Pigmented ganglioneuroblastoma: a tumour cell ‘storage disease’?

We report a composite nodular ganglioneuroblastoma, distinctive for the presence of coarsely granular black intracellular pigment, which was histochemically indistinguishable from.melanin. Ultrastructurally, the pig-ment was resolved as numerous heterogeneous lyso-somes, containing material compatible with oxidized lipid residues, the accumulation of which may reflect. acquired lysosomal dysfunction in individual tumour cells-a tumour cell 'storage disease'.     

Comparison of Ultrastructural Features Among Neuroblastic Tumors: Maturation from Neuroblastoma to Ganglioneuroma

Neuroblastic tumors have the unique ability to differentiate and mature. This family of tumors is composed of the neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. These tumors are derived from primordial neural crest cells that form the sympathetic nervous system. The purpose of this study was to characterize the ultrastructural features of neuroblastic tumors in a pediatric population. Forty-five neuroblastic tumors (15 neuroblastomas, 15 ganglioneuroblastomas, and 15 ganglioneuromas) were examined using standard transmission electron microscopic techniques. Undifferentiated neuroblastomas were composed of primitive cells with rare neurite-like processes containing clear secretory vesicles and no Schwann cell differentiation. Poorly differentiated and differentiating neuroblastomas showed more frequent neuritic processes with infrequent dense core granules and infrequent immature Schwann-like cells. Ganglioneuroblastomas possessed an admixture of cell types, including immature ganglion cells without associated satellite cells, intermediate cells, and differentiating neuroblasts. The neuropil contained immature Schwann cells encasing haphazardly arranged neuritic processes. Ganglioneuromas were composed of mature ganglion cells with occasional binucleation. The neuropil contained mature Schwann cells with well-organized neuritic processes and abundant collagen deposition. Differentiation or maturation of tumor cells, neuritic processes, and Schwann cells may thus be discerned ultrastructurally in primary neuroblastic tumors in pediatric patients.     

Peripheral neuroblastic tumors of the adrenal gland: clinicopathologic features and important molecular alterations

Peripheral neuroblastic tumors are the most common extracranial solid tumor of childhood and commonly present as an adrenal mass in a young child. This group of tumors is notable for wide clinical heterogeneity and variable clinical outcome. Peripheral neuroblastic tumors are defined by the amount of Schwannian stroma and degree of neuroblastic differentiation and categorized by the International Pathology Neuroblastoma Committee (INPC) as neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Molecular testing remains critical for risk stratification of patients. Important prognostic factors include age, stage, histologic classification, and amplification of MYCN. Advancements in our understanding of clinical and biologic prognostic factors continue to provide valuable knowledge for risk stratification and potential therapeutic targets.     

Peripheral Neuroepithelioma with Ganglion Cells: Report of Two Cases and Review of the Literature

Peripheral neuroepitheliomas, also known as peripheral primitive neuroectodermal tumors, are by definition primitive embryonal lesions generally composed of poorly differentiated neuroectodermal elements. We have examined two cases that paradoxically contain extensive foci of ganglionic differentiation similar to that of ganglioneuroblastoma, in addition to primitive elements. One tumor arose from the chest wall of an 8-year-old male and the other from the abdominal wall of a 15-year-old male. Differentiation into a mature ganglionic phenotype was confirmed by immunohistochemistry in one case. Rare peripheral neuroepitheliomas have a capacity for maturation that is not generally appreciated. These lesions should not be confused with ganglioneuroblastomas, which are genotypically unrelated neoplasms. Received August 27, 1997; accepted March 27, 1998.     

Intracerebral Ganglioneuroblastoma with Intracytoplasmic Microtubular Aggregates: Case Report and Ultrastructural Study

Cerebral ganglioneuroblastoma is a rare entity that is not well characterized at the ultrastructural level. We report a case of such a neoplasm with some unique fine structural features. The tumor contains both small cells with little evidence of differentiation and large, bizarre multinucleate cells, many of which contain intracytoplasmic aggregates of 22- to 24-nm micro-tubules that displace the nucleus and cytoplasmic organelles to the periphery of the cell. Inclusions of this type have not previously been described in this entity.     

Cytomorphology and morphometry of small round-cell tumors in the region of the kidney

Small round-cell tumors (SRCTs), with malignant cell components measuring 10 m or less in diameter with scanty cytoplasm in alcohol-fixed smears, pose a diagnostic challenge at fine-needle aspiration cytology (FNAC), especially when they are situated in and around the kidney and need facilities such as electron microscopy, immunohistochemistry, tissue culture, and cytogenetics for their subtyping. A precise cytodiagnosis of SRCTs is important because a definite diagnosis is mandatory in preoperative diagnostic workup for presurgical chemotherapy in these cases. With this view in mind, an attempt has been made to diagnose SRCTs in the region of the kidney based on cytomorphology and morphometry alone so as to facilitate its diagnosis in a simple cytology laboratory of a developing country where facilities for auxiliary techniques are not easily available. Of 2,028 abdominal aspirates in a 12-yr period, 36 SRCTs were diagnosed in the region of the kidney by correlating with histology, radiology, and clinical features. The smears were studied for cellularity, morphology, pattern of cell arrangement, and smear background and morphometrically analyzed using an ocular micrometer. An aspirate with preponderant malignant round cells that were larger or double the size of red blood cells in air-dried smears or measured less than 10 micro in diameter in alcohol-fixed smears was considered as a small blue-cell tumor. Twenty-one were diagnosed as Wilms' tumor (WT), 10 were diagnosed as neuroblastoma (NB), 3 were ganglioneuroblastoma (GNB), 1 was a cellular congenital mesoblastic nephroma (CMN), and 1 was an adrenocortical carcinoma (ACC). Cell clusters with neuropil and cytoplasmic processes were diagnostic of NB, ganglion cells of GNB, and blastema with tubular differentiation in WT. Aspirates from CMN and ACC were considered as simulators/mimickers of SRCT because they had superficial resemblance to SRCT and their differentiating cytomorphological features observed at histology were too subtle to be noted at cytology. The latter were appreciated only on retrospective analysis after histological confirmation.Thus, morphometry in correlation with cytology, clinical history, physical findings, and radiological data is helpful in guided FNA for a definite diagnosis of SRCT in the region of the kidney. One needs to keep in mind the mimickers of small round-cell lesions at this anatomic site.     

A case of cerebral composite ganglioneuroblastoma: an immunohistochemical and ultrastructural study

Summary An unusual cerebral tumor is reported in a 14-year-old boy. On light and electron microscopy, the constituent cells were very complex; the majority of the neoplastic cells were primitive neuroectodermal cells dispersed in myxomatous or fibrous stroma. Neoplastic neuronal cells and hypertrophic astrocytes were also observed in these areas. The neuronal cells showed a continuous spectrum of differentiation from very primitive to mature ganglion cells. Furthermore, the tumor contained a highly cellular discrete area consisting of neuroblasts and their precursor cells. From these findings, a diagnosis of composite ganglioneuroblastoma was made.     

后纵隔神经节细胞瘤和神经节神经母细胞瘤的CT和MRI诊断

目的分析后纵隔神经节细胞瘤和神经节神经母细胞瘤的CT和MRI表现及其诊断.材料和方法分析经手术、病理证实的神经节细胞瘤7例,神经节神经母细胞瘤2例的CT和MRI表现.结果肿瘤常呈半圆形或椭圆形,纵径比横径或前后径长,边缘锐利,1例神经节细胞瘤呈哑铃形.CT示肿瘤实性部分呈等或稍低于胸壁肌密度,囊变部分的密度比脑脊液稍高.T1WI示肿瘤等或稍低于胸壁肌信号,囊变部分稍高于脑脊液的低信号;T2WI呈高信号.结论肿瘤纵径长于其它径是重要征象,CT和MRI能显示肿瘤形态特征,提示诊断.