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PubMedDate not supplied

Primitive neuroectodermal tumor of the uterus. A case report.

BACKGROUND: Primitive neuroectodermal tumor (PNET) is a rare tumor derived from fetal neuroectodermal cells. These tumors occur in the central nervous system and in peripheral locations. Histologic diagnosis is the standard since most of these tumors are detected at an advanced stage. CASE: A 17-year-old female presented with persistent vaginal bleeding. Physical examination revealed a 4-cm, hard, barrel-shaped cervix. A cervicovaginal smear was obtained. The specimen was hypercellular, with small to medium-sized, round, malignant cells. A diagnosis of PNET was made from the histologic sections of the surgical specimen. CONCLUSION: When numerous small round cells in a diffuse pattern are seen on a Pap smear, the differential diagnosis is long and difficult. However, with careful evaluation of the cytologic features, a few reasonable differential diagnoses can be reached. Furthermore, with liquid-based Pap smears, material is available for immunohistochemical staining to narrow the range even more. Using all resources, including a good clinical history, a cytopathologist can give the clinician an early diagnosis for intervention and treatment.

Adolescent↗
PubMed1994

Primitive neuroectodermal tumor arising in the pancreas.

Peripheral primitive neuroectodermal tumors (PNETs) are extra cranial primitive small round blue cell tumors showing histologic, immunohistochemical or electron microscopic evidence of neuroectodermal differentiation. They are most commonly encountered in the soft tissue or bone in children and young adults. We report an unusual case of a PNET arising in the pancreas. A 17-yr-old male presented with a pancreatic mass and underwent a pancreatoduodenectomy. Histologically, the neoplasm was composed of sheets of small round cells that stained for cytokeratin, neuron specific enolase, and 12E7 but not muscle specific action, desmin, common leukocyte antigen, chromogranin, synaptophysin, or other islet cell markers. The diagnosis of PNET in this unusual location was confirmed by cytogenetic analysis which showed the chromosomal translocation characteristics of PNETs, t(11;12)(q24;q12). This case highlights the difficulty in the diagnosis of PNET when it is present in visceral organs where other neuroendocrine neoplasms and adenocarcinomas are more common.

Adolescent↗
PubMed1991

Primitive neuroectodermal tumor of the leptomeninges.

Primitive neuroectodermal tumor (PNET) without an obvious intraparenchymal mass lesion and with diffuse spread in the subarachnoid space is rare. We report such a rare case and discuss the computed tomography (CT) and magnetic resonance (MR) features.

Adolescent↗
PubMed1985

Epididymal melanotic neuroectodermal tumor of infancy.

Six melanotic neuroectodermal tumors of infancy arising in the epididymis were reported previously. Each followed a benign clinical course. In the seventh case of such a tumor, reported herein, an elevated serum alpha-fetoprotein level returned to normal following excision of the tumor. Urinary and serum tumor markers may be useful for following the course of these tumors in the future.

Chorionic Gonadotropin↗
PubMed1990

[Induction of primitive neuroectodermal tumors by oncogene complementation].

Primitive neuroectodermal tumors (PNET) represent a family of undifferentiated neural neoplasms which predominantly occur in children. PNETs are likely to originate from precursor cells and exhibit a marked potential for neuronal, glial and ependymal differentiation. A prominent example is the medulloblastoma of the cerebellum. In a model system using a novel transgenic CNS transplantation model, we have introduced a combination of ras and myc oncogenes into cell suspensions from fetal forebrain (E14) and postnatal cerebellum (P2) of the rat. Oncogene transfer into fetal forebrain grafts resulted in a high incidence of anaplastic neural tumors predominantly derived from glial precursors. Cell lines established from these neoplasms expressed high levels of both oncogenes. In a second experiment, the ras/myc vector was introduced into cell suspensions from neonatal cerebellum. The transformed cells were cultured for 3 weeks. Following stereotaxic transplantation, tumors of a similar morphology were observed. However, one animal developed a neoplasm with features of a cerebellar medulloblastoma. A cell line which exhibits a marked capacity for neurite extension and synaptogenesis was established from this tumor. Since these cells neither express ras nor myc, an insertion mutagenesis event appears to be responsible. Experiments to characterize this mutation are in progress. Our results indicate a potent transforming effect of ras and myc on neural precursor cells in vivo.

Animals↗
PubMed1999

Peripheral primitive neuroectodermal tumors of bone. A review of three cases.

The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.

Adult↗
PubMed2003

Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature.

STUDY DESIGN: Report of four cases and a review of the literature. OBJECTIVES: To study the clinical features and prognosis of primitive neuroectodermal tumor or extraskeletal Ewing sarcoma arising around the spinal column. SUMMARY OF BACKGROUND DATA: Primitive neuroectodermal tumor or extraskeletal Ewing sarcoma that originates around the spinal column is very rare, and its prognosis is very poor. METHODS: Four patients were diagnosed and underwent treatment. RESULTS: Although all the patients received high-dose chemotherapy with or without radiotherapy after surgery, three patients died of the disease. Only one patient who received en bloc resection of the tumor combined with multiagent chemotherapy followed by high-dose chemotherapy with peripheral blood stem cell transplantation remains alive and continues to be disease free. CONCLUSION: The prognosis of the patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column is very poor. Multiagent chemotherapy combined with en bloc resection and radiation therapy is the preferred treatment for patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column.

Adolescent↗
PubMed1983

Melanotic neuroectodermal tumor of infancy. A review of seven cases.

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare, usually benign, pigmented neuroectodermal tumor which most often involves the maxilla. The authors reviewed seven cases of MNTI, with patient ages of our patients ranged from nine weeks to 18 months; six of the seven were less than six months old at initial diagnosis. Four patients were males, and all were white. One tumor each was located in the femur, the temporal bone, and the epididymis; the remaining lesions occurred in the maxilla. Three of the four maxillary tumors recurred locally; the epididymal and femoral tumors metastasized. Two of these cases had unique clinical or pathologic features. The case of the femoral tumor is remarkable in that it is the first reported one of MNTI presenting in a long bone. This tumor was aggressively malignant; within two months after its discovery, a large mass of similar tumor was formed in the pelvis, and the tumor resulted in the patient's death. To the authors' knowledge, the case of the temporal bone tumor is the first one of MNTI in which neuronal differentiation of the neuroblastic cells is convincingly demonstrated. This finding provides additional evidence in support of the neuroectodermal theory of origin of these neoplasms.

Female↗
PubMed1998

Peripheral primitive neuroectodermal tumors in adults: documentation by molecular analysis.

PURPOSE: The Ewing tumor (ET) family of peripheral primitive neuroectodermal tumors (pPNETs) are primitive small round-cell tumors (SRCTs) of the bone and soft tissue that occur predominantly in children and adolescents. However, pPNETs only rarely enter the differential diagnosis of bone and soft tissue SRCTs in adults. Recently, gene fusions between the EWS gene and different members of the ETS transcription factor family have been shown to occur in virtually all pPNETs and thus constitute a pathognomonic marker for this tumor subclass. The aim of the present study was to document EWS/ETS fusion gene expression in suspected pPNETs of adults as objective evidence for the existence of this tumor family in older patients. PATIENTS AND METHODS: The three contributing molecular diagnostic laboratories retrospectively compiled a cohort of all SRCT cases in which EWS/ETS gene fusions had been shown by molecular analysis. This cohort was surveyed for cases that occurred in patients aged 40 years or older, which were then analyzed for their clinical and pathologic features. RESULTS: Nine patients between 40 and 65 years of age were found to have tumors positive for EWS/ETS gene fusions. Standard histopathologic and clinical features of these cases, other than age, were similar to those of childhood pPNETs. Patients were initiated on appropriate therapy after molecular analysis confirmed the diagnosis of pPNET. CONCLUSION: Identification of an EWS/ETS gene fusion is useful in providing objective evidence of the diagnosis of pPNET in patients over the age of 40 years. This diagnosis should be considered in adults who present with bone and soft tissue SRCTs and appropriate biopsy specimens should be collected for molecular analysis at the time of diagnosis.

Adult↗
PubMed2001

Peripheral primitive neuroectodermal tumor with neuronal and glial differentiation: report of a case arising in suprarenal region.

It is well known that embryonal neuroectodermal tumors of the central nervous system (CNS) not infrequently display varying amount of neoplastic cells acquiring glial differentiation. In contrast, glial differentiation rarely occurs in primitive neuroectodermal tumors outside the CNS being documented in less than ten cases. The author presents herein a case of peripheral primitive neuroectodermal tumor with prominent glial differentiation identified by the presence of glial fibrillary acidic protein (GFAP) arising in the right suprarenal region of a 32-year-old man, histologically indistinguishable from an ordinary neuroblastoma.

Adult↗
PubMed2004

Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management.

Primitive neuroectodermal tumors are in the Ewing's sarcoma family of tumors and are composed of small round cells. Because of their rare occurrence, optimal therapy is challenging, particularly if they occur in the head and neck. Diagnosis is based on history, immunostaining with at least 2 neural markers, ultrastructural examination, and evidence of an abnormal t(11;22)(q24;q12) translocation as the hallmark for the Ewing's sarcoma family. The prognosis in general is poor because of overt metastasis at the time of diagnosis. Of 27 reported patients with primitive neuroectodermal tumors of the head and neck, 23 were less than 20 years of age. Most patients presented with a tumor in the nasal cavity, paranasal sinuses, or neck. Symptoms developed rapidly (3.6 months, on average), and a lethal outcome occurred in 9 patients. This highly malignant tumor requires an aggressive combination of radical resection, chemotherapy, and radiotherapy. A close follow-up with regular radiographic examination for at least 5 years is mandatory.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings.

One hundred ninety-eight children were entered on POG 8633, "Prolonged Postoperative Chemotherapy and Delayed Radiation for Children <3 years of age with Malignant Brain Tumors" (1986-1990). Thirteen manifested supratentorial nonpineoblastoma primitive neuroectodermal tumors, making this the second most common supratentorial tumor in the study. Symptoms and signs included seizures, nausea, vomiting, lethargy, irritability, headache, focal motor weakness, and increased head circumference. Twelve of 13 had symptoms for less than 1 month before diagnosis. The average tumor size was 5.96 cm (+/- 0.37) x 5 cm (+/- 0.28) x 5.15 cm (+/- 0.31). Eight tumors were predominantly hemispheral, and five were midline. Computed tomographic scans on nine patients revealed tumor hyperdensity (nine), midline shift (eight), hydrocephalus (seven), cysts (six), well-defined borders (five), and calcification (four). No patients exhibited peritumoral edema. Contrast enhancement, primarily heterogeneous, was present in all patients. Magnetic resonance imaging appearance of the tumor (six patients) demonstrated midline shift (four), well-defined margins (four), necrosis (two), cysts (three), and hemorrhage (two). No peritumoral edema was present. Most enhanced heterogeneously. The diagnosis of supratentorial nonpineoblastoma primitive neuroectodermal tumors should be suspected when a large, sharply marginated, hyperdense supratentorial mass is observed in a young child, particularly when no peritumoral edema is present.

Child, Preschool↗
PubMed1983

Primitive neuroectodermal tumor in a 57-year-old man.

Primitive neuroectodermal tumors are rare cerebral neoplasms previously described only in children and young adults. This report describes such a tumor arising in the left frontal lobe of a 57-year-old man. After surgical resection and radiation therapy to the primary site, the patient developed extensive central nervous system metastases that led to his death. The histopathologic, radiographic, and clinical features of this case suggest that future therapeutic protocols for primitive neuroectodermal tumor should be similar to those for childhood medulloblastoma or neuroblastoma.

Age Factors↗
PubMed1992

Peripheral primitive neuroectodermal tumors. A flow cytometric analysis with immunohistochemical and ultrastructural observations.

Flow cytometry of classical neuroblastoma has provided provocative evidence that cell cycle and ploidy analysis generate prognostically useful information. To determine whether such analyses of peripheral primitive neuroectodermal tumors might yield similar results, formalin-fixed, paraffin-embedded tissue specimens from 19 peripheral primitive neuroectodermal tumors, each previously characterized by immunohistochemical or ultrastructural study, were assessed. An acceptable histogram was obtained in 16 cases. Of these, nine neoplasms were diploid and seven contained aneuploid DNA. Among patients with diploid lesions, four were free of disease, whereas three had persistent or recurrent disease, and two had died of tumor. Among patients with aneuploid neoplasms, four were free of disease, one had recurrence, and two had died. There was no apparent correlation between immunophenotype and proliferative activity with the clinical outcome. Among aneuploid peripheral primitive neuroectodermal tumors, DNA index did not predict survival. Hence, cell cycle and DNA ploidy analyses do not appear to contribute to the prognostic assessment of peripheral primitive neuroectodermal tumors, as they do to presumably related neoplasms of the central and peripheral nervous system.

Aneuploidy↗
PubMed1999

Cell cycle-associated proteins in melanotic neuroectodermal tumor of infancy.

OBJECTIVE: The purpose of the present study was to compare the immunohistochemical expression of cell cycle-associated proteins in neuroblastic and melanocytic cell populations of melanotic neuroectodermal tumor of infancy. STUDY DESIGN: Three cases of melanotic neuroectodermal tumor of infancy were selected. The immunohistochemical expression of MDM-2, p53, proliferating cell nuclear antigen, cyclin D1, and cyclin A was assessed through use of the streptavidin-biotin-peroxidase complex technique. RESULTS: Positive immunostaining for MDM-2, proliferating cell nuclear antigen, cyclin D1, and cyclin A was occasionally observed in the large melanin-containing epithelioid cells. CONCLUSIONS: These data suggest that MDM-2 expression may be important for the development of melanotic neuroectodermal tumor of infancy and that the melanocytic cell population, not the neuroblastic one, is the proliferative component of the tumor.

Cell Cycle Proteins↗
PubMedDate not supplied

Cytogenetic and molecular genetic abnormalities in primitive neuroectodermal tumors of the central nervous system.

Primitive neuroectodermal tumors constitute a large class of pediatric brain tumors. Despite notable recent advances in improving treatment and survival, the pathogenesis and the molecular genetic bases of these malignancies remain poorly understood. Combined cytogenetic and molecular genetic approaches have been used to identify genomic alterations in different histologic tumor types. Translation of these advances from basic science to clinical application is currently underway. Goals for the future include the development of more efficacious treatment strategies while simultaneously lessening toxicity. The most important cytogenetic and molecular genetic abnormalities documented to date together with their potential prognostic significance are reviewed.

Aneuploidy↗
PubMed2003

Primary leptomeningeal primitive neuroectodermal tumor.

Leptomeningeal metastases are a common complication of medulloblastomas and other primitive neuroectodermal tumors (PNETs). Much rarer are PNETs apparently arising in the leptomeninges. An 8-year-old boy presented with headache and vomiting, due to neoplastic meningitis from primary neuroectodermal tumor without an identifying mass. After craniospinal irradiation and chemotherapy (carboplatin, vincristine, cyclophosphamide and lomustine) the boy was in remission for 14 months, then suffered several relapses despite various chemotherapy regimens. After the initial presentation of 3.5 years the boy began to suffer from prolonged refractory non-convulsive status epilepticus and later expired from progression of primary leptomeningeal PNET.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

A para-testicular primitive neuroectodermal tumor in an adult: a case report and literature review.

OBJECTIVE: The authors describe the salient clinical, radiologic and histopathologic features of an extremely rare para-testicular primitive neuroectodermal tumor in a 25 year-old man. INTERVENTION: Excisional biopsy of the tumor en bloc was performed. Adjuvant VAdriaC-based chemotherapy (Vincristine, Doxorubicin, and Cyclophosphamide) was given post-operatively. MAIN OUTCOME MEASURES: Histopathologic examination and immunohistochemical studies were performed on formaldehyde-fixed, paraffin-embedded tumor tissue. RESULTS: Histologic examination showed an undifferentiated small cell tumor. The tumor cells stained positively with MIC-2, a marker specific for primitive neuroectodermal tumors. The patient is 12 months post surgery and has completed adjuvant chemotherapy with no evidence of recurrent disease. CONCLUSIONS: This highly unusual, peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of undifferentiated small cell neoplasms of the genitourinary system in adults, from the kidney to the testicle. We present a patient with a PNET treated based on a Ewing's family of tumors protocol.

Adult↗