1/10. choroid plexus papilloma. Report of a case with cytologic differential diagnosis.The cytopathologic features of choroid plexus papilloma observed in the ventricular fluid of a 9-month-old boy are reported and compared with other pediatric central nervous system neoplasms. The cytologic features of choroid plexus papilloma are similar to those of normal choroid plexus and may be difficult to distinguish from those of a well-differentiated papillary ependymoma. However, the cell clusters are distinct from those associated with choroid plexus carcinoma and primitive neuroectodermal tumors.- - - - - - - - - - ranking = 1keywords = neuroectodermal (Clic here for more details about this article) |
2/10. Gastrointestinal autonomic nerve tumours: a case report with ultrastructural and immunohistochemical studies.A case of gastrointestinal autonomic nerve tumour with light microscopic, immunohistochemical and ultrastructural examination is reported. The tumour was composed of spindle cells or large cells with clear cytoplasm and showed intense staining for vimentin and focal staining for neuron-specific enolase, chromogranin, synaptophysin, gastrin, P substance and S-100 protein. Ultrastructural examination showed long processes with dense core granules and the absence of features characteristic of other gastrointestinal stromal tumours. In addition we noted small traces of basal lamina and the absence of synaptic vesicles. It seems that the biological behaviour of gastrointestinal autonomic nerve tumours is aggressive but there are too few reports on which to conclude anything about their prognosis. Our findings suggest that tumour has a neuroectodermal differentiation.- - - - - - - - - - ranking = 1keywords = neuroectodermal (Clic here for more details about this article) |
3/10. Fine needle aspiration cytology of primitive neuroectodermal tumors. A report of these cases.Primitive neuroectodermal tumor (PNET) is a small round cell malignancy arising in soft tissue and bone, predominantly in older children and adolescents. We report the cytomorphologic features and findings of ancillary studies of eight fine needle aspiration (FNA) biopsies from three patients (7-year-old male, 12-year-old female, 9-year-old female). Two of the biopsies suggested the initial diagnosis of PNET of the chest wall, while the remaining six documented recurrent or metastatic disease. In one of these cases the primary diagnosis made by FNA biopsy enabled the pediatric oncologists to give specific therapy for the unresectable tumor and achieve remission. Local recurrences included the chest wall (two cases), pleura (one case) and pericardium (one case), while metastatic disease involved the supraclavicular lymph node and breast. All the cases consisted of small malignant cells with a high nuclear/cytoplasmic ratio and hyperchromatic nuclei without prominent nucleoli. Homer Wright rosettes were seen in only two of the aspirates, and neuropil and ganglion cells were not present. Ancillary studies, including electron microscopy (two cases), immunocytochemistry (four aspirates from two cases) and cytogenetics (11/22 translocation, one case) performed on the aspirated material were aids in making a specific diagnosis and excluded other small round cell tumors of childhood, such as malignant lymphoma, rhabdomyosarcoma and Ewing's sarcoma. The differential diagnosis between PNET and neuroblastoma can be difficult on the basis of an FNA biopsy alone, although light microscopic morphologic differences exist. Clinical features (e.g., age, primary site, metastatic patterns), catecholamine levels, electron microscopy and cytogenetics are necessary in establishing the correct diagnosis.- - - - - - - - - - ranking = 5keywords = neuroectodermal (Clic here for more details about this article) |
4/10. Primitive neuroectodermal tumor: CT, MRI, and angiographic findings.Primitive neuroectodermal tumors (PNETs) are uncommon CNS neoplasms found usually in the first decade of life. This article presents a proven case of this lesion in a 14-month-old boy located deep in the left frontal lobe. This lesion was studied by CT, MRI, and cerebral angiography. The radiologic findings of this tumor were assessed and compared with those cases reported in the medical literature.- - - - - - - - - - ranking = 5keywords = neuroectodermal (Clic here for more details about this article) |
5/10. Primitive neuroectodermal tumors of the uterus: a report of four cases.Four cases of primitive neuroectodermal tumor (PNET) of the uterine corpus are reported, bringing the total number of reported PNETs in this site to seven. The four women were in their seventh decade of life and presented with abnormal vaginal bleeding and, in two cases, an enlarged uterus. The patients underwent total or subtotal abdominal hysterectomy and bilateral salpingo-oophorectomy and, in one patient, pelvic lymphadenectomy. Three patients received postoperative radiation therapy, chemotherapy, or both. Gross examination revealed fleshy polypoid masses filling the endometrial cavity and, in two cases, deeply invading the myometrium. Histologic, immunohistochemical, and, in two cases, ultrastructural examination revealed typical PNETs that exhibited variable degrees of neural, glial, ependymal, and medulloepithelial differentiation. Two PNETs were admixed with other neoplasms: in one case a grade I endometrial adenocarcinoma and in the other a low-grade endometrial stromal sarcoma. The prognosis of the tumors was related to their stage: two patients with stage I tumor were alive with no evidence of disease at 5 and 6 years, whereas two patients with stage III or IV tumor died of tumor at 6 and 12 months. Although it has been suggested that uterine PNETs may be derived from displaced germ cells or implanted fetal tissues, evidence provided by this study, including the advanced ages of the patients and an admixture with neoplasms of unquestioned mullerian origin, suggests a mullerian origin for these tumors in at least some cases.- - - - - - - - - - ranking = 5keywords = neuroectodermal (Clic here for more details about this article) |
6/10. Ultrastructural evidence for neural crest origin of the malanotic neuroectodermal tumor of infancy.A case of malanotic neuroectodermal tumor of infancy, which occurred in the anterior maxilla of a 3-month-old girl, was studied by electron microscopy. Polymorphic cellular elements were revealed to be present: large melanin-producing cells with some of the characteristics of a neuroepithelium, small non-pigmented cells resembling immature neuroblasts, and many intermediate forms resembling differentiating neuroblasts. All cell types clearly demonstrated ultrastructural features of neurogenic cells, e.g. neurite-like cytoplasmic processes, fine filaments and microtubules indistinguishable from those seen in normal neurites, synaptic-like structures, and neurosecretory-like vesicles. Based on these fine structures, the lesion was presumed to represent a hamartoma of primitive neuroectodermal cells with a tendency toward the differentiation into melanin-producing cells. The neural crest origin of this tumor was convincingly supported by ultrastructural analysis.- - - - - - - - - - ranking = 6keywords = neuroectodermal (Clic here for more details about this article) |
7/10. Primitive neuroectodermal tumor of the endometrium: report of two cases, one with electron microscopic observations.Two primary primitive neuroectodermal uterine malignant tumors are reported. One initially had the appearance of neuroblastoma but after radiotherapy showed both glial and ganglionic differentiation. The second neoplasm exhibited medulloepithelial canals, as well as glial, neuroblastic, and focal neuronal differentiation. Both patients died with widespread metastatic disease after both radiotherapy and chemotherapy.- - - - - - - - - - ranking = 5keywords = neuroectodermal (Clic here for more details about this article) |
8/10. Extracranial primitive neuroectodermal tumor.An 11-year-old white girl was admitted to The Children's Hospital of philadelphia in July 1983 for evaluation of metastatic tumor. She had been well until July 1982, when a mass developed over the right scapula. Treatment with warm compresses and antibiotics resulted in no improvement, and incision and drainage were unproductive. A biopsy at another hospital was interpreted as showing a primitive neuroectodermal tumor. There was no evidence of metastatic disease. She then underwent excision of the tumor with the underlying scapula. No further treatment was administered. She remained well until February 1983, when she began to complain of occasional lower back and thigh pains. Her symptoms worsened over the succeeding 3 months, despite treatment with analgesics and physical therapy. By May 1983, she was no longer able to attend school because of weakness and pain, and had sustained a 10% weight loss during the previous 2 months. She was admitted to her original hospital, where bone scan and bone marrow biopsy showed disseminated tumor; she then came to The Children's Hospital of philadelphia. On examination, she appeared acutely and chronically ill. It was very uncomfortable for her to move, and she walked with a slow, stooped, shuffling gait. She complained of tenderness in the lower back and both sides. There were no other abnormalities on examination. The hemoglobin level was 9.7 gm/dl, following transfusion at the other hospital; white blood cell count was 6,900/mm3 with a normal differential, and the platelet count was 480,000/mm3. A 24-hr urine test for VMA excretion was normal. She underwent bone marrow aspiration and biopsy, and the radiographs and pathology slides from the other hospital were reviewed.- - - - - - - - - - ranking = 5keywords = neuroectodermal (Clic here for more details about this article) |
9/10. Spinal metastases. A rare mode of presentation of brain tumors.An analysis of more than 18,000 primary central nervous system (CNS) tumors revealed only 18 cases (0.01%) in which dropped spinal metastases had caused the presenting symptoms. This group included 11 males and 7 females in whom there was no history of surgical intervention or irradiation. Primitive neuroectodermal tumors ( PNET , medulloblastoma), comprised the largest group (11 patients) followed by high-grade astrocytomas (anaplastic and glioblastoma) (5 patients). One case each of germinoma and ependymoma were also identified. The clinicopathologic data of these cases, and a brief review of the literature are presented.- - - - - - - - - - ranking = 1keywords = neuroectodermal (Clic here for more details about this article) |
10/10. Primitive neuroectodermal tumor of the chest wall in a patient with Jeune's syndrome and renal transplant.Jeune's syndrome is a rare autosomal disorder characterized by osseous dysplasia, fetal respiratory distress, and renal failure in later life. We describe a 27-year-old man with Jeune's syndrome who underwent renal transplantation and 6 years later developed a sarcoma (primitive neuroectodermal tumor [PNET]) in the soft tissue of the chest wall, a principal site of dysplasia in this disorder.- - - - - - - - - - ranking = 5keywords = neuroectodermal (Clic here for more details about this article) |
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