Cases reported "Choristoma"

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21/88. tongue gastric choristoma: failure to localize by technetium-99m pertechnetate scan.

    Congenital rests of gastric epithelium have been reported in a variety of head and neck locations. Presenting symptoms of these lesions range from an asymptomatic cyst to one which is compromising the airway. The true diagnosis is rarely suspected prior to excision. A case of a gastric choristoma of the tongue is presented which was noted at birth as an intermittently bleeding ulcer. Complete excision of the mass was difficult to ascertain due to poor delineation of the tumor margins from the tongue musculature. A postoperative technetium-99m pertechnetate scan failed to demonstrate residual gastric mucosa. Conservative management resulted in only transient healing of the ulcer. Repeat excision demonstrated abundant residual gastric mucosa. technetium-99m pertechnetate scanning may not be a reliable indicator of ectopic gastric mucosa in the head and neck region.
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22/88. Heterotopic gastrointestinal cyst of the oral cavity.

    A case of an oral heterotopic gastrointestinal cyst of the tongue is described and a review of the literature is presented. The oral gastrointestinal cyst is a developmental lesion found more commonly in males, with a wide age range, but mostly occurring in the young. It presents as an asymptomatic swelling and most likely arises from misplaced embryonal tissue. Conservative surgical excision is the treatment of choice and no recurrence is expected.
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23/88. Oral congenital cystic choristomas: a case report.

    Solitary choristomas containing gastrointestinal mucosa occur rarely in the tongue and the oral mucosa and usually present as an asymptomatic mass. This report documents the occurrence of two congenital intraoral cystic choristomas in a 5-month-old male infant. The cysts were located on the dorsum of the base of the tongue and left floor of mouth anterior to the lingual cyst. Both cysts were lined by nonkeratinizing squamous epithelium and gastric, intestinal, and respiratory epithelium. The cysts were excised by an oral approach.
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24/88. Dual-site thyroid ectopy in a mother and son.

    We found familial thyroid ectopy in a mother and son, each of whom had an anterior neck mass corresponding to a perihyoid ectopic thyroid. In addition, technetium scintigraphy revealed another focus of aberrant uptake at their tongue roots. Neither patient showed hypothyroid symptoms or abnormal biochemical data other than a mildly elevated TSH level in the son. No other member of the family had a thyroid disorder. To our knowledge this is the first documented instance of familial thyroid ectopy in multiple sites. The observed similarity in distribution of ectopic tissue suggests a hereditary pathogenic factor.
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25/88. prenatal diagnosis of enteric duplication cyst of the tongue.

    cysts located on the tongue are rare and usually diagnosed in childhood. Here we report on the prenatal diagnosis of an unusual cystic malformation of the tongue with heterotopic intestinal tissue explored by prenatal ultrasound, magnetic resonance imaging and on its surgical treatment and histological examination. The prenatal differential diagnoses as well as perinatal management of cystic lesions of the tongue are reviewed.
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26/88. New case of thyroid dysgenesis and clinical signs of hypothyroidism in williams syndrome.

    The authors report a female presenting with congenital heart defects, liver hemangiomas, and facial dysmorphisms admitted to hospital at 3 months of age because of feeding difficulties and poor growth. She had hypotonia and large tongue, "coarse" face, and umbilical hernia in presence of complex congenital cardiovascular malformations. In spite of normal neonatal screening we performed serum levels of thyroid hormones. thyrotropin level was very high (>50 microU/ml; normal value 0.2-4 microU/ml), while serum free T(3) (FT3) and free T(4) (FT4) levels were normal (FT3 3.6 pg/ml, normal value 2.8-5.6 pg/ml; FT4 11.6 pg/ml, normal value 6.6-14 pg/ml); antithyroid autoantibodies were absent. Thyroid scintigraphy with sodium 99m Tc pertechnetate showed a small ectopic thyroid located in sublingual position, so treatment with L-thyroxine 37.5 microg/24 hr was started with rapid improvement of the clinical picture. At 17 months of age the patient developed the complete characteristic phenotype of williams syndrome (WS); the clinical diagnosis was proven by fluorescent in situ hybridization (FISH) analysis which showed hemizygous deletion of the elastin gene on chromosome 7. Recently a case of thyroid hemiagenesis in a child with WS has been reported; our patient underscores the association of hypothyroidism and WS. Moreover, our case shows that clinical manifestations of hypothyroidism may be present and the treatment may be necessary as it is in isolated congenital hypothyroidism.
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27/88. lingual thyroid causing dysphonia: evaluation and management. Case report.

    CONTEXT: lingual thyroid gland is a rare clinical entity that is caused by the failure of the thyroid gland to descend to a normal cervical location during embryogenesis. The occurrence of an ectopic thyroid gland located at the base of the tongue may cause problems for the patient, with symptoms of dysphagia, dysphonia, upper airway obstruction or even hemorrhage at any time from infancy through adulthood. CASE REPORT: We report on a case of lingual thyroid gland in a 41-year-old female patient. The embryology and diagnosis of ectopic thyroid are discussed and its management is outlined. Features of the diagnostic and therapeutic evaluation are described with attention to the clinical findings, laboratory tests, thyroid scan and computed tomography imaging studies employed in the confirmation of diagnosis and planning of appropriate treatment. The history of the condition is reviewed and a treatment strategy is outlined. Surgical excision of the gland is reserved for cases of gland enlargement that result in compromised airways (dysphagia or dysphonia) or recurrent hemorrhage.
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28/88. lingual thyroid gland: clinical evaluation and management.

    The authors present three illustrative cases of lingual thyroid gland in paediatric age group with a protocol for diagnosis and management of the condition. elements in the diagnostic and therapeutic evaluation are outlined with attention to the clinical findings, laboratory tests and radiographic imaging studies employed in confirming the diagnosis and planning appropriate treatment. Presentations varied from swelling noticed on routine evaluation for upper respiratory tract infection in one, to dysphagia in the next and sore throat in the third patient. On examination they had swelling on the tongue just behind the circumvallate line. 99mTc thyroid scan confirmed the swelling as lingual thyroid with no thyroid tissue in the normal location. All three children had raised TSH levels and were put on suppressive L-thyroxine. At follow up patients are asymptomatic.
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29/88. Congenital melanotic macules and Sebaceous choristoma arising on the tongue of a newborn: epidermal choristoma?

    Oral hyperpigmentation is a common event in older individuals, however, is exceptional in neonates (congenital melanotic macules). Conversely, 70-80% of people have sebaceous glands in the oral mucosa, with the tongue representing an ectopic location and termed sebaceous choristoma by some authors. We report a case that fulfills both conditions in a tongue lesion. A 1-month-old boy presented with a pigmented macula on his tongue noted at birth. An excisional biopsy was performed showing a lesion lined by an epidermal-like epithelium with basal pigmentation, under which, sebaceous glands, abortive hair follicles and ductal structures mimicking apocrine glands were found. Seven cases of congenital melanotic macules of the tongue have been reported, however, none of them showed sebaceous glands under the lesion. Furthermore, there has not been a reported case of sebaceous choristoma of the tongue present at birth. We present a case that shares clinical and histological features of both conditions and propose the name 'epidermal choristoma'.
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30/88. Sclerosing papillary carcinoma arising in a lingual thyroid: report of a case.

    Ectopic lingual thyroid tissue is an uncommon congenital anomaly. Tumors with identical pathological characteristics to those arising in thyroid tissue may be present in ectopic locations, but there are very few cases of malignant ectopic thyroid tumors reported in the literature. We present a review of this phenomenon and report a case of papillary carcinoma of the base of the tongue, located in ectopic lingual thyroid tissue, in a 30-year-old woman. The patient's presenting symptoms were dysphagia and oral bleeding, and we performed radical resection of the neoformation at the base of the tongue with part of the muscles of the floor of the mouth and the body of the hyoid bone, as well as total thyroidectomy. Histological examination revealed a "sclerosing" papillary carcinoma. The patient was treated with 131I and substitutive thyroid hormonal therapy. An ultrasonogram done 5 years later showed bilateral laterocervical lymph node recurrence, which was effectively treated with bilateral laterocervical lymphectomy.
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