Cases reported "Choristoma"

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1/20. Unusual choristoma of the parotid gland in a girl. A possible trichoadenoma.

    An 8-year-old girl had a painless circumscribed nodule in the right parotid gland for 4 weeks. A tumour (1.3 cm diameter) within the salivary gland parenchyma showed small cystic spaces with horn-like material macroscopically. The tumour tissue contained solid squamous cell formations and cystic spaces limited by multilayered squamous epithelium and covered by layers of ortho- and parakeratotic cells. The cystic spaces contained keratotic lamellae. In some areas pin-like epithelial proliferations were seen. All epithelial cells were characterized by uniform nuclei, and no atypical mitoses were seen. Keratinizated masses with partial calcification were occasionally located in the interstitial tissue and replaced by multinucleated giant cells. The tumour was classified as a choristoma and resembled a trichoadenoma. The ectodermally derived oral and salivary gland epithelium may be the source of skin-like or adnexal tumours.
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2/20. Double ileoileal intussusception caused by a giant polypoid mass of heterotopic pancreas in a child.

    Heterotopic pancreatic tissue has been found in several abdominal and intrathoracic locations. In the ileum, it is a rare, usually asymptomatic, incidental finding. CONCLUSION: A unique case of a recurrent ileoileal intussusception in an 11-year-old girl is presented caused by a giant polypoid mass composed of ectopic pancreatic tissue that remained undetected during several diagnostic tests during two previous admissions and laparoscopic abdominal exploration.
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3/20. Highly aggressive brown tumour of the maxilla as first manifestation of primary hyperparathyroidism.

    A case is presented of a 62-year-old man with a right maxillary swelling for the previous three months. The lesion was expansive and osteolytic, with invasion of the adjacent maxillary sinus, nasal and pterygomaxillary fossae and floor of the orbit. histology revealed the presence of an intrabony giant cell lesion. blood tests demonstrated elevations in calcium (16.2 mg/dl) and parathyroid hormone (PTH) concentrations (841 pg/ml). This suggested the diagnosis of hyperparathyroidism initially manifesting as a brown tumour of the maxilla. Posterior explorations confirmed the existence of an underlying ectopic parathyroid adenoma as the cause of the condition.
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4/20. A giant ectopic ovary.

    A young woman with a congenital chest-wall defect presented with abdominal pain, irregular menses, and a palpable mass at the defect. The mass, removed laparoscopically, was found to be a functioning ovary that tracked along the retroperitoneum, reflecting its embryologic origin.
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keywords = giant
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5/20. Incidentally detected giant oncocytoma arising in retroperitoneal heterotopic adrenal tissue.

    A nonfunctional retroperitoneal oncocytoma incidentally discovered in a 40-year-old woman is described. The tumor, which was 17 cm in largest dimension, was completely separated from the kidneys and adrenal glands and consisted of nests of polygonal cells with large, granular, eosinophilic cytoplasm. Significant nuclear atypia, necrosis, and mitosis were absent. Ultrastructural analysis confirmed the oncocytic nature of the neoplastic cells. Since neoplastic cells were not immunoreactive for chromogranin and did not contain dense-core secretory granules, the diagnosis of oncocytic paraganglioma was excluded. Cells immunoreactive for 3beta-hydroxysteroid dehydrogenase, the enzyme catalyzing the conversions of pregnenolone to progesterone and dehydroepiandrosterone to androstenedione, were identified in the tumor, thus strongly indicating adrenocortical tissue origin. Multiple nests of 3beta-hydroxysteroid dehydrogenase-positive cells were detected in the loose retroperitoneal connective tissue. These findings strongly support the origin of the tumor from heterotopic retroperitoneal rests of the adrenal gland. To our knowledge, only 1 similar case has been described in the literature to date.
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6/20. Primary giant cell tumor of soft tissue. Report of a case with fine needle aspiration cytologic and histologic findings.

    BACKGROUND: So-called primary giant cell tumor of soft tissue of low malignant potential is the rare soft tissue analogue of giant cell tumor of bone, occurring primarily in superficial soft tissue. To our knowledge, the cytologic findings in bulky giant cell tumor of deep soft tissue were described only once, and no further report on the subcutaneous giant cell tumor could be retrieved from the literature. CASE: A 58-year-old woman presented with a well-demarcated, 1.5-cm-diameter dermal tumor. Fine needle aspiration smears contained numerous osteoclastlike giant cells and mononuclear cells showing bland and vesicular nuclei. A small fragment of branching vasculature and 1 mitosis were found. Those cytologic findings were enough to suggest a diagnosis of giant cell tumor of soft tissue, confirmed as a deep dermal giant cell on surgical resection. CONCLUSION: Primary giant cell tumor of soft tissue of low malignant potential should be considered in the differential diagnosis of bland-looking giant cell-rich lesions. awareness of its existence and knowledge of its cytologic features are important for a correct preoperative cytologic diagnosis.
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ranking = 13
keywords = giant
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7/20. fetus in fetu or giant epignathus protruding from the mouth.

    A huge and extremely organoid mass, protruding from the mouth of a newborn and causing respiratory embarrassment, was resected. It was difficult to make a clear distinction whether this mass was fetus in fetu or a highly organoid epignathus dealing with the findings obtained from its gross and histological examination of the mass.
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ranking = 4
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8/20. Subcortical nodular heterotopia: a functional MRI and somatosensory evoked potentials study.

    Subcortical nodular heterotopia (SNH) associated with refractory epilepsy may be surgically treated, and a positive outcome can be expected following the complete excision of the malformed tissue. Recent functional neuroimaging studies have suggested the possible functional relevance of cerebral malformations, and may make it possible to improve presurgical planning, thus allowing extended resections and minimising post-operative deficits. We here report the case of a 19-year-old man with epilepsy and a giant SNH associated with diffused abnormal gyrations of the right temporal-parietal regions. Cortical functional organisation was investigated by means of functional magnetic resonance imaging (MRI) during sensory and motor tasks, and somatosensory evoked potentials. The results revealed enlarged and displaced motor and sensory cortical areas with heterotopic tissue functional activation. The relevance of these findings is discussed in the light of the possible surgical treatment of drug-refractory epilepsy associated with cerebral malformations: surgical treatment based on conventional MRI studies alone, without taking the functional nature of dysplastic tissues into account, may lead to considerable side effects.
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9/20. Parapharyngeal neuroglial heterotopia presenting as a growing single locular cyst: MR imaging findings.

    Heterotopic brain presenting as a giant, growing, single locular cyst at the parapharyngeal space has not been reported before, to our knowledge. We present such a case, with MR imaging findings, in a 13-month-old girl. A well-demarcated giant cystic mass was noted in the left parapharyngeal space from the skull base to the submandibular region. Airway compression and deformity of the left mandible with subluxation of the temporomandibular joint were noted. The cyst contained a clear fluid that was isointense to CSF with all pulse sequences. Wall enhancement was noted on contrast-enhanced T1-weighted images. No connection to intracranial structures was noted. Histologic findings were compatible with neuroglial heterotopia.
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ranking = 2
keywords = giant
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10/20. Adrenal cortical adenoma in the spinal canal of an 8-year-old girl.

    An ectopic adrenal cortical adenoma containing high levels of androstenedione but without clinically detectable virilizing effects was found in the spinal intradural space of an 8-year-old girl. The tumor, which was located at the L2 level, manifested itself clinically by a short history of bilateral leg pain. It was well encapsulated; therefore, total surgical removal was accomplished. The light microscopic appearance of the tumor was typical of adenomatous adrenal cortical tissue. Ultrastructurally, it also showed characteristic features of steroid-producing tumors, including very abundant smooth endoplasmic reticulum and giant mitochondria with tubulovesicular and circular cristae. Frozen tissue analyzed by radioimmunoassay was found to contain almost 20 times the normal tissue level of androstenedione. There was no elevation of cortisol or aldosterone levels in the tumor. Postoperative magnetic resonance imaging (MRI) scan of the retroperitoneum showed no abnormalities in the patient's adrenal glands. serum androstenedione levels were normal. We postulate that the adenoma developed from congenital ectopic rests of intraspinal adrenal tissue. Although ectopic occurrence of adrenal cortical tissue has been recorded in other areas, neither such rests nor tumors developing from them have been previously reported within the spinal canal.
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