Schwannoma of the uterine cervix: a rare case report

Schwannomas are neurogenic tumors, which are among the most varied tumors with respect to morphology, clinical associations, and presentations; they occur in a wide variety of sites. The uterine cervix is a rare site of occurrence and only 15 cases of schwannomas of the uterine cervix, including 5 benign and 10 malignant cases, have been reported to date. Thus, schwannomas of the uterine cervix may pose diagnostic difficulty. Here, we report a case of benign schwannoma of the uterine cervix in a 37-year-old female, who presented with vaginal spotting.

Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1 / 영남의대학술지

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

Immunohistochemical Markers for Schwannomas, Neurofibromas and Malignant Peripheral Nerve Sheath Tumors­What Can the Recent Literature Tell Us?

Introduction Schwannomas and neurofibromas are the two most common benign neoplasms of the peripheral nerve sheath, and although they are generally easy to distinguish, in some cases, they can closely resemble one another. Furthermore, malignant peripheral nerve sheath tumors (MPNSTs), another example of peripheral nerve sheath neoplasm, may likewise constitute, due to their morphology and lack of specific immunohistochemical markers, a challenging diagnostic. Objective To bring attention to new and promising biomarkers for schwannomas, neurofibromas and MPNSTs and to outline, based on the recent literature, a immunohistochemical profile for each neoplasm at hand, as well as to emphasize the need for further studies that could help us understand their diagnostic potential and disrupt our dependence of limited and nonspecific biomarkers. Methods An overview of the recent literature published in English on both the classical promising immunohistochemical markers of schwannomas, neurofibromasand MPNSTs was performed. We discarded case reports. Conclusions There is still a lack of specific biomarkers for peripheral nerve tumors. However, plenty of new immunohistochemical markers have been coming to light with presumed higher specificity and more diverse helpful uses than the classical ones. For example, Sox10 is a good biomarker for differentiating schwannomas and neurofibromas from sarcomas, calretinin schwannomas from neurofibromas, TLE1 and HMGA2 MPNSTs from sarcomas, and nestin, EGFR, p16 and Ki-67 MPNSTs from different types of schwannomas and neurofibromas. There is still need for further studies; however, the potential of some of these promising markers, among others, should not be disregarded.

Introdução Schwannomas e neurofibromas são as duas neoplasias benignas mais comuns a acometer o tecido nervoso periférico, e apesar de geralmente serem facilmente distinguíveis, em alguns casos, elas podem ser muito semelhantes. Além disso, os tumores malignos da bainha dos nervos periféricos (TMBNPs), outro exemplo de neoplasia da bainha do nervo periférico, podem da mesma forma constituir, pela sua morfologia e falta de marcadores imuno-histoquímicos específicos, um diagnóstico desafiador. Objetivo Chamar a atenção para novos e promissores biomarcadores para schwannomas, neurofibromas e TMBNPs e delinear, a partir da literatura atual, um perfil imuno-histoquímico para cada neoplasia em questão, além de enfatizar a necessidade de futuros estudos que possam elucidar-nos acerca de seu potencial diagnóstico e, por ventura, romper nossa dependência de biomarcadores inespecíficos e limitados. Método Foi feita uma revisão da literatura recente incluindo artigos em língua inglesa sobre os marcadores imunohistoquímicos clássicos e os promissores para schwannomas, neurofibromas e TMBNPs. Descartamos relatos de caso. Conclusão Ainda há uma falta de biomarcadores específicos para as neoplasias acima. Contudo, vários novos marcadores imuno-histoquímicos têm surgido, e com futuros estudos poderemos talvez definir biomarcadores específicos e indispensáveis para os casos desafiadores de neurofibromas, schwannomas e TMBNPs. Por exemplo, o Sox10 é um bom biomarcador para diferenciar schwannomas e neurofibromas de sarcomas; a calretinina é um bom marcador para diferenciar schwannomas de neurofibromas; os biomarcadores TLE1 e HMGA2 podem ajudar a diferenciar TMBNPs de sarcomas, e a nestina, o receptor do fator de crescimento epidérmico (EGFR), o gene p16 e a proteína Ki-67 podem diferenciar TMBNPs de diferentes tipos de schwannomas e neurofibromas. Ainda há necessidade de novos estudos; contudo, o potencial de alguns desses marcadores, dentre outros, não deveria ser negligenciado.

Widely Infiltrating Epithelioid Malignant Peripheral Nerve Sheath Tumour of Skull Base

The epithelioid variant of malignant peripheral nerve sheath tumours is a rare histological entity, and the occurrence of a malignant peripheral nerve sheath tumour in the skull base is even more unusual. We report a case of a 52-year-old man who presented with reduced hearing in the left ear, giddiness and left-sided facial weakness of short duration. He was a known hypertensive. On examination, left-sided 7th to 12th cranial nerve palsies were noted. Computed tomography (CT) and brain magnetic resonance imaging (MRI) were reported as an ill-defined heterogeneously enhancing mass left skull base suggestive of chondrosarcoma. Left tympanotomy and biopsy of the lesion were carried out. On light microscopy and immunohistochemical examination of the biopsy, a diagnosis of epithelioid malignant peripheral nerve sheath tumour was established. The patient underwent left extended modified radical mastoidectomy and selective neck dissection. Histopathological study of the resected surgical specimen confirmed left-sided extensive tumour involvement of skull base structures, as well as neck nodal metastases.

Concurrence of Malignant Peripheral Nerve Sheath Tumor at the Site of Complex Regional Pain Syndrome Type 1: A Case Report

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare sarcomas derived from various cells in the peripheral nerve sheath. Malignant peripheral nerve sheath tumors have a known association with neurofibromatosis type 1. Diagnosis of MPNSTs is difficult in patients with chronic pain, when MPNST occurs at an overlapping area of chronic pain. Therefore, the diagnosis can be missed unless clinicians pay attention to the possibility of this disease. Here in, we report a case of concurrent malignant peripheral nerve sheath tumor with complex regional pain syndrome type 1. A 44-year female patient, who was diagnosed with complex regional pain syndrome (CRPS) type 1 in her left ankle, visited our clinic because of aggravated pain. The cause of the aggravated pain was revealed as concurrent MPNST in the left common peroneal nerve territory, which overlapped the site of pain from CRPS.