1/22. Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location.A peripheral primitive neuroectodermal tumor arising from the abdominopelvic cavity is reported in a 24-year-old young male without any previous remarkable pathology. He was admitted to our hospital with complaints of urinary symptoms (acute urinary retention) and mild intestinal occlusion that had been present for three months. physical examination and CT scan revealed a pelvic mass occupying the entire pelvic cavity. The diagnostic workup included a CT-guided biopsy which defined the tumor as a sarcomatous type. Radical surgery was performed including tumor resection, pelvic exenteration (bladder and prostate gland) and urinary and fecal diversion. Adjuvant chemotherapy (VAIA) was delivered once the histology was confirmed. We reviewed the available literature focusing on the varied nomenclature of this tumor (peripheral neuroepithelioma, Askin's tumor, Ewing's extraosseous tumor, peripheral adult neuroblastoma, peripheral primitive extracranial neuroectodermal tumor (PPNET), the clinical features, the role of diagnostic imaging techniques, pathologic assessment and controversial therapeutic management. In addition, the prognosis and survival of this rare condition were analyzed.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/22. Presacral neuroblastoma in a child: a case report.Presacral location of neuroblastoma is rare. Resection entails an abdomino-sacral approach. This case report discusses clinical, imaging and successful surgical management of a presacral neuroblastoma in a one-year-old male child.- - - - - - - - - - ranking = 6keywords = neuroblastoma (Clic here for more details about this article) |
3/22. Bilateral testicular neuroblastoma. Scintigraphic depiction and therapy with I-131 MIBG.The authors describe a 29-year-old man who had a 16-year history of neuroblastoma and uncommon manifestations. At age 13, he was diagnosed with stage III retroperitoneal ganglioneuroblastoma that was resected. Ten years later, bilateral testicular enlargement and a pelvic mass from infiltration of the neuroblastoma became palpable. Metastatic involvement was depicted with MIBG, a radiotracer that concentrates in tissues of the sympathetic nervous system. Using I-131 MIBG, the tumors were treated with therapeutic doses of radiation and a partial response was obtained. This case is unique because of the massive degree of bilateral testicular infiltration and its occurrence as a late manifestation of neuroblastoma in early adulthood.- - - - - - - - - - ranking = 8keywords = neuroblastoma (Clic here for more details about this article) |
4/22. Bilateral pelvic ganglioneuroma: clinico-pathological findings: a case report.ganglioneuroma is a rare benign neurogenic tumor originating from the autonomic nervous system, and is considered the benign counterpart of neuroblastoma, lacking the immature neuroblastic cells. A case of pelvic ganglioneuroma with bilateral pelvic spread is described.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
5/22. Fine-needle aspiration of ganglioneuroma.ganglioneuroma is a rare soft tissue neoplasm, arising most frequently in the mediastinum or retroperitoneum in children and young adults. Correct evaluation of this lesion is of crucial importance, as there are some malignant neoplasms that have similar clinical presentations. As reports of the cytologic appearance of ganglioneuroma are few, the author describes the cytologic features of three patients examined initially by fine-needle aspiration (FNA) with correlation to histology and clinical data. This paper shows that the cytologic features of ganglioneuromas can be diagnostic if both spindle cells and ganglion cells are present in smears. The absence of immature cells and necrosis/inflammation differentiates ganglioneuromas from their malignant counterparts, ganglioneuroblastomas and neuroblastomas. Immunocytochemistry can help to establish the neurogenic origin of the tumor cells.- - - - - - - - - - ranking = 2keywords = neuroblastoma (Clic here for more details about this article) |
6/22. Presacral cystic neuroblastoma: case report and review of the literature.Cystic neuroblastoma (CN) is highly unusual; since 2005 only 55 cases have been reported in the medical literature. The authors report a case of pelvic (presacral) CN in a 2-month-old boy. Computed tomography scan confirmed a cystic, septated, and encapsulated mass. Complete tumor excision was achieved. Histopathology diagnosed a stroma-poor and undifferentiated neuroblastoma with multiple calcifications. meta-iodobenzylguanidine scan, radionuclide bone, and a bone marrow aspiration were normal. N-myc amplification was absent. The infant was classified stage I neuroblastoma and received no further therapy (4 y of follow-up). To the authors' knowledge, this is the first report of a CN in a pelvic-presacral location.- - - - - - - - - - ranking = 7keywords = neuroblastoma (Clic here for more details about this article) |
7/22. Pelvic neuroblastoma.Neuroblastoma usually presents as an upper abdominal mass arising from the adrenal gland. Recent experience with neuroblastoma of the spermatic cord, bladder and pelvis demonstrates the propensity of this tumor to arise in unusual areas. The need for unusual and unexpected masses in children to be evaluated thoroughly for neuroblastoma and for therapy to be individualized based on histological criteria, patient age and location of the tumor is demonstrated.- - - - - - - - - - ranking = 6keywords = neuroblastoma (Clic here for more details about this article) |
8/22. Neuroblastoma in adults. A report of 3 cases.Well documented as one of the more frequent tumours of childhood, neuroblastomas in adults are rare. Correct diagnosis, and distinction from other small-cell neoplasms, is thus often delayed. Three further cases are described in 2 women and 1 man between the ages of 26 years and 47 years. Metastatic disease existed at the time of initial presentation in 1 patient, while another had experienced multiple recurrences at the primary site before correct diagnosis. All, however, had a favourable response to chemotherapy, albeit in the short term. Primary diagnosis was made by fine-needle aspiration cytology, together with electron microscopic evaluation of aspirated cells in 1 case. The histological, cytological and electron microscopic characteristics of neuroblastomas are presented and illustrated, and their differentiation from other small cell tumours in adults discussed.- - - - - - - - - - ranking = 2keywords = neuroblastoma (Clic here for more details about this article) |
9/22. Malignant rhabdoid tumour of soft tissue. An ultrastructural and immunohistological study of a pelvic tumour.A case of extrarenal malignant rhabdoid sarcoma arising in the pelvic soft tissues of a 12-year-old girl is described. By routine light microscopy the tumour resembled, in some areas, an embryonal rhabdomyosarcoma and, in other areas, a neuroblastoma. Electron microscopy revealed characteristic cytoplasmic aggregates of intermediate filaments, often with central clusters of organelle membranes surrounded by these filaments. Immunohistochemical stains showed strong cytoplasmic reactivity for vimentin. Staining for cytokeratin, myoglobin, desmin, neurofilaments, neurone specific enolase, S-100 protein and leucocyte common antigen was negative. A histogenetic origin from primitive mesenchymal cells is favoured. We strongly support the use of electron microscopy for the definitive diagnosis of small round cell undifferentiated sarcomas of childhood.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
10/22. adult neuroblastoma. Report of three cases and review of the literature.adult neuroblastoma is an uncommon malignancy. The authors report three additional cases and review the 39 reported cases in the world literature. Presentation in the abdomen is the most common; a high rate of lower limb presentation is also observed. incidence is equal between sexes with median age of presentation of 34 years. survival is greatest after surgical intervention (median, 20.5 months) compared with no surgery (median, 12.5 months). Chemotherapy may benefit individual patients but does not have a major impact on survival. radiotherapy is indicated for localized, inoperable primaries or painful metastases. The survival rates of this group of patients parallels that of childhood neuroblastoma, Stage III-IV.- - - - - - - - - - ranking = 6keywords = neuroblastoma (Clic here for more details about this article) |
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