An unusual case of glandular schwannoma

Glandular differentiation is exceedingly rare in peripheral nerve sheath tumors. In this report, an exceptional case of retroperitoneal glandular schwannoma is described in which the glandular element is markedly atypical, whereas the schwannian component is benign by morphologic analysis. To the best of our knowledge, the biologic behavior for such a lesion is unknown because similar cases have not yet been described in the literature.     

Papilledema secondary to vestibular schwannoma: An atypical case without intracranial hypertension

In most cases, vestibular schwannomas with papilledema are associated with intracranial hypertension secondary to hydrocephalus (obstructive or communicating). We describe the atypical case of a 39-years-old man who presented with bilateral papilledema revealing a vestibular schwannoma, but without hydrocephalus and with normal intracranial pressure. Ophtalmologic signs were completely resolved after tumor removal. The pathophysiological mechanism generally described to explain bilateral papilledema in such cases is tumor-induced hyperproteinorachia. However, in the absence of hydrocephalus or intracranial hypertension, this case raises the question of the mechanisms involved in the visual impairment related to vestibular schwannoma.     

Malignant peripheral nerve sheath tumor of the vulva,an unusual differential diagnosis for vulvar mass

INTRODUCTION

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, up to one half of the MPNSTs occur in patients with neurofibromatosis type-1 (NF-1), while the rest are sporadic. Here, we present a 52-year-old woman with MPNST of the vulva without NF-1. We will discuss basics of the disease, treatment options and follow-up strategies.

PRESENTATION OF CASE

52-year-old female admitted to our hospital with complaint of abnormal uterine bleeding and rapidly growing vulvar mass. Excisional biopsy of the mass showed MPNST of the vulva. Afterwards, the patient underwent radical vulvectomy with inguinofemoral lymph node dissection. Short after the surgery, multiple lung metastasis were shown and responded to chemotherapy, but rapid local recurrence occurred short after the completion of the chemotherapy.

DISCUSSION

The primary treatment option in MPNSTs is surgical excision with or without adjuvant therapy. There is not enough data about the role of systemic chemotherapy in the management of MPNSTs and it still remains controversial.

CONCLUSION

In general, radiation therapy has not been demonstrated to improve overall survival. Complete surgical resection of the primary tumor is the mainstay of the treatment.     

Endoscopic resection of benign esophageal schwannoma: Three case reports and review of literature

BACKGROUNDEsophageal schwannomas are uncommon esophageal submucosal benign tumors and are usually treated with surgery. CASE SUMMARYHere, we report three cases of middle/lower thoracic esophageal schwannoma treated successfully with endoscopic resection. These lesions were misdiagnosed as leiomyoma on preoperative imaging. During the endoscopic resection of such tumors, there is a risk of esophageal perforation due to their deep location. If possible, submucosal tunneling endoscopic resection should be used. CONCLUSIONFor larger schwannomas, endoscopy combined with thoracoscopy can be considered for en bloc resection. We performed a mini literature review in order to present the current status of diagnosis and treatment for esophageal schwannoma.     

Expression of the neurofibromatosis 2 tumor suppressor gene product,merlin, in Schwann cells

The neurofibromatosis 2 (NF2) tumor suppressor gene encodes a protein with sequence similarity to a family of molecules linking cell membrane proteins to the actin cytoskeleton. Individuals affected with NF2 develop schwannomas at an increased frequency. In this paper, we report that merlin is expressed in Schwann cells, where it is localized in vitro to the cell membrane by immunohista chemistry and subcellular fractionation. Exogenous expression of merlin fragments confirms this subcellular distribution and suggests that both the N-terminal and C-terminal portions of the molecule are required for this localization. In addition, merlin is expressed in rat sciatic nerve Schwann cells at paranodal membranes, where it colocalizes with RhoA. Lastly, expression of the NF2 gene increases during postnatal rat sciatic nerve development, consistent with its role as a negative growth regulator for Schwann cells. These results collectively suggest that merlin may function at the cell surface to modulate cell growth in Schwann cells and to link cell membrane proteins to the cytoskeleton. © 1996 Wiley-Liss, Inc.     

Prognostic factors of facial nerve function after vestibular schwannoma removal via translabyrinthine approach

Background: Prediction of facial function is a major concern when proposing surgery for patients with vestibular schwannoma (VS).

Aims/objectives: To evaluate postoperative facial function of patients who underwent operation of VS via a translabyrinthine approach (TL), and to analyze factors that influence facial functions.

Material and methods: A total of 91 VS patients, who were operated via a TL approach, between March 1997 and December 2016, were analyzed. Demographics, tumor-related factors, and operative findings were collected. Facial function was assessed according to the House-Brackmann (HB) grading system before surgery, immediately after surgery, and 1-, 3-, 6-months, and 1 year after surgery.

Results: In cases of patients that had a tumor that extended to the CPA, an unsatisfactory facial outcome was noted in 12 (30.0%) patients. FN outcomes after tumor removal depend on tumor size (p?=?.040). Among FN-related factors, only the FN recovery timing was correlated with facial outcomes (p?=?.030). Univariable and multivariable analysis revealed that tumor size and the timing of FN recovery were significant as favorable prognostic factors for good facial outcomes.

Conclusions and significance: Tumor size and the FN recovery timing are significant prognostic factors of facial outcome in VS patients who underwent operations via a TL approach.     

Intraparotid facial nerve schwannoma: a 17-year,single-institution experience of diagnosis and management

Background: Intraparotid facial nerve schwannoma (IFNS) is rare and its definite preoperative diagnosis is challenging.

Objective: To improve available knowledge regarding the diagnosis of IFNS and to suggest an appropriate treatment plan.

Material and methods: We retrospectively analyzed medical records of IFNS patients at our hospital. Inclusion criteria were surgery (from January 2000, to December 2016) for a parotid mass, pathologically diagnosed as a schwannoma.

Results: The study included 42 eligible patients who had undergone tumor resection from 5977 parotid tumor patients. Mostly presented hard-textured (18/39) or medium-textured (15/39), with limited mobility (21/39) mass (three tumors were not palpable). Their facial nerve function outcomes were House–Brackmann Grade I (n?=?14), Grade II (n?=?7), Grade III (n?=?11), Grade IV (n?=?5), Grade V (n?=?3), and Grade VI (n?=?2). Significant differences were noted in results based on different surgical methods used (p?=?.000) and tumor involvement (p?=?.002).

Conclusions and significance: A hard-textured tumor with limited mobility mass in the parotid gland should prompt the diagnosis of a schwannoma. Tumors involving main trunk usually lead to unsatisfactory facial nerve outcomes. Facial nerve preservation should always be essential, and stripping surgery or intracapsular enucleation could be the preferred surgical methods of choice.