Cases reported "Head and Neck Neoplasms"

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1/160. Fourth branchial arch sinus: clinical presentation, diagnostic workup, and surgical treatment.

    OBJECTIVES/HYPOTHESIS: Abnormalities of the fourth branchial arch are much less common than those of the second arch and present in a different manner. The authors report their experience with five cases of fourth arch sinuses. methods: Retrospective chart review of patients at a tertiary care center. RESULTS: All patients presented in the first or second decade of life, and all but one had left-sided neck involvement. Four patients presented with recurrent low neck inflammatory episodes, and one with respiratory compromise. Diagnostic studies performed included ultrasound, computed tomography (CT) scan, barium swallow, magnetic resonance imaging, and fine-needle aspiration. barium contrast studies and CT scan were the most useful in demonstrating a fourth arch sinus tract preoperatively. Surgical treatment with an emphasis on complete exposure of the recurrent laryngeal nerve and exposure of the lateral piriform sinus to facilitate complete sinus tract excision was successful in all patients. CONCLUSION: The clinical history of recurrent low neck inflammatory episodes in young patients, especially on the left side, should raise the suspicion of this entity. Investigation using barium swallow in combination with CT scanning is useful. Excision of the sinus tract taking care not to injure the recurrent laryngeal nerve is curative.
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2/160. Midline posterior cervical cystic hygroma.

    Cystic hygromas are among the most common congenital neck masses. They have a predilection for the left posterior triangle and manifest early in life. Although various locations of cystic hygromas are found in the literature, we believe our case of a 9-month-old girl represents the first reported occurrence of a posterior cervical midline cystic hygroma.
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3/160. head and neck infantile myofibromatosis--a report of three cases.

    Infantile myofibromatosis (IM) is a proliferative disorder characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures. This proliferation may occur at any anatomical site, but in 30% of the cases it involves the head and neck. We report here three cases of head and neck IM occurring in young children and presenting as solitary lesions. The clinical heterogeneity and the misleading histopathological appearances can make the diagnosis difficult. The treatment is surgical but the low rate of recurrence and the possibility of spontaneous tumoral regression may lead to conservative surgery or therapeutic abstention.
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4/160. Incidental sampling of branchial remnants: a potential source of error in fine-needle aspiration of neck lesions-a case report.

    Remnants of the branchial apparatus can produce lesions in the head and neck region in later life, often amenable to fine-needle aspiration (FNA) diagnosis. Yet such remnants or rudimentary lesions can remain clinically undetected and can later interfere with the cytologic interpretation of other deep lesions of the neck, as the present case demonstrates. In this case the lesion, which by a subsequent resection turned out to be a neurilemmoma, had been adequately sampled by the FNA, yet the cytologic diagnosis was sidetracked by the presence in the specimen of immature squamous epithelial tissue fragments and other elements (multinucleated histiocytes, calcifications), on the basis of which the diagnosis of an epithelial lesion, likely malignant, was made. The neck surgery and a preceding endoscopic examination of the mouth, pharynx, and larynx did not identify such a lesion, but a detailed microscopic examination of the fibroadipose tissue between the tumor and the peripharyngeal region revealed the presence of epithelial microfragments with morphology partly corresponding to that of the FNA cytology, highly indicative of a branchiogenic lesion in the peripharyngeal region. The basic embryology of the branchial apparatus resulting in such defects is presented, as well as tentative guidelines for recognizing material deriving from accidental sampling of such lesions during FNA investigations of deep-seated masses of the neck. Diagn. Cytopathol. 2000;22:157-160.
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5/160. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure.

    A case of scleredema diabeticorum of Buschke associated with nuchal fibroma and organic solvent exposure is reported. The patient presented with a neck mass causing discomfort and restriction of movement. Histological examination showed this to be a nuchal fibroma. Additionally, there was widespread induration of the skin of his trunk which was asymptomatic. A biopsy showed features of scleredema. This is the first reported association of these two conditions, both of which show increased and thickened collagen bundles without significant fibroblast proliferation. They differ by the occurrence of mucin in scleredema, although this is not always demonstrable, particularly in late lesions. The possibility that nuchal fibroma is an end stage, localized form of scleredema is canvassed. The patient's medical history included insulin-dependent diabetes mellitus with complications of retinal vessel thrombosis and peripheral neuropathy. The patient also had significant past exposure to a wide variety of chemicals, including organic solvents.
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6/160. Intraosseus teratoma of the iliac bone.

    We present a 4-year-old child with a large iliac bone mass incidentally discovered in a plain abdominal radiograph. The pathological examination revealed a benign mature teratoma. To the best of our knowledge, the occurrence of intraosseous mature teratoma has not been previously reported. The child had had an immature teratoma of the neck discovered in a fetal ultrasound, and resected on day 6 of life. The neck teratoma recurred twice, at 16 months and at 3.5 years of age. In these two recurrences the lesion appeared progressively more mature. At the time of discovery of the iliac bone teratoma there was no evidence of residual neck disease. The radiological and pathological characteristics, differential diagnosis, and clinical course are discussed.
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7/160. Malignant peripheral nerve sheath tumor arising in benign ancient schwannoma: a case report with an immunohistochemical study.

    A rare example of malignant transformation in an ancient schwannoma arising in the right side of the neck of a 51-year-old man without any clinical manifestations suggesting neurofibromatosis is described. The tumor, approximately 4 cm at its largest dimension, was well circumscribed and had a direct connection with the sympathetic nerve. Microscopically, the central portion of the tumor showed features of ancient schwannoma characterized by extensive hyalinization with cystic degeneration, scattered spindle cells with hyperchromatic and tapered nuclei, and some symplastic changes. However, predominantly in the outer portion, a proliferation of spindle-shaped cells with enlarged nuclei was present. The nuclei of these cells showed irregular contours, coarse granular chromatin texture, and conspicuous nucleoli. Mitotic figures and small necrotic foci with scattered apoptotic bodies were also seen. Immunohistochemically, S-100 protein was almost negative in areas consisting of overtly atypical cells where the mitotic index evaluated with MIB-1 antibody was 30.5%. In contrast, S-100-positive bland spindle cells were scattered in an extensively hyalinized area with a labeling index less than 3%. P53 protein was strongly positive in atypical spindle cells. Although it is a very uncommon event, definite nuclear atypia, frequent mitotic figures, and the existence of small necrotic foci should be recognized as indicating a diagnosis of malignant degeneration of benign schwannoma. immunohistochemistry would be useful as an ancillary technique in such a setting.
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8/160. A review of myeloproliferative disease with presentation in the head and neck region.

    The diagnosis of an essential thrombocytosis is demonstrated in this presentation of a well-looking 53 year old man who had a five-year history of increasing facial asymmetry as evidenced by deviation of his mandible to the right and malocclusion. The enlarged mandibular condyle was the first manifestation of his underlying myeloproliferative disorder. His management will be discussed. Neoplastic diseases of the multipotent haematopoietic stem cells result in four major diseases: chronic myelogenous leukaemia (CML); polycythaemia vera (PV); agnogenic myeloid metaplasia with myelofibrosis (AMM/MF); essential thrombocytosis (ET). CML: demonstrates increased production of neutrophils and marked splenomegaly. It is divided into a chronic phrase typified by hyperplasia of mature bone marrow elements and a blastic or acute phase which evolves into a proliferation of immature marrow elements and can develop into acute myelogenous leukaemia. PV: associated with increased production of all myeloid cells but dominated by increased red blood cells with splenomegaly. AMM/MF: allows the neoplastic stem cells to proliferate and lodge in multiple sites outside the bone marrow. splenomegaly and fibrosis of marrow spaces also occurs. ET: resulting in a markedly elevated platelet count in the absence of a recognizable stimulus. Treatment revolves around measures to maintain hydration, to relieve arthralgias, to prevent thrombotic episodes, and to prevent infections.
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9/160. Cystic hygroma of the head and neck--a long-term follow-up of 44 cases.

    Cystic hygroma of the neck is a relatively rare congenital malformation usually diagnosed during the first years of infancy. Complete surgical extirpation may be impossible without sacrificing important neurovascular structures. This paper reports the long-term outcome of surgical treatment during a 35-year period. A follow-up examination of 44 patients (24 males and 20 females) treated in our departments during the last 35 years was performed. Median age at first operation was 1.5 years (0-28 years) The observation period ranged between 1 and 36 years, median 16.2 years. Indications for operation were space occupying lesion, haemorrhage, dysphagia, difficulties in pronunciation or breathing and infection or nerve lesions. Fifty percent of the patients revealed residual or recurrent hygroma at the time of follow-up. Forty-four percent suffered from impaired speech, food intake, breathing or swallowing. Thirty-six percent were cosmetically bothered, and only 11% reported reduced quality of life. A significant correlation was noted between the extension of the lesion and (i) the number of operations performed and (ii) the rate of recurrent or residual hygroma. The rate of residual or recurrent hygroma was statistically higher for the suprahyoid lesions compared with the infrahyoid lesions. The localization and extent of the lesion is related to the surgical outcome. Surgical intervention should be centralized and should be considered carefully. Neurovascular structures should not be damaged in an attempt to effect complete removal. The above-mentioned results lead to a search for a new therapeutic modality, and the authors have recently taken up the Japanese way of treating hygromas by intralesional injection of OK-432. The first two patients treated by this technique had total regression of the lesion, and the method seems to be promising as an alternative to surgery.
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10/160. Intravascular papillary endothelial hyperplasia (Masson's tumor) manifesting as a lateral neck mass.

    Intravascular papillary endothelial hyperplasia is a benign lesion of vascular origin that is caused by an excessive proliferation of endothelial cells in normal blood vessels or vascular malformations. We report the case of a 26-year-old man who had such a lesion deep within the soft tissues of his neck. Imaging studies revealed a 6-cm-diameter mass, with its epicenter in the right retromandibular space. The mass extended into the right parapharyngeal space and compressed the pharynx. The mass was excised, and the patient recovered uneventfully. We discuss the management of this lesion, with emphasis on radiologic and histologic assessment and the differential diagnosis.
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