Cases reported "Nasopharyngeal Neoplasms"

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1/800. Extramedullary tumors of myeloid blasts in adults as a pattern of relapse following allogeneic bone marrow transplantation.

    BACKGROUND: Extramedullary tumors of lymphoid and myeloid blasts outside the well-defined sanctuaries following allogeneic bone marrow transplantation (allo-BMT) are rare. Little is known about the biology, treatment, and outcome of these tumors in this setting. methods: In this retrospective analysis, 134 consecutive patients with acute myeloid leukemia (AML) or chronic myeloid leukemia (CML) who underwent allo-BMT at a single institution between 1990 and 1998 were reviewed. Five cases of isolated extramedullary myeloid sarcoma that occurred as patterns of recurrence following allo-BMT between 1990 and 1998 are reported. These patients were treated with radiotherapy, systemic chemotherapy, or a second allo-BMT. Clinical outcome is compared with posttransplantation bone marrow relapses observed during the same period at the same institution. The literature on the clinical characteristics, currently available treatment, and outcome of posttransplantation myeloid sarcoma patients was reviewed. RESULTS: Excluding isolated skin and central nervous system recurrences, the frequency of extramedullary myeloid sarcoma encountered as a relapse pattern following allo-BMT was determined to be 3.7% among patients with acute or chronic leukemia of myeloid origin. The survival of patients who were managed with radiotherapy and systemic chemotherapy was less than 4 months. A patient who underwent a second allo-BMT following local radiotherapy is alive and in complete remission more than 33 months after the diagnosis of myeloid sarcoma. The median survival of 17 patients with posttransplantation bone marrow relapse following allo-BMT was 2.2 months. When posttransplantation medullary recurrences are analyzed, patients with CML had a median survival of 12 months, with a significantly better 5-year survival rate than patients with AML (0 vs. 60%, P = 0.015; median survival, 12 months). CONCLUSIONS: The clinical outcomes of patients with recurrent isolated extramedullary myeloid sarcoma following allo-BMT are poor, as in any leukemic relapse, with the exception of patients with CML in this setting. ( info)

2/800. Epignathus: a germ-cell tumour presenting as neonatal respiratory distress.

    A full-term neonate developed acute upper airway obstruction immediately after birth secondary to a polypoidal mass in the oropharynx. After the child's airway had been secured, the mass was excised and found to be a nasopharyngeal teratoma, a rare congenital germ cell tumour which is frequently associated with other congenital malformations. It is sometimes possible to diagnose these tumours in utero, thereby enabling appropriate precautions to be taken during the delivery, otherwise if the diagnosis is unknown, then it is essential for any attending clinician to urgently secure the airway by means of either intubation or tracheostomy. Such a case is presented with a review of the possible management options. This case emphasises the fact that although many conditions are uncommon, the total incidence of rare conditions is surprisingly high, and that care needs to be taken at all times in the management of patients, in order not to overlook such life-threatening diagnoses. ( info)

3/800. The blastic variant of mantle cell lymphoma arising in Waldeyer's tonsillar ring.

    We present three cases of blastic mantle cell lymphoma with an unusual initial manifestation in Waldeyer's ring with methods for differentiating it from other blastic neoplasms of the head and neck. All cases presented with a feeling of fullness in the area of the mass. Morphologically, the tumours were blastic with a high mitotic rate (three to nine per high power field). All were B-cell phenotype with coexpression of CD43. In all cases cyclin d1 and bcl-2 were positive and CD23 negative. Blastic mantle cell lymphoma occurring in Waldeyer's tonsillar ring may be mistaken for other high grade haematopoietic neoplasms. immunohistochemistry and awareness of this type of lymphoma are helpful in differentiating it from other neoplasms. ( info)

4/800. hemangiopericytoma of the nose: a case for both internal and external localization.

    BACKGROUND: Nasal and paranasal cavities are sites of predilection for hemangiopericytoma (HPC), a rare vascular tumor described for the first time in 1942. Because of their characteristics, i.e. clinical presentation, age distribution and biological behavior, nasal HPCs are frequently reported as "hemangioperycitoma-like intranasal tumors" although such a distinction from other HPCs is debated. patients, methods and results: out of nearly 64,000 autopsies and 336,000 surgical and endoscopic biopsies performed at the University of Trieste over 30 years, only three cases of HPC have been found, two of them affecting the external nose; for both there was a good agreement between histology--supported by immunohistochemistry and flow cytometry--and biological behavior. CONCLUSIONS: On the basis of the sparse literature on skin HPC and of our two cases, we suggest that not only the internal, but also the external nose should be considered a predilection site for HPC. ( info)

5/800. Aggressive giant pituitary adenoma presenting as a nasopharyngeal mass: magnetic resonance imaging and pathologic findings.

    We report a giant pituitary adenoma with aggressive histologic features that prominently invaded the nasopharynx. magnetic resonance imaging (MRI) demonstrated a large heterogeneous nodular mass that was hypointense to isointense on T1-weighted images and mixed hypointense, isointense, and hyperintense on T2-weighted images. The mass measured 7.5 x 5 x 7 cm, extending from the nasopharynx posteriorly through the clivus, and superiorly through the paranasal sinuses, and sellar-suprasellar region. After contrast administration, heterogeneous nodular enhancement was noted. A nasopharyngeal neoplasm extending into the sella was suspected because voice change and nasal speech long preceded the patient's visual symptoms. A biopsy disclosed an aggressive, infiltrating, hemorrhagic tumor, which was diagnosed as a non-secreting pituitary macroadenoma. This report indicates that pituitary adenomas may grow invasively to tremendously large sizes resulting in their initial presentation as nasopharyngeal masses. ( info)

6/800. Hyperfractionated radiotherapy followed by adjuvant chemotherapy for nasopharyngeal cancer: report of seven cases.

    Cases of hyperfractionated radiotherapy and adjuvant chemotherapy for nasopharyngeal cancer are reported. Seven patients received hyperfractionated radiotherapy (76.8-81.6 Gy/64-68 fractions to primary tumor) and two cycles of cisplatin (80 mg/m2 i.v. on day 1) plus 5-FU (800 mg/m2 continuous infusion on days 2-6). mucositis was the most frequent side effect in hyperfractionated radiotherapy. Moderate leukopenia was the major side effect of adjuvant chemotherapy. With a mean follow-up time of 34 months (range 25-48 months), five of the seven patients were locoregionally controlled. Two developed distant metastases. Two patients suffered late complications (posterior nasopharyngeal wall necrosis and brain necrosis). These results suggested that our regimen was almost well tolerated and might be of use in locoregional control of nasopharyngeal cancer. However, it carries some risk of late complications and might be inadequate for preventing distant metastases. A three-dimensional conformal boost irradiation technique and adequate dose intensity chemotherapy might be encouraged. ( info)

7/800. 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. ( info)

8/800. retropharyngeal abscess. A rare presentation of nasopharyngeal carcinoma.

    Early symptoms of nasopharyngeal carcinoma (NPC) can often be deceptive and confusing. Most patients with nasopharyngeal carcinoma present at an advanced stage with metastatic cervical nodes present at the time of diagnosis. A deep neck abscess as the presenting feature has not been reported. We report two cases of nasopharyngeal carcinoma which presented with retropharyngeal abscesses and persistent lymphadenopathy. These two patients illustrate that refractory lymphadenopathy, despite adequate treatment of the associated infection, should prompt a search for underlying disease. The relationship between nasopharyngeal carcinoma and retropharyngeal abscess is discussed. ( info)

9/800. Fractionated stereotactic radiation therapy for locally recurrent nasopharynx cancer: report of three cases.

    BACKGROUND: This article reports on experience with fractionated stereotactic radiation therapy (FSRT) for locally recurrent nasopharynx cancer. methods: Three patients with locally recurrent nasopharynx cancer were given FSRT as reirradiation between September 1995 and August 1996. Application of FSRT was the third radiation therapy in two patients. Authors used the individually made relocatable Gill-Thomas-Cosman (GTC) stereotactic frame, and the radiation dose planning was performed using XKnife-3. The total doses to the recurrent tumor were 45 Gy/18 fractions in two patients, who were given concurrent chemotherapy as a radiosensitizer, and 50 Gy/20 fractions in the other patient. In all three patients the dose per fraction was 2.5 Gy, and the fraction schedule was to give five daily treatments per week. RESULTS: Authors observed satisfactory symptomatic improvement and remarkable objective tumor size decrease through the magnetic resonance (MR) images taken one month post-FSRT in all three patients. No neurological side effect was observed. All three patients died with regional and distant seeding outside the FSRT field at seven, nine, and nine months, respectively. CONCLUSION: FSRT as reirradiation for locally recurrent nasopharynx cancer seemed to be effective and safe. ( info)

10/800. Melanin pigmented oncocytic metaplasia of the nasopharynx.

    A 64-year-old man presented with a history of discomfort of the throat of a few weeks' duration. Nasoscopic examination revealed multiple small, brown pigmentations at the left suprapharynx, the base of the left nasal cavity and the pharyngeal openings of the auditory tube on both sides. Microscopically, the lesion showed a glandular pattern of oncocytic epithelium with abundant pigmented granules and melanophages in the surrounding stroma. Immunohistochemically, the dendritic cells in the basal layer were positive for S-100 protein. Electron microscopic study revealed numerous fully melanized melanosomes and hypertrophied mitochondria in the oncocytic cells. Oncocytic cells do not produce melanin for themselves, melanin granules apparently being transferred from the adjacent dendritic cells to the oncocytic cells. ( info)
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