Cases reported "Nasopharyngeal Neoplasms"

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1/30. Re-irradiation for recurrent rhabdomyosarcoma of the nasopharynx in a child.

    There are few clinical situations where high dose re-irradiation with curative intent is indicated in children. Because of the potential for serious late sequelae, re-irradiation is considered only in the unusual situation where there is just local recurrence and surgery and/or chemotherapy are unlikely to provide long-term control. The authors present a case of nasopharynx rhabdomyosarcoma in a child which recurred after high dose irradiation and chemotherapy and was then re-irradiated to a high dose. The child is well without any serious sequelae more than 13 years following completion of the second course of radiotherapy.
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keywords = rhabdomyosarcoma
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2/30. rhabdomyosarcoma of the adult nasopharynx.

    rhabdomyosarcoma of the adult head and neck is rare, particularly beyond 40 years of age. In this region, the nasopharynx is an unusual site. Most nasopharyngeal rhabdomyosarcomas are of an alveolar variety. We report a case of embryonal or spindle cell rhabdomyosarcoma in the nasopharynx of a 47-year-old man. The histology of this tumour revealed clear cells that have not been described in embryonal rhabdomyosarcomas occurring in the head and neck. The pathology is discussed and current literature reviewed.
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ranking = 0.6
keywords = rhabdomyosarcoma
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3/30. Nasopharyngeal rhabdomyosarcoma in a patient with hypohidrotic ectodermal dysplasia syndrome.

    Hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome) is characterized by partial or complete absence of sweat glands, hypotrichosis, hypodontia, prominent frontal ridges and chin, saddle nose, sunken cheeks, thick, everted lips, large ears and sparse hair. While association of other ectodermal dysplasia syndromes with tumors such a non-Hodgkin's lymphoma, hamartoma, keratoakanthoma, Merkel-cell cancer, squamous-cell carcinoma, syringofibroadenomatosis has been reported, association of hypohidrotic ectodermal dysplasia and a tumor has not been reported. In a five-year-old male patient admitted with nasal obstruction and nasal mass complaints, we have reported firstly an association of hypohidrotic ectodermal dysplasia and nasopharyngeal rhabdomyosarcoma.
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keywords = rhabdomyosarcoma
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4/30. Clinics in diagnostic imaging (90). Childhood nasopharyngeal carcinoma.

    An 11-year-old boy presented with a nasopharyngeal mass that was thought to represent a juvenile angiofibroma based on the initial clinical and radiological evaluation. Partial tumour resection was performed. Resected specimen revealed histological diagnosis of undifferentiated carcinoma. Further evaluation of the tumour including MR imaging, radioisotope bone scan, CT thorax and abdomen were performed. Differential diagnoses of childhood nasopharyngeal masses were discussed. The differences between childhood NPC and adult NPC, rhabdomyosarcoma, malignant lymphoma and juvenile nasopharyngeal angiofibroma were also discussed.
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keywords = rhabdomyosarcoma
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5/30. Nasopharyngeal rhabdomyosarcoma and multiple lentigines syndrome: a case report.

    An 8-year-old boy with multiple lentigines syndrome presented with a nasopharyngeal rhabdomyosarcoma. The tumour failed to respond to chemotherapy. This is a further association of rhabdomyosarcoma with a congenital syndrome.
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ranking = 1.2
keywords = rhabdomyosarcoma
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6/30. Long-term follow-up and prognosis of orbital apex syndrome resulting from nasopharyngeal rhabdomyosarcoma.

    PURPOSE: Nasopharyngeal rhabdomyosarcoma may present with a variety of ophthalmic symptoms. Direct extension of the tumor into the orbital apex can lead to ophthalmoplegia and loss of vision. The prognosis for recovery of vision and ocular motility in patients with an orbital apex syndrome due to nasopharyngeal rhabdomyosarcoma is examined. DESIGN: Retrospective case series. methods: Six eyes from four patients with nasopharyngeal rhabdomyosarcoma who presented to the Children';s Hospital of philadelphia with a clinical orbital apex syndrome were identified. Complete ophthalmic examination, including visual acuity and extraocular motility at the time of presentation, was reviewed. Tumor extension into the orbital apex was confirmed radiographically. Follow-up ophthalmic evaluations were reviewed for all patients with an average follow-up of 5.5 /- 3.1 years (range 1 to 8 years). RESULTS: Six eyes of four patients had limited ocular ductions along with marked loss of vision at presentation. All patients were treated with chemotherapy and radiation, with reduction of tumor mass. Ocular motility recovered in all patients, occurring by an average of 2.2 /- 1.8 months after initiation of therapy. Four of six eyes had little or no recovery of visual acuity detected at long-term follow-up. CONCLUSIONS: patients with ocular motor deficits in orbital apex syndromes caused by extension of nasopharyngeal rhabdomyosarcoma have an excellent prognosis for recovery after treatment of the tumor. The long-term prognosis for visual recovery, however, is poor.
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ranking = 1.6
keywords = rhabdomyosarcoma
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7/30. parathyroid hormone expression in a patient with metastatic nasopharyngeal rhabdomyosarcoma and hypercalcemia.

    Ectopic PTH secretion by tumor cells has been described as the cause of hypercalcemia associated with malignancy in the absence of osteolytic bone lesions. Although there have been case reports of elevated PTH and hypercalcemia in patients with rhabdomyosarcoma, to date ectopic PTH secretion by malignant cells has not been definitively shown. The possibility of PTH production by pleural-based metastatic nasopharyngeal rhabdomyosarcoma cells in a 62-yr-old Japanese male with hypercalcemia was investigated. The patient's serum PTH level was found to be elevated at 62.22 pmol/L, and pleural fluid PTH level was 47.28 pmol/L and PTHrP level was 3.7 pmol/L. RT-PCR of mRNA extracted from rhabdomyosarcoma cells in the pleural fluid was performed with the addition of PTH and PTHrP exonic primer sets yielded only a cDNA fragment of approx 150 bp consistent with the expected PTH fragment. sequence analysis of a nested primer PCR fragment confirmed PTH mRNA sequence. We believe this patient to have had hypercalcemia secondary to ectopic PTH secretion, as we have identified the presence of PTH mRNA in tumor cells. We speculate that the overexpression of PTH in rhabdomyosarcoma cells results from molecular rearrangement of the PTH gene. The finding of a normal PTH dna sequence of the PCR fragment suggests the likelihood of alterations in regulatory sequences.
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ranking = 1.6
keywords = rhabdomyosarcoma
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8/30. Embryonal rhabdomyosarcoma of adult nasopharynx.

    adult rhabdomyosarcomas in the head and neck are extremely rare and carry a poor prognosis. They should be considered as a distinct clinical entity. The authors report a case of embryonal rhabdomyosarcoma in an adult nasopharynx treated with a combined modality treatment of chemotherapy and radiotherapy as per the European International Society of Paediatric Oncology (SIOP) MMT 89 study, group D. The patient responded to treatment and 10 years later, he is still alive with no signs of metastatic disease.
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ranking = 1.2
keywords = rhabdomyosarcoma
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9/30. Acquired Chiari malformation type I following fractionated radiation therapy to the anterior skull base in a 20-month-old boy. Case report.

    The authors present the case of a 20-month-old boy who underwent fractionated radiation therapy to the paranasal sinuses and anterior skull base during treatment for nasopharyngeal parameningeal rhabdomyosarcoma. Subsequent magnetic resonance imaging demonstrated progressive development of a Chiari malformation Type I (CM-I) and partial hypoplasia of the posterior fossa. Since the tonsillar herniation was discovered, the child, now 3 years old, remains asymptomatic except for mild, intermittent neck discomfort. For the time being, his family has elected for him to undergo clinical and neuroimaging follow up. The authors believe this is the first report of a progressive acquired CM-I after cranial irradiation in the pediatric population.
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ranking = 0.2
keywords = rhabdomyosarcoma
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10/30. Lipid-rich rhabdomyosarcoma--a potential source of diagnostic confusion.

    rhabdomyosarcoma is an often primitive tumor capable of diverse morphologic manifestations. The article describes three cases of childhood rhabdomyosarcoma in which a significant population of lipid-rich tumor cells was present. The lack of specificity and potentially confusing nature of this feature are discussed, and the ultrastructural diagnosis of rhabdomyosarcoma is briefly reviewed.
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ranking = 1.2
keywords = rhabdomyosarcoma
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