Cases reported "Choristoma"

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1/107. Salivary duct carcinoma in the mandible: report of a case with immunohistochemical studies.

    Salivary duct carcinoma is rare. We describe a 56-year-old man who developed salivary duct carcinoma in the mandible 10 years after removal of the right second and third molars. The tumour originated in the retromolar gland or the ectopic minor salivary gland in the mandible. The panoramic radiograph showed a radiolucent, poorly circumscribed area about 40 x 30 mm in size and distal to the lower right first molar. This tooth, together with all neoplastic tissue, was removed, and histopathological examination showed it to be a salivary duct carcinoma in the mandible. On immunohistochemical staining, keratin antibodies stained the ductal structure, 1A4 antibody stained myoepithelial cells, but S-100 protein and vimentin were not seen. The patient was well and with no sign with recurrence 6 years postoperatively.
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keywords = mandible, lower
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2/107. Phakomatous choristoma may be located in the eyelid or orbit or both.

    PURPOSE: Phakomatous choristoma is a rare congenital tumour of lenticular origin. The exact location at clinical presentation is controversial, but herein we provide evidence that phakomatous choristoma may be located in the lower eyelid or orbit or both. methods: Case report of an infant presenting with a mass in the lower eyelid at birth, a systematic review of previous cases of phakomatous choristoma and an outline of the histopathological features in normal mid-facial embryonic development at different gestational ages. RESULTS: The histopathological features of the present case were consistent with those of a phakomatous choristoma. The preoperative imaging studies and clinical findings at surgery suggested that the tumour occupied parts of both the lower eyelid and anterior orbit. Histopathological sections of a normal human embryo showed that at the 26-mm stage of development the embryonic lens is formed but the bony walls defining the orbit are not yet present. CONCLUSION: Phakomatous choristoma arises in a setting of undifferentiated mesenchymal tissue which later may develop into the lower eyelid or orbit depending on the choristomatous elements being deposited superficial or deep to the embryonic surface.
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ranking = 0.032807144145214
keywords = lower
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3/107. Ectopic thymus presenting as a solid submandibular neck mass in an infant: case report and review of literature.

    Solid ectopic cervical thymus is an extremely uncommon etiology of a neck mass in an infant. It occurs in the line of descent of the thymus from the angle of the mandible to the superior mediastinum. Nine cases of ectopic cervical thymus in infants have been reported in the literature. Only two of nine cases were solid, the remaining seven were thymic cysts. A preoperative diagnosis is seldom considered and is often misdiagnosed as a possible malignancy or a lymph node. We present a case of a 2-month-old infant with an asymptomatic enlarging right neck mass. Patient underwent complete excision of the mass.
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ranking = 0.14168545913767
keywords = mandible
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4/107. Phakomatous choristoma: a case report and review of the literature.

    Phakomatous choristoma is a rare congenital lesion of the eyelid that can be clinically and/or histologically mistaken for a cyst, cutaneous adnexal neoplasm, or an ocular adnexal oncocytoma. Only 13 such cases have been previously described, mostly in the English language ophthalmic literature. Zimmerman reported the first case in 1971 and proposed the lesion to be of lenticular anlage origin, a theory that has been widely accepted. We report an additional case occurring in an 8-week-old male infant with a firm nodule of the right lower eyelid that was present since birth. A 15 x 12 x 2 mm circumscribed solid nodule with a homogenously white cut surface was surgically excised. Histologically, this lesion was comprised of cuboidal cells forming cystically dilated and irregularly branched ducts and cords within a densely fibrotic stroma. Also present were eosinophilic basement membranelike material, psammoma body-like calcifications and intraluminal degenerated ghost cells. The immunohistochemical profile of the epithelial cells included strong immunoreactivity for vimentin, focal weak staining for S-100, and negative staining for cytokeratin, epithelial membrane antigen, synaptophysin, and chromogranin. The irregularity of the ducts and cords of epithelial cells within the densely fibrotic stroma resembled an infiltrative neoplasm of cutaneous adnexal or lacrimal duct origin. However, the site of involvement, the peculiar basement membrane material, ghost cells, and immunohistochemical profile were features that helped to distinguish phakomatous choristoma from an infiltrative carcinoma. The correct identification of this lesion is essential to avoid an aggressive surgical excision, thus sparing the eyelid and lacrimal system. The purpose of this article is to bring attention to this rare entity, because it has not been described in either the dermatology or dermatopathology literature and furthermore, is not mentioned in any of the major dermatopathology texts.
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ranking = 0.0082017860363036
keywords = lower
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5/107. Phakomatous choristoma of the eyelid.

    This article describes the first report of phakomatous choristoma of the eyelid in korea. A six-month-old boy underwent excision of a congenital inferonasal orbital mass arising from the left lower lid. A dermoid cyst was suspected, however a diagnosis of phakomatous choristoma was made following conventional histology. An immunohistochemical study of this rare benign congenital tumor was conducted. The cuboidal epithelial cells comprising this choristoma showed strongly positive cytoplasmic staining with S-100 protein and vimentin. They also showed focally positive staining with a neuron-specific enolase, while they showed no immunoreactivity to cytokeratin or epithelial membrane antigen. The results of the immunohistochemical study support the conclusion that this tumor is of lenticular anlage origin.
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keywords = lower
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6/107. Ectopic lacrimal gland cyst of the orbit.

    Lacrimal duct cysts are not common. It is extremely rare when a lacrimal duct cyst and an ectopic lacrimal gland develop in the orbital cavity. A unique case of an ectopic lacrimal gland cyst of the orbit is presented. A 33-year-old man had a palpable mass above the inferior medial orbital rim for nearly two years. An ocular examination was normal except for a movable, firm mass found in the anterior nasal inferior orbit of the right eye. An echogram revealed a homogeneous, hypoechoic cystic mass. Computed tomography of the orbit showed a well-encapsulated lesion in the lower orbit of the right eye near the inferior rectus muscle, without bony erosion. A tense, thin-walled, clear fluid-filled cyst measuring 15 x 12 x 13 mm in size was completely enucleated without rupture by anterior orbitotomy. Pathologic examination disclosed a small nest of normal gland tissue surrounded by a cystic lesion lined with two layers of lacrimal duct epithelium cells. No recurrent signs were noticed during a 12-month period of follow-up.
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ranking = 0.0082017860363036
keywords = lower
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7/107. Retroperitoneoscopic heminephrectomy of the right upper collecting system emptying into an ectopic ureterocele in a 5-year-old girl: a case report.

    A 5-year-old girl with a history of recurrent urinary tract infection since the age of 14 months was diagnosed as having a right duplicated urinary collecting system with the upper ureter ectopically opening in the urethra. She underwent retroperitoneoscopic heminephrectomy for a right dysplastic kidney and open ureterocelectomy and reimplantation of the refluxing lower ureter via Pfannenstiel incision. She survived the procedure without serious complications and resumed normal daily activities by day 6. To the best of our knowledge, this case is the 16th case of laparoscopic heminephrectomy for pediatric patients and the first case treated by the retroperitoneal approach in the English literature.
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ranking = 0.0082017860363036
keywords = lower
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8/107. Ultrasound diagnosis and Doppler monitoring of a pelvic spleen in pregnancy.

    We describe a patient with a pelvic spleen diagnosed during pregnancy and monitored through gestation which we believe to be the first reported case. A 40-year-old woman was referred at 8 weeks of gestation because of a chronic intense pain in the left iliac cavity which had spread to her lower back. Clinical examination revealed a poorly defined pelvic mass. Pelvic ultrasound demonstrated a gestational sac containing a viable embryo whose size was consistent with the period of amenorrhea. While the splenic area in the left hypocondrium was found to be empty, a homogeneous and elongate mass measuring 152 x 123 mm with a maximum thickness of 53.4 mm was observed in the left iliac cavity above the uterus. This mass, the ectopic spleen, was monitored by Doppler velocimetry at monthly intervals until delivery and no variation throughout gestation was observed; therefore, despite the occasional occurrence of heavy pain, it was possible to exclude circulatory complications such as thrombosis or torsion. Doppler ultrasound proved to be a useful tool for the differential diagnosis of this rare anatomical variation.
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ranking = 0.0082017860363036
keywords = lower
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9/107. Heterotopic neuroglial tissue causing airway obstruction in the newborn.

    BACKGROUND: Heterotopic neuroglial (brain) tissue is a rare cause of airway obstruction in newborns. Fewer than 30 cases have been reported in the English literature. brain heterotopias can mimic more common congenital anomalies of the head and neck. OBJECTIVE: To review our experience in the diagnosis and treatment of children with heterotopic pharyngeal neuroglial tissue. DESIGN: Case series. SETTING: Tertiary care children's hospital. patients: Four newborns with airway obstruction caused by heterotopic neuroglial tissue. RESULTS: All patients were infants (3 full-term girls and a 32 weeks' gestation boy) who had airway obstruction in the newborn period. All patients underwent preoperative computed tomography and magnetic resonance imaging, which revealed a heterogeneous mass involving the pharynx, neck, and parapharyngeal space. Bony deformities of the skull base and mandible were present in all patients, although intracranial connection was absent. Multiple surgical procedures were performed in all 4 patients. tracheotomy was performed in 2 patients, gastrostomy tube placement was required in 3, and a nasopharyngeal tube was used in 1. Combined cervicofacial and transoral approaches were used for resection, preserving vital structures. Histopathologic evaluation revealed mature glial tissue and choroid plexus-like structures. CONCLUSIONS: Heterotopic neuroglial tissue must be considered in the differential diagnosis of airway obstruction in the newborn. Management is surgical resection, with attention to vital structures and function-analogous to surgery for lymphangioma. Multiple surgical procedures might be necessary in the treatment of these patients.
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ranking = 0.14168545913767
keywords = mandible
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10/107. Retropharyngeal aberrant thymus.

    INTRODUCTION: Upper airway obstruction from a retropharyngeal mass requires urgent evaluation. In children, the differential diagnosis includes infection, trauma, neoplasm, and congenital abnormalities. Aberrant cervical thymic tissue, although occasionally observed on autopsy examination, is rarely clinically significant. We present the case of an infant with respiratory distress attributed to aberrant thymic tissue located in the retropharyngeal space. CASE: A 6-week-old infant was brought to the emergency department for evaluation of stridor associated with periodic episodes of cyanosis. Lateral neck radiograph revealed widening of the retropharyngeal soft tissues. The patient's symptoms did not improve with intravenous ampicillin-sulbactam. magnetic resonance imaging (MRI) performed on the seventh day of hospitalization revealed a retropharyngeal mass that extended to the carotid space. The mass was easily resected using an intraoral approach. Microscopic examination demonstrated thymic tissue. A normal thymus was also observed in the anterior mediastinum on MRI. The patient recovered uneventfully and had no further episodes of stridor or cyanosis. DISCUSSION: Aberrant cervical thymic tissue may be cystic or solid. Cystic cervical thymus is more common, and 6% of these patients present with symptoms of dyspnea or dysphagia. Aberrant solid cervical thymus usually presents as an asymptomatic anterior neck mass. This case is unusual in that solid thymic tissue was located in the retropharynx, a finding not previously reported in the English literature. Additionally, the patient presented in acute respiratory distress, and the diagnosis was confounded by the presence of mild laryngomalacia. In retrospect, our patient likely had symptoms of intermittent upper airway obstruction since birth. The acute respiratory distress at presentation was likely the result of laryngomalacia exacerbated by the presence of aberrant thymic tissue and a superimposed viral infection. Aberrantly located thymic tissue arises as a consequence of migrational defects during thymic embryogenesis. The thymus is a paired organ derived from the third and, to a lesser extent, fourth pharyngeal pouches. After its appearance during the sixth week of fetal life, it descends to a final position in the anterior mediastinum, adjacent to the parietal pericardium. Aberrant thymic tissue results when this tissue breaks free from the thymus as it migrates caudally. Therefore, aberrant thymic tissue may be found in any position along a line from the angle of the mandible to the sternal notch, and in the anterior mediastinum to the level of the diaphragm. In an autopsy study of 3236 children, abnormally positioned thymic tissue was found in 34 cases (1%). The aberrant thymus was most often located near the thyroid gland (n = 19 cases) but was also detected lower in the anterior neck (n = 6 cases), higher in the anterior neck (n = 8 cases), and at the left base of the skull (n = 1 case). The presence of thymic tissue in the retropharyngeal space in our patient is more unusual given the typical embryologic origin and descent of the thymus in the anterior neck to the mediastinum. Children with aberrant thymus may have associated anomalies. Twenty-four of 34 children (71%) with aberrant thymus detected at autopsy had features consistent with digeorge syndrome, and only 5 of the remaining 10 patients had a normal mediastinal thymus present. Our patient had normal serum calcium levels after excision and a mediastinal thymus was visualized on MRI. Biospy is required for diagnosis of cervical thymus and should also be considered to exclude other causes. MRI is helpful in delineating the presence, position, and extent of thymic tissue. Immunologic sequelae or recurrence after resection of an aberrant cervical thymus has not been reported.
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ranking = 0.14988724517397
keywords = mandible, lower
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