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PubMed1998

Identification of CD56 and CD57 by flow cytometry in Ewing's sarcoma or primitive neuroectodermal tumor.

CD56 and CD57 are commonly considered as natural killer and neuroectodermal markers, but their expression has been identified in a wide spectrum of neoplasms including some cases of Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET). We report two cases of small, round blue cell tumor (SRBCT), in which flow cytometry immunophenotyping (FCI) detected strong expression of CD56 and CD57 (one case). Immunohistochemical staining with Leu-19 and Leu-7 confirmed the FI results. Although CD56 and CD57 expression is consistent with ES/PNET, it can be potentially misleading if results of FCI are interpreted in the absence of other findings. These cases suggest the utility of FCI in undifferentiated SRBCT. The literature on CD56 and CD57 expression in ES/PNET is reviewed and discussed.

Adolescent↗
PubMed1998

Peripheral primitive neuroectodermal tumor presenting with diffuse cutaneous involvement and 7;22 translocation.

We report an unusual case of peripheral primitive neuroectodermal tumor (pPNET) in an infant presenting with congenital cutaneous nodules and a t(7;22)(p21;q11.2). The biologic behavior of the tumor diverged over time from a slowly growing tumor with multiple cutaneous nodules to a more aggressive neoplasm characterized by pulmonary metastases and a soft tissue mass showing additional cytogenetic alterations.

Chromosomes, Human, Pair 22↗
PubMed1996

Peripheral primitive neuroectodermal tumors. CT and MRI evaluation.

The clinical, radiographic, and pathologic features of 17 patients with documented peripheral primitive neuroectodermal tumor (PNET) were evaluated in a retrospective study. The age at diagnosis ranged from 9 months to 46 years (median, 15.8 years). Primary sites of involvement were the abdomen (n = 8), extremities (n = 5), chest (n = 1), temporal bone (n = 1), maxilla (n = 1), and diploe (n = 1). At the time of diagnosis, six patients had distant metastases; all of these patients died, with an average survival of 8.8 months. Radiologic workup included standard radiographs, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and bone scintigraphy. The radiographic appearance of these tumors was not specific for differentiation of PNETs from other types of bone and soft tissue tumors. The typical appearance resembled large non-calcified, soft tissue masses with cystic or necrotic areas. Heterogeneous enhancement with intravenous contrast agents was evident on CT, as was an intermediate signal intensity on Tl-weighted images and hyperintense signal on T2-weighted and STIR sequences. After gadolinium administration, variable enhancement was seen. MRI and CT were useful in predicting resectability, in detecting distant metastases, and in the evaluation of response to treatment. Surgery was performed in all cases, either for definitive diagnosis or for therapy. All patients received combined chemotherapy and radiotherapy and five patients received autologous bone marrow transplantation. Clinical follow-up was obtained over a mean period of 3 years (range 1 day to 6 years). Prognosis was poor with a median survival of 3.4 years. Our experience in 17 patients with peripheral neuroectodermal tumors indicates that although their radiologic features are non-specific, they should be included in differential diagnosis of soft tissue tumors of aggressive behavior, especially in a young age group. CT and MRI are useful in delineating the extent and resectability of tumor and in monotoring treatment.

Adolescent↗
PubMed1995

Primary cutaneous Ewing's sarcoma/peripheral primitive neuroectodermal tumors in childhood. A molecular, cytogenetic, and immunohistochemical study.

Childhood cutaneous and subcutaneous malignancies are rare and include metastatic tumors of diverse histogenesis as well as primary lesions, such as sweat gland carcinomas. Some cutaneous malignancies exhibit a small round cell tumor morphology with few definitive differentiating features; they can thus pose a significant diagnostic problem. We describe two primary malignancies of the skin and superficial subcutis, which were originally diagnosed as sweat gland carcinomas on the basis of their morphological features. A cytogenetic analysis performed on one of these lesions showed the t(11;22)(q24;q12) rearrangement, believed to be unique to the Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) group of neoplasms. In view of this unexpected result, reverse transcriptase-polymerase chain reaction analysis was performed on both lesions and showed that they expressed EWS/FLI-1 fusion gene mRNA transcripts, the molecular equivalent of t(11;22)(q24;q12). The two tumors also had an immunohistochemical profile suggesting ES/pPNET, including strong expression of the MIC2 antigen. Both patients were treated with wide local excision, and one was given a course of chemotherapy. Neither patient showed evidence of tumor elsewhere after follow-up periods of 2 years and 16 years. These findings suggest that these tumors are indeed a form of primary ES/pPNET arising in the skin or superficial subcutis, which may be of low-grade malignancy and curable by local surgery.

Base Sequence↗
PubMed2006

Primitive neuroectodermal tumor of the adrenal gland.

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.

12E7 Antigen↗
PubMed1995

Primitive neuroectodermal tumor (PNET). A case report.

The authors present a case of relatively rare tumor of the central nervous system (CNS) in a 19-year-old female, who died 18 months after the first manifestation of meningismus, increased intracranial pressure and secondary hydrocephalus. Brain autopsy revealed abundant neoplastic infiltrations, which spread through the subarachnoid space. Neoplastic infiltrations were also present in the third ventricle and in a form of small subependymal nodules along the whole ventricular system. The microscopical examination showed that neoplasm consisted of small cells, which formed neuroblastic Homer Wright rosettes. Immunohistochemical studies (for synaptophysin, chromogranin A, GFAP, vimentin) together with morphology and localization of neoplasm suggested diagnosis of primitive neuroectodermal tumor (PNET) that spread mainly in the leptomeninges and caused obliteration of subarachnoid space.

Adult↗
PubMed1992

Desmin positivity in primitive neuroectodermal tumors of childhood.

In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.

Abdominal Neoplasms↗
PubMed1997

Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

PURPOSE: To determine the feasibility of detecting Ewing's sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS: Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS: Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION: In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.

Chromosomes, Human, Pair 11↗
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↗
PubMed1990

Congenital primitive neuroectodermal tumor with epithelial and glial differentiation. An ultrastructural and immunohistochemical study.

A congenital primitive neuroectodermal tumor associated with epithelial and glial elements is described. This soft-tissue tumor present on the right temple of a newborn boy consisted mainly of small round cells of the primitive neuroepithelial type, occasionally forming rosettes. The other components were focal glandular structures producing mucin, and aggregates of epithelioid cells bearing clear cytoplasm, both being distributed throughout the entire tumor. Additional glandular and clear cell components were strongly positive for various epithelial markers, such as carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin. Epithelioid cells were also positive for neuron-specific enolase and S100 protein. Glial differentiation was evidenced in some of the epithelioid cells by localization of cytoplasmic glial fibrillary acidic protein. These findings suggest that this tumor derives from a remnant of a neural crest, and the possibility of a special type of peripheral primitive neuroectodermal tumor is considered.

Epithelium↗
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↗
PubMed2003

Primitive neuroectodermal tumors, embryonal tumors, and other small cell and poorly differentiated malignant neoplasms of the central and peripheral nervous systems.

Many different types of small cell, embryonal, and poorly differentiated neoplasms originate within the central and peripheral nervous systems. Because appropriate treatment is based on a correct diagnosis, the surgical pathologist must be familiar both with basic characteristics of each of the numerous entities as well as the spectrum of morphologic features that each may display. The nosology and nomenclature of these tumors have a rich and varied history. One basic distinction is between primitive neuroectodermal tumors of the central nervous system (cPNETs) and primitive neuroectodermal tumors of the peripheral nervous system (pPNETs), which are clinicopathologically and genetically distinct. Among the cPNETs are medulloblastoma, pineoblastoma, cerebral neuroblastoma, ependymoblastoma, medulloepithelioma, primary rhabdomyosarcoma, and atypical teratoid/rhabdoid tumor, whereas the pPNETs comprise the more differentiated end of a spectrum of neoplasms that include skeletal and extraskeletal Ewing's sarcoma.

Central Nervous System Neoplasms↗
PubMed1995

Atypical cytogenic aberrations in two childhood peripheral primitive neuroectodermal tumors.

Atypical cytogenetic abnormalities were detected in peripheral primitive neuroectodermal tumors (PPNET) of the extremity in two children. One had an osseous tumor with a balanced reciprocal translocation, t(5;9)(q22;q32), and had a complete response to therapy. The other had a non-osseous tumor with an interstitial deletion, del(18)(q12.2q21.2), was resistant to combination therapy, and at autopsy had evidence of possible clonal evolution with the karyotype 46,XX der(8)t(8;8)(p11.2;q13), inv(16)(p13.2q12),del(18)(q12.2q21.2). Neither tumor demonstrated the t(11;22)(q24;q12) typically found in Ewing's sarcoma and PPNET, suggesting heterogeneity of the cytogenetic aberrations seen in this rare childhood malignancy.

Bone Neoplasms↗
PubMed2005

Intracranial peripheral primitive neuroectodermal tumor manifesting as abducens nerve palsy.

INTRODUCTION: Peripheral primitive neuroectodermal tumor (PNET) occurring as an extra-axial lesion within the intracranial space and extending to the subarachnoid space is extremely rare. CASE REPORT: An 18-month-old girl presented with an intracranial peripheral PNET manifesting as abducens nerve palsy. Magnetic resonance imaging on admission revealed a lesion affecting the trigeminal and abducens nerves. The tumor was partially removed via the subtemporal approach. Histological examination showed a high-grade, undifferentiated neoplasm of small cell type with positive immunostaining for MIC2. The histological diagnosis was peripheral PNET. OUTCOME: Craniospinal radiotherapy reduced the tumor size, but adjuvant chemotherapy designed for Ewing's sarcomas and PNETs was not effective. She died 1 month after the last chemotherapy, despite whole craniospinal irradiation (total dose 53.2 Gy) and chemotherapy.

Abducens Nerve↗
PubMed2003

Primary primitive neuroectodermal tumor (PNET) of the kidney with venous thrombus.

Primitive neuroectodermal tumors of the kidney are rare, the diagnosis usually being made at histopathology. A young adult presented with a painful left renal mass. CT Scan of the abdomen revealed a large necrotic tumor of the left kidney. At surgery the patient was found to have a venous thrombus confined to the renal vein. Radical nephrectomy was done. Histopathology showed a round cell neoplasm with typical Homer Wright rosette formation and positive staining for neuron specific enolase (NSE) and MIC-2 on immunohistochemistry. The patient is undergoing multidrug chemotherapy and is alive and well at a follow up of nine months.

Adult↗
PubMed1998

Placental metastasis from maternal primitive neuroectodermal tumor.

The occurrence of placental metastasis from maternal primitive neuroectodermal tumor (PNET) is extremely rare. A 33-year-old woman at 33 weeks' gestation was admitted to the hospital. She died of the disease on hospital day 28 because the unresectable tumor was located over a wide area of the retroperitoneum. A neonate was delivered by a caesarean section without evidence of disease and has survived to 1 year of age. Pathologically, the tumor consisted of monotonous small round cells with a high nuclear cytoplasmic ratio, including several pseudorosettes and Homer-Wright-type rosettes. On immunohistochemical examination, the tumor cells were positively stained for neuron-specific enolase. Microscopically, the placenta showed intervillous metastasis from the maternal PNET. This case may be the first one of placental metastasis from PNET ever reported in the literature.

Adult↗
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↗
PubMed2005

Long-term survival of high-risk pediatric patients with primitive neuroectodermal tumors treated with antineoplastons A10 and AS2-1.

Primitive neuroectodermal tumors (PNETs) are usually successfully treated with craniospinal radiation and chemotherapy; however, difficulties with standard treatment can be encountered in very young children, in adult patients at high risk of complication from standard treatment, and in patients with recurrent tumors. Thirteen children, either with recurrent disease or high risk, were treated in phase II studies with antineoplastons (ANP). The median age of patients was 5 years, 7 months (range, 1-11). Medulloblastoma was diagnosed in 8 patients, pineoblastoma in 3 patients, and other PNET in 2 patients. Previous treatments included surgery in 12 patients (1 had biopsy only, suboccipital craniotomy), chemotherapy in 6 patients, and radiation therapy in 6 patients. Six patients had not received prior chemotherapy or radiation. The treatment consisted of intravenous infusions of 2 formulations of ANP, A10 and AS2-1, and was administered for an average of 20 months. The average dosage of A10 was 10.3 g/kg/d and of AS2-1 was 0.38 g/kg/d. Complete response was accomplished in 23%, partial response in 8%, stable disease in 31%, and progressive disease in 38% of cases. Six patients (46%) survived more than 5 years from initiation of ANP; 5 were not treated earlier with radiation therapy or chemotherapy. The serious side effects included single occurrences of fever, granulocytopenia, and anemia. The study is ongoing and accruing additional patients. The percentage of patients' response is lower than for standard treatment of favorable PNET, but long-term survival in poor-risk cases and reduced toxicity makes ANP promising for very young children, patients at high risk of complication of standard therapy, and patients with recurrent tumors.

Antineoplastic Agents↗