Papillary glioneuronal tumor with highly degenerative pseudopapillary structure accompanied by specific abortive glial cells: a case report

Recently, unusual examples of tumors of the mixed glioneuronal type have been reported, including the papillary glioneuronal tumor (PGNT). A 23-year-old woman with a 2-3 months history of headache and insomnia presented with a tumor. Neuroimaging showed a right temporal lobe cystic tumor with a mural nodule enhanced by contrast medium. She underwent gross total resection of the tumor. The tumor was histologically marked by a mixture of glial and neural components. A pseudopapillary component was comprised of highly hyalinized vessels surrounded by a single layer of thin spindle cells stained for glial fibrillary acidic protein. Specific abortive glial cells stained for vimentin/S-100 protein accompanied pseudopapillary structure. Intervening neural areas were occupied by neural cells of varying size, including neurocyte-like cell and ganglionic cells. Ganglionic cells demonstrated abnormal cluster, lack of normal polarity and epiperikaryal immunoreactivity for synaptophysin staining suggesting neural neoplastic nature. No mitotic activity or necrosis was noted. A MIB-1 labeling index was 1.8%. Our patient remains free of disease 33 months after surgical treatment.     

Clinical,radiological, and pathological features of 16 papillary glioneuronal tumors

Background

Papillary glioneuronal tumors (PGNTs) are a novel distinct intracranial neoplastic entity. In this study, the authors aimed to analyze the clinical, radiological, and pathological features of PGNT.

Methods

Clinical charts and radiographs of 16 cases of PGNT surgically treated between 2006 and 2013 were retrospectively reviewed. Follow-up evaluations and a literature review were performed.

Results

The study included nine males and seven females with a mean age of 23.8 years. The most common preoperative symptom was headache (68.8 %, 11 of 16 patients). Radiological results showed that the frontal lobe (25.0 %) was the most common portion of the brain involved, and 13 lesions (81.3 %) presented with cystic appearance with or without solid elements. All patients were misdiagnosed as non-PGNT tumors. Complete resection was achieved in 12 patients (75.0 %). Ki67 staining was positive in <1 % of cells present in eight lesions and varied in the other eight lesions, with a range of 1 % to 13 %. The mean follow-up duration was 56.2 months, and no recurrence was observed. Seventy-seven PGNTs (40 males, 51.9 %) have been reported, and 49 patients stained positive for Ki67/MIB-1. Of these cases, 28 (57.1 %) had positive staining rates of ≥1 %. In 51 patients for whom outcome information was available, six (11.8 %) exhibited recurrence, and the recurrence rates for complete resection and incomplete resection were 5.1 % and 33.3 %, respectively.

Conclusions

PGNTs displayed a wide spectrum of clinical and radiological phenotypes, and they were more frequently observed in the frontal lobe and in young patients without sex predilection. Fair outcomes could be achieved by complete resection. Although PGNT displayed indolent pathobiology, atypical appearances were observed. More patients and longer follow-up periods are needed to further elucidate the biological features of PGNTs.     

Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: case report

BACKGROUND: Tumors composed of both neurocytic and astrocytic cells are uncommon and poorly understood. We describe the clinicopathologic features of a very rare rosette-forming glioneuronal tumor of the fourth ventricle and propose bromocriptine as a useful therapeutic agent for cerebellar mutism after posterior fossa surgery. CASE DESCRIPTION: A fourth ventricle tumor was incidentally discovered in an 18-year-old woman. Magnetic resonance imaging revealed ventriculomegaly and a solid tumor with low-intensity signals on T1-weighted images and high-intensity signals on T2-weighted images. There was slight gadolinium enhancement. The tumor was subtotally resected. Although its lower half was well circumscribed, its upper half manifested invasive growth. Histologically, 2 components were identified, synaptophysin-positive neurocytic cells forming perivascular pseudorosettes and glial fibrillary acidic protein-positive astrocytic cells with Rosenthal fibers. Overall, cellular atypia was minimal and the MIB-1 labeling index was low. On the basis of these histologic findings, the tumor bore striking similarity to the recently described rosette-forming glioneuronal tumors of the fourth ventricle. Postoperatively, the patient manifested cerebellar mutism. The administration of bromocriptine improved her neurological status dramatically. CONCLUSION: The natural history of rosette-forming glioneuronal tumors of the fourth ventricle is not yet fully understood. Therefore, careful and long-term follow-up monitoring of the tumor hosts is necessary. Bromocriptine therapy may promote recovery from mutism after posterior fossa surgery.     

Fourth ventricle rosette-forming glioneuronal tumor. Case report

The authors describe a rosette-forming glioneuronal tumor of the fourth ventricle in a 29-year-old woman. She had been experiencing dizziness for 1 year and headaches for 1 month. Cranial computed tomography revealed a relatively circumscribed mass involving the inferior cerebellum and floor of the fourth ventricle with extension into the ventricle. Histologically, much of the tumor was piloid with Rosenthal fibers as well as telangiectatic blood vessels; other areas contained complete or incomplete neurocytic rosettes. This tumor type must be differentiated from pilocytic astrocytomas, other gliomas with a piloid glial component, and glioneuronal tumors arising from the floor of the fourth ventricle or inferior cerebellum. Recognition of, and long-term follow up for, this recently described pathological entity may clarify the nature of this lesion and strategies for its optimal management.     

Infratentorial localisation of a dysembryoplastic neuroepithelial tumor. A case report

Neuroepithelial dysembryoplastic tumor (DNT) is usually considered as a supratentorial benign neoplasm. DNT of the posterior fossa is a very rare entity and only four previous cases were reported in the literature. We describe a case of a 26-Year-old woman presenting recurrent episodes of vertigo. Magnetic resonance imaging revealed four cystic lesions located in the cerebellum, hypointense on T1-weighted images and hyperintense on T2-weighted images, without gadolinium enhancement. After partial resection, histological examination showed small glial cells, oligodendrocytes-like, lying in an eosinophilic alveolar matrix with some floating neurons. Due to this specific glioneuronal element, the diagnosis of DNT was retained. We discuss the clinical and radiological particularities of this infratentorial location and compare our case with those previously described in the literature.     

Papillary glioneuronal tumor: unexplored entity

Papillary glioneuronal tumors represent a new and rare entity of an uncommon morphologic subtype of low-grade mixed neuronal-glial neoplasms with an unclear etiology. They are described as benign lesions with extraventricular localization. We report the second case of papillary glioneuronal tumor with recurrent nature after gross-total resection, and the third case of this tumor with intraventricular localization. While conventional magnetic resonance imaging of papillary glioneuronal tumors is well described in literature, there are no data based on advanced magnetic resonance techniques. The present article represents a review of clinicopathological and both conventional and advanced magnetic resonance imaging characteristics of papillary glioneuronal tumors, with focus on 2 cases with atypical course and localization.     

Electrocorticographic–Histopathologic Correlations Implying Epileptogenicity of Dysembryoplastic Neuroepithelial Tumor

Based on intracranial-video electroencephalography (EEG), histopathological features, and postoperative seizure outcome, we elucidated the epileptogenicity in patients with dysembryoplastic neuroepithelial tumor (DNT). Five patients (P1–P5) pathologically diagnosed with DNT underwent intracranial-video EEG to identify the ictal onset zone and irritative zone. We evaluated the correlations of ictal onset zone and irritative zone with the magnetic resonance imaging-visible lesion (MRI-lesion) and their histopatho-logical features. Intracranial-video EEG located the ictal onset zone adjacent to the MRI-lesion margin in four patients with complex/simple forms of DNT subcategory, and on the MRI-lesion in P3 with a nonspecific DNT form. The irritative zone extended to surrounding regions of the ictal onset zone in all patients. Histopathologically, MRI-lesions were characterized by specific glioneuronal elements, whereas the ictal onset zone and irritative zone were represented with dysplastic cortex accompanying oligodendroglia-like cells in four (P1, P2, P4, and P5) of five patients. Cortical dysplasia was identified with typical histopathologic features in the irritative zone remote from the MRI-lesion in P5. P3, with a nonspecific form, indicated prominent component of dysplastic cortex with oligodendroglia-like cells scattered in the MRI-lesion. Lesionectomy of MRI-lesion with additional cortical resections (including the ictal onset zone and irritative zone) yielded postoperative seizure freedom (Engel Class I) in P3, P4, and P5, while P1 and P2 (with only lesionectomy) experienced postoperative residual seizure (Class II and III in each patient). Our results suggest the intrinsic epileptogenicity of DNT. The topographical correlation indicated that the dysplastic cortex accompanying oligodendroglia-like cells was more epileptogenic than the specific glioneuronal elements itself. Meticulous intracranial-video EEG analysis delineating the MRI nonvisible ictal onset zone and the irritative zone may yield better seizure outcome.     

胚胎发育不良性神经上皮瘤的诊治

目的探讨胚胎发育不良性神经上皮瘤（DNT）的诊断和治疗。方法2001年11月至2005年2月共收治DNT患者18例。对临床表现、影像学检查、术前诊断、治疗及病理作回顾性分析。结果癫痫为主要临床表现;磁共振成像（MRI）为无占位效应的病变;病理上特异性胶质神经元和多发结节为其特点;外科手术治疗,癫痫控制良好。结论DNT为良性病变,需外科治疗,手术时全切除肿瘤及术中采用皮层脑电图监护可提高手术疗效。     

Gastrointestinal stromal tumor in ileal neobladder

Abstract  Gastrointestinal stromal tumor (GIST) is a recently described mesenchymal tumor that can develop in any portion of the gastrointestinal tract. The occurrence of a GIST in the urinary tract is rare, but GIST can present as tumor of the urinary tract or invade the urinary tract. This is the first reported case of GIST in the ileal neobladder, which presented as a submucosal tumor. The patient underwent an open exploration and partial resection of the neobladder pouch.     

Inferior vena caval thrombus associated with double neoplasms of the retroperitoneum and kidney: Report of a case

We report herein the case of a patient we recently encountered who was found to have a giant retroperitoneal tumor coincident with tumor thrombus in the inferior vena cava (IVC). On the assumption that the origin was retroperitoneal, the two lesions were resected using an intracaval filter. However, histopathological examination of the specimens revealed neurilemoma for the retroperitoneal tumor and adenocarcinoma for the tumor thrombus. A right renal tumor was subsequently detected, which led to right nephrectomy being performed en bloc with part of the IVC using a venoarterial bypass. Microscopy revealed carcinoma of the Bellini duct, or collecting duct, which is an extremely rare tumor. Retrospectively, a lesion with soft tissue density was noted in the renal vein. Surgical management focused on the prevention of pulmonary embolism. We describe this case because of its extreme rarity and its significance from the viewpoint of diagnosis and therapeutic strategies.     

Plexiform fibrohistiocytic tumor of the hand: a case with late recurrence

A case of a rare and recently described neoplasm of the superficial soft tissue that occurs mainly in children and young adults is described. The lesion demonstrates morphological features reminiscent of both fibrous histiocytoma and fibromatosis. Microscopically, this tumor is characterized as a multinodular or plexiform proliferation of histiocyte- and fibroblast-like cells associated with multinuclear giant cells. Because of its unpredictable biological behavior, high recurrence rate, and potential for regional lymphatic metastasis, the diagnosis of plexiform fibrohistiocytic tumor raises important treatment considerations.     

Contribution of perfusion magnetic resonance imaging in a patient with cerebral ganglioglioma

A 48-year-old woman was admitted to the Neurosurgery Department for a large frontal lobe tumor revealed by partial seizures. The patient was conscious and alert. Neurological examination was normal. MRI study showed a right frontal lobe tumor compounded of an anterior solid mass strongly enhanced after gadolinium injection and a posterior voluminous cyst with important mass effect. The cerebral blood volume (CBV) map showed no area of elevated CBV within the tumor consistent with a low-grade tumor. The patient was operated on with a presumed diagnosis of anaplastic oligodendroglioma. Postoperative course was uneventful. Histopathological examination was consistent with a benign ganglioglioma. The patient did not undergo an additional treatment. One year later, the patient was healthy and neurological and neuropsychological examination were normal. MRI study did not show any recurrence. This case emphazises the relevance of perfusion MR imaging in the preoperative workup of glioneuronal and glial tumors.     

胃肠间质瘤预后相关因素

胃肠间质瘤（GIST）是一组起源于胃肠道间叶源性的肿瘤,其发病率低,恶性程度有时难以评估。即使完整切除GIST后,部分病人术后仍容易发生肿瘤复发或远处转移,准确的评估GIST恶性程度及预后影响因素对指导临床个体化治疗有着重要的意义。近年来,随着临床医师对其认识的不断加深,根据其预后因素制定了多种复发危险度分级标准以判断GIST预后的评估模型。GIST预后的影响因素有肿瘤部位、大小、核分裂像数、肿瘤是否破裂、基因突变类型以及肿瘤免疫组化标记物等。     

Verteilungsmuster von Fuß- und Sprunggelenkstumoren

BackgroundUnclear bone lesions and soft tissue masses of the foot and ankle are not uncommon, but pseudotumorous lesions must be strictly differentiated from real neoplasia. Delayed and incorrect diagnoses are more common here than in other localizations of the musculoskeletal system, sometimes with serious consequences for affected patients.Awareness of true musculo-skeletal tumours is low due to the rarity of these diseases and neoplasia is often not considered.In the scarce literature on this topic there is no uniform definition of the term foot tumor, which makes it difficult to compare and correctly classify the different entities. The aim of this study is to show incidence and distribution patterns of foot and ankle tumors of a university tumor institute in order to improve the understanding of this rare and heterogeneous disease pattern, and to present a simple definition of the term foot tumor.MethodsIn a retrospective, monocentric study, data of patients treated between June 1997 and December 2015 for foot and ankle tumors at a musculoskeletal university tumor center were analyzed with respect to epidemiological data, entity and localization. This included all cases with foot and ankle tumors defined according to the WHO. Exclusion criteria were incomplete data on patient or entity and all pseudotumorous lesions.ResultsOut of a total of 7487 musculo-skeletal tumors, 413 cases (5.52%) of foot and ankle tumors were indentified in 409 patients (215 male and 198 female patients). The average age of the affected patients was 36 ± 18 years (min.3 years, max.92 years). There were 266 bone tumors (64%), among them 231 (87%) benign and 35 (13%) malignant tumors. Of 147 soft tissue tumors (36%) 104 (71%) were benign and 43 (29%) malignant. The most frequent benign osseous tumor lesions included juvenile / simple bone cysts, enchondromas and osteochondromas. By far the most frequent malignant bone tumor was chondrosarcoma. Common benign soft tissue tumors included tenosynovial giant cell tumor (including pigmented villo-nodular synovitis), superficial fibromatosis and schwannoma, while the most frequent malignant entities were synovial arcoma and myxofibrosarcoma. In terms of anatomical localization, the hindfoot was most frequently affected.ConclusionsKnowledge of incidence and the corresponding distribution patterns of foot and ankle tumors can help to correctly assess unclear bumps and lumps and to initiate the right steps in further diagnosis and treatment. Ignorance can lead to delayed diagnosis and inadequate treatment with serious consequences for the affected patient.     

Endoscopic observation for detection and monitoring ofN-butyl-N-(4-hydroxybutyl)nitrosamine — induced bladder tumor in rats

Recent advances in tumor carbohydrate biochemistry have demonstrated antitumor effects of locally administered G_M3 ganglioside on mouse MBT-2 tumor. When intravesical therapy inN-butyl-N(4-hydroxybu-tyl)nitrosamine (BBN)-induced rat bladder tumor is attempted, it is essential to identify the tumor, to classify its size before therapy and to monitor the effect of the therapy. To establish a more reliable experimental therapeutic system, we assessed the development of BBN-induced rat bladder tumor by endoscopic observation. BBN-induced bladder tumors in rats were observed serially using a 4.2-F flexible fiberscope. The endoscopic findings were compared with the histopathological findings. Intravesical tumor growth varied greatly between individual rats. The smallest change detected by endoscopy was a small edematous lesion histologically proved to be papilloma. The largest nodular lesion was determined to be a papillary, transitional cell carcinoma. This noninvasive method makes the BBN rat experimental system more reliable by allowing confirmation of tumor formation and classification of the tumor volume prior to therapy.     

Malignant transformation of a dysembryoplastic neuroepithelial tumor. Case report

A 29-year-old man presented in 1984 with a recent onset of partial seizures marked by speech arrest. Electroencephalography identified a left frontotemporal dysrhythmia. Computerized tomography (CT) scanning revealed a superficial hypodense nonenhancing lesion in the midleft frontal convexity, with some remodeling of the overlying skull. The patient was transferred to the London Health Sciences Centre for subtotal resection of what was diagnosed as a "fibrillary astrocytoma (microcystic)." He received no chemotherapy or radiation therapy and remained well for 11 years. The patient presented again in late 1995 with progressive seizure activity. Both CT and magnetic resonance imaging demonstrated a recurrent enhancing partly cystic lesion. A Grade IV astrocytoma was resected, and within the malignant tumor was a superficial area reminiscent of a dysembryoplastic neuroepithelial tumor (DNT). Data on the lesion that had been resected in 1984 were reviewed, and in retrospect the lesion was identified as a DNT of the complex form. It was bordered by cortical dysplasia and contained glial nodules, in addition to the specific glioneuronal element. The glial nodules were significant for moderate pleomorphism and rare mitotic figures. The Ki67 labeling index averaged 0.3% in the glial nodules and up to 4% focally. Cells were rarely Ki67 positive within the glioneuronal component. This case is the first documented example of malignant transformation of a DNT. It serves as a warning of the potential for malignant transformation in this entity, which has been traditionally accepted as benign. This warning may be especially warranted when confronted with complex forms of DNT. The completeness of resection in the benign state is of paramount importance.     

Dumbbell neurogenic tumor of the mediastinum: A report of three cases undergoing single-staged complete romoval without thoracotomy

Of a total thirteen patients who underwent surgery for a neurogenic tumor in the posterior mediastinum 4 (30.8 per cent) presented with dumbbell type development of the tumor. Along with a comparatively greater incidence in the number of cases of dumbbell neurogenic tumors in the posterior mediastinum, resection has also recently become more popular, necessitating the establishment of a standard operative approach for this type of tumor. We successfully removed dumbbell neurogenic tumor from the posterior mediastinum in our 3 most recent casesvia a dorsal approach by virtue of a laminectomy and resection of a small portion of the neighbouring rib root without opening the parietal pleura at all. These three aptients were a 14 year old female, a 54 year old male and a 68 year old female, respectively, and the largest diameter in cm and level of origin of the tumors were 5.5 at Th 1 in case 1, 3.0 at Th 2 in case 2 and 3.7 at Th 11 in case 3. The operative approach described herein was easy to perform, felt secure and was less invasive and better tolerated by the patients than the thoractomy approach. Avoiding a thoractomy in such cases has many advantages to enumerate, but does not seem to have been clearly aimed at by others to date. We therefore propose our technique as a standard approach for dumbbell neurogenic tumors in the posterior mediastinum.     

Update on Wilms tumor

This article reviews of the current evidence-based treatment standards for children with Wilms tumor. In this article, a summary of recently completed clinical trials by the Children's Oncology Group is provided, the current diagnostic evaluation and surgical standards are discussed, and the surgical impact on current risk stratification for patients with Wilms tumor is highlighted.

Glomus Tumor in a Segmental Bronchus: A Case Report

Glomus tumors originate from a neuroarterial structure called the glomus body, and grow mostly in soft tissue. It is rare for glomus tumors to develop in the respiratory system. The patient of the present case had an abnormal shadow in the right lung on chest X-ray, and computed tomography (CT) findings displayed a lung tumor in the right S6. Bronchoscopy was performed for the diagnosis of the lung tumor, and a polypoid bronchial tumor was unexpectedly found to occupy the right B3. The bronchial tumor was diagnosed as a glomus tumor, and the lung tumor was diagnosed as an adenocarcinoma. The bronchial glomus tumor was cauterized by argon plasma coagulation (APC). Three weeks after the cauterization by APC, the right lower lobectomy was performed for the treatment of the lung adenocarcinoma. The patient has remained disease free for 2 years.