Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Neuroectodermal Tumors, Primitive”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 235 records · Page 13Linked to original sources
PubMedDate not supplied

Cerebral primitive neuroectodermal tumor in an adult male. A case report.

BACKGROUND: Primitive neuroectodermal tumors (PNETs) are very rare. Malignant tumors of the cerebrum in young individuals are composed predominantly of undifferentiated cells, with moderate differentiation along either neuronal or glial lines. To our knowledge, cerebral PNETs in adults are extraordinarily rare and have been reported in only 11 cases, with little cytologic documentation in the literature. The cytopathologic, immunohistochemical and ultrastructural features of cerebral PNET arising in an adult male are presented. CASE: A cystic tumor, on computed tomography and magnetic resonance imaging, arose from the left frontal lobe in a 39-year-old man and contained histopathologic features of PNET. Specimens obtained from surgery revealed the presence of an undifferentiated type of PNET with moderate neuronal and glial differentiation and mild characteristic findings of peripheral PNET. The cytologic and histologic specimens showed evidence of a scattered pattern of blastic and undifferentiated tumor cells and a neural arrangement with Homer-Wright-like rosettes. Immunohistochemically, the tumor cells were glial fibrillary acidic protein, neuron-specific enolase, synaptophysin and CD-99 positive and epithelial membrane antigen, S-100 protein and vimentin negative. Ultrastructurally, neither microtubular structures nor intermediate filaments, except neurosecretory granules, were found in the tumor cells. CONCLUSION: Both immunohistochemical and ultrastructural studies on cytologic and histologic slides were important for the diagnosis of PNET because of establishing not only undifferentiated tumor cells but also neural and glial differentiation.

Adult↗
PubMed2005

Intracranial peripheral primitive neuroectodermal tumors of the cavernous sinus: a diagnostic peculiarity.

Peripheral primitive neuroectodermal tumors (pPNETs) are aggressive, poorly differentiated neoplasms that occur in children and young adults. These tumors are associated with a peak incidence in the second decade and a slight male preponderance. Recently, Ewing sarcoma and pPNET tumors have been proven to carry identical translocations, the most common being t(11;22)(q24;q12). Intracranial Ewing sarcoma/pPNETs have rarely been described in the literature. We studied a case of intracranial pPNET arising in the right cavernous sinus of a 46-year-old man. On imaging, the tumor had both sellar and suprasellar components and was centered within the right parasellar region. Histologically, the tumor was composed of intermediate to large cells with round to oval hyperchromatic nuclei with distinct nucleoli. The cells contained a moderate amount of slightly basophilic cytoplasm. The tumor was markedly fibrotic and had collagen bands surrounding both individual and groups of cells. A large immunohistochemical panel was positive only for CD99 and vimentin. Fluorescence in situ hybridization did not show translocations associated with Ewing sarcoma/pPNET. However, a small percentage of these tumors can be negative for this translocation. In these cases, histology and immunohistochemical techniques in the absence of an alternative diagnosis are the only tools available to establish the diagnosis.

Brain Neoplasms↗
PubMed2001

Intense p53 staining is a valuable prognostic indicator for poor prognosis in medulloblastoma/central nervous system primitive neuroectodermal tumors.

UNLABELLED: Intense p53 immunostaining may predict for a poor prognosis in central nervous system primitive neuroectodermal tumor of childhood. BACKGROUND: Medulloblastoma is a common childhood primary brain tumor. Potential prognostic indicators for patients with local disease are age, extent of resection, and gender. However, none of these are well established. Immunohistologic staining is a potentially useful means to identify high-risk patients. The purpose of this clinical pathologic study was to investigate the prognostic significance of GFAP, synaptophysin, Ki-67, and p53 immunostaining in medulloblastoma/central nervous system primitive neuroectodermal tumors (CNS PNETs.) MATERIALS AND METHODS: The records of 40 patients with CNS PNETs were reviewed. Their surgical specimens were immunostained for p53, glial fibrillary acidic protein (GFAP), synaptophysin, and Ki-67. The p53 specimens were scored blindly for the intensity of staining of nuclei (intense vs weak) and the quantity of cells stained. The Ki-67, GFAP, and synaptophysin specimens were analyzed for quantity of cells stained. RESULTS: Ten patients' specimens stained intensely for the p53 protein. Eleven had weakly staining nuclei. Nineteen specimens had no staining. The patients with specimens that stained intensely had a statistically significant decreased disease free survival (P = 0.03). Mere presence or quantity of p53 nuclear staining did not correlate with disease free survival. Immunohistochemical staining for Ki-67, GFAP, and synaptophysin did not correlate with disease free survival. Clinical parameters of age, gender, and extent of resection also did not approach statistical significance for disease free survival. CONCLUSION: Intense nuclear staining for p53 was the only variable in this clinical pathologic study that reached statistical significance for disease free survival. This suggests that intense staining for p53 may be the most important prognostic indicator for non-metastatic CNS PNETs. p53 Immunostaining with antibodies against p53 in CNS PNETs should be studied in a multi-institutional setting with larger numbers of patients.

Adolescent↗
PubMed1995

Dynamics of cell proliferation and cell death during the emergence of primitive neuroectodermal tumors of the immature central nervous system in transgenic mice.

Cell proliferation and cell death play critical roles in embryonic development, postnatal tissue maintenance, and tumor formation. To understand the interplay between cell proliferation and death in tumor formation, we studied these two processes in nascent primitive neuroectodermal tumors that arose postnatally from neuroepithelial cells ventral to the median eminence of transgenic mice (designated rTH-Tag mice) carrying a Simian virus 40 large T antigen transgene driven by a rat tyrosine hydroxylase promoter. Cell proliferation continued in the neuroepithelium of the ventral median eminence in wild-type and transgenic animals for the first 2 weeks of postnatal life but subsided completely in the wild-type mice after 2 weeks of age. In contrast, mitotic activity persisted in these progenitor cells of the rTH-Tag mice, and there was a dramatic increase in mitotic activity after 10 weeks leading to the formation of primitive neuroectodermal tumors despite sustained cell death activity. We conclude that primitive neuroectodermal tumors originate from progenitor cells in the ventral median eminence of rTH-Tag mice in early postnatal life when progenitors fail to respond to signals to exit the cell cycle. Thus, the disruption of mechanisms that regulate cell proliferation and cell death in the developing brain may underlie the emergence of primitive neuroectodermal tumors in the rTH-Tag mice.

Animals↗
PubMed1985

Primitive neuroectodermal tumor: a newly recognized cause of early fetal death.

Reviews in the literature of fetal and neonatal neoplasms deal only with congenital tumors, that is, tumors presenting at or shortly after birth. Virtually nothing is known about neoplasms occurring in the embryonal period or early fetal life. We report five small macerated fetuses with a very primitive embryonal tumor of neuroectodermal origin. The tumor characteristically showed massive vascular permeation. Expulsion of the fetus occurred during the fourth or fifth month of pregnancy. It is suggested that this primitive neuroectodermal tumor is a "new" cause of spontaneous late abortion.

Blood Vessels↗
PubMed2000

Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy.

BACKGROUND: The outcome of a child with a primitive neuroectodermal tumors arising supratentorially (SPNET) is not well characterized and may differ from the outcome of a patient with a histologically similar cerebellar tumor (medulloblastoma [MB]). Recently, 5-year progression free survival rates as high as 80% have been reported for children with MB treated with craniospinal radiation (CRT) and chemotherapy including cisplatin, lomustine (CCNU), and vincristine (VCR). METHODS: The authors reviewed the outcome of 22 consecutive patients age 3 years and older (mean age, 10 years; range, 3-18 years) with SPNET who were treated at the study institutions between 1981 and 1996. Tumor location included was 13 pineal, 6 cortical, and 3 thalamic or suprasellar. Five patients had disease dissemination at diagnosis. All patients underwent surgery and staging, followed by CRT and chemotherapy with cisplatin, CCNU, and VCR. RESULTS: Of the 22 patients, 13 had developed disease progression and 10 had died at the time of last follow-up. Overall progression free survival (PFS) was 47% +/- 11% at 3 years and 37% +/- 11% at 5 years. There was a significant difference in PFS between patients with localized disease versus those with disseminated disease (P = 0.04). There was no statistical association between tumor location and survival. Although not significant (P = 0.21), there was a trend toward better survival of those patients with complete or near-complete resection compared with those with partial resection or biopsy. CONCLUSIONS: The results of the current study demonstrate that the outcome for children with SPNET treated with radiation and chemotherapy appears worse than for children with MB treated with identical therapy. This suggests that there may be biologic differences between supratentorial and infratentorial primitive neuroectodermal tumors, thus requiring refinements in treatment.

Adolescent↗
PubMed2000

Peripheral primitive neuroectodermal tumor of the small bowel mesentery: a case showing perforation at onset.

A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with an uncommon clinical onset is reported. A 40-year-old man was admitted to hospital because of acute severe abdominal pain. Chest X-ray revealed a free air sign beneath the diaphragm. At emergency surgery a mass measuring 11.0 x 8.0 cm with perforation was located in the jejunal mesenteric region. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming abortive Homer Wright rosettes. Some spindle-shaped cells showed perivascular pseudorosettes. Immunohistochemical study revealed that the tumor cells expressed positivity against CD99 (MIC2), neuron-specific enolase, synaptophysin and vimentin. To the authors' knowledge this is the first documentation of peripheral primitive neuroectodermal tumor of the small bowel mesentery with perforation at onset.

Abdomen, Acute↗
PubMed2006

Primary intramedullary primitive neuroectodermal tumor (PNET)--case report and review of the literature.

Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.

Adolescent↗
PubMed1998

[Primary primitive neuroectodermal tumor of the urinary bladder. Clinicopathologic case report and differential small cell tumor diagnosis of this site].

We report a rare case of primary primitive neuroectodermal tumour of the bladder in an adult. A huge tumour with extensions into pelvic and retroperitoneal tissue was found radiologically in a 62-year-old man. The patient did not complain about remarkable clinical symptoms until 4 days before admission to hospital. Histology of diagnostic transurethral tumour resection showed a small round-cell tumour with focal necrosis and scattered Homer-Wright rosettes. Immunohistochemical analysis revealed that tumour cells stained positively with 013, a monoclonal antibody which recognizes the membrane glycoprotein p30/32MIC2. Focally, tumour cells stained positively for vimentin, NSE, S-100 protein and synaptophysin. The patient died 3 weeks later because of fulminant pulmonary embolism and autopsy revealed a huge, partly exophytic but mainly endophytic tumour of the bladder with extensions into the rectum and retroperitoneal tissue. The differential diagnosis of small round-cell tumours in this location is discussed.

Adult↗
PubMed2002

Primitive neuroectodermal tumor of the cervix: a clinicopathologic and immunohistochemical study of two cases.

Two cases of primary primitive neuroectodermal tumors of the cervix are presented. The two female patients are 35 and 51 years of age who presented with abnormal uterine bleeding of several weeks' duration. On gynecologic examination, a mass in the cervical area was palpated and a biopsy was obtained. The initial biopsy was interpreted as possible small cell carcinoma in both women. A radical hysterectomy was performed in both patients. Grossly, in both cases, the uterine cervix showed an ill-defined tumor involving the ectocervix and endocervix, measuring 3.0 and 4.0 cm in greatest dimension, respectively, and showing areas of necrosis and hemorrhage. Histologic sections showed the presence of a malignant neoplasm arranged in cords and with a vague nesting pattern. Areas of hemorrhage and necrosis were also present. The neoplastic cells were characterized by having indistinct cell borders, small round to oval nuclei, and inconspicuous nucleoli. Mitotic figures were easily identified. In one patient, the tumor had metastasized to lymph nodes. Immunohistochemical studies revealed the neoplastic cells to be positive for antibodies for CD99 and focally for synaptophysin, while keratin, chromogranin, smooth muscle actin, desmin, and neurofilament protein were negative. Both patients received adjuvant chemotherapy and remain alive 5 and 18 months after initial diagnosis, respectively. The present cases highlight the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix.

Adult↗
PubMed1996

Primitive neuroectodermal tumor of the myocardium: a case report, review of the literature, immunohistochemical, and ultrastructural study.

We report the case of a primitive neuroectodermal tumor (PNET) arising in the heart of a 63-year-old man. The neuroectodermal nature of this tumor was confirmed by the immunohistochemical positivity for O13 (CD99) (the P30/32MIC2 gene product) neuron specific enolase (monoclonal and polyclonal), synaptophysin and vimentin. Other markers, such as actin, desmin, myoglobin, chromogranin, keratin, and leukocyte common antigen were negative. The diagnosis was made on an endomyocardial biopsy and was confirmed in sections from the myocardial tumor found within the heart excised during cardiac transplant. Primitive neuroectodermal tumors have been reported in a variety of sites, most commonly in the extremities. No case has ever been reported within the myocardium, although one has been reported in the pericardium. In addition to morphological similarities, PNET and extraskeletal Ewing's sarcoma have been shown to possess the same chromosomal translocation, t11;22, and the same cell surface antigen, P 30/32. Separation of this case from extraskeletal Ewing's sarcoma was possible because of the absence of PAS positivity, as well as the immunohistochemical positivity for at least two neural markers, as extraskeletal Ewing's sarcoma is only positive for neuron specific enolase.

Biopsy↗
PubMed1994

The impact of perioperative factors on subsequent intelligence quotient deficits in children treated for medulloblastoma/posterior fossa primitive neuroectodermal tumors.

BACKGROUND: Despite success in treating children with medulloblastoma/posterior fossa primitive neuroectodermal tumor (PF PNET), some children survive with significant neurocognitive sequelae. This study was performed to understand better the significance of perioperative factors on subsequent full scale intelligence quotient (FSIQ) deterioration in these children. METHODS: Twenty-eight children who underwent prospective and serial neurocognitive testing were studied. All children underwent surgery followed by radiotherapy with or without chemotherapy between 1983 and 1987 for medulloblastoma/PF PNET and were disease free when this study was conducted. IQ testing was performed before surgery and after the completion of radiation therapy. The clinical courses of the patients were correlated with changes in the corresponding intelligence quotients of each child. Factors correlating with neurocognitive declines were examined by chi-square or Fisher exact test analysis. Differences in mean IQs were examined by the t test. Factors found to be significant were analyzed by exact logistic regression analysis. RESULTS: The presence of adverse factors such as neurologic deficits, meningitis, or shunt infections, or the need for repeat surgery was correlated significantly with IQ deficits after treatment. Of the subset of children with one or more of these factors, 13 of 16 (81%) sustained decreases in FSIQ; 7 of 16 (43.8%) had decreases of 20 points of more. In contrast, only 3 of 12 (25%) of the children without the factors sustained FSIQ decreases, and no child sustained a decrease of more than 13 points. The mean FSIQ change after treatment in the group with factors was -15.7 (95% confidence interval [CI]: -24.0, -8.4), and the median was -18. The mean FSIQ change in the group without factors was 4.8 (95% CI: -0.5, 10.1), and the median was 5. The difference in mean FSIQ change between the two groups was significant (P < 0.0001). On univariate analysis, both the presence of adverse factors and an age less than six years correlated with neurocognitive deficit. On regression analysis, only the presence of adverse factors was significant (odds ratio 11.53; 95% CI, 1.65-116.58; P = 0.009), whereas age was not (P = 0.27). CONCLUSIONS: Perioperative events or complications may account for some of the neurocognitive deterioration seen in these children after treatment, especially in the very young. The occurrence of these factors is associated with a significantly greater risk of IQ deterioration. Studies of the neurocognitive effects of treatment for children with medulloblastoma/PF PNET should include an analysis of these postoperative factors.

Adolescent↗
PubMed1996

Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant.

Primary spinal cord primitive neuroectodermal tumor (PNET) is a rare entity. In all 13 cases have been reported in the literature, including 3 with intracranial seeding. A 3-month-old girl with involvement of the spinal cord below the mid-thoracic level is described. The brain MRI revealed findings indicative of seeding along the intracranial subarachnoid space. Biopsy, duraplasty and removal of laminotomy flap were done. In spite of a good response to the first cycle of postoperative "8-drugs-in-a-day' chemotherapy, further treatment was refused. She died 21 days after the onset of leg weakness, which reveals the rapid progression of untreated cases. To our knowledge, this is the first case of spinal cord PNET with parenchymal involvement that has been described in an infant.

Fatal Outcome↗
PubMed2006

Primitive neuroectodermal tumor arising in the colon: report of a case.

Peripheral primitive neuroectodermal tumors (pPNETs) are usually found in the soft tissue of the extremities, paravertebral region, and chest wall. We report a rare case of a pPNET arising in the colon. A 59-year-old man underwent left hemicolectomy for an infiltrative ulcerating tumor, 11 cm long, in the descending colon. Histological examination of the resected specimen revealed small, round cell proliferation with rosette-like structures, and confirmed regional lymph node involvement and peritoneal dissemination near the primary tumor. Immunohistochemically, the tumor cells were positive for synaptophysin and MIC2 (CD 99). ESW-FLI1 chimeric mRNA was detected in the tumor by reverse transcriptase-polymerase chain reaction. The patient underwent resection of recurrence in the retroperitoneum 3 months later, but metastasis rapidly developed and he died of the disease 7 months after his first operation.

Base Sequence↗
PubMed1994

Primitive neuroectodermal tumor of the retroperitoneal cavity.

We report a case of primitive neuroectodermal tumor (PNET) arising from the retroperitoneal cavity. Because of infiltration, the tumor was excised en bloc with the left kidney. Despite postoperative adjuvant chemotherapy and local radiation therapy, the tumor recurred and the patient died of the disease 3 months postoperatively. Serum levels of neuron-specific enolase (NSE) correlated with those of lactate dehydrogenase (LDH), and it is suggested that monitoring NSE and LDH is useful in evaluating tumor recurrence. The clinical course and the treatment of this aggressive tumor are reviewed.

Adult↗
PubMed1997

Congenital primitive neuroectodermal tumor of the hand: a case report.

A congenital peripheral primitive neuroectodermal tumor of the hand demonstrating aggressive behavior by rapid growth and ulceration, as well as early diffuse metastasis is presented. Management consisted of below-elbow amputation and chemotherapy. Despite the tumor's initial response, intracranial metastases occurred 7 months later. The patient died shortly thereafter, 15 months after presentation.

Female↗
PubMed2005

Mouth metastasis of peripheral primitive neuroectodermal tumor.

We describe a case of oral metastasis of peripheral primitive neuroectodermal tumor (pPNET) in a 68-year-old man, who presented the primary lesion in the chest. Oral metastasis of pPNETs is very rare and we have not found any similar case reported in the English literature. Clinical examination showed an extensive and ulcerated fleshy mass measuring 3.0 x 3.5 cm in the right lower gingivae. Microscopic examination showed sheets of proliferating small, hyperchromatic, round cells. Tumor cells were reactive to neuron-specific enolase (NSE), vimentin and MIC-2 gene by immunohistochemistry, consistent with PNET. The patient died 3 weeks later because of respiratory insufficiency.

Aged↗
PubMed2007

Primary spinal primitive neuroectodermal tumor: case series and review of the literature.

Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.

Adolescent↗