1/164. Peripheral primitive neuroectodermal tumors of bone. A review of three cases.The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.- - - - - - - - - - ranking = 1keywords = neuroectodermal (Clic here for more details about this article) |
2/164. Intra-articular benign osteoblastoma of the acetabulum: a case report.The acetabulum is an unusual location for benign osteoblastoma, usually seen in adolescents and young adults. This article reports a case of an intra-articularly expanding benign osteoblastoma of the acetabulum in a 6-year-old boy. The diagnostic and surgical features of the disease in this unusual location at this young age are discussed. Excisional biopsy and curettage of the lesion followed by bone grafting resulted in healing of the acetabular lesion without acetabular dysplasia, but with a resultant coxa magna.- - - - - - - - - - ranking = 0.0057288625515807keywords = dysplasia (Clic here for more details about this article) |
3/164. Intraosseus lipoma.The authors present a case of intraosseus lipoma seen at the Orthopaedic Clinic of the University of Bari, bringing the total in the world literature to thirty cases. The tumour is a benign primary neoplasm of bone, made up of mature adipose tissue containing atrophic bone trabeculae. This affection is a definite entity quite distinct from parosteal lipomas arising in periosteum, fasciae and muscles which only affect the bone secondarily by compression or direct invasion. Emphasizing the extreme rarity of the tumour, the authors discuss its clinical, radiological, anatomical and pathological features. The differential diagnosis is with certain dysplasic and neoplastic lesions of bone such as: bone cyst, non-ossifying fibroma, aneurismal cyst, monostotic fibrous dysplasia, and solitary myeloma. Complete removal is followed by cure.- - - - - - - - - - ranking = 0.0057288625515807keywords = dysplasia (Clic here for more details about this article) |
4/164. Cytologic diagnosis of a melanotic neuroectodermal tumor of infancy occurring in the cranial bones.Melanotic neutroectodermal tumor of infancy (MNTI) is a rare, usually benign tumor commonly occurring in the maxilla. MNTIs at unusual sites like the cranium clinically mimic malignant small round cell tumors. Consequently, a correct preoperative cytologic diagnosis of MNTI at these sites helps in the surgical management of the patient. We report on a cytologically diagnosed case of MNTI in the frontotemporal region of the skull in an infant. Aspirates from the lesion were cellular, with a bimodal population mainly of small neuroblast-like cells admixed with a few large epithelioid cells with melanin granules. In the present case, following the cytologic diagnosis a wide local excision was carried out, and the histologic examination confirmed the cytologic diagnosis. Diagn. Cytopathol. 1999;21:280-283.- - - - - - - - - - ranking = 0.57142857142857keywords = neuroectodermal (Clic here for more details about this article) |
5/164. Dysplasia epiphysealis hemimelica of the proximal tibia showing epiphyseal osteochondroma in an adult.A 33-year-old woman was referred to our hospital complaining of pain and a tumorous lesion in her left knee joint in the absence of any history of trauma. Radiological examinations demonstrated an osseous mass originating from the epiphysis of the proximal tibia, with a continuous osteoblastic lesion involving the lateral half of the epiphysis. The pathological diagnosis of these lesions was compatible with that of osteochondroma. The clinical and pathological features of this case were considered to be identical with those of dysplasia epiphysealis hemimelica, although this patient was older than patients described in previous reports, and demonstrated no other symptoms such as valgus or varus deformity or limb-length discrepancy.- - - - - - - - - - ranking = 0.0057288625515807keywords = dysplasia (Clic here for more details about this article) |
6/164. osteosarcoma in a patient with McCune-Albright syndrome and Mazabraud's syndrome.Sarcomas infrequently develop in osseous sites of fibrous dysplasia. We report a patient with Mazabraud's syndrome (polyostotic fibrous dysplasia and soft tissue myxomas) complicated by the development of osteogenic sarcoma in a bone affected by fibrous dysplasia. This is the third case of osteosarcoma within the small population of reported patients with Mazabraud's syndrome. There may be an increased incidence of malignant transformation in these individuals' dysplastic bones above that associated with patients suffering from fibrous dysplasia alone.- - - - - - - - - - ranking = 0.022915450206323keywords = dysplasia (Clic here for more details about this article) |
7/164. Synovial sarcoma, histologically mimicking primitive neuroectodermal tumor/Ewing's sarcoma at distant sites.We report a case of synovial sarcoma (SS) showing unusual histology at distant sites. A 47-year-old man was aware of a tumor on the sole of his left foot. After preoperative chemotherapy with a diagnosis of SS, wide excision was performed. During postoperative chemotherapy, multiple tumorous lesions developed in the bone (including the whole spine) and both lungs. The patient died 1 year later. Histologically, the excised tumor of the foot showed a biphasic cellular pattern typical of SS, whereas at autopsy the bone and lung lesions were composed only of undifferentiated small round cells with cytoplasmic fibrillar processes. Homer-Wright rosettes were also observed. Immunohistochemically, 80% of the bone and lung tumor cells expressed MIC2 protein homogeneously. To clarify whether the bone and lung round cell tumors were metastatic lesions or second malignancies, especially primary primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES), we performed reverse transcription-polymerase chain reaction (RT-PCR) analysis of tumor type-specific fusion gene transcripts. The SYT/SSX fusion transcript was identified in both the foot and lung lesions, whereas the EWS/FLI1 transcript was not detected in either lesion. Therefore, we concluded that the multiple bone and lung tumors were poorly differentiated metastatic tumors, which arose from the SS of the foot. We also conclude that the identification of chimeric fusion transcripts can be successfully applied to poorly differentiated sarcomas and will help in the differential diagnosis of tumors that cannot be distinguished by conventional morphological examinations. Also, it should be remembered that cytoplasmic staining for MIC2 protein may occur in sarcomas other than PNET/ES.- - - - - - - - - - ranking = 0.71428571428571keywords = neuroectodermal (Clic here for more details about this article) |
8/164. Examination of tumor and tumor-like conditions of bone.Surgical pathology specimens composed of bone, ranging from core biopsy to limb amputation specimens, require special attention, processing, and often unique equipment. This readily translates into additional handling steps and time, especially when one factors in clinical correlation with the surgeon and radiologic review of all images with a knowledgeable musculoskeletal radiologist. When these factors are superimposed on the rarity of these lesions in routine practice, it is not surprising that most trainees, as well as seasoned pathologists, are wary of these lesions. In this report, we use a case of osteofibrous dysplasia (Campanacci's disease) to demonstrate the dissection of such a surgical specimen and complete the report with a brief discussion of the entity.- - - - - - - - - - ranking = 0.0057288625515807keywords = dysplasia (Clic here for more details about this article) |
9/164. Two cases of secondary angiosarcoma arising from fibrous dysplasia.Angiosarcoma associated with fibrous dysplasia is very rare. We have recently experienced two cases of angiosarcoma that secondarily arose from fibrous dysplasia. The first patient, a 55 year-old man, had noticed a deformity in the right upper arm since he was five years old. At the age of 25 years, polyostotic fibrous dysplasia was diagnosed by X-ray examination. The patient complained of swelling and pain around the left shoulder. The diaphysis of the humerus was mostly non-observable due to severe bone destruction by tumor invasion and there was a large soft tissue tumor. biopsy examination revealed grade II or III hemangioendothelioma with typical histologic findings of fibrous dysplasia. After interscapulothoracic amputation for wide tumor resection, he died of DIC. autopsy revealed multiple liver metastatic lesions of angiosarcoma. The second patient was a 66-year-old woman. She presented with a pathological fracture in the right tibia, due to an osteolytic lesion with cortical bone swelling and thinning. The histologic diagnosis was fibrous dysplasia without any sarcomatous changes. However, after a 3-time recurrence, angiosarcoma arose from the same lesion. She is now still alive with disease-free period of 6.5 years after amputation.- - - - - - - - - - ranking = 0.051559762964227keywords = dysplasia (Clic here for more details about this article) |
10/164. Cystic fibrous dysplasia mimicking giant cell tumor: MRI appearance.We report the case of a 43-year-old man who presented with an osteolytic and expansive lesion in the left distal femur mimicking a giant cell tumor. Magnetic resonance imaging (MRI) showed that most of the lesion was cystic, and histological examination revealed fibrous dysplasia with marked cystic degeneration. Radiographic findings of cystic fibrous dysplasia in the end of a long bone may be similar to those of a giant cell tumor, and a biopsy is essential for the final diagnosis.- - - - - - - - - - ranking = 0.034373175309484keywords = dysplasia (Clic here for more details about this article) |
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