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PubMed1987

Esthesioneuroblastoma masquerading as extramedullary plasmacytoma.

Nine years after biopsy and radiation therapy for an extramedullary plasmacytoma of the nasal cavity, a 64-year-old man developed locally recurrent tumor. Repeat biopsy revealed tumor identical in hematoxylin and eosin stain (H & E) appearance to that found originally, leading to the diagnosis of recurrent extramedullary plasmacytoma. Neither the original tumor or its recurrence demonstrated features characteristic of esthesioneuroblastoma (e.g., nests, rosettes, ganglion cells, or fibrillary background). However, Gremilus staining and neuron specific enolase tests (immunoperoxidase method), done strictly as a teaching exercise, were strongly positive and eventually resulted in an altered diagnosis to esthesioneuroblastoma. This case underscores the difficulty that may be encountered in distinguishing these two pathologic entities of the nasal cavity solely on the basis of H & E staining. In view of differences in staging, evaluation, prognosis, and management, accurate differentiation of these two relatively uncommon tumors of the nasal cavity is of immediate clinical importance. These observations suggest that care should be taken to exclude the possibility of esthesioneuroblastoma before making the histologic diagnosis of extramedullary plasmacytoma of the nasal cavity.

Diagnosis, Differential↗
PubMed1989

Olfactory neuroblastoma.

Fifteen patients with olfactory neuroblastoma were treated during the 17-year period of 1969 to 1986. Data was analyzed with respect to age at presentation, sex, presenting signs and symptoms, stage, and results of treatment. Age ranged from 4 to 67 years with the median age being 27 years. Median follow-up was 8 years. Local control was achieved in nine of nine patients or 100% with successful surgical resection, i.e., minimal residual disease, followed by postoperative radiation therapy (45 to 65 Gy) was employed. There were no distant failures when the primary site was controlled. Regional lymph node metastases were infrequent: only 13% (two of 15 patients) presented with positive nodes. Three of four patients treated initially with surgery alone had a local recurrence, two of which were successfully salvaged by combined therapy. There were four patients treated with radiation therapy alone: three had persistent disease after radiation therapy, and one patient was controlled with 65 Gy. Olfactory neuroblastoma has a propensity to recur locally when treated with surgery alone. The authors' experience suggests excellent local control can be achieved with surgery immediately followed by radiation therapy. Thus the authors recommend planned combined treatment for all resectable lesions.

Adolescent↗
PubMed1989

Neuroepithelial small cell carcinoma of the vagina.

We studied two patients with primary vaginal small cell carcinomas. Their tumors show histologic and ultrastructural neuroepithelial elements similar to those reported in the literature in the cervix and the lung. The clinical behavior of the tumors was aggressive in both patients. Ultrastructural study of small cell genital tract neoplasms is indicated to help classify this disease. Adjunctive therapy may be required for neoplasms of neuroepithelial origin.

Aged↗
PubMed1990

Pathogenesis of vertebral metastasis and epidural spinal cord compression.

The authors have studied the sequential events in the process of vertebral metastasis that result in spinal cord compression. Different tumor cell lines were injected into the systemic arterial circulation of syngeneic or nude mice, and they were killed at timed intervals after injection or when they became paraplegic. The following observations were made. The tumor cells lodged and grew in the hematopoietic bone marrow of the vertebrae. Cancer cells in the vertebral marrow cavity invaded into the spinal canal through the foramina of the vertebral veins rather than destroying the cortical bone. Tumor cell lines that grew in an infiltrative fashion migrated toward a posterior location in the spinal canal, and compressed the spinal cord from a posterior direction. Tumor cell lines that grew as compact tumors formed a tumor mass at the same location from which the cells emerged from the vertebra, and compressed the cord predominantly from an anterior direction. Radiographic evidence of vertebral metastasis was a late event, and commonly associated with significant compression of the cord and extraosseous tumor. These experimental findings may help to establish better diagnostic and treatment strategies for patients with metastatic disease of the spine.

Animals↗
PubMed1991

Basic fibroblast growth factor and somatomedin C in human medulloepithelioma.

Two published cases of medulloepithelioma, a rare malignant pediatric brain tumor composed of a mixture of primitive neuroepithelium and its differentiated neuronal and glial descendants, were examined by immunohistochemical staining for the presence of growth factors. From a panel of antibodies, those identifying basic fibroblast growth factor and insulin-like growth factor I, formerly known as somatomedin C, were strongly immunoreactive within the neuroepithelial cell population of the tumors. Immunoblots of purified recombinant basic fibroblast growth factor and insulin-like growth factor I showed antibody specificity without cross-reactivity. In controls, immunostaining of tissue sections was abolished by preabsorption of primary antibodies with the appropriate growth factor polypeptide antigen. Preabsorption with inappropriate growth factor did not reduce the intensity or alter the distribution of staining. The congruent histologic patterns of immunoreactivities suggest that more than one type of growth factor may be produced by the neuroepithelial component of medulloepithelioma. These growth factors may stimulate proliferation and differentiation of tumor cells by autocrine molecular mechanisms.

Animals↗
PubMed1992

Sinonasal primitive neuroectodermal tumor arising in a long-term survivor of heritable unilateral retinoblastoma.

BACKGROUND: Patients who survive retinoblastoma (RB) are at risk for having second nonocular tumors, usually osteosarcomas, which often are fatal. Such patients almost always have bilateral RB. METHODS: This article reports a woman who, at the age of 1 year had been cured of a unilateral RB by radiation therapy and enucleation. Eighteen years later, she had a sinonasal small cell tumor that rapidly recurred and proved fatal 2 months after surgical debulking. The tumor was studied by immunohistochemistry and electron microscopic (EM) examination. RESULTS: It showed diffuse neuron-specific enolase staining, focal weak staining for chromogranin, synaptophysin, and Leu-7 monoclonal antibodies in paraffin-embedded, B5-fixed tissue (Great Lakes Diagnostics, Troy, MI). EM study showed an undifferentiated primitive neuroectodermal tumor with many polyribosomes, simple cell junctions, few microtubules, and rare dense core granules. CONCLUSIONS: The combined immunohistochemical, ultrastructural, and clinical features of the tumor were interpreted as a sinonasal primitive neuroectodermal tumor with early neuronal differentiation. The tumor was pathologically indistinguishable from poorly differentiated olfactory neuroblastoma (ONB) and Ewing sarcoma.

Chromogranins↗
PubMed1994

Gangliosides as diagnostic markers of human astrocytomas and primitive neuroectodermal tumors.

BACKGROUND: Limitations of classification schemes for brain tumors based solely on morphology have stimulated searches for molecular markers of nosologic and prognostic value. Gangliosides are logical candidates because there are high concentrations of them in the nervous system, there is evidence of their roles in regulation of growth and differentiation, and data from small series suggest correlations between ganglioside composition and glioma type. METHODS: Ganglioside compositions were determined for 70 primary human brain tumors: 16 low grade astrocytomas (LG), 12 anaplastic astrocytomas (AA), 34 glioblastoma multiformes (GBM), and 8 primitive neuroectodermal tumors (PNET). This method involved identification and quantitation of specific gangliosides using chemical analysis and immunoanalysis. RESULTS: Among all tumor types, histologic grade correlated with a progressive loss of 1b gangliosides (P < 0.0001). GQ1b was higher in LGs than in AAs (P < 0.001). Both GT1b and GD1b were higher in AAs than GBMs (P < 0.01 and 0.05, respectively) and lower in PNETs than in GBMs (P < 0.05). GM3 was higher in PNETs than in any astrocytoma group and higher in GBMs than in either AAs or LGs. There was a significant difference in the content of 3'-LM1 among all groups (P < 0.005), between AAs and GBMs (P < 0.05), and between low grade ordinary and juvenile pilocytic astrocyomas (P < 0.01). The lacto-series ganglioside 3'-isoLM1 was present in all groups except PNET. CONCLUSIONS: These results indicate that patterns of gangliosides could be of considerable value in refining the classification and diagnosis of primary human brain tumors.

Adult↗
PubMed2000

A phase I dose escalation of combination chemotherapy with granulocyte-macrophage-colony stimulating factor in patients with neuroblastoma.

BACKGROUND: Dose intensity is important in the response to chemotherapy in patients with advanced neuroblastoma. The aim of the current study was to determine the maximum tolerated dose of a combination chemotherapy regimen in the treatment of patients with recurrent neuroblastoma and peripheral neuroepithelioma (primitive neuroectodermal tumor [PNET]) and whether the use of growth factor would allow increased dose intensity. METHODS: Twenty-nine patients diagnosed with recurrent neuroblastoma or PNET were treated with a combination chemotherapy regimen of cisplatin, 160 mg/m(2)/96 hours; doxorubicin, 40 mg/m(2)/96 hours; and escalated doses of etoposide and ifosfamide. Granulocyte-macrophage-colony stimulating factor (GM-CSF) was administered beginning 24 hours after the completion of the chemotherapy. Courses were repeated at 28-day intervals. Once the maximum tolerated dose (MTD) was defined the interval between courses was shortened by administering the next course as soon as the patient's neutrophil and platelet counts had recovered to > 1500/microL and > 75,000/microL, respectively. RESULTS: Sixteen patients were treated at 3 dose levels. The MTD was defined as 10 g/m(2)/96 hours of ifosfamide and 800 mg/m(2)/96 hours of etoposide. Thirteen additional patients then were treated at 1 level below the MTD to try and decrease the interval between courses. A total of 12 of 29 patients developed a dose-limiting toxicity (DLT) after the first course of therapy. The most common DLT was gastrointestinal toxicity followed by hematologic toxicity. Twenty-seven patients developed standard National Cancer Institute criteria Grade 3 or 4 toxicity after the first course of treatment and 7 patients achieved a complete or partial response to the first course. The use of GM-CSF did not allow further dose intensification. CONCLUSIONS: This chemotherapy combination achieved a 31% overall response rate. A further increase in the dose intensity of this regimen may require supportive measures other than GM-CSF to decrease toxicity.

Adolescent↗
PubMed2004

Second malignant neoplasms in childhood acute lymphoblastic leukemia: primitive neuroectodermal tumor of the chest wall with germline p53 mutation as a second malignant neoplasm.

About 80% of children treated for acute lymphoblastic leukemia (ALL) will be long-term survivors. Second malignant neoplasm (SMNs) are a devastating sequelae observed on these children, with an estimated cumulative risk of 2-3.3% fifteen years after diagnosis. Primitive neuroectodermal tumor of bone (PNET) is rarely observed as a SMN following treatment of childhood ALL. The authors described the occurrence of a chest wall PNET of the bone at the site of a central line placement associated with both germ-line and tumor cell p53 mutation in a 8-year-old boy 1 year after completing therapy for standard risk ALL. A review of the literature of 25,051 children treated for ALL discovered 230 SMNs (0.99%), and only one case of PNET of the bone was noted among this group. The occurrence of a SMN in children treated for ALL is a rare event. Such an occurrence, in particular the development of an unusual SMN, should be evaluated for a germline p53 mutation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Dysembrioplastic neuroepithelial tumor: cytological diagnosis by intraoperative smear preparation.

The dysembrioplastic neuroepithelial tumor (DNT) is a benign and slow-growing temporal lobe cortical lesion that presents with clinically unresponsive partial and complex seizures, with onset before age 20 yr. DNT shows a multinodular architecture and the presence of mixed glioneural elements. The authors report on a case of DNT diagnosed by the intraoperative smear cytological technique. We describe the cytological characteristics of smear preparations of DNT, with emphasis on the differential diagnosis. The authors present a 5-yr-old male with partial seizures for the preceding 3 yr. The patient showed no other neurological deficits. Magnetic resonance identified a lesion in the cortical area of the right temporal lobe. The smear cytology showed small, round, oligodendroglial-like cells arranged in lobules, with microcystic spaces containing ganglion cells floating in a fine fibrillary background. Histology confirmed the diagnosis of DNT. This report shows that the smear preparation can be trustworthy for the intraoperative diagnosis of DNTs, helping to determine the appropriate neurosurgical procedure and thus avoiding extensive and unnecessary resections.

Astrocytoma↗
PubMedDate not supplied

Esthesioneuroblastoma: cytologic features with differential diagnostic considerations.

A case of recurrent esthesioneuroblastoma was diagnosed by fine-needle aspiration of a midcervical mass. The cytologic features that characterize this uncommon neoplasm include uniform, small, round-to-oval cells with coarsely granular chromatin, multiple small nucleoli, prominent nuclear membranes, and scant cytoplasm. Morphologically and ultrastructurally, esthesioneuroblastoma cells resemble those of sympathetic neuroblastomas. Both tumors show rosette formation and minimal cellular pleomorphism, and, by electron microscopy, dense-core cytoplasmic granules can be identified. The cytologic recognition of esthesioneuroblastoma is important since it metastasizes to cervical lymph nodes accessible to fine-needle aspiration biopsy. In addition, the histogenesis of this unusual neuroectodermal tumor and its behavior are discussed.

Biopsy↗
PubMedDate not supplied

Esthesioneuroblastoma: is there a role for elective neck treatment?

A retrospective review of the Memorial Sloan Kettering Cancer Center experience (MSKCC) with esthesioneuroblastoma was performed. From 1975 to 1985 14 cases were identified. Overall 5- and 10-year survival was 86% and 70%, respectively. Four local failures were observed and 4 neck failures were observed. A review of the literature since 1966 revealed an unexpectedly high incidence of neck failure, and of the 21 of 110 patients with neck failures that were identified through the literature, there were 6 subsequent deaths.

Adult↗
PubMedDate not supplied

Esthesioneuroblastoma and neck metastasis.

Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the cribriform olfactory epithelium. Its aggressive biologic behavior is characterized by inapparent submucosal spread, local recurrence, atypical distant metastases, and poor long-term prognosis. Historically, the suspicion of neck metastasis has not been associated with ENB. A retrospective review of all ENB patients treated at the University of North Carolina Hospitals since 1972 has revealed an unusually high incidence of cervical metastases (four of four patients). This prompted a review of the ENB surgical literature. All English-literature series published since the widespread use of craniofacial surgery with accruals of eight or more patients were examined for reports of neck metastasis. Although the cumulative cervical metastatic rate reached 27% (55 of 207 patients), most authors failed to recognize the potential risk of cervical metastasis in ENB. Furthermore, in patients suffering from Kadish stage C disease, the cervical metastatic rate climbed to 44% (25 of 57 Kadish stage C patients). This further emphasizes the importance of the neck as a high-risk metastatic site. These findings suggest that cervical metastases may be an important consideration in the evaluation and treatment of patients suffering from ENB. Strategies for evaluation and management of the neck are discussed.

Adult↗
PubMedDate not supplied

Olfactory neuroblastoma: the results of modern treatment approaches at the University of Michigan.

A retrospective study was undertaken to review patterns of treatment and survival for patients with olfactory neuroblastomas at the University of Michigan since the introduction of craniofacial resection in the late 1970s. Recent results were compared to previous reports. Disease-free and overall survival for patients with Kadish stage B tumors were unchanged from prior years. Improvement in both disease-free intervals and overall survival was evident for patients with stage C tumors. Interestingly, in two patients with "unresectable" local disease, complete responses to induction chemotherapy followed by radiotherapy were noted.

Adolescent↗
PubMedDate not supplied

Olfactory neuroblastoma: a retrospective clinicopathologic study.

The clinical course of olfactory neuroblastoma was not predictable by histologic criteria. Local recurrences were common (62%) and were often treated with multiple surgical procedures and radiation therapy. Metastatic neoplasm occurred in 28% of the patients. The recurrence-free interval decreased in those patients treated with radiation therapy initially, compared with those who had surgery initially. It is justifiable with respect to this study to treat disease in stages A and B with surgery and to reserve radiation therapy for recurrence. Stage C disease should be treated with combined therapy. Craniofacial resection should be promoted.

Adolescent↗
PubMed1984

Surgical pathology of olfactory neuroblastoma.

The natural history of a personal group of eight patients with olfactory neuroblastoma is reviewed with particular reference to surgical pathology as revealed by a study of the operative specimens in seven of these patients. This reveals that spread to the cranial surface of the overlying dura must be considered to be present in all patients irrespective of the radiologic findings. Craniofacial resection is therefore considered to be an essential part of any treatment regimen.

Ethmoid Bone↗
PubMedDate not supplied

Esthesioneuroblastoma metastatic to the trachea.

Esthesioneuroblastoma is an uncommon nasal neoplasm of neural crest origin, which is recognized for its propensity for local recurrence and distant dissemination by both lymphatic and hematogenous routes. Hematogenous spread occurs most frequently to the lungs and bones, but metastases to the liver, spleen, scalp, face, breast, adrenal, aorta, and ovary have been reported. We present a patient who developed metastatic esthesioneuroblastoma of the trachea 1 year after successful craniofacial resection of his primary tumor. The patient was treated by YAG laser resection of the metastatic tumor followed by radiotherapy. He was free of tracheal disease 1 year after treatment of the metastases.

Adult↗