1/8. Endometrial endometrioid carcinomas associated with Ewing sarcoma/peripheral primitive neuroectodermal tumor.Three uterine tumors, each consisting of endometrioid carcinoma and Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) are described. The diagnosis of ES/pPNET in each case was first established in the hysterectomy specimen because each ES/pPNET was misinterpreted on the endometrial biopsy specimens as a high-grade homologous sarcoma. The ES/pPNET element in each case consisted of solid masses of small- to medium-sized round cells without Homer-Wright pseudorosettes, glial or ganglion cells, true rosettes with central lumens, or medulloepithelial tubules. Each ES/pPNET exhibited focal positive immunostaining for neuron-specific enolase, diffuse staining for vimentin, and strong cell membrane immunoreactivity for O13 (CD99), the last finding providing the first clue to the diagnosis of ES/pPNET in each case. The diagnosis in each case was confirmed by detection of EWS/FLI-1 fusion transcript through reverse transcription polymerase chain reaction. We also examined O13 immunoreactivity retrospectively in 40 cases of malignant mixed mullerian tumors (MMMT) with homologous or heterologous elements. O13 immunoreactivity was not observed in the malignant epithelium or in the homologous or heterologous sarcomas. The immunoreactivity of O13 in round cell endometrial sarcomas provides a clue to the diagnosis of ES/pPNET.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/8. Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin.The authors report a case of sinonasal teratocarcinosarcoma in a 37-year-old man, which was located in the anterior skull base and extended to the right nasal cavity and paranasal sinuses. The tumor was surgically resected twice, but it could not be removed completely. Microscopically, it was mainly composed of primitive cell nests within a moderately cellular stroma. The components of squamous cell epithelia with focal teratoid appearance and adenocarcinomatous differentiation were observed. There were many rhabdomyoblasts scattered in the nests and stroma. Ultrastructurally, the primitive cells had many neural processes with parallel microtubules, resembling olfactory neuroblastoma. Rhabdomyoblasts showed various degrees of skeletal muscle differentiation. Some of the stromal spindle cells had actin filaments with dense patches and dense core granules. Immunohistochemically, the primitive cells were positive for epithelial markers, neuron-specific enolase, synaptophysin, and myogenic regulatory proteins. The rhabdomyoblasts showed immunoreactivity for myoid markers, cytokeratin, epithelial membrane antigen, and synaptophysin. Most of the stromal spindle cells were positive for smooth muscle actin, neuron-specific enolase and synaptophysin. The immunohistochemical and ultrastructural findings suggest that primitive cells had the most primitive phenotype of placodes, and support the possibility that sinonasal teratocarcinosarcoma is essentially a neuroectodermal tumor with divergent differentiation.- - - - - - - - - - ranking = 0.2keywords = carcinoma (Clic here for more details about this article) |
3/8. Primary primitive neuroectodermal tumor of the cauda equina.Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification has been controversial, PNETs are well recognized primary tumors of both central and peripheral nervous systems. PNETs must be distinguished from other round-cell tumors, including Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and small cell carcinoma. Intraspinal PNETs are rare neoplasms that are usually metastatic in origin. We describe the eighth reported primary PNET of the cauda equina that developed in a 52-year-old man with no significant medical history. The tumor was characterized by Homer-Wright rosettes and immunoreactivity for CD99, glial fibrillary acidic protein, neuron-specific enolase S100, and synaptophysin. The anatomic location of primary intrathecal PNETs is important as those arising in the spinal cord develop in the central nervous system, whereas those arising in the cauda equina develop in the peripheral nervous system. The histogenesis of intrathecal PNETs may be multifactorial.- - - - - - - - - - ranking = 0.2keywords = carcinoma (Clic here for more details about this article) |
4/8. Pigmented intraosseous odontogenic carcinoma of the maxilla: a pediatric case report and differential diagnosis.We report a pigmented intraosseous odontogenic carcinoma of the maxilla occurring in a 6-year-old Japanese boy. Grossly, the tumor showed solid, gray-yellow, and markedly pigmented appearance. histology showed neoplastic growths of atypical epithelial cells that occasionally contained melanin pigments. melanocytes with dendritic processes were often found in the tumor cell clusters, and solitary or aggregated melanophages were scattered within the dense fibrovascular stroma. The tumor cells were diffusely positive for cytokeratins and epithelial membrane antigen, and focally positive for vimentin, neuron specific enolase, neurofilament protein, carcinoembryonic antigen, and amelogenin. Ultrastructural studies showed well-developed intercellular junctions, mainly desmosomes, and glycogen particles. In addition, some tumor cells contained melanosomes and/or a few neurosecretory granules. We consider that the present tumor suggests a close association of ectoderm, mesenchyma, and neuroectoderm in embryogenesis of the tooth, and can raise a diagnostic confusion with melanotic neuroectodermal tumor.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
5/8. Teratoid carcinosarcoma of the ovary with prominent neuroectodermal differentiation.We present what we believe to be only the second report of ovarian teratoid carcinosarcoma. The patient, a 59-year-old woman, was admitted to hospital complaining of a pelvic mass and of abdominal fullness. Advanced ovarian cancer was diagnosed, and a tumorectomy was done. The tumor occupied the pelvis, and metastasis was found in the liver and spleen. The solid tumor was composed of chondrosarcoma, squamous cell carcinoma, adenocarcinoma and malignant neuroectodermal components, which contained ganglioneuroblastoma-like and medulloepithelioma-like areas. Immunohistochemically, the neuroectodermal cells were positive for both neural and epithelial markers. This ovarian tumor consisted of frankly malignant components, with prominent neuroectodermal elements mixed with epithelial and mesenchymal elements in an organoid fashion; a quite rare tumor.- - - - - - - - - - ranking = 0.4keywords = carcinoma (Clic here for more details about this article) |
6/8. Rapid diagnosis of germline p53 mutation using the enzyme mismatch cleavage method.The p53 tumor suppressor gene is the most commonly altered gene in human cancers. Germline mutations in p53 are the genetic alteration underlying predisposition to multiple cancers in li-fraumeni syndrome and Li-Fraumeni-like syndrome. We describe a patient who presented with developed adrenocortical carcinoma at age 19 months and a cerebral primitive neuroectodermal tumor at age 5 years. The patient did not have a family history of cancer. We used the enzyme mismatch cleavage (EMC) method to screen for mutations in the p53 gene and found a germline mutation in exon 7 (codon 248). loss of heterozygosity analysis in one tumor revealed loss of the wild-type p53 allele. In our report we demonstrate the EMC method to be a rapid and sensitive method for mutation detection.- - - - - - - - - - ranking = 0.2keywords = carcinoma (Clic here for more details about this article) |
7/8. Primary neuroendocrine tumor of the breast.adult neuroendocrine tumors may present with a wide range of clinical symptoms that share specific ultrastructural and biochemical features. A 63-year-old post-menopausal female patient was admitted to the hospital with a mass in her right breast and the diagnosis was primary neuroendocrine tumor of the breast. Although neuroendocrine tumors can originate in various parts of the body and breast carcinoma with neuroendocrine differentiation has been described, primary neuroendocrine tumor of the breast is very unusual. This case is now presented and the current literature is reviewed.- - - - - - - - - - ranking = 53.842347406856keywords = neuroendocrine, carcinoma (Clic here for more details about this article) |
8/8. Vertical ramus compartment resection of the mandible for deeply invasive tumors.PURPOSE: Vertical ramus compartment resection of the mandible is indicated in the management of pathology that originates in the ascending ramus or condyle of the mandible and invades the pterygomaxillary fossa, infratemporal space, parapharyngeal space, masseter muscle, or medial pterygoid muscle. This article describes the technique, presents a review of the relevant literature, and reports the outcome of five cases. patients AND methods: A retrospective review of all cases operated on by the senior author (R.A.O.) over the last 5 years at the University of maryland was performed. The records of these cases were reviewed to identify patient sex, age, pathologic diagnosis, use of adjuvant therapy, and status after resection. RESULTS: Five surgical cases in which a vertical ramus compartment resection of the mandible was necessary were identified. The histologic diagnoses of these five cases were as follows: grade II chondrosarcoma, peripheral neuroectodermal tumor, high-grade central mucoepidermoid carcinoma, invasive squamous cell carcinoma, and odontogenic keratocyst. Four of the five patients are alive and well at follow-up of 16 to 43 months. The only death occurred in a 70-year-old man with squamous cell carcinoma that recurred intracranially by local extension. CONCLUSIONS: When indicated by local extension or tumor biology, vertical ramus compartment resection of the mandible is a viable surgical modality that allows clearance of local disease in most well-selected cases.- - - - - - - - - - ranking = 0.6keywords = carcinoma (Clic here for more details about this article) |