Cases reported "Hamartoma"

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1/25. Intrasellar neuronal hamartoma associated with pituitary adenoma. Case report.

    46-year-old acromegalic women presenting high level of growth hormone (32 ng/ml) in the serum underwent surgery. The intrasellar tumor, 16 mm in diameter, has been removed. The biopsy material consisted of two types of closely adjacent and intermingled tissues, one of which was growth hormone positive acidophilic adenoma, the second component were haphazardly oriented ganglion cells of various size and shape, also multinuclear, with bundles of unmyelinated fibers. The cytoplasm and processes of ganglion cells were immunopositive for neurofilaments and for synaptophysin on cellular membranes and processes. There were none glial fibrillary acidic protein positive cellular elements. The authors discuss commonly used name of choristoma for this type of tumor and the problem of possible neurosecretory stimulation of pituitary adenoma by neuronal hamartoma.
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keywords = membrane
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2/25. Two cases of ectopic hamartomatous thymoma.

    Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm. Since it was named by Rosai et al. in 1984, 24 cases have been reported. We herein report two cases of EHT, one of which presented with massive myoid cells, and review the literature related to EHT. Both of our cases displayed the typical features of EHT: (1) nests of epithelial cells, including solid, cystic, or glandular epithelial islands; (2) spindle cells dominating the microscopic picture; and (3) adipose cells which intermingle haphazardly to impart a hamartomatous quality to the tumor. In this paper, we observed massive myoid cells and the transition from spindle epithelial cell to myoid cell in one of our cases. Immunohistochemical examinations showed that the main component of EHT, spindle cells, was positive for cytokeratin and epithelial membrane antigen (EMA). Intriguingly, the myoid cells simultaneously expressed cytokeratin, EMA, myoglobin, and creatine kinase-mm, suggesting that myoid cells may originate from epithelial cells and are an intermediate state between epithelial cells and muscular cells.
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keywords = membrane
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3/25. tail gut cyst.

    The tail gut is a blind extension of the hindgut into the tail fold just distal to the cloacal membrane. Remnants of this structure may form tail gut cyst. We report a 14-year-old girl with tail gut cyst that presented as acute abdomen. The patient recovered after cyst excision.
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keywords = membrane
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4/25. Tailgut cyst with carcinoid: a case report.

    Tailgut cyst, an unusual presacral lesion, is lined by a variety of epithelial types including squamous, columnar, transitional and cuboidal epithelium. Disorganized fascicles of smooth muscle may be seen in the cystic wall. Tailgut cysts are usually multiloculated, and often afflict adult women. In rare cases, adenocarcinoma arises within the tailgut cyst. We present a carcinoid tumor developing from the tailgut cyst. The tumor cells grow in ribbon and festoon patterns and are positive for argyrophilic granules. Immunostains for neuron-specific enolase are also positive. Ultrastructurally, dense-core, membrane-bounded neurosecretory granules are present.
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ranking = 1
keywords = membrane
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5/25. Presumed combined hamartoma of the retina and retinal pigment epithelium with preretinal neovascularization.

    PURPOSE: To describe a case of presumed combined hamartoma of the retina and retinal pigment epithelium associated with preretinal neovascularization. DESIGN: Observational case report. methods: We report clinical and angiographic findings of a 26-year-old woman. RESULTS: The patient presented with mild vitreous hemorrhage and slowly decreasing vision in the right eye. A combined hamartoma of the midperipheral retina and retinal pigment epithelium with an epiretinal membrane causing traction to the macula was found. fluorescein angiography showed areas of capillary nonperfusion and a large preretinal neovascularization peripheral to the hamartoma. CONCLUSIONS: A combined hamartoma may be associated with retinal capillary nonperfusion and preretinal neovascularization, suggesting that significant retinal ischemia can occur with a combined hamartoma.
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ranking = 1047.8354465518
keywords = epiretinal membrane, epiretinal, membrane
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6/25. Congenital bilateral diaphragmatic eventration with membranous chest wall hamartoma.

    The case of a 2-month-old girl with congenital bilateral eventration of the diaphragm is reported. After normal delivery from cephalic position, the patient developed respiratory distress. Mechanical ventilation started immediately but extubation was difficult. A membranous lesion was found on computed tomography of the chest. Resection of the membrane between the right middle and lower lobes and bilateral diaphragmatic plication was performed. Histologically the membrane was a chest wall hamartoma. The patient was extubated on 6 day postoperatively and is alive and well 4 months after surgery.
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ranking = 2
keywords = membrane
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7/25. Successful treatment of subfoveal choroidal neovascularization associated with combined hamartoma of the retina and retinal pigment epithelium.

    PURPOSE: To describe a patient with subfoveal choroidal neovascularization (CNV) associated with combined hamartoma of the retina and retinal pigment epithelium (CHRRPE), treated successfully by submacular surgery. DESIGN: Interventional case report. methods: A 12-year-old girl was referred to our clinic for evaluation. visual acuity was 20/30 in the affected left eye. ophthalmoscopy disclosed juxtapapillary CHRRPE and subfoveal pigmented CNV. Vitreous surgery was carried out because of visual deterioration to 20/60. RESULTS: The posterior vitreous was strongly attached to glial tissue at the superior margin of the optic disk in the CHRRPE region. The CNV, which was not connected with the CHRRPE, was carefully removed, resulting in visual improvement to 20/20 5 months after surgery. Histologically, the excised membrane showed fibroblast-rich cellular component and a type 2 configuration. CONCLUSION: Submacular surgery can be effective for the treatment of secondary CNV associated with CHRRPE.
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ranking = 1
keywords = membrane
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8/25. Lymphatic malformation of the sphenoid sinus in a pediatric patient.

    Lymphatic malformation is a benign vascular lesion resulting from lymphatic tissue being isolated from the remainder of the lymphatic system. They are present at birth and up to 90% are diagnosed by 2 years of age. More aggressive lesions are usually diagnosed earlier, with low-grade lesions presenting later with fewer complications. These lesions are hamartomas and not true neoplasms. The term hamartoma is used to describe an abnormally large mass of histologically normal tissue in a normal location. The lymphatic malformation is composed of lymph-filled channels lined with a single layer of flat endothelial cells on a basement membrane. They present with either generalized edema and poorly defined borders (microcystic) or a localized area of multilocular cysts (macrocystic). The term lymphatic malformation has replaced many other outdated terms, such as lymphangioma, cystic hygroma, lymphangioma circumscriptum, and lymphangiomatosis. In this study, we present a case report of a pediatric lymphatic malformation of the sphenoid sinus. To our knowledge, this lesion has not been described in the pediatric population and has been described only once in an adult in the German literature. EBM rating: C.
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ranking = 1
keywords = membrane
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9/25. Splenic hamartoma: immunohistochemical and ultrastructural profile of two cases.

    Splenic hamartoma (SH) is a rare, benign lesion. We present 2 cases, both in females (2 and 30 years, respectively) with multiple urinary tract infections, and left upper quadrant abdominal pain. Immunohistochemical staining with factor VIII displayed intense diffuse staining in the SH with corresponding weak staining in the adjacent spleen. CD31 showed a reverse pattern from that of factor viii. CD34 staining pattern was identical in both the spleen and the SH. Ultrastructurally, the SH showed endothelial cells with relatively empty cytoplasm, scattered Weibel Palade bodies, and lining by basement membrane surrounded by fibrous long-spacing collagen. Our study highlights the unique immunohistochemical profile of SH. The ultrastructural features are interesting, although their diagnostic significance remains to be confirmed in future studies.
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ranking = 1
keywords = membrane
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10/25. Nasal chondromesenchymal hamartoma in older children and adults: series and immunohistochemical analysis.

    CONTEXT: Nasal chondromesenchymal hamartoma is a benign mass lesion of the nasal cavity predominantly described in young infants. These unusual lesions are composed of a proliferation of mesenchymal and cartilaginous elements. Their pathogenesis is unknown, but they may be derived from embryologic rests. To our knowledge, only 1 case in an older child has been reported, and no cases have been reported in adults. OBJECTIVE: To report 4 cases of nasal chondromesenchymal hamartoma occurring in older children and adults, including immunohistochemical analysis of these unusual lesions. DESIGN: Cases identified from our archives were examined to confirm the diagnosis of nasal chondromesenchymal hamartoma. Immunohistochemical analysis was performed using a panel of antibodies (epithelial membrane antigen, smooth muscle actin, all muscle actin, cytokeratin, S100, and KP1) to evaluate for epithelial, smooth muscle, neural, chondroid, and histiocytic differentiation. RESULTS: Four cases of nasal chondromesenchymal hamartoma in patients of 11, 69, 17, and 25 years of age demonstrated histologic evidence of mesenchymal and cartilaginous elements underlying a chronically inflamed respiratory mucosa. Bony and adipose elements and rare glandular elements were interspersed. Cartilaginous elements stained strongly with S100, whereas mesenchymal regions showed variable and weaker staining. Smooth muscle differentiation was seen primarily in the mesenchymal areas. Epithelial membrane antigen was focally positive in all cases. CONCLUSIONS: Nasal chondromesenchymal hamartomas can rarely occur in the older child and adult. Mesenchymal areas show both myofibroblastic and cartilaginous differentiation. We speculate that inflammation or a recapitulation of developmental signals may be components in the pathogenesis of these lesions.
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ranking = 2
keywords = membrane
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