Segmental Schwannomatosis in the Upper Extremity: A Case Report and Review of Literature

Schwannomas, the most frequently occurring benign tumors of the peripheral nerve sheath, generally remain as painless swellings for several years before diagnosis. Multiple schwannomas involving different nerves within the same extremity are rare. We report a rare case of a 61-year-old female who presented with multiple schwannomas in the palmar common and proper digital nerves, 15 years after the resection of a median nerve schwannoma within the same upper extremity. Using preestablished diagnostic criteria, she was diagnosed with segmental schwannomatosis. After careful surgical resection, biopsy confirmed the diagnosis and she recovered without neurological symptoms or limitations in the range of motion. Literature review revealed only four case series on segmental schwannomatosis, indicating its rarity. Postoperative sensory deficits are more likely in cases with multiple schwannomas in the common and proper digital nerves. We demonstrate that such complications can be avoided by meticulous dissection and separation of the tumors from the nerve fibers.

Schwannoma of the medial plantar nerve: a case report

We report the case of a 60-year-old woman with a schwannoma of the medial plantar nerve. She presented with the complaint of pain for about 2 years and a painful lesion in the medial plantar region of the left forefoot and on the second toe. Physical examination showed well-defined, firm tumor masses painful to palpation on the medial and plantar surface of the left forefoot and second toe. We surgically explored the area and excised 3 lesions, resulting in complete pain relief and no aesthetic complaints. Schwannomas of the medial plantar nerve are rare tumors, with only a few reports of cases extending to the forefoot. The finding of multiple schwannomas may be a red flag for the possible existence of local, painful, nerve lesions despite a negative Tinel sign. Appropriate surgical planning contributed to our successful intervention, without aesthetic, painful, or functional sequelae for the patient. Level of Evidence V; Therapeutic Studies; Expert Opinion.

Multiple schwannomas of the facial nerve mimicking cervical lymphoma: a case report

Background In this report, we describe the first case in literature of a patient with multiple schwannomas of the marginal mandibular branch of the facial nerve. Case presentation A Caucasian patient presented with a sudden onset of left lower facial nerve palsy House–Brackmann score III for 1 month. Computed tomography imaging was performed to exclude a cerebral event and revealed multiple tumors within the left parotid gland. Duplex ultrasound and magnetic resonance imaging scans delineated multiple, hypoechoic tumors, round in shape and well defined without a hilar structure along the left mandible. For histological verification, a left-side partial parotidectomy and extirpation of an intraparotideal node was performed with use of a nerve-integrity monitor. Histomorphological analysis of the resected tissue revealed a benign schwannoma. Facial nerve function remained unchanged since the operation. The size of the nonresected tumors is currently monitored regularly by ultrasonography. Fibromatosis has been excluded. Conclusions If multiple tumors occur in the parotid gland and the angle of the jaw, schwannomas need to be considered as a differential diagnosis. To plan the right diagnostic surgical intervention and prevent nerve damage, a thorough ultrasound examination is essential in preoperative diagnostic work-up for any suspicious lesion of the parotid gland and jaw region.

[18F]FDG Positron emission tomography with whole body magnetic resonance imaging ([18F]FDG-PET/MRI) as a diagnosis tool in Schwannomatosis

Schwannomatosis is a rare autosomal dominant genetic syndrome characterized by the presence of multiple schwannomas. The main symptom is neurogenic pain. The diagnosis requires the presence of several schwannomas and whole-body [18F]FDG-PET/MRI might help detect extra schwannomas in patients when the diagnosis is uncertain. Among the 25 patients treated for Schwannomatosis in our tertiary center, three men and two women had had a [18F]FDG-PET/MRI performed, and the number of schwannomas detected by [18F]FDG-PET/MRI outnumbered the number of schwannomas suspected during the clinical examination. The majority of schwannomas exhibited a radiolabeling (median of 66.7%, range 28–93%). Our findings show that [18F]FDG-PET/MRI could prove useful when suspecting schwannomatosis to accelerate diagnosis and offer optimal care to patients.

A Linear Variant of Segmental Schwannomatosis Localized to the Upper Extremity

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.

A case report of primary multiple obturator nerve schwannomas

Primary multiple obturator nerve schwannomas originate from Schwann cells and are extremely rare. Patients with schwannomas are asymptomatic and a retroperitoneal schwannoma is often misdiagnosed as an adnexal mass. In the present study, we describe a 58-year-old woman in whom a right adnexal mass accompanied by endometrial polyp was found incidentally through transvaginal ultrasound. The mass was diagnosed as multiple obturator nerve schwannomas after laparoscopy. Immunohistochemical assay confirmed the schwannomas to be positive for SOX10. To our knowledge, this is the first report to demonstrate a case of multiple schwannomas originating from the obturator nerve and treated by laparoscopic resection.

Multiple Schwannomas of the Lower Extremity

This case study presents a 72-year-old male patient who presented with multiple schwannomas. The patient underwent several soft-tissue mass excisions performed by general surgery and podiatry. The physical examination displayed multiple nodules on the left foot, ankle, and previously in the groin. Magnetic resonance imaging displayed high–T2-weighted signal masses in the left plantar heel and ankle. These masses were excised and, through pathologic analysis, were diagnosed as schwannomas. Immunohistochemistry displayed a mosaic pattern of INI1 expression, a gene associated with tumor suppression. The patient demonstrated findings consistent with multiple schwannomas (eg, schwannomatosis or neurofibromatosis type 2). Because of the patient's medical history, neurofibromatosis type 2 was more probable.