1/88. A case of chordoma in association with rectal carcinoma.A 74-year-old male patient presented with anal and sacral pain 18 months after abdomino-perineal resection for rectal cancer. Computerized tomography (CT) of the pelvis demonstrated a well defined mass anterior to the lower sacrum, posteriorly infiltrating and destroying the fourth and fifth sacral nerves and invading the right gluteal fossa. A 7.5 x 15 x 2 cm encapsulated mass was demonstrated during the operation using a posterior approach and the lower sacral segments together with the tumour were removed by amputation at S3 level. Histopathology revealed chordoma. This case is unique because of the rarity of chordoma in association with rectal tumour at the sacrococcygeal region.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/88. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases.AIMS: Angiosarcomatous differentiation represents the least common form of heterologous differentiation in malignant peripheral nerve sheath tumours (MPNST), and is seen most frequently in patients with neurofibromatosis type 1. More rarely, it has been reported in patients without stigmata of neurofibromatosis, or in benign nerve sheath tumours and peripheral nerves. This study was undertaken to confirm this rare association. methods AND RESULTS: Four cases of angiosarcoma arising in a peripheral nerve, in a long-standing schwannoma and in two MPNST are described. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. An intraneural high-grade epithelioid angiosarcoma arose in the left posterior tibial nerve of a 78-year-old man, a well to moderately differentiated angiosarcoma was seen in an ancient schwannoma of the lateral neck in a 73-year-old women, and an angiosarcoma of varying grades of differentiation developed in a recurrent MPNST in the thigh of 38-year-old man. In addition a high-grade MPNST in the axillary region of a 30-year-old man showed foci of heterologous high-grade angiosarcomatous differentiation. The neural and endothelial lines of differentiation were confirmed in each case by positive immunohistochemical staining for neural and endothelial markers, respectively. In all cases tested, the neural differentiated cells stained immunohistochemically positive for antibodies against vascular endothelial growth factor. CONCLUSIONS: This study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and malignant peripheral nerve sheath tumours.- - - - - - - - - - ranking = 11keywords = nerve (Clic here for more details about this article) |
3/88. Therapy-related megakaryoblastic leukemia with pituitary involvement following treatment for non-Hodgkin's lymphoma.A case of a 66-year-old Japanese man developed therapy-related megakaryoblastic leukemia with pituitary involvement after chemotherapy for non-Hodgkin's lymphoma. alkylating agents had been administered for the treatment of non-Hodgkin's lymphoma and 6 years later, megakaryoblastic leukemia with myelofibrosis and myelodysplasia developed. The blast cells expressed CD41, and immature antigens also. These findings were compatible with therapy-related megakaryoblastic leukemia. An autopsy revealed blast-cell infiltration into multiple organs including the posterior pituitary lobe. Therapy-related megakaryoblastic leukemia is very rare, and pituitary involvement may be associated with immaturity of blast cells.- - - - - - - - - - ranking = 0.44329019368001keywords = organ (Clic here for more details about this article) |
4/88. Parotid carcinoma as a second malignancy after treatment of childhood acute lymphoblastic leukemia.The occurrence of second malignant neoplasms (SMN) in children who survive their primary malignancy is a major cause for concern. Two children with diagnoses of intermediate-risk acute lymphoblastic leukemia (ALL) at 22 months and 2 years of age were treated with multiagent chemotherapy and prophylactic cranial irradiation. They experienced painless parotid swelling 6 and 7 years after successful treatment of the ALL. The patients underwent total parotidectomy, and a diagnosis of mucoepidermoid carcinoma was made. Both patients experienced transient facial nerve paresis. The incidence of SMN in children successfully treated for primary malignancies is 3% to 12%. Salivary gland tumors are being increasingly described in this setting. Long-term follow-up for survivors of childhood ALL is recommended with prompt assessment and resection of parotid swellings, particularly in children who have received cranial irradiation.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
5/88. Multifocal neuroblastoma: biologic behavior and surgical aspects.BACKGROUND: Although multifocal neuroblastoma is rare, its incidence has increased because of recent improvements in diagnostic tools and the introduction of mass screening. Among the 106 neuroblastoma cases treated at the authors' hospital between 1984 and 1998, 8 were multifocal neuroblastoma. methods: The authors examined clinicopathologic findings and biologic features, including MYCN amplification, NTRK1 and Ha-ras p21 expression, cellular dna content, and telomerase activity in these 8 multifocal neuroblastoma cases. Moreover, clinicopathologic findings were investigated with a review of 53 published cases of multiple neuroblastoma in the literature published in English between 1966 and 1999. RESULTS: Among these eight cases, five were detected by mass screening and three were incidental neuroblastomas. Histologically, all tumors were classified as ganglioneuroma or favorable neuroblastoma except one advanced case. All tumors lacked the MYCN gene amplification and expressed NTRAK1 mRNA and Ha-ras p21 protein. Cellular dna content showed that half of these tumors were near-triploid, and the proliferative index (%S-phase) of all tumors was less than 25%. High telomerase activity was detected in none of these cases. Four patients underwent multistage operation and five patients with bilateral adrenal neuroblastomas underwent tumor enucleation to preserve adrenal function. Currently, all patients are disease free and none have required corticosteroid replacement therapy. Among the previously reported 53 cases with multifocal neuroblastoma, 25 were incidentally detected, 18 had familiar history, and most patients without other major complications also had extremely good prognoses. CONCLUSIONS: These findings suggested that most multifocal neuroblastomas have favorable biologic features. Clinically, surgical approaches should be attempted to preserve organ function, especially adrenal function, and minimal invasive surgery should be performed. In cases of thoracoabdominal neuroblastoma, multistage surgery is effective and safe.- - - - - - - - - - ranking = 0.44329019368001keywords = organ (Clic here for more details about this article) |
6/88. Granular cell traumatic neuroma: a lesion occurring in mastectomy scars.BACKGROUND: Granular cell changes can be observed in a variety of benign and malignant tumors, and are seen more commonly in granular cell tumors, which in about 5% of cases develop in the breast. Granular cells also have been observed in sites of previous trauma, such as surgery, and are found to be inflammatory reactions of histiocytic origin. methods AND RESULTS: We investigated, morphologically and immunohistochemically, 2 granular cell lesions occurring in mastectomy scars after surgery for carcinoma. Both lesions were composed of strands and nests of large granular cells, haphazardly set in a background of fibrous tissue, with sparse inflammatory infiltrates. Several tortuous hypertrophic nerve bundles were also embedded in the fibrous tissue. A few of these nerve bundles showed degenerative changes and contained granular cells. Immunohistochemically, granular cells were positive for S100 protein, neuron-specific enolase, vimentin, and CD68 antigen. CONCLUSIONS: We consider these proliferative lesions of peripheral nerves to have the features of both granular cell tumor and traumatic neuroma. These cases indicate that traumatic neuroma can undergo extensive granular cell changes and constitute a previously unrecognized entity, which we provisionally label granular cell traumatic neuroma. Granular cell traumatic neuroma has to be taken into consideration when evaluating lesions occurring at mastectomy scars and should be differentiated from malignant tumors with granular cells, such as apocrine carcinoma and alveolar soft part sarcoma.- - - - - - - - - - ranking = 3keywords = nerve (Clic here for more details about this article) |
7/88. Malignant peripheral nerve sheath tumor arising in benign ancient schwannoma: a case report with an immunohistochemical study.A rare example of malignant transformation in an ancient schwannoma arising in the right side of the neck of a 51-year-old man without any clinical manifestations suggesting neurofibromatosis is described. The tumor, approximately 4 cm at its largest dimension, was well circumscribed and had a direct connection with the sympathetic nerve. Microscopically, the central portion of the tumor showed features of ancient schwannoma characterized by extensive hyalinization with cystic degeneration, scattered spindle cells with hyperchromatic and tapered nuclei, and some symplastic changes. However, predominantly in the outer portion, a proliferation of spindle-shaped cells with enlarged nuclei was present. The nuclei of these cells showed irregular contours, coarse granular chromatin texture, and conspicuous nucleoli. Mitotic figures and small necrotic foci with scattered apoptotic bodies were also seen. Immunohistochemically, S-100 protein was almost negative in areas consisting of overtly atypical cells where the mitotic index evaluated with MIB-1 antibody was 30.5%. In contrast, S-100-positive bland spindle cells were scattered in an extensively hyalinized area with a labeling index less than 3%. P53 protein was strongly positive in atypical spindle cells. Although it is a very uncommon event, definite nuclear atypia, frequent mitotic figures, and the existence of small necrotic foci should be recognized as indicating a diagnosis of malignant degeneration of benign schwannoma. immunohistochemistry would be useful as an ancillary technique in such a setting.- - - - - - - - - - ranking = 5keywords = nerve (Clic here for more details about this article) |
8/88. Development of a biologically distinct EBV-related lymphoproliferative disorder following autologous bone marrow transplantation for an EBV-negative post-renal allograft Burkitt's lymphoma.Post-transplant lymphoproliferative disorder (PTLD) is a known complication of both solid organ transplantation and allogeneic bone marrow transplantation (BMT) but is rarely seen following autologous BMT. We report the case of a 45 year-old female who developed Burkitt's lymphoma eight years after a renal allograft. This PTLD was found to have lambda light chain restriction, contained del(8)(q24) and add(14)(q32), and was negative for EBV on immunohistochemical and dna-based PCR analyses. Immunoglobulin heavy chain (IgH) PCR studies revealed a prominent clonal rearrangement. She responded to intravenous cyclophosphamide and proceeded to high-dose chemoradiotherapy and mafosfamide-purged autologous BMT. Thirty-nine days post-BMT she presented with cough and fever and developed hepatic dysfunction; abnormal lymphoplasmacytoid cells were noted in the peripheral blood. Investigations revealed kappa light chain restriction, an oligoclonal IgH rearrangement, a normal karyotype and PCR studies for EBV were positive, consistent with a clinically and biologically distinct PTLD. She initially improved following discontinuation of immunosuppression, but then deteriorated abruptly and died 58 days post-BMT. It is likely that the two separate episodes of PTLD in this patient, one of which was atypical, arose as a result of both the chronic use of cyclosporine and the impairment of cell-mediated immunity associated with autologous BMT. The sequence of events in this patient should contribute to a better understanding of late-onset, EBV-negative PTLD.- - - - - - - - - - ranking = 0.44329019368001keywords = organ (Clic here for more details about this article) |
9/88. Comparative risk assessment of secondary cancer incidence after treatment of Hodgkin's disease with photon and proton radiation.Probabilities for secondary cancer incidence have been estimated for a patient with Hodgkin's disease for whom treatment has been planned with different radiation modalities using photons and protons. The ICRP calculation scheme has been used to calculate cancer incidence from dose distributions. For this purpose, target volumes as well as critical structures have been outlined in the CT set of a patient with Hodgkin's disease. Dose distributions have been calculated using conventional as well as intensity-modulated treatment techniques using photon and proton radiation. The cancer incidence has been derived from the mean doses for each organ. The results of this work are: (a) Intensity-modulated treatment of Hodgkin's disease using nine photon fields (15 MV) results in nearly the same cancer incidence as treating with two opposed photon fields (6 MV). (b) Intensity-modulated treatment using nine proton fields (maximum energy 177.25 MeV) results in nearly the same cancer incidence as treating with one proton field (160 MeV). (c) Irradiation with protons using the spot scanning technique decreases the avoidable cancer incidence compared to photon treatment by a factor of about two. This result is independent of the number of beams used. Our work suggests that there are radiotherapy indications in which intensity-modulated treatments will result in little or no reduction of cancer incidence compared to conventional treatments. However, proton treatment can result in a lower cancer incidence than photon treatment.- - - - - - - - - - ranking = 0.44329019368001keywords = organ (Clic here for more details about this article) |
10/88. cerebellopontine angle metastasis from papillary carcinoma of the thyroid: case report and literature review.BACKGROUND: Papillary thyroid carcinoma is the most common type of well-differentiated thyroid malignancy and typically has an excellent prognosis and a low incidence of distant metastasis. However, once metastasis has developed in a distant site, prognosis is markedly diminished. brain metastases from papillary thyroid carcinoma are extremely rare. Currently, there are no established therapeutic guidelines for treating brain metastases from thyroid carcinoma. CASE DESCRIPTION: We report on the case of a patient who presented with worsening neurological symptoms 3 years after resection of a thyroid papillary carcinoma. magnetic resonance imaging identified a lesion of the cerebellopontine angle that encased the lower cranial nerves. The patient underwent a left retrosigmoid craniotomy with a 98% resection of the mass and received postoperative adjuvant radioiodine therapy, external beam radiation, and Gamma Knife radiosurgery. The patient tolerated the procedure well and demonstrated significant progressive improvement in her neurological symptoms postoperatively. After the multimodal approach to therapy, she remains symptom-free at 3-year follow-up. Radiographic monitoring of the small tumor remnant reveals a decrease in size from the postoperative period. CONCLUSION: This article is the first formal case report of cerebellopontine angle metastasis from papillary thyroid carcinoma. Therapeutic protocols for brain metastases of papillary thyroid carcinoma are not firmly established. This case illustrates the unique event of a cerebellopontine angle metastasis from papillary thyroid carcinoma, which was successfully treated with a combination of surgical, chemical, and radiological modalities. This aggressive course of therapy has resulted in an excellent outcome in this instance.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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