1/15. Intracranial Ewing sarcoma/'peripheral' primitive neuroectodermal tumor of dural origin with molecular genetic confirmation.Ewing sarcoma/'peripheral' primitive neuroectodermal tumor (ES/pPNET) is the designation given to a family of small cell neoplasms that typically arise in bone or soft tissue and are unified by their common expression of the MIC2 antigen and specific translocations involving a gene on chromosome 22q12 [the most common being t(11;22)(q24;q12)]. ES/pPNET of intracranial origin is extraordinary. We report the case of a 6-year-old boy with a large left frontal region mass that adhered to dura and was extracerebral at surgery. Histologic study revealed a high-grade, undifferentiated-appearing neoplasm of small cell type that was negative on immunostudy for glial fibrillary acidic protein, synaptophysin, desmin, leukocyte common antigen, smooth muscle actin and epithelial membrane antigen, but positive for vimentin and neuron-specific enolase and diffusely labeled by antibody O13 (which recognizes the MIC2 gene product). rna-based polymerase chain reaction assay confirmed the diagnosis of ES/pPNET by demonstrating fusion transcripts indicative of t(11;22) translocation. Bone scan, computerized tomography of the chest and bone marrow examination revealed no systemic tumor. The limited observations published to date suggest that primary intracranial ES/pPNET is most likely to present in childhood as a circumscribed, contrast-enhancing and dural-based extracerebral mass. It must be distinguished from a variety of small cell neoplasms, particularly PNETs of central neuroepithelial origin.- - - - - - - - - - ranking = 1keywords = body (Clic here for more details about this article) |
2/15. Primitive neuroectodermal tumor of the uterus. A case report.BACKGROUND: Primitive neuroectodermal tumors (PNETs) of the uterus are very rare. Even preoperative curettage specimen morphology does not permit establishing a proper histogenetic diagnosis. CASE: A 16-year-old woman was admitted to the hospital because of metrorrhagia of three months' duration. The curettage specimen was interpreted as poorly differentiated sarcoma. The patient was operated on, and a total abdominal hysterectomy with bilateral salpingo-oophorectomy with omentectomy was performed. Histologically the tumor was composed of uniform, rounded, oval and sometimes spindle shaped cells with a narrow rim of eosinophilic cytoplasm. The cells were positive for neurogenic marker protein gene product, neuron-specific enolase and Ewing's sarcoma-related HBA-71. All cells were uniformly negative for Ber EP4 monoclonal antibody recognizing an epithelium-specific surface antigen. A diagnosis of PNET was rendered. The patient received combined therapy, external radiation to the pelvis and chemotherapy. Four years later she was alive, without signs of recurrent tumor. CONCLUSION: In spite of the generally recognized aggressive behavior of PNET, it can have a long disease-free survival rate when appropriately treated.- - - - - - - - - - ranking = 1keywords = body (Clic here for more details about this article) |
3/15. Peripheral primitive neuroectodermal tumor presenting in the retroperitoneum: a case report with immunohistochemical study.It is now believed that malignant peripheral primitive neuroectodermal tumor (PNET) form a distinct tumor entity from other malignant small round cell tumors exhibiting neuroectodermal differentiation by morphologic, immunohistochemical or electron microscopic analyses. A 17-year-old Ethiopian boy was found to have a big upper extra osseous retroperitoneal tumor mass not associated with peripheral nerve that had infiltrated the body of the pancreas. Histologic sections from excised biopsy showed neoplastic cells with a high nuclear-cytoplasmic ratio and had an indistinct cytoplasm with numerous Homer-Wright rosettes. Immunohistochemically, isolated tumor cells and the centre of rosettes disclosed strong positivity to neural markers, synaptophysin and chromogranin. To the best of our knowledge, this case report represents the first patient described in ethiopia.- - - - - - - - - - ranking = 1keywords = body (Clic here for more details about this article) |
4/15. retinoblastoma, microphthalmia, coloboma, and neuroepithelioma of the pineal body.A one-month-old infant boy was examined early in life because his mother had bilateral retinoblastoma and his father had bilateral microphthalmia. The ophthalmologist found his right eye was normal size with a coloboma of the iris, choroid, and retina. The left eye was microphthalmic with a coloboma of the uveal tract and retina. A vascularized fluffy white mass in the posterior pole was diagnosed clinically as a retinoblastoma. The tumor regressed with radiation. When the patient was four years of age, a large tumor was found in the region of the pineal recess, causing hydrocephalus and seizures. A biopsy showed an undifferentiated malignant neuroepithelial neoplasm. The patient died within three months of diffuse central nervous system tumor. The unusual findings of a retinoblastoma in a microphthalmic eye with bilateral colobomas and a neuroepithelial neoplasm of the pineal gland are discussed.- - - - - - - - - - ranking = 4keywords = body (Clic here for more details about this article) |
5/15. Concurrent benign teratoid medulloepithelioma and pineoblastoma.Teratoid medulloepithelioma of the ciliary body is a rare tumor usually occurring in young children. Primary tumors of the pineal gland are also quite rare and have a similar age distribution. We report the case of a 2-year-old girl who had a leukocoria of the right eye. cataract surgery was performed; however, the patient developed a total retinal detachment refractory to treatment. She eventually developed early changes of phthisis bulbi and underwent an evisceration. Histopathologic examination revealed a benign teratoid medulloepithelioma containing islands of hyaline cartilage and rhabdomyoblasts. During the course of her evaluation, a pineoblastoma was discovered and surgically excised. While the association of pineal tumors with retinoblastomas has been well established, no such association has been reported for medulloepitheliomas. To our knowledge, this is the first recorded case of a benign teratoid medulloepithelioma occurring concurrently with a pineoblastoma.- - - - - - - - - - ranking = 4588.5348629984keywords = ciliary body, body (Clic here for more details about this article) |
6/15. Esthesioneuroblastoma presenting as sudden unilateral blindness. Histopathologic confirmation of optic nerve demyelination.We report here a case of esthesioneuroblastoma an 11-year-old girl presenting as acute loss of vision with minimal evidence of orbital, nasal, or paranasal sinus disease, a rare presenting symptom for this tumor. The initial diagnosis was postviral optic neuritis, a pattern of presentation not previously reported. When vision failed to improve, magnetic resonance imaging revealed a lesion in the posterior ethmoid and sphenoid sinuses. After a biopsy, the tumor was excised through the cranium and paranasal sinuses. A mass completely surrounding the optic nerve without invasion was found. Histochemical staining suggested demyelination secondary to compression, confirming the clinical impression of optic neuritis. Anti-Leu 7 monoclonal antibody is useful in characterizing of this tumor, since other immunochemical stains can be misleading. radiation and chemotherapy were given after the tumor was removed. Two years later, the patient has had neither recurrence nor complications.- - - - - - - - - - ranking = 1keywords = body (Clic here for more details about this article) |
7/15. Unilateral benign medulloepithelioma associated with bilateral retinal detachment.A two-month-old girl presented with a unilateral benign cystic medulloepithelioma from the ciliary body associated with bilateral retinal non-attachment. The authors discuss the histopathologic findings and the pathogenesis of the congenital retinal non-attachment: are these incidental occurrences or do they arise from a common pathogenetic mechanism?- - - - - - - - - - ranking = 4588.5348629984keywords = ciliary body, body (Clic here for more details about this article) |
8/15. Peripheral primitive neuroectodermal tumor associated with the anterior mandible: a case report and review of the literature.neuroectodermal tumors may arise in many places throughout the body including the diverse tissues of the head and neck. The primitive neuroectodermal tumor is a predominately neural, nonepithelial neoplasm similar to Ewing sarcoma. This article describes an 18-year-old female patient with a highly malignant peripheral primitive neuroectodermal tumor located in the soft tissue anterior to the mandibular symphysis. The clinical and radiographic presentation as well as the histopathology and immunohistochemistry of this rare entity is discussed. A review of the literature with respect to this tumor, as well as the current management of this tumor, is presented.- - - - - - - - - - ranking = 1keywords = body (Clic here for more details about this article) |
9/15. Glioneuroma associated with colobomatous dysplasia of the anterior uvea and retina. A case simulating medulloepithelioma.The left eye of an otherwise healthy child was enucleated at the age of 2 months because of an enlarging mass involving the temporal iris, ciliary body, and anterior retina. The initial histopathologic diagnosis was malignant medulloepithelioma with orbital extension. Closer study revealed a superotemporal chorioretinal and ciliary body coloboma; dysplasia of the adjacent retina; a glioneuromatous mass replacing the temporal ciliary body, chamber angle structures, and iris and extending through the sclera to involve the insertion of the lateral rectus muscle; neuroepithelial elements resembling medulloepithelioma; and abnormally developed iris pigment epithelium, and dilator and sphincter muscles. immunohistochemistry demonstrated that the main mass consisted of neurons positive for neuron-specific enolase (NSE), synaptophysin and neurofilaments, and glial cells expressing vimentin, glial fibrillary acidic protein, and S-100 protein. The neuroepithelial elements reacted positively for cytokeratins and S-100 protein, in addition to NSE and vimentin, suggesting ciliary epithelial rather than embryonic retinal origin. The tumor was rediagnosed as glioneuroma, which in this case was part of a widespread colobomatous dysplasia of the anterior uvea and retina. The patient is alive without metastases or local recurrence 2 years following enucleation and subtotal removal of the lesion.- - - - - - - - - - ranking = 13765.604588995keywords = ciliary body, body (Clic here for more details about this article) |
10/15. Recurrent medulloepithelioma of the ciliary body. Immunohistochemical characteristics.A predominantly benign medulloepithelioma of the ciliary body was diagnosed in an 8-year-old girl and resected by iridocyclectomy. It recurred twice during 30 months. Highly malignant histopathologic features developed, and the eye finally perforated and had to be enucleated. No recurrence or metastases have subsequently developed. Histologically, the tumor was a nonteratoid medulloepithelioma consisting of elements resembling embryonic retina, nonpigmented ciliary epithelium, and neuroblasts, but had also areas of obvious glial and neuronal differentiation as judged by immunohistochemistry. The neuroepithelial tumor cells were positive for neuron-specific enolase, vimentin, and often for S-100 protein. The neuroblastic cells were generally positive for neuron-specific enolase and synaptophysin, but were intermixed with glia-like tumor cells positive for vimentin, glial fibrillary acidic protein, and S-100 protein. The results suggest that even a nonteratoid medulloepithelioma may be, unlike retinoblastoma, a truly multipotential tumor.- - - - - - - - - - ranking = 22942.674314992keywords = ciliary body, body (Clic here for more details about this article) |
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