), while on T2-weighted image (B) it presented as high signal (arrows); C, D: Transverse magnetic resonance images; C: T1-weighted image; D: The lesions show significant enhancement after administration of contrast agent (arrows).

Figure 2

Photomicrograph of the tumors. A and B: Pathological examination shows a biphasic architecture of Antoni A (dense) and Antoni B (loose) and nuclei palisading with multiple fascicles (hematoxylin and eosin); C and D: The tumor cells stain positive for S100 protein. Adapted with permission from Jiang S, Shen H, and Lu H. Multiple schwannomas of the digital nerves and common palmar digital nerves: An unusual case report of multiple schwannomas in one hand. Medicine (Baltimore) 2019; 98: e14605.

Figure 3

Schwannomas. A: A solitary, palpable lesion on the volar of the left hand; B: A solitary, encapsulated well-defined surface of schwannomas involving the median nerve branch was found during operation; C: The lesion, of 22 mm in diameter, was removed carefully without damaging the nerve. Courtesy of Lu Hui, Orthopedics, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

Figure 4

Neurofibromatosis type 1 patient with neurofibromas on the radius of the right middle finger underwent surgical operation. Adapted with permission from Lu H, Chen Q, and Shen H. Hamartoma compress medial and radial nerve in neurofibromatosis type 1. Int J Clin Exp Med 2015; 8: 15313-15316.

Figure 5

Neuroma. A: Partial rupture of the median nerve with neuroma formation in the right hand; B and C: Surgical neuroma resection with nerve grafting was performed in this patient. Courtesy of Lu Hui, Orthopedics, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

Figure 6

Digital nerve neuromas. A: A patient with amputation of the second and third fingers suffered neuralgia; B and C: Digital nerve neuromas were found and neurectomy with radiofrequency ablation was performed to decrease the possibility of recurrence. Courtesy of Lu Hui, Orthopedics, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

Figure 7

Magnetic resonance imaging of intraneural ganglion cyst of the ulnar nerve. A: Hyperintense lesion in the ulnar nerve on T2-weighted (T2W) in transverse section; B: Hyperintense lesion in the ulnar nerve on T2W in coronal section; C: Hyperintense beaded lesion in the ulnar nerve on T2W in median sagittal section. Adapted with permission from Li P, Lou D, and Lu H. The cubital tunnel syndrome caused by intraneural ganglion cyst of the ulnar nerve at the elbow: a case report. BMC neurology 2018; 18: 217.

Figure 8

Magnetic resonance imaging of multiple intraneural glomus tumors in the digital nerve. A: A hyperintense signal on T1-weighted; B: A hyperintense signal on T2-weighted; C: Contrast-enhanced magnetic resonance imaging showing a significantly enhanced signal. Adapted with permission from Wang Y and Lu H, Multiple intraneural glomus tumors in different digital nerve fascicles. BMC Cancer 2019; 19: 888.

Figure 9

Two lesions in the digit nerve and multiple intraneural glomus tumors. A and B: Two lesions in the digit nerve were found during the operation and complete resection of multiple intraneural glomus tumors was performed in this case. Adapted with permission from Wang Y and Lu H, Multiple intraneural glomus tumors in different digital nerve fascicles. BMC Cancer 2019; 19: 888.