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PubMedDate not supplied

Primitive neuroectodermal tumor of the kidney. A report of two cases diagnosed by fine needle aspiration cytology.

BACKGROUND: Primitive neurocetodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectrodermal origin most often presenting as bone or soft tissue masses. There are very few reported cases of PNET of the kidney and none diagnosed by fine needle aspiration cytology (FNAC), to the best of our knowledge, in the world literature. We present two cases of renal PNET diagnosed on cytology. CASES: Two patients with renal masses were diagnosed as having PNET on FNAC. Cytologically the tumors showed a dispersed population of malignant small round cells with focal rosette formation and perivascular arrangement of tumor cells. Immunohistochemistry on the cell blocks in both cases showed strong membrane positivity for CD99 (MIC2). Cytogenetic studies in both cases showed the characteristic t(11;22)(q24;q12) translocation, with additional chromosomal abnormalities in case 2. CONCLUSION: PNET of the kidney is a distinct entity and can be diagnosed on fine needle aspiration smears and confirmed with immunohistochemistry and cytogenetic studies. A diagnosis of PNET must be included in the differential diagnosis of renal masses in adolescents and young adults.

12E7 Antigen↗
PubMedDate not supplied

[Peripheral primitive neuroectodermal tumors of the soft tissues and bones].

Large group of small-round-cell tumours of soft tissues and bone represents a complex diagnostic problem for the pathologists. Neuronal nature of many tumours from this group is proven by means of new methods--immunophenotypic analysis, tissue culture, cytogenetics. Peripheral neuroepithelioma, Ewing tumour, primitive neuroectodermal tumour (PNET), Askin tumour belong to these neoplasms. These tumours anatomically have no connection with the structures of the central nervous system or autonomous sympathetic nervous system.

Adolescent↗
PubMedDate not supplied

Radiologic characteristics of primitive neuroectodermal tumors in children.

Radiographic findings are reviewed in 31 children with primitive neural ectodermal tumors seen at the Hospital for Sick Children from 1962 through 1983. Seventeen children were initially evaluated with computed tomography (CT). Ten of these had both CT studies and angiography. The tumors were large, irregular, typically iso- to hyperdense, and showed dense, heterogeneous contrast enhancement. Cysts were present in 65% and calcifications in 71% of cases. The angiographic findings were nonspecific, ranging from avascular to markedly vascular. Although these tumors were usually found in the cerebral hemisphere, particularly the frontal lobes, two cases are included of tumors arising elsewhere: one in the pineal and one in the suprasellar region. Epidemiologic data are reviewed.

Adolescent↗
PubMed2000

[Cardiac manifestations of peripheral primitive neuroectodermal tumor (pPNET): a rare case].

HISTORY: A 44-year-old man presented to his general practitioner with increasing exertional dyspnoea. After a syncope he was admitted to hospital. INVESTIGATIONS: Echocardiography on admission demonstrated a large pericardial effusion (45 mm over the right and 57 mm over the left ventricle) which, in view of its haemodynamic relevance, had to be removed by pericardiocentesis. Cytological examination of the fluid showed various blood constituents but no atypical cells. Computed tomography (CT) of the thorax revealed a homogeneous hyperdense structure in the area of the left atrium. After several more pericardiocenteses echocardiography showed a pericardial tumour between the left atrium and ventricle. Gastroscopy, coloscopy, abdominal CT, an octreotide scan and positron-emission tomography did not indicate an extracardiac tumour. Coronary angiography and video-assisted thoracoscopy were performed. They confirmed an epicardial tumour at the level of the left atrium. TREATMENT AND COURSE: The entire round and solid tumour of about 3 cm in diameter, at the tip of the left atrial appendage was removed under extracorporeal circulation. No adjuvant treatment was given. Histological and immunohistological tests of the resected specimen (CD-99 and NSE-positive) provided the diagnosis of malignant peripheral primitive neuroectodermal tumour (pPNET). CONCLUSION: There are at present no adequate data on optimal treatment of pPNET in the heart. This case demonstrates that even with a large such tumour long remission is possible after complete removal without any adjuvant treatment.

Adolescent↗
PubMed1998

[Two children with unusual causes of torticollis: primitive neuroectodermal tumor and Grisel's syndrome].

Two children were presented with a torticollis. One, a 2-year-old girl, had a primitive neuroectodermal tumour of the cervical spinal cord which could not be radically removed surgically (the patient died three days later), the other, a 9-year-old boy, had the syndrome of Grisel. He was treated with antibiotics, head traction and atlantoaxial posterior spondylodesis. Acquired torticollis is a frequent innocent symptom in children with cervical lymphadenopathy following a respiratory tract infection. These cases demonstrate that additional investigation should not be delayed if relief of symptoms is not achieved very soon.

Atlanto-Axial Joint↗
PubMed1991

Immunohistochemical characterization of primitive neuroectodermal tumors and their possible relationship to the stepwise ontogenetic development of the central nervous system. 1. Ontogenetic studies.

Aim of the present study was to establish different immunohistochemical staining patterns for a subsequent comparison with those of primitive neuroectodermal (PNET) subsets, i.e. PNET-NOS (not otherwise specified) or PNET with focal neuronal, astrocytic or ependymal differentiation, to relate neoplastic to embryonal development. Tissue of the developing central nervous system, with special emphasis on the stepwise development of the rhombencephalon, the cerebellar and the retinal anlage, from 20 different human embryos and fetuses ranging from 3 to 30 weeks of gestational age (GA) was examined. Six neuronal markers, synaptophysin, chromogranin A, neuron-specific enolase (NSE), neurofilament protein (NFP; 160 kDa, 200 kDa, 70 and 200 kDa) and six other markers, glial fibrillary acidic protein (GFAP), S-100 protein, vimentin, myoglobin, desmin, cytokeratin, were assessed immunohistochemically. GFAP and S-100 protein appeared at the 6th week of GA in primitive glial cells of the cerebellar anlage, brain stem, rhombencephalon, and developing spinal cord, together with--as first neuronal marker--chromogranin A, then NFP (70 and 200 kDa, and 160 kDa) from the 8th week onward. NSE started in the 11th week and synaptophysin not earlier than the 16th week of GA. Interestingly, the differentiation of the retinal anlage started rather late with NSE positivity beginning from the 16th week and positive reactions to synaptophysin and NFPs only from the 25th and chromogranin A from the 28th week of GA onward.

Central Nervous System↗
PubMed1982

Primitive neuroectodermal tumor arising in the ulnar nerve. A case report.

Malignant peripheral nerve tumors are rare. A neuroectodermal tumor arose in a 59-year-old man from the ulnar nerve. Typically, the tumor reproduces some of the appearances of cells of the primitive central nervous system. The possibility that a primary tumor of the sympathetic nervous system, especially of the adrenal glands, should be considered in the differential diagnosis. The tumor may arise from a major peripheral nerve and show features of growth patterns of the embryonic central nervous system. Blood stream spread is not uncommon; awareness of this proclivity makes early diagnosis mandatory.

Humans↗
PubMed2004

Expression of SV40 T antigen gene in the oligodendroglia induced primitive neuroectodermal tumor-like tumors in the mouse brain.

Primitive neuroectodermal tumors (PNET) are classified as the embryonal tumors developed in the brain, except for the cerebellum. Although many studies have been reported, the origin and pathogenesis of PNET are still unclear. In this study, we observed the development of undifferentiated tumors indistinguishable from PNET in the transgenic mice which expressed simian virus 40 T antigen (SV40-Tag) selectively in the oligodendroglia under the control of mouse myelin basic protein gene promoter. These PNET-like tumors reproducibly developed in the brain stem of the founder mice and the transgenic progeny derived from one founder mouse. Oligodendroglia-specific expression of SV40-Tag in these transgenic mice was observed by immunohistochemical analysis. Furthermore, expression of the oligodendroglia-specific marker genes was decreased in the tumors as well as in the transgenic brains. These findings suggested that tumors developed in transgenic mice were indistinguishable from PNET, and one of them showed oligodendroglia-like characteristics. Consequently, this transgenic line is a useful animal model to study the pathogenesis of undifferentiated tumor.

Animals↗
PubMed1989

[Primitive neuroectodermal tumor with peritoneal metastasis through a ventriculoperitoneal shunt. Case report].

A 13-month-old boy admitted with lethargy and hydrocephalus was found to have a right thalamic mass. Ventricular drainage was instituted, and the tumor mass was reduced by partial resection and local irradiation. A ventriculoperitoneal shunt was then placed. However, the tumor recurred 16 months later, with extensive ventricular seeding and peritoneal metastasis through the shunt tube. The child died 22 months after onset. Histological study of surgical specimens of the primary tumor and autopsy specimens of the brain and peritoneal metastatic tumors revealed poorly differentiated, small, round cells with numerous mitotic figures. In addition, autopsy specimens of the brain tumor contained areas of ependymal, oligodendroblastic, and spongioblastic differentiation. On immunohistochemical study, the tumor cells of each specimen were positive for anti-neuron specific enolase and anti-neurofilament antibodies, but negative for anti-glial fibrillary acidic protein antibodies. Electron microscopy revealed some zonulae adherens. These findings strongly suggest that the tumor originated from primitive multipotential cells capable of differentiating into ependymal, glial, and neuronal lines.

Brain Neoplasms↗
PubMed1990

Extracranial primitive neuroectodermal tumor of the head and neck.

Malignant peripheral neuroectodermal tumors are rare neoplasms of the head and neck. We describe a case of a teenage girl presenting with this neoplasm in the head and neck. The clinical histological and ultrastructural features are reviewed and presented, along with guidelines for the management of this highly aggressive neoplasm.

Adolescent↗
PubMed1985

A classification system for primitive neuroectodermal tumors (medulloblastoma) of the posterior fossa.

Tumors of the posterior fossa are described. Although the preoperative staging system of Chang and associates has proven prognostic relevance, a proposed new system may have even greater impact on prognosis because it considers the tumor burden postoperatively. This system (MAPS: metastasis, age, pathology, and surgery) incorporates age and pathologic characteristics into the staging system. The value of this proposed staging system, in particular, the impact of pathology, must be assessed in controlled trials.

Age Factors↗
PubMed2005

Atypical teratoid/rhabdoid tumor of the brain: cytopathologic characteristics and differential diagnosis.

BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive neoplasm with a unique cytogenetic profile. Although the clinicopathologic and radiologic features of AT/RT have been described previously, to the authors' knowledge the cytomorphologic profile of this tumor has not been studied well. METHODS: Nine samples of AT/RT from 8 patients were analyzed from the pathology files of 2 large institutions in a 10-year period (1993-2002). Material consisted of slides made from scraping and smearing (SS) or squash preparation (SP) of the tissue cores (six slides), fine-needle aspiration (FNA) (two slides), and cerebrospinal fluid (one slide). Smears were stained with Diff-Quik, Papanicolaou, and hematoxylin and eosin stains. RESULTS: There were 4 males and 4 females who ranged in age from 1-16 years (mean age, 7.1 years). Cytomorphologic features consisted of hypercellularity (eight of eight tumors); predominantly large tissue fragments with tumor cells surrounding proliferating capillaries depicting a "papillary-like" appearance (five of eight tumors); large, round, "plasmacytoid" cells and characteristic "rhabdoid" cells (i.e., intermediate-sized cells with granular to fibrillary, brightly eosinophilic cytoplasm with or without globoid "inclusions"; large, eccentrically located, round-to-reniform nuclei with single prominent nucleoli; eight of eight tumors); small, round, primitive "neuronal-appearing" cells with a high nuclear to cytoplasmic ratio (five of eight patients); and bizarre, multinucleated giant cells (two of eight tumors). Also seen were numerous apoptotic bodies, mitoses, and significant necrosis (seven of eight tumors), and prominent dystrophic calcification (four of eight tumors). CONCLUSIONS: AT/RT is extremely rare. Cytologic examination by SS, SP, or FNA offers a useful alternative to frozen section during intraoperative consultation. Cytomorphologic features are unique and lead to an accurate diagnosis in the right clinicoradiologic context. The differential diagnosis includes medulloblastoma (in cerebellar tumors), primitive neuroectodermal tumor (in suprasellar tumors), choroid plexus carcinoma, and malignant glioma.

Adolescent↗