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PubMed1995

[Melanotic neuroectodermal tumor in an infant].

BACKGROUND: Melanotic neuroectodermal tumor is a mostly benign tumor, rare in childhood, essentially located in the head and neck region. CASE REPORT: A two-month-old girl was seen for a rapidly increasing odontogenic tumor which appeared cystic at the CT scan. After enucleation, this premaxillar tumor recurred one month later with an osteogenic aspect at the CT scan. Urine catecholamine excretion was normal; Methyl Iodo Benzyl Guanidin scintigraphy failed to show any fixation and electron microscopy examination of the biopsy showed several varieties of melanocytes. A partial maxillectomy was performed by oral approach. The patient is well 4 1/2 years later. CONCLUSION: This observation confirms the recurrence potential of this tumor and the cosmetic interest of the oral surgical approach.

Female↗
PubMed1995

Establishment of a human cell line secreting neuron-specific enolase from a primitive neuroectodermal tumor of the retroperitoneal cavity.

Primitive neuroectodermal tumor (PNET) is one of the small round cell malignancies of presumed neural crest origin for which an effective treatment has not yet been established. In the present study, a human cell line, designated KU-9, was established from a 27-year-old male patient with PNET of the retroperitoneal cavity and has been successfully maintained in nude mice and in culture. On histological examination, the primary tumor was composed of poorly differentiated small round cells arranged in clusters showing a variety of mitotic changes, and contained Homer-Wright rosettes. The histopathological appearance of the KU-9 xenografts was similar to that of the primary tumor. Electron microscopy revealed neurosecretory granules and cytoplasmic processes in the xenograft. No significant amplification of N-myc gene was observed in the KU-9 cells. The KU-9 cells showed chromosome numbers ranging from 56 to 61 with consistent structural abnormalities being add(2)(q31), +add(11)(p11.2), +add(13)(p11.1), and +del(22)(q12). Cultured KU-9 cells grew exponentially with a doubling time of about 50 h and a time-dependent increase in medium levels of neuron-specific enolase (NSE) was noted. Serum levels of NSE in KU-9 tumor-bearing nude mice were significantly elevated and a linear relationship between the serum NSE levels and the tumor NSE content or tumor volume was observed, suggesting that serum levels of NSE may reflect the PNET tumor burden and tumor extent. These results indicate that the KU-9 cell line provides a reproducible model system which could be useful in gaining some insight into the histogenesis and oncogenesis of PNET and in establishing an effective treatment for PNET.

Adult↗
PubMed1990

Melanotic neuroectodermal tumor of infancy: a case report.

The melanotic neuroectodermal tumor of infancy is a rare benign neoplasm that appears as a rapidly enlarging mass, most commonly in the anterior part of the palate. The tumor is believed to be of neurocrestal origin. The case report of a 3-month-old infant with a rapidly growing melanotic neuroectodermal tumor of infancy is presented. The tumor was surgically excised, and no evidence of recurrence has been found.

Biopsy↗
PubMed1999

[A case of primitive neuroectodermal tumor of the kidney].

A primitive neuroectodermal tumor (PNET) is a solid tumor originating from the neural crest. This tumor is known to occur in the central nervous system and soft tissue, but recently determined to also invade the kidney. Although primary renal PNET is very rare, we encountered a case of primary renal PNET with multiple lung metastasis. This case was a 35-year-old man with a chief complaint of macroscopic hematuria, which he noticed in January 1996. He was hospitalized because of right renal tumor detected with ultrasonography. On CT scan, a low-density solid tumor with a maximum diameter of 8 cm was visualized in the right kidney. Low-signal and high-signal tumorous lesions were demonstrated in the same region on T 1-weighted and T 2-weighted MR images, respectively. Then right radical nephrectomy was performed on Feb. 5, 1996. On histopathological observation, the tumor was composed of small tumor cells with solid growth. Immunohistochemical staining revealed that the tumor was positive for CD 99. Thus our final diagnosis was primary right renal PNET. Although 2 metastatic lesions with a diameter of about 1 cm were observed in the right lower lung before the time of surgery, pulmonary metastatic lesions markedly increased in number 3 months after surgery. Thus we initiated CAP chemotherapy with cyclophosphamide, doxorubicin, and CDDP. After 3 cycles, pulmonary metastatic lesions disappeared at CT scan, indicating complete remission. However, a tumor with a maximum diameter of about 10 cm recurred in the retroperitoneum 7 months after complete remission. Although 2 cycles of CAV/PE therapy with cyclophosphamide, doxorubicin, vincristine, CDDP, and VP-16 were performed following resection of the retroperitoneal tumor, he showed no response and decreased 24 months after surgery.

Adult↗
PubMed2003

Organ-confined primitive neuroectodermal tumor arising from the kidney.

Primitive neuroectodermal tumor (PNET) of the kidney is a rare entity with high malignant potential. Of the reported cases of renal PNET, patients presented with metastatic disease and had a poor response to therapy. The authors describe a rare case of an organ-confined primary PNET arising from the kidney in a 16-year-old girl who presented with vague abdominal pain. Tumor behavior and treatment modalities are discussed.

Abdominal Pain↗
PubMed2005

Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma.

The supratentorial primitive neuroectodermal tumors (PNETs) are a group of highly malignant lesions primarily affecting young children. Although these tumors are histologically indistinguishable from infratentorial medulloblastoma, they often respond poorly to medulloblastoma-specific therapy. Indeed, existing molecular genetic studies indicate that supratentorial PNETs have transcriptional and cytogenetic profiles that are different from those of medulloblastomas, thus pointing to unique biological derivation for the supratentorial PNET. Due to the rarity of these tumors and disagreement about their histopathological diagnoses, very little is known about the molecular characteristics of the supratentorial PNET. Clearly, future concerted efforts to characterize the molecular features of these rare tumors will be necessary for development of more effective supratentorial PNET treatment protocols and appropriate disease models. In this article the authors review existing molecular genetic data derived from human and mouse studies, with the aim of providing some insight into the putative histogenesis of these rare tumors and the underlying transforming pathways that drive their development. Studies of the related but distinct pineoblastoma PNET are also reviewed.

Animals↗
PubMed1998

Effects of all-trans retinoic acid and interferon alpha in peripheral neuroectodermal tumor cell cultures and xenografts.

Peripheral neuroectodermal tumors (PNET) have an unsatisfactory outcome when treated with standard approaches. Among novel treatments, the use of biological response modifiers has rarely been reported in this group of malignancies. We have previously demonstrated that both all-trans retinoic acid (ATRA) and interferon á (IFNá) can inhibit proliferation of human PNET cells and that ATRA can up-regulate IFNá receptor expression in vitro. In this study we evaluated the anti-tumor effects of ATRA and IFNá in PNET cells in vitro and in a human PNET xenograft model, using CHP100 cells. A synergistic inhibitory effect of ATRA and IFNá was observed on CHP100 cells in vitro. On the contrary, a significant inhibition of tumor growth was observed in mice treated with ATRA alone, whereas neither IFNá nor the combination of ATRA and IFNá, reached a statistically significant anti-tumor effect. Histologic examination of tumors revealed the presence of necrosis upon treatment with IFNá, whereas almost no necrosis, but a more differentiated morphology, confirmed by electron microscopy analysis, was associated with the ATRA containing treatments. Taken together these data show an in vitro and in vivo anti-tumor activity of ATRA in human PNET cells, although no synergism of ATRA and IFNá was observed in our xenograft model.

Animals↗
PubMedDate not supplied

[Melanotic neuroectodermal tumor of infancy].

BACKGROUND: The melanotic neuroectodermal tumor of infancy is a rare and so far as being classified neoplasm with a high rate of recurrence for one year after diagnosis. Since Krompecher described 1918 the tumor at first, only about 200 cases are reported until today, mostly with manifestation in the maxillary region. CASE-REPORTS: The authors present two infants at the age of six and eight weeks with first clinical manifestation of the tumor in the maxillary region. Although there were no other common signs, the tumor destroyed wide areas of the mid-face. In spite of a treatment with radical surgery, recurrences occur rapidly in the first living year. CONCLUSIONS: Our clinical and histological findings show characteristics of local malignant growth. For these facts the radical resections of the primary tumor and its recurrences are individually the therapeutical consequences. A follow up of seven years of one infant shows a hypoplasm of the mid-face as a result of the inhibition of further growth by the loss of germs after maxillary hemisection.

Humans↗
PubMed1993

Reverse transcriptase PCR amplification of EWS/FLI-1 fusion transcripts as a diagnostic test for peripheral primitive neuroectodermal tumors of childhood.

The peripheral primitive neuroectodermal tumors (pPNETs) of childhood, including Ewing's sarcoma, peripheral neuroepithelioma, and Askin's tumor, often present significant diagnostic challenges for the anatomic pathologist. One consistent feature of these tumors is the presence of the t(11;22)(q24;q12) in tumor cells, and this translocation has been useful as a marker for this group of tumors. The recent cloning of the t(11;22) breakpoint has revealed the fusion of the human FLI-1 gene on chromosome 11q24 with a gene of unknown function called EWS on 22q12, and fusion transcripts have been detected. These findings have raised the possibility of using molecular genetic analysis as a tool to diagnose pPNETs. To this end, we have tested pPNETs for the presence of EWS/FLI-1 fusion transcripts by reverse transcriptase-polymerase chain reaction (RT-PCR) using EWS and FLI-1 specific primers. Eight (80%) of 10 pPNET cell lines were positive for amplified products using this technique. These results were confirmed by Southern analysis, which revealed rearrangements of EWS using genomic EWS probes in all eight positive cell lines. We then tested 20 primary pPNET tumors, and identified fusion transcripts by RT-PCR in 18 (90%) of these cases. Cloning and sequencing of PCR products confirmed the presence of EWS and FLI-1 sequences in these products. Furthermore, fusion transcripts were not detected by this technique in a series of non-pPNET pediatric solid tumors. Detection of EWS/FLI-1 fusion transcripts by RT-PCR therefore provides a novel adjunctive tool in the diagnosis of pPNETs.

Adolescent↗
PubMed2000

Primary primitive neuroectodermal tumor of the cauda equina.

Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification has been controversial, PNETs are well recognized primary tumors of both central and peripheral nervous systems. PNETs must be distinguished from other round-cell tumors, including Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and small cell carcinoma. Intraspinal PNETs are rare neoplasms that are usually metastatic in origin. We describe the eighth reported primary PNET of the cauda equina that developed in a 52-year-old man with no significant medical history. The tumor was characterized by Homer-Wright rosettes and immunoreactivity for CD99, glial fibrillary acidic protein, neuron-specific enolase S100, and synaptophysin. The anatomic location of primary intrathecal PNETs is important as those arising in the spinal cord develop in the central nervous system, whereas those arising in the cauda equina develop in the peripheral nervous system. The histogenesis of intrathecal PNETs may be multifactorial.

Cauda Equina↗
PubMed2005

Treatment strategies for high-risk medulloblastoma and supratentorial primitive neuroectodermal tumors. Review of the literature.

Primitive neuroectodermal tumors (PNETs) are malignant tumors with a high propensity to disseminate throughout the cerebrospinal fluid. Current treatment guidelines are largely determined by clinically based prognostic factors, the most important of which are tumor location and the extent of tumor spread. Although the cure rate for high-risk PNETs has improved, the irreversible sequelae of craniospinal axis radiation treatment in patients who survive have motivated researchers to investigate more fully which patients can safely receive less treatment. The author reviews the literature, describes currently available treatment options for patients with high-risk PNETs, and discusses strategies aimed at improving outcome and refining prognosis that are currently being explored.

Age Factors↗
PubMed1979

Malignant melanotic neuroectodermal tumor of infancy: a clinical, pathologic, ultrastructural and tissue culture study.

The melanotic neuroectodermal tumor of infancy is an uncommon neoplasm typically of early childhood which has a predilection for the head and neck region, particularly the maxilla. Except for one previous example in the literature, this tumor has consistently behaved in a benign fashion. This study documents the clinical course and pathologic findings of a tumor which began in the maxilla of a 4-month-old boy, followed by a local recurrence, metastasis to a cervical lymph node and finally, widespread dissemination and death at 18 months, 24 months and 38 months, respectively. The tumor was initially composed of nests consisting of melanin-containing cells and small dark cells. An elevated vanillylmandelic acid level was recorded during the course of the disease. At autopsy, the tumor in lymph nodes, liver, bone and soft tissues had a monotonous pattern of small dark cells similar to a conventional neuroblastoma. Previous ultrastructural studies indicate that the melanotic neuroectodermal tumor of infancy is composed of melanocytes and neuroblast-like cells. Our case provided the unique opportunity to examine in sequence the ultrastructural and in vitro characteristics of a recurring and eventually metastasizing melanotic neuroectodermal tumor. Although the neuroblast-like cells were initially difficult to identify by electron microscopy, a melanin-producing cell line and a separate nonpigmented cell line were successfully isolated from various tumor explants. Various stages of melanosome development were identified in the pigmented cells from the local recurrences and in vitro. Dibutyryl cAMP accentuated the formation of pigment and dendritic development in the melanocytes and dendrites only in the small nonpigmented cells. Electron dense granules were observed in the cultured smaller cells and also in the lymph node and soft tissue metastases. Tyrosine hydroxylase activity was demonstrated in the neuroblast-like cells. In the final biopsy and autopsy material, only the neuroblast-like cells remained and the tumor resembled a conventional neuroblastoma.

Culture Techniques↗
PubMed2003

Medulloblastomas and central nervous system primitive neuroectodermal tumors.

Significant advances in the treatment of medulloblastoma and primitive neuroectodermal tumors have been made in the past three decades. Maximal surgical resection is a mainstay of therapy. However, unlike many other central nervous system neoplasms, medulloblastoma and primitive neuroectodermal tumors are radiation and chemotherapy responsive. Despite this response, the prognosis for patients with these tumors remains variable and is relatively poor in infants and patients with metastatic disease. These tumors most commonly arise in children, thus most clinical trials emphasize the reduction of long-term sequelae, in addition to improving survival. All newly diagnosed patients who are eligible should be offered participation in a clinical trial. If a patient is ineligible or declines consent/assent for a clinical trial, the best current treatment approach is surgical resection, followed by radiation therapy (except for children younger than 3 years) with weekly vincristine. For high-risk patients, 36 Gy of craniospinal irradiation should be delivered plus a boost of 19.8 Gy to the posterior fossa/primary tumor bed and sites of bulk metastatic disease. For average-risk patients, the craniospinal irradiation dose may be lowered to 23.4 Gy plus 32.4 Gy to the posterior fossa/tumor bed. After radiation therapy, intensive multimodal chemotherapy should be used for all patients.

Antineoplastic Agents↗
PubMed2000

Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction.

Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.

12E7 Antigen↗
PubMed2006

Transverse myelitis after therapy for primitive neuroectodermal tumors.

Traditional therapy for malignant primitive neuroectodermal tumors in children includes surgery, multi-agent chemotherapy, and radiation. Given the poor prognosis with conventional therapy alone, newer treatment approaches have incorporated high-dose chemotherapy followed by autologous stem cell rescue. Treatment with chemotherapy and radiation is not without unanticipated and unwanted side effects. Specifically, radiation-induced damage to the central nervous system can occur, though the frequency is thought to be acceptably low. This report describes two cases of treatment-related transverse myelitis in patients who received induction chemotherapy and craniospinal irradiation followed by high-dose chemotherapy with autologous stem cell rescue. Other patients treated with a similar strategy but different sequence and timing of treatment did not experience symptoms of myelitis, suggesting that the specific timing of radiation in relationship to the chemotherapy may be of critical importance.

Adolescent↗
PubMed2005

Molecular detection of EWS-Ets fusion transcripts and their clinicopathologic significance in Ewing's sarcoma/peripheral primitive neuroectodermal tumor.

BACKGROUND: Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is often difficult to distinguish from other small round cell tumors. The EWS-Ets gene fusions that result from chromosomal translocations in this tumor provide potential molecular diagnostic markers. To apply these molecular markers to commonly available archival materials, we evaluated the feasibility of detecting EWS-Ets including EWS-Fli1 and EWS-ERG fusion transcripts in paraffin-embedded tissues and its diagnostic value for detecting ES/pPNET. METHODS: Thirteen paraffin-embedded samples of ES/pPNETs were retrieved from archives. Thirteen cases of other tumors with small round cell features (including rhabdomyosarcoma, neuroblastoma, lymphoma, small cell carcinoma, and desmoplastic small round cell tumor) were used as negative controls. Beta-actin and beta2-microglobulin were used as internal controls. A nested reverse transcriptase-polymerase chain reaction (RT-PCR)-based assay was performed to detect the EWS-Fli1 and EWS-ERG fusion transcripts. RESULTS: Beta-actin and beta2-microglobulin were detected in 10/13 and 13/13 ES/pPNETs, respectively. EWS-Fli1 fusion transcripts were detected in 11 of 13 (85%) ES/pPNETs. Three chimeric transcripts, all EWS-Fli1, were detected in ES/pPNET samples. Among 11 EWS-Fli1-positive cases, 7 cases had a type I fusion transcript involving fusion of EWS exon 7 with Fli1 exon 6, 2 cases had a type II fusion transcript involving EWS exon 7 with Fli1 exon 5, and 2 cases expressed fusion transcripts involving EWS exon 7 and Fli1 exon 8. Type I EWS-Fli1 fusion predominated over other types. Fusion types could not be distinguished in the remaining 2 cases. Thirteen negative controls did not show detectable chimeric messages. There was a significant relationship between EWS-Fli1 fusion transcripts and CD99 expression. CONCLUSIONS: Molecular detection of EWS-Fli1 fusion transcripts in formalin-fixed paraffin-embedded material by nested RT-PCR is feasible and is useful for the diagnosis and differential diagnosis of ES/pPNETs.

Adolescent↗
PubMed1986

Peripheral and central primitive neuroectodermal tumors. A nosologic concept seeking a consensus.

The term primitive neuroectodermal tumor is widely used in the literature for a group of small, round-cell tumors in the central and sympathetic nervous systems and soft tissues as well as a specific diagnostic term for individual neoplasms; however, the contention that these various clinicopathologic entities (neuroblastoma, medulloblastoma, and peripheral neuroepithelioma) are histogenetically related is an unproved hypothesis. Morphologic, cytogenetic, immunohistochemical, biochemical, and in vitro studies have established phenotypic similarities among these putatively related neoplasms whether they originate in the brain, adrenal gland, or soft tissues. Because one tumor resembles another in terms of its phenotypic expression, that does not necessarily imply a common histogenesis. This point has been made by previous investigators. The purpose of this review is to evaluate and discuss the present status of our understanding and some of the controversial aspects of this enigmatic category of neoplasms, mainly occurring in children, known as the primitive neuroectodermal tumors.

Cell Differentiation↗
PubMed1993

Metastatic pigmented neuroectodermal tumor with cranial CT-pathology correlation.

Pigmented neuroectodermal tumors are a relatively rare clinicopathologic entity, presenting in a variety of locations. Their histogenesis suggests a transformation from common pleuripotential neural crest cells with varying degrees of differentiation to neural elements and melanocytes. We present a case of just such a tumor, which presented initially in the mandible of a leukemic patient who received irradiation to the skull base, as well as systemic chemotherapy, for suspected cranial leukemic involvement. Subsequent development of intracranial spread was documented both by imaging and pathology. Extracranial spread was also found at autopsy. This unusual presentation and widespread dissemination of the neuroectodermal tumor in our case confirms its aggressiveness and potential for multifocality.

Acute Disease↗