A Linear Variant of Segmental Schwannomatosis Localized to the Upper Extremity

Journal of Hand and Microsurgery ◽

10.1055/s-0040-1715424 ◽

2020 ◽

Author(s):

Ashkaun Shaterian ◽

Tuna Ozyurekoglu

Keyword(s):

Upper Extremity ◽

Surgical Excision ◽

Case Presentation ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Distinct Subset ◽

Nerve Sheath ◽

Single Nerve ◽

Nerve Fascicle ◽

Multiple Schwannomas

Abstract Introduction Schwannomas represent benign peripheral nerve sheath tumors. Their phenotypic presentations in schwannomatosis and segmental schwannomatosis have been well described. To date, however, cases of schwannomatosis or multiple schwannomas localized to a single nerve fascicle have been limited in the literature. Case Presentation In this study, we identify a case of a 48 year-old non-neurofibromatosis male who presented with symptomatic schwannomas localized to a single nerve fascicle of the upper extremity. Intraoperative exploration revealed four schwannomas arising from a 15-cm segment of ulnar nerve fascicle. Surgical excision was successful, without neurological deficit or recurrence. Conclusion This study identifies a case of schwannomatosis localized to a single nerve fascicle that may represent a linear variant of segmental schwannomatosis. The presentation may represent a temporary linear appearance in progression to “nonlinear” segmental variant; however, a molecularly distinct subset of schwannomas cannot be excluded.

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Recurrent schwannoma of the tongue in a pediatric patient—report of a rare case with an updated review of literature

The Egyptian Journal of Otolaryngology ◽

10.1186/s43163-021-00101-0 ◽

2021 ◽

Vol 37 (1) ◽

Author(s):

Anup Singh ◽

Vaisakh Kuzhikkali ◽

Arvind Kumar Kairo

Keyword(s):

Pediatric Population ◽

Surgical Excision ◽

Review Of Literature ◽

Case Presentation ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Cervical Sympathetic ◽

Mobile Tongue

Abstract Background Head and neck is a relatively common site of occurrence for the peripheral nerve sheath tumors, and majority of these tumors are seen involving neck, involving vagus nerve, and cervical sympathetic chain. Schwannomas involving mobile tongue are rarely encountered, especially in the pediatric population Case presentation We present a case of recurrent tongue schwannoma in a 13-year-old female successfully managed with transoral excision. At a follow-up of 3 years, no recurrence is observed. Conclusion Surgical excision is the recommended modality of treatment for lingual schwannomas, and when excised adequately, recurrences are not expected. A clear margin of surrounding normal tissue should be aimed for to avoid possible recurrence.

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The Surgical Management of Symptomatic Benign Peripheral Nerve Sheath Tumors of the Neck and Extremities: An Experience of 442 Cases

Neurosurgery ◽

10.1093/neuros/nyx076 ◽

2017 ◽

Vol 81 (4) ◽

pp. 568-580 ◽

Cited By ~ 9

Author(s):

Ketan I. Desai

Keyword(s):

Peripheral Nerve ◽

Surgical Excision ◽

Extent Of Resection ◽

Motor Deficit ◽

Neurogenic Pain ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Upper Cervical ◽

The Mean

Abstract BACKGROUND The occurrence of benign peripheral nerve sheath tumors (PNSTs) is not uncommon. The surgical excision of symptomatic benign PNSTs along with preservation of the affected nerve and its function is an ideal treatment option. OBJECTIVE To analyze the outcome with respect to morbidity, extent of resection, and recurrence, and to review and compare our results with those reported in literature. METHODS A retrospective review of clinical and radiological findings of 442 patients with benign PNSTs involving the neck and extremities treated surgically from 2000 to 2014 was performed. RESULTS In our series, benign PNSTs involved the extremities in 290 (65.6%) patients and the brachial plexus in 146 (33%) patients, and 6 (1.4%) patients had tumors of the extracranial portion of the vagus and hypoglossal nerves in the neck. The mean age of patients was 38 yr. The presenting features were painful mass and paresthesia. Preoperative motor weakness in the extremity was noted in 15.6% of patients. The common nerves involved by the tumors were the ulnar nerve (15.8%), sciatic nerve (12.7%), and upper cervical roots (11.5%). The excision was total in 81.2%, gross total (>90%) in 17.9%, and subtotal (>50%) in 0.9% patients. In 17.6% of patients, there was severe postoperative neurogenic pain. In 28 (6.3%) patients, a new motor deficit was noted following surgery. Recurrence was seen in 2 patients in our series. The mean follow-up was 30.2 mo. CONCLUSION Benign PNSTs have excellent clinical outcome, and the goal for surgical treatment is total to gross total excision of the tumor with neural preservation.

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