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1/179. Follicular dendritic cell tumor of the parapharyngeal region.

    BACKGROUND: Follicular dendritic cell (FDC) tumors are rare. A majority of the reported cases were confined to the lymph nodes. We report a case of FDC tumor occurring in the parapharyngeal region in a 45-year-old woman. methods: Characteristic histopathologic features of the excised primary and recurrent parapharyngeal tumors in conjunction with immunohistochemistry and electron microscopy helped us to arrive at a diagnosis of FDC tumor. RESULTS: Histopathology of primary excision revealed a lobulated tumor with a suggestion of ill-defined whorls. The most striking feature was regular occurrence of aggregates of lymphocytes within the tumor, especially around the blood vessels. The anatomic location together with the histology indicated the possibilities of either a meningioma, a salivary gland tumor, or a nerve sheath tumor. Immunostains for cytokeratin (CK), S-100 protein, and smooth muscle actin (SMA) were negative. However, the tumor cells showed strong immunoreactivity for epithelial membrane antigen (EMA) and vimentin. A diagnosis of parapharyngeal meningioma appeared to be the closest possibility. One year later, the patient developed a recurrence at the same site. A reexcision showed an identical tumor with an additional feature of lymphatic embolization and angioinvasion. A review of the entire case with further immunoreactivity for CD21 and CD35 confirmed the diagnosis of FDC. CONCLUSIONS: Follicular dendritic cell tumor has distinctive morphologic features and immunohistochemical profile. It is also characterized by considerable potential for recurrences.
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2/179. Primary orbital leiomyoma and leiomyosarcoma.

    A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.
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3/179. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.

    Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.
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4/179. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.

    Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.
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5/179. Malignant rhabdoid tumor of the vulva. Case report.

    The poor prognosis of malignant rhabdoid tumor is emphasized and histopathological criteria for distinction from epithelial sarcoma of the vulva are discussed. Immunohistochemical analyses were performed by using nine different antigens including vimentin, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, S-100 protein, AP-15, neuron specific enolase. This is the sixth reported case of a malignant rhabdoid tumor of the vulva. The patient died eight months after the initial diagnosis in spite of a combination of surgery, adjuvant chemotherapy and external radiotherapy.
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6/179. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.

    A demarcated 6 x 5 cm right paravertebral tumor at the level of T6 in a 39-year-old male was removed surgically. Histologically, the tumor consisted of monomorphous benign-looking, low-cellular spindle cells embedded in desmoplastic stroma. Ten years later, the tumor recurred locally with metastasis to systemic organs, including the occipital skin. Malignancy was histologically evident by the increased cellularity, cellular atypia and mitotic activity. The patient died of respiratory failure at the age of 49. Retrospectively reviewed, the primary lesion was low-grade fibrosarcoma-like spindle cell tumor, with secondary transformation into a highly malignant form. The differential diagnoses included sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma and malignant peripheral nerve sheath tumor. Immunohistochemically, the spindle cells in the primary and recurrent tumors consistently expressed epithelial membrane antigen, vimentin, type 4 collagen and laminin. The tumor cells in the present case showed a differentiation toward perineurial cells, which are normally positive for these immunohistochemical markers. Hence, the appropriate diagnostic term should be 'malignant perineurioma', a subtype of malignant peripheral nerve sheath tumor.
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7/179. Intraosseous squamous cell carcinoma arising in association with a squamous odontogenic tumour of the mandible.

    We report a rare occurrence of intraosseous squamous cell carcinoma (SCC) arising in association with a squamous odontogenic tumour (SOT), which had not previously been documented in the literature. A 53-year-old man had, for 5 years, a well-demarcated radiolucency attached to the impacted third molar of the mandible. The enucleated specimen had a characteristic pattern of SOT, but in which a few epithelial islands showed atypical features suggestive of SCC. Intense p53-, proliferating cell nuclear antigen- and Ki-67-positive cells were detected in carcinoma areas. Within 2 months, aggressive bone destruction showing typical findings of intraosseous SCC appeared. The present tumour is presumably a malignant variant of SOT.
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8/179. Intraductal papillary tumors of the major salivary glands: case reports of benign and malignant variants.

    Intraductal papilloma is an extremely rare benign salivary gland tumor that occurs most commonly in the minor salivary glands. To our knowledge, a malignant counterpart of intraductal papilloma has not been described previously. We report one case each of benign and malignant intraductal papillary tumors. The benign tumor occurred in the sublingual gland and was a typical example of intraductal papilloma, with the exception that we found no previously published reports of this type of tumor in this location. The other patient had a left parotid gland tumor that was architecturally similar to the intraductal papilloma, with the addition of cytologic atypia, intraductal extension, microinvasion, and lymph node metastases. This tumor was diagnosed as intraductal papillary adenocarcinoma with an invasive component. Both patients were alive and well without evidence of recurrence 2 years and 6 months (case 1) and 6 years (case 2) after surgery. Immunohistochemical examination revealed that the tumor cells resembled duct luminal cells in both cases. The 2 tumors had different immunoreactivities for carcinoembryonic antigen, p53, and Ki-67. The malignant counterpart of intraductal papilloma should be considered in the differential diagnosis of salivary gland tumors with a predominantly papillary structure, even though this tumor is extremely rare.
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9/179. Radical resection and intraoperative radiotherapy for a recurrent endometrial cancer after prolonged remission following aggressive salvage therapy: case report.

    The prognosis of recurrent endometrial carcinomas is generally poor, except for isolated vaginal relapse. We report a case of recurrent endometrial cancer in a 58-year-old woman who initially received a type I extended hysterectomy with bilateral salpin-go-oophorectomy and bilateral para-aortic and pelvic lymph node dissection. The first recurrence occurred in the left parametrium 7 months after the primary surgery. The salvage therapy consisted of radiotherapy combined with hormonal therapy (tamoxifen and Megace). Complete remission was achieved initially. Subsequently, the patient accepted six courses of chemotherapy (cisplatin and Adriamycin) for progressive elevation of cancer antigen 125 (CA-125). The CA-125 levels remained elevated with titers fluctuating around 100 U/ml until a second recurrence at the left iliac 75 months following salvage therapy. The second salvage treatment consisted of maximal debulking of the pelvic mass and intraoperative radiotherapy (IORT) followed by four courses of chemotherapy with paclitaxel and carboplatin. Complete remission was again accomplished, with clinical investigations and molecular markers returning to normal. The patient has been clinically free of disease for more than 2 years since the second relapse of cancer. In this particular case, we found that repeated recurrence could occur after a long complete remission following salvage therapy; however, the disease could be recontrolled with further aggressive salvage efforts. A multimodality approach with combinations of radical resection, IORT, and paclitaxel-based chemotherapy can be offered to patients with localized recurrent or repeatedly recurrent endometrial carcinoma after previous cisplatin-based chemotherapy and pelvic radiation.
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10/179. Superior vena cava obstruction due to prostate carcinoma.

    Superior vena cava obstruction (SVCO) is considered an oncologic emergency commonly associated with lung carcinoma. The case presented here is that of a 48-year-old man presenting with SVCO, which was diagnosed as metastatic prostate carcinoma localized to the chest. He was treated with goserelin and aggressive radiotherapy with a drop in his prostate-specific antigen levels and symptomatic relief that lasted approximately 12 months. SVCO recurred locally in the chest and the patient died 24 months after diagnosis. This represents a rare presentation of prostate carcinoma and underlines the necessity for tissue diagnosis before local radiotherapy.
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