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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.
We report a rare case of cystic metastases to the liver from a peripheral primitive neuroectodermal tumour in a child. Ultrasound and CT appearances are described with a discussion on the histological findings and the differential diagnosis.
Peripheral neuroepithelioma is a rare tumor, comprising less than 1% of all soft tissue malignancies arising from the peripheral nonautonomic nervous system. Most peripheral neuroepitheliomas reported were located in the extremities, thoraco-pulmonary region, and pelvic areas, and as many as 30% of cases were associated with peripheral nerve. We report one case of peripheral neuroepithelioma arising in the kidney, mimicking renal cell carcinoma on the CT scan.
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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.
Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sacral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, and died 29 months after the first diagnosis. In a 17-year-old male adolescent, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinical, radiological, histopathological and cytogenetic findings of both patients are presented and the relevant literature is reviewed. Particular attention is given to the histogenetic relationship between peripheral primitive neuroectodermal tumour and Ewing's sarcoma.
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.
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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.
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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.
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.
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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.
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.
The investigation for a plausible explanation of the development of massive spontaneous hemothorax during exercise in two patients led to the disclosure of two different malignancies. The first patient (pleural fibrosarcoma) passed away shortly after diagnosis. The second patient is alive without signs of disease (peripheral neuro-ectodermal tumor, PNET) since the diagnosis was made 3 years before. Spontaneous hemothorax (SH) arising during exercise does not exclusively designate trauma and full search for anatomical abnormality is warranted.
The peripheral primitive neuroectodermal tumour (pPNET) is a rare and highly malignant soft tissue neoplasm in children and young adults. We present a case of pPNET of the maxilla in a 49-year-old woman.
PURPOSE: The purpose of this study was to determine the clinical outcome for pediatric patients with peripheral neuroepithelioma treated with combined modality therapy and followed long enough to account for late relapses. PATIENTS AND METHODS: Fifteen patients, ages 3 3/12 to 19 10/12 years, with peripheral neuroepithelioma (median follow-up 91 months) were diagnosed at The Children's Hospital, Denver, Colorado over the period 1980-1989. All of these malignancies originated in the soft tissues. A critical review of these cases was performed with particular consideration given to the site and stage of the tumor and to the radiographic findings at presentation. Thirteen patients had bulk (> 5 cm in the greatest dimension) or metastatic disease. Four patients had primary tumors involving the chest wall. All patients received chemotherapy, which included at least doxorubicin, vincristine, and cyclophosphamide. Definitive surgical resections were performed on 13 of 15 patients. RESULTS: Five patients relapsed. Three were late relapses 24-44 months after diagnosis. Three of the five patients who relapsed had chest wall primaries. There were three deaths in this series due to peripheral neuroepithelioma and one due to sepsis. The overall survival was 68.5%, and the recurrence-free, survival 55.2%. Two patients with pulmonary relapses were treated with surgery and intensive chemotherapy and remain free of disease > 51 months following recurrence. CONCLUSIONS: Combined treatment modalities appear to be important for optimal outcome. This series represents the first report of favorable outcome of peripheral neuroepithelioma using a series with follow-up that is long enough to account for late relapses.