Cases reported "Nose Neoplasms"

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1/17. Nasal angiocentric lymphoma: an entity that should be remembered.

    After four nasal aesthetic functional surgeries in a period of 18 months, a 46-year-old woman was evaluated who presented with moderate functional alteration, saddle-nose deformity, and total loss of the septal cartilage. Four months before presentation the patient sustained severe nasal trauma, resulting in depression of the nasal bridge without loss of function. Her problem was diagnosed initially as a consequence of an infected septal hematoma and loss of the septal cartilage. Based on this diagnosis, the patient was subjected, in an 18-month period, to four reconstructive surgeries by different specialists, without any improvement and with worsening of clinical presentation. During the authors' physical examination of the patient, she demonstrated marked nasal cutaneous retraction, atrophic nasal conchae with total loss of the septal cartilage, and a large loss of septal bone. Three nasal mucosa biopsies were acquired and the authors proceeded to carry out complete nasal reconstruction using external cranial table and rib cartilage. Histopathologically, a lesion was noted that was compatible with angiocentric lymphoma, for which treatment was administered according to this type of illness. The authors point out the importance of establishing an adequate diagnosis in the face of an apparently obvious clinical case, present cross-disciplinary treatment, and discuss the study protocol that should be used for this type of pathology. They present their reconstructive technique of the nasal structure using a combination of bone tissue and cartilage, the results, and the current state of the patient.
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2/17. Nasofrontal dermoid sinus cyst: report of two cases.

    OBJECTIVE AND IMPORTANCE: Nasofrontal dermoid sinus cysts are rare. The embryological origin, presentation, treatment, and genetic associations of two cases of these cysts are discussed. Emphasis is placed on physical findings and the importance of addressing both the intracranial and extracranial components. CLINICAL PRESENTATION: The first patient, a 33-year-old woman, sought care for chemical meningitis. As a child, she was differentiated from her identical twin sister by a dimple on the tip of her nose. The second patient, a 34-year-old man, sought care for new-onset seizures. Since birth, he had a dimple on the tip of his nose. As a child, he had undergone resection of a nasal cyst. Imaging studies in both patients indicated a midline anterior cranial base mass within the falx and a defect in the crista galli. INTERVENTION: Both patients underwent biorbitofrontal nasal craniotomy. A bifrontal craniotomy was performed first, then removal of the orbitonasal ridge. The dermoid and involved falx were resected. The sinus tract was followed through the crista galli and resected up to the osteocartilaginous junction in the nose. The remainder of the tract was resected via a small incision through the nares. The dura was closed primarily by mobilizing the dura along the sides of the crista galli. After surgery, both patients still possessed their sense of smell. CONCLUSION: Nasofrontal dermoid sinus cysts have a unique embryological origin. A midline basal frontal dermoid associated with a dimple on the nasal surface with or without protruding hair and sebaceous discharge is the pathognomonic presentation. It is important to address both the intracranial and extracranial component surgically. Although concomitant anomalies and familial clustering have been described, most cases are spontaneous occurrences.
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3/17. Chondroid chordoma of the nasal septum.

    Chondroid chordoma is an uncommon malignant tumor of bone that occurs mostly in patients of early middle age. To our knowledge, this tumor has not been previously described in the nasal septum as a primary lesion. We describe a 39-year-old woman who presented with a 1-year history of nasal obstruction. Results of her physical examination and imaging studies demonstrated a nasoseptal mass. We also discuss the histologic and radiographic characteristics of the chondroid chordoma and compare it with other tumors that arise in the nasoseptal area to highlight the different prognoses and approaches to management.
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4/17. Use of radial forearm free flap with palmaris longus tendon in reconstruction of total maxillectomy with sparing of orbital contents.

    Nasal paragangliomas are extremely rare. The most adequate treatment is total excision. After surgical excision requiring total maxillectomy, there has been no ideal technique for reconstruction. A 47-year-old man was admitted to our clinic because of recurrent epistaxis, which lasted for 2 months. He was also suffering from nasal airway obstruction. The physical examination revealed a mass originating from the medial aspect of the middle turbinate of the right nasal cavity. It invaded the anterior maxillary wall and hard and soft palate. Endoscopic examination showed that the mass pushed the nasal septum to the left side and protruded into the nasopharynx. The mass was fleshy and had a rich capillary network. Conventional paranasal sinus radiographs were normal. Computerized tomography of the skull showed the mass protruding into the nasopharynx. A total maxillectomy was performed. Histopathological evaluation showed neoplastic tissue consisting of round, oval, or slightly elongated cells, altogether of a rather monomorphous appearance, tending to arrange themselves in clusters adjacent to or around capillary blood vessels. The blood vessels were numerous and branched. reticulum staining showed a typical Zellballen arrangement of the neoplastic cells to provide a firmer basis for the diagnosis of paraganglioma. To reconstruct the total maxillectomy defect, a radial forearm free flap with the palmaris longus tendon was elevated to inlay the nasal cavity and the oral cavity and to suspend the ocular globe. The flap was placed into the defect, and the palmaris longus tendon was medially and laterally anchored to the periosteum of the frontal bone to suspend the ocular globe in the orbital cavity. One part of the skin island was used to close the defect of the nasal mucosal cavity, and the other part was used to repair the oral mucosal defect of the palate. Consequently, speech was considered near normal; the patient was able to eat an unrestricted diet and to retain both solid and liquid food inside the oral cavity without drooling, and there was no diplopia or enophthalmos. Six months later, porous polyethylene was inserted and fixed to the zygomatic bone with a miniplate and miniscrews to restore malar contour. No further procedure was believed to be necessary later on. Two years later, a satisfactory and functional esthetic result was obtained, providing an acceptable suspension of the ocular globe and filling of the total maxillectomy defect. We believe that a total maxillectomy is indicated if it is needed in nasal paragangliomas and that microsurgical repair with the composite radial forearm-palmaris longus free flap has several advantages: 1) it can offer en bloc reconstruction of the entire defect after a total maxillectomy in terms of good function and cosmesis; 2) it can repair mucosal defects; and 3) it can anchor and suspend the ocular globe in its original anatomical location, protecting against gravity through the sling effect of the palmaris longus tendon. The composite radial forearm-palmaris longus free flap has not been described previously for suspension of the ocular globe.
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5/17. Aggressive psammomatoid ossifying fibroma of the inferior turbinate and lateral nasal wall.

    OBJECTIVE: Fibroosseous lesions are rare entities of the nose and paranasal sinuses, the terminology and classification of which are still confusing. Psammomatoid or aggressive (juvenile) ossifying fibroma is a benign messenchymal tumor usually met in the young age (5-15 years), in the sinonasal tract, bearing distinctive histomorphologic features and a tendency towards locally aggressive behaviour. METHODOLOGY: We report here a rare case of an aggressive psammomatoid ossifying fibroma of the inferior turbinate and the lateral nasal wall, with obstruction of the nasolacrimal duct, in a 68-year-old woman. RESULTS: diagnosis was based on physical examination, CT scan imaging and histopathological examination. Treatment consisted of endoscopic intranasal resection of the tumor accompanied by removal of the lateral nasal wall. CONCLUSIONS: Fibro-osseous tumors of the nose and paranasal sinuses require aggressive surgical approach in order to avoid recurrence. Complete surgical excision may not always be possible mainly due to the tumor's extent and location.
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6/17. Endoscopic sinus surgery treatment for a huge sinonasal fibroma.

    Fibromas are rare tumors of the nasal cavity, which may result from progressive inflammation or fibroblastic proliferation of the nasal mucosa. The tumors are usually too small to cause symptoms. We present a 47-year-old woman suffering through right nasal obstruction, purulent rhinorrhea and severe headaches for 6 months. A gray-white, smooth-surfaced, gigantic firm mass occupying the right nostril was found in physical examination. Sinus computed tomography revealed 4 x 3 x 3 cm soft-tissue-density mass in the right nasal cavity and right maxillary sinusitis. The huge sinonasal fibroma measuring 4.5 x 3 x 3-cm in the right posterior ethmoid sinus, which was successfully endoscopically resected. The final diagnosis of fibroma was made histologically, according to light microscopy and immunohistochemical stain examinations, which were important for determining the patient's treatment. After endoscopic resection, her initial signs and symptoms were relieved and no recurrence was noted after 2 years of follow up.
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7/17. Recurrent epistaxis from an intranasal glomus tumor: the 22nd case report.

    The case of a 66-year-old Thai woman who was treated at King Chulalongkorn Memorial Hospital due to recurrent epistaxis for two years is presented. Her physical examination revealed a 0.7-cm tumor mass on the right nasal septum. A biopsy was subsequently done and the tissue was sent for pathological examination. The lesion was then diagnosed as "glomus tumor of the nasal septum". Histologically, the tumor was rather well-circumscribed and located in the submucosal region. The neoplastic cells were uniform, round to ovoid in shape and contained bland-looking, finely chromatic nuclei and moderate amount of cytoplasm with ill-defined cell borders. These cells were intervened by tortuous vascular structures. Immunohistochemically, the tumor cells were strongly reactive to smooth muscle actin and negative to cytokeratin and S-100 protein. A glomus tumor is rare in the sinonasal region. So far, there have been only 21 reported cases in the literature. Almost all cases of sinonasal glomus tumor are benign and are usually cured by complete excision.
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8/17. Body cavity-based presentation of natural killer cell lymphoma.

    We describe an unusual case of a 31-year-old Mexican woman who presented with pleural and peritoneal effusions involved by Epstein-Barr virus-positive non-Hodgkin's lymphoma of natural killer (NK)-cell lineage. The patient had no symptoms that could be related to her nasal region, and physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other extranodal masses. Thus, this case clinically mimicked body cavity-based lymphoma. Extranodal NK/T-cell lymphoma of nasal type is the current designation for these neoplasms in the recently proposed world health organization classification of lymphoid neoplasms. These tumors previously have been referred to many other names, including lethal midline granuloma, midline malignant reticulosis, polymorphic reticulosis, angiocentric immunoproliferative lesion, and angiocentric lymphoma. Nasal-type NK/T-cell lymphomas typically involve the nasal region, but may involve other extranodal sites, such as skin and gastrointestinal tract. The malignant cytologic features and the presence of azurophilic granules within the cell cytoplasm observed in Wright-Giemsa-stained cytocentrifuge preparations led to immunophenotypic and molecular genetic studies that were essential in establishing the correct diagnosis. As demonstrated in the case reported, extranodal NK/T-cell lymphomas of nasal-type can be clinically aggressive and may be associated with paraneoplastic phenomena.
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9/17. Recurrent inverted papilloma of a pediatric patient: clinico-radiological considerations.

    Inverted papilloma (IP) of the nasal cavity and paranasal sinuses is an uncommon pathology, especially in the pediatric population. We present a pediatric case of recurrent IP in the left nasal cavity. The physical examination of a 9-year-old girl revealed a polypoid mass filling the middle meatus in the left nasal cavity. Purulent secretion and adenoid vegetation obstructing the choanea were also noted. Coronal and axial maxillo-facial computed tomography demonstrated a mass in the nasal cavity, enlarging the left middle meatus. We discussed the imaging findings and treatment of IP and concluded that it should have been included in the differential diagnosis of nasal mass lesions in the pediatric population.
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10/17. Misdiagnosis of olfactory neuroblastoma.

    OBJECT: Olfactory neuroblastoma (ON) is a rare neoplasm arising from the olfactory epithelium and found in the upper nasal cavity. The authors studied the frequency with which ON is misdiagnosed with other tumors of the paranasal sinuses such as neuroendocrine carcinoma (NEC), pituitary adenoma, melanoma, lymphoma, and sinonasal undifferentiated carcinoma (SNUC). Based on the belief that misdiagnosis commonly occurs, they emphasized the importance of establishing the correct diagnosis, because the treatment regimens and prognosis of these tumor types are often significantly different. methods: Twelve consecutive patients in whom ON was diagnosed were referred to the Department of neurosurgery at the M. D. Anderson Cancer Center between January 1998 and March 2000. Demographic data were collected, physical findings and mode of treatments were documented, and neuroimaging studies were assessed. Pathologists at the authors' institute reviewed the histological specimens. Only in two of 12 patients was the diagnosis of ON confirmed. Lesions in 10 patients were misdiagnosed; there were two cases of melanoma, three cases of NEC, three cases of pituitary adenoma, and two cases of SNUC. Eight of 10 patients in whom lesions were misdiagnosed required significant alteration in the initially proposed treatment plan. CONCLUSIONS: Neurosurgeons should be acutely aware of the variety of neoplasms that occur in the paranasal region. The correct diagnosis should be ensured before initiating treatment to provide the optimum therapy and spare the patients from needless and potentially toxic treatment.
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