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PubMedDate not supplied

Immunohistology, cytogenetics, and molecular studies of small round cell tumors of childhood. A review.

Malignancies of childhood include a well-defined spectrum of hematolymphoid, organ specific (adrenal, kidney, liver), soft tissue, bone, and nervous system (central and peripheral) neoplasms with variable biology. Small round cell neoplasms, a subset of childhood malignancies, are histologically similar but differ markedly in their histogenesis, therapy, and prognosis. Traditionally, clinical information and light microscopy, with the aid of histochemistry and ultrastructural evaluation, establish a diagnosis or at least narrow the differential diagnosis. Additionally, immunohistology, cytogenetics, and molecular studies have become important in diagnosis and in defining phenotype/genotype, patient treatment modalities, and prognosis in specific cases. The 11;22 chromosomal translocation typifies Ewing's sarcoma, primitive neuroectodermal tumor, and Askin's tumor, as does the resultant chimeric transcript, while expression and amplification of N-myc oncogene are predictive of the prognosis in neuroblastoma. Furthermore, studies of genes and gene products are elucidating mechanisms of oncogenesis and tumor progression.

Child↗
PubMed1984

Esthesioneuroblastoma: ultrastructural, immunohistological and biochemical investigation of one case.

A case of esthesioneuroblastoma, the pathological diagnosis of which almost always causes great difficulties, was investigated ultrastructurally, biochemically, and immunohistologically, using antibodies against the five known types of intermediate filaments [keratin, vimentin, desmin, glial fibrillary acidic protein (GFAP) and neurofilaments]. The tumour cells did not react with antibodies against any of the five intermediate filament proteins. Ultrastructural investigations showed dense cored secretory granules in the cytoplasm and cell processes. Thus, immunohistology offers by "exclusion" a differential diagnosis to avoid often misdiagnosed tumours (undifferentiated carcinomas, embryonal rhabdomyosarcomas, and malignant lymphomas), since carcinomas react with antikeratin, embryonal rhabdomyosarcomas with antibodies to desmin and malignant lymphomas show immunofluorescence with antibodies to vimentin. The biological behaviour (age distribution, tendency to metastasize), the normal values of biochemical parameters, homovanillic acid and vanilmandelic acid (HVA, VMA), and the absence of neurofilaments distinguish this type of tumour from the peripheral sympathetic neuroblastoma.

Cytoplasmic Granules↗
PubMed1987

[Peripheral neuroepithelioma in the retroperitoneum; a case report and a review of the literature].

A 30-year-old man with peripheral neuroepithelioma which was present in the retroperitoneum underwent surgical treatment under the diagnosis of germ cell tumor. Subsequently he died of local recurrence and multiple metastases to the liver seven months after surgery. To our knowledge, this is the second case of peripheral neuroepithelioma which was observed in the retroperitoneal space in the literature of the world. We also reviewed 20 cases of peripheral neuroepithelioma reported in Japan.

Adult↗
PubMed1991

Adult peripheral neuroepithelioma in Meckel's cave.

A case of peripheral neuroepithelioma arising from the trigeminal nerve in Meckel's cave is presented. The discussion emphasizes the pathological criteria for the diagnosis of a peripheral neuroepithelioma and the current controversy about the classification of this and related tumors.

Humans↗
PubMed1995

Benign and malignant nerve tumors.

Tumors of peripheral nerves in the upper extremity are uncommon. All too frequently this diagnosis is made intraoperatively or postoperatively with the potential to compromise the outcome of treatment. An appropriate index of suspicion is necessary for inclusion of peripheral nerve tumors and a differential diagnosis of an upper extremity mass. Knowledge of this group of neoplasms will help direct evaluation and treatment.

Arm↗
PubMed1984

Peripheral neuroepithelioma in childhood.

Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. Fifteen of the 38 previously reported cases have involved children from birth to 17 years of age. The authors observed the course of a 3-month-old girl who presented with an enlarging mass in the left arm and manifested hepatic metastases at the time of diagnosis. The urinary level of vanillylmandelic acid (VMA) was moderately elevated. The primary lesion was excised and metastatic foci showed response to a regimen of vincristine, cyclophosphamide, Adriamycin (doxorubicin), and cisplatin. However, tumor recurred in the brain and liver and the child died 14 months after diagnosis. At autopsy, there was no involvement of adrenal glands or sympathetic ganglia and the liver contained numerous involuted lesions as well as active metastases. It is suggested that this is a unique neoplasm, derived from neural crest but distinct from neuroblastoma, which can be characterized by peripheral origin, a histologic pattern of confluent pseudorosettes, and aggressive clinical behavior.

Adolescent↗
PubMed1985

Immunohistochemical diagnosis of olfactory neuroblastoma.

Olfactory neuroblastoma (OLF NB) is an uncommon neurogenic tumor arising in the nasal cavity. To identify the immunohistochemical reactivity of OLF NB, we examined specimens from ten patients for the presence of neuron-specific enolase (NSE), S-100 protein (S-100) and glial fibrillary acidic protein (GFAP). Formalin-fixed paraffin sections were treated with rabbit antisera using the peroxidase-antiperoxidase (PAP) and avidin-biotin immunofluorescence methods. Results were compared with those observed in four neuroblastomas (two central and two peripheral sites), 17 medulloblastomas, four pheochromocytomas and five paragangliomas. All OLF NB were positive for NSE and S-100. NSE was uniformly distributed throughout clusters of tumor cells within tumor nodules. The S-100 reactivity was distributed in neoplastic cell nuclei and long cytoplasmic processes of cells which interconnected to form a network at the periphery of tumor cell nests. GFAP was observed only in astrocyte-like cells in one tumor. Although the S-100-positive cells in OLF NB resembled S-100-positive sustentacular cells of paraganglioma and pheochromocytoma, the network pattern was distinctive in OLF NB. The immunodiagnostic pattern of OLF NB includes the following: (a) NSE present in irregular clusters of tumor cells, (b) S-100 present in peripheral cells which form a marginal network, (c) and GFAP rarely present.

Adult↗
PubMed1991

Single-cell detection of ets-1 transcripts in human neuroectodermal cells.

The genes of the ets family are thought to code for a novel class of transcriptional factors. These proteins have a specific DNA-binding domain different from the basic domain of both the helix-loop-helix and leucine zipper families of DNA-binding proteins. The ets-1 gene product has been shown to bind to the enhancer region of the human T-cell receptor alpha gene during thymocyte ontogeny. This finding explains the high expression of ets-1 observed in T cells and the correlation between ets-1 expression and the expression of the T-cell receptor gene during fetal development. The ets-1 gene is also possibly biologically active in neural cells. By using RNA in situ hybridization analysis, we demonstrate the presence of ets-1 transcripts in cells of peripheral embryonal neuroectodermal tumors, specifically neuroepithelioma and neuroblastoma. In addition, the gene is found transcribed in Ewing's sarcoma, postulated to be ontogenetically related to tumors derived from the neural crest.

Gene Expression↗
PubMed1991

Immunohistochemical analysis of Ewing's sarcoma cell surface antigen p30/32MIC2.

Monoclonal antibody (MAb) HBA71, which was raised against Ewing's sarcoma cells, recognizes a cell-surface glycoprotein, p30/32MIC2, that is encoded by the MIC2 gene in the pseudoautosomal region of human chromosomes X and Y. This immunohistochemical study evaluates the specificity and sensitivity of MAb HBA71 for tumor diagnosis. Frozen and paraffin-embedded tissues of more than 300 tumors of diverse histologic type, including more than 100 small round cell tumors of childhood and adolescence, were tested with this MAb by the avidin-biotin immunoperoxidase procedure. The authors found HBA71 immunoreactivity in 61 of 63 Ewing's sarcomas studied and 9 of 11 primitive neuroectodermal tumors and peripheral neuroepitheliomas. HBA71-negative tumors included neuroblastomas (0 of 24), melanomas (0 of 13), an esthesioneuroblastoma, small cell osteosarcomas (0 of 2), a malignant ectomesenchymoma, desmoplastic SRCT (0 of 5), and medulloblastomas (0 of 5). Heterogeneous expression of HBA71 immunostaining was found in some embryonal rhabdomyosarcomas (3 of 14) and astrocytomas (4 of 7), and in a few neuroendocrine tumors (4 of 26), carcinomas (3 of 94), and lymphomas (6 of 30). Because Ewing's sarcomas are consistently HBA71 positive, the authors searched for antigen-positive normal cells that may represent precursors for these tumors; however, no obvious candidate for the elusive cell of origin for Ewing's sarcoma was identified in the normal fetal tissues tested. Their findings indicate that HBA71 is a highly restricted cell-surface antigen of Ewing's sarcomas and primitive neuroectodermal tumors, and immunohistochemistry employing this antibody may be of value in the differential diagnosis of selected small round cell tumors in childhood and adolescence.

12E7 Antigen↗
PubMed1994

Peripheral neuroepithelioma presenting as a spinal cord tumour.

A case of spinal cord tumour in a 41-year-old man is presented. The initial diagnosis was of metastatic small cell tumour. One year postoperatively he presented with a left cerebellopontine angle tumour which resolved with radiotherapy. Three years after initial presentation he remains well with no evidence of a primary tumour. Histological review shows the tumour to be a peripheral neuroepithelioma, which has not previously been reported to affect the spinal cord. The differential diagnosis of small cell spine tumours is discussed.

Adult↗
PubMed1987

Treatment of peripheral neuroepithelioma in children and young adults.

Seventeen patients with peripheral neuroepithelioma were treated with an intensive chemotherapy regimen of vincristine, Adriamycin (Adria Laboratories, Columbus, OH), and cyclophosphamide (VADRIAC) in combination with radiation therapy. Fifteen patients with stage III (seven) or stage IV (eight) at presentation were treated on a more intensive regimen including total body irradiation (TBI) (8 Gy). Two patients with stage I (one) or II (one) disease received a less intensive chemotherapy regimen of VADRIAC. Therapy was completed within 6 to 7 months in all patients. The disease arose in the chest wall in 12 patients, pelvis in three patients, and extremity in two patients. Sixteen of the 17 (94%) patients achieved a complete remission. With a median follow-up of 18 months, ten patients remain in complete remission with an actuarial survival of 68% and an actuarial relapse-free survival of 56% at 12 months. On the basis of our initial experience with this tumor, we believe that peripheral neuroepithelioma is a chemoresponsive and radioresponsive tumor.

Adolescent↗
PubMed1981

Soft tissue sarcomas of childhood: the differential diagnostic dilemma of the small blue cell.

In its histologic features, embryonal rhabdomyosarcoma (RMS), the prototype of malignant soft tissue tumors in childhood, summarizes the problems associated with the diagnosis of this entire group of neoplasms. Many of the tumors that do not fulfill the criteria for RMS have been designated "sarcomas of uncertain histogenesis." The introduction of the concept of a soft tissue equivalent of Ewing's sarcoma may have eased the semantic anxiety without improving our conceptual understanding. It is thought that the embryonal RMS, Ewing's sarcoma, and other are derived from a primitive mesenchymal cell. Another separate category of "small blue cell tumors" are those which presumably originate from the primitive neuroepithelium. Some of the diagnostic terms applied to this category are "neuroepithelioma," "medulloepithelioma," and "peripheral neuroblastoma." Because most of these tumors are hormonally inactive and electron microscopy is not performed, the diagnosis is infrequently considered or proved. The recently described small cell tumor of thoracopulmonary origin is likely a malignant neuroepithelial neoplasm. Hematopoietic tumors, such as non-Hodgkin's malignant lymphomas, granulocytic sarcoma, and malignant histiocytosis, may appear in the soft tissues as the initial manifestation of these system diseases. A final group of malignant soft tissue tumors are the fibrohistiocytic ones with a biphasic pattern of small round cells and spindle cells. It now has become increasingly difficult for the pathologist to satisfy his clinical colleagues with the diagnosis of "undifferentiated malignant tumor" in a child.

Bone Neoplasms↗