Cases reported "Nose Neoplasms"

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1/67. Transnasal endoscopic repair of congenital defects of the skull base in children.

    OBJECTIVE: To examine imaging findings and methods of endoscopic treatment of congenital skull base defects in children. DESIGN: Retrospective study and case series. SETTING: Academic tertiary care center. patients: Four patients (aged 12 and 14 months and 8 and 13 years) were included from 1995 to 1997. Three presented with a nasal glioma, which was recurrent in 1 case. The fourth patient presented with bacterial meningitis due to a spontaneous cerebrospinal fluid leak. Computed tomography and magnetic resonance imaging were used to locate the defect of the skull base. INTERVENTION: Transnasal endoscopic resection of the glioma or the meningocele, with immediate repair of the skull base defects using free mucosal flaps and/or pediculized mucosal flaps and/or conchal cartilage together with fibrin glue and nasal packing during a 3-week period. RESULTS: None of the 4 patients has experienced recurrent cerebrospinal fluid leaks or postoperative meningitis. CONCLUSIONS: The transnasal endoscopic repair of congenital meningoceles is a reliable technique in select pediatric patients. Computed tomography and magnetic resonance imaging provide information that can be used to help the surgical procedure.
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2/67. Small cell neuroendocrine carcinoma with skeletal muscle differentiation: report of three cases.

    Three cases of neuroendocrine carcinoma showing skeletal muscle differentiation are presented. The tumors were located in the skin and subcutaneous tissue, the urinary bladder, and the nasal cavity respectively, and were composed by two cell types admixed intimately with each other. One cell type had features identical to those seen in conventional small cell neuroendocrine carcinoma, including scanty cytoplasm, round nuclei with fine granular chromatin, immunohistochemical reactivity for neuron-specific enolase, chromogranin and cytokeratins, and electron-dense granules on ultrastructural examination. The second cell type was either plasmacytoid or elongated and straplike, with abundant eosinophilic cytoplasm and irregular nuclei with prominent nucleoli. These cells showed immunohistochemical positivity for desmin, sarcomeric actin, myoglobin, and myogenin. They also exhibited ultrastructural evidence of rhabdomyoblastic differentiation in the form of contractile filaments with abortive Z-band formation. An origin from a cell capable of dual differentiation toward neuroendocrine and rhabdomyoblastic elements is postulated for these tumors.
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3/67. Intracranial nasal dermoid sinus cyst associated with colloid cyst of the third ventricle. Case report and new concepts.

    A case of a 16-year-old male with both a nasal dermoid sinus cyst (NDSC) and a third ventricle colloid cyst is presented. The NDSC was excised via a single-stage combined intracranial-extracranial approach and the third ventricle colloid cyst was resected endoscopically. The pathogenetic theories of NDSC and third ventricle colloid cyst are discussed, and an embryological explanation for the simultaneous development of the two lesions in this patient is explored. This case is best classified among congenital developmental malformations in a category we propose to call 'anterior neuropore corridor defects.'
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4/67. Extraintestinal polyps in peutz-jeghers syndrome: presentation of four cases and review of the literature. Deutsche Peutz-Jeghers-Studiengruppe.

    peutz-jeghers syndrome (PJS) is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. Extraintestinal polyps have rarely been reported. Possible sites include the respiratory tract, urogenital tract, and gallbladder. We here describe four cases of extraintestinal polyps in PJS patients and review the literature on the need for operative therapy of extraintestinal polyps in PJS. Three nonrelated patients were examined who had PJS and polyps in the gallbladder; the fourth patient had PJS and recurrent choanal polyps. Surgery has so far been performed only for symptomatic polyps: one laparoscopic cholecystectomy and removal of the choanal polyps for recurrent infections of the respiratory tract. The remaining two patients reported no symptoms from the extraintestinal polyps. No malignant transformation was found in these patients, nor has such been reported in the literature on PJS. The frequent observation of this manifestation in our patients raises the question of clinical management: Is prophylactic surgery indicated? Since malignant transformation of PJS polyps in the intestine is extremely rare we see no reason for operative therapy as long as the polyps are small and asymptomatic. Regular sonographic controls are recommended since the risk of malignant transformation cannot be ruled out at present.
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5/67. Case report: Sinonasal teratocarcinosarcoma.

    Sinonasal teratocarcinosarcoma (SNTCS) is a rare, teratoma-like lesion of the nasal cavity and paranasal sinus. To the best of our knowledge, SNTCS is highly malignant. We report a case of SNTCS arising in the nasal cavity of a 71-year-old male who complained of nasal obstruction and epistaxis. In spite of intensive treatment, the tumor recurred three times and the patient died from a local extension into the anterior cranial fossa 7 years after initial onset. The resected tumors consisted of variegated components, such as epithelial elements, including cystic, ductal and glandular structures occasionally associated with squamous differentiation, neuroectodermal ones exhibiting neural rosette formation, and mesenchymal ones with prominent rhabdomyoblastic differentiation. Immunohistochemical and ultrastructural studies clearly demonstrated characteristic cellular differentiation of each component. These three principal elements were often topographically related and showed morphological transition with each other. These findings suggest the derivation of divergent components from common progenitor cells. Although the cellular atypia of the primary lesion was inconspicuous, the recurrent tumors became anaplastic and mitotically active. Histologic anaplasia may be somewhat related with aggressiveness of recurrent lesions. Appropriate sampling of specimens, and awareness of this rare teratoid tumor are important to make the correct diagnosis.
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ranking = 475.50915645217
keywords = rosette, formation
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6/67. A case of solitary nasal schwannoma.

    A case of solitary neurilemmoma is presented. The patient was a 35-year-old male with progressive nasal obstruction due to a neoformation of the left vestibule. After the surgery the pathological diagnosis was neurilemmoma.
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7/67. Collision of squamous-cell carcinoma with melanoma in situ in a child with xeroderma pigmentosum.

    Coexistence (collision) of two different neoplasms in the same lesion has previously been documented by several authors. In this report, we describe a 13-year-old boy with xeroderma pigmentosum presenting with squamous-cell carcinoma and melanoma arising at the same site on the nose. Histopathologically, the melanoma component of the lesion was located mainly eccentrically to the squamous-cell carcinoma component. Immunohistochemical stains confirmed the histopathologic findings. Mutations for p53 assessed using single-strand conformation polymorphism, and sequencing analysis revealed a CC-to-TT transition at codon 159 of the p53 gene in the squamous-cell component but not in the melanoma component. This finding suggests a possible role for UV in the pathogenesis of at least the squamous-cell component of the tumor. To the best of our knowledge, this is the first report of a collision tumor comprising squamous-cell carcinoma and melanoma arising in childhood.
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8/67. Malignancies arising in oncocytic schneiderian papillomas: a report of 2 cases and review of the literature.

    Oncocytic schneiderian papillomas (OSPs) are uncommon benign neoplasms that arise from the sinonasal schneiderian epithelium. Malignancies arising in OSPs are rare, and, to our knowledge, only 14 such instances have been reported in the medical literature. We report 2 additional cases--a small cell carcinoma and a sinonasal undifferentiated carcinoma arising in OSPs and presenting synchronously with the benign neoplasm. The potential for malignant transformation in OSPs is small, but warrants that these papillomas be completely excised to exclude a coexisting carcinoma.
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9/67. Nasal dermoid sinus cysts and the role of open rhinoplasty.

    All suspected congenital abnormalities of the nose require further evaluation. The nasal dermoid sinus cyst (NDSC) is one of the many midline nasal masses that often pose diagnostic and treatment dilemmas for the plastic and reconstructive surgeon. NDSCs are distinct from other facial dermoids in their potential for involving deeper contiguous structures, and intracranial extension. Accurate diagnosis and effective treatment are essential to avoid craniofacial skeletal deformation, cyst rupture, and infection that could cause cutaneous, ocular, or intracranial complications. A comprehensive discussion of the embryogenesis, pathogenesis, diagnosis, and surgical management of the NDSC is presented to delineate the role of open rhinoplasty in optimizing the management of this congenital nasal deformity.
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10/67. Fine needle aspiration cytology of metastatic olfactory neuroblastoma: a case report.

    BACKGROUND: Olfactory neuroblastoma (ONB) is an uncommon tumor, presenting as a polypoid mass arising from the upper nasal cavity. This tumor has been seldom diagnosed by direct fine needle aspiration (FNA). CASE: Metastatic ONB was diagnosed by FNA. The patient was a 40-year-old female with a polypoid mass in the nasal cavity and ipsilateral cervical lymphadenopathy. The punch biopsy of the nasal tumor revealed a smudged small round cell neoplasm with neuroendocrine differentiation, consistent with ONB. In FNA smears from the cervical lymph node, there were well-preserved, small, monotonous cells with hyperchromatic nuclei, fibrillary cytoplasm and indistinct cell borders. Also noteworthy were occasional pseudorosettes as well as rare true rosettes. By immunocytochemistry, tumor cells were positive for cytokeratin, chromogranin and synaptophysin. CONCLUSION: ONB, like adrenal neuroblastoma, shows distinctive cytologic features, including a rosette or pseudorosette and fibrillary network. FNA can accurately demonstrate these characteristic findings, and in some cases it may be a better diagnostic modality than incisional biopsy.
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ranking = 1898.0366258087
keywords = rosette
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