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11/23. Benign mixed epithelial and stromal tumor of the kidney: imaging findings.

    Three cases of mixed epithelial and stromal tumor of the kidney with their imaging findings are described; these cases have not been reported previously in the radiology literature. This benign tumor contains epithelial and spindle cell stromal components and arises exclusively in adult women. It is characterized morphologically by a solid and cystic nature with delayed enhancement.
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12/23. Spindle-cell epithelial thymoma. Fine-structural and tumor lymphocyte observations.

    A fine-structural study of a spindle-cell epithelial thymoma in a patient with pemphigus and autoimmune hemolytic anemia is presented and compared with the few previously described. Because light-microscopic features suggested hemangiopericytoma, critical fine-structural comparisons between spindle-cell epithelial thymoma and hemangiopericytoma are detailed. Based upon groups of tonofilaments with desmosomal insertions, abundant well-formed desmosomes, negligible numbers of pinocytotic vesicles, and an absence of myofilaments and dense bodies, an epithelial origin for this tumor is proposed. Langerhans' cell granules, a new observation in thymoma, were found in cells of probably histiocytic origin. Tumor lymphocyte studies revealed that more than 95% of cells formed E rosettes, 36% formed EAC rosettes, yet none contained surface immunoglobulin. The significance of these observations is discussed.
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keywords = spindle
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13/23. Mixed epithelial and stromal tumor of the kidney.

    A 42-year-old female presented with intermittent left flank pain for 2 years. She developed gross hematuria 1 month prior to seeking help. Intravenous pyelography showed filling defects within the lower portion of the left collecting system and renal pelvis causing hydronephrosis. Abdominal computed tomography revealed a huge cystic heterogenic tumor about 20 cm in largest diameter occupying the entire left kidney. A left radical nephrectomy was performed without complications. The pathology report confirmed the diagnosis of mixed epithelial and stromal tumor of the kidney. From pathologic survey, the spindle cells of this tumor were positive for muscle markers and expressed estrogen and/or progesterone receptors. We suggest that a mixed epithelial and stromal tumor of the kidney should be considered in all cystic renal tumors presenting in perimenopausal women.
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14/23. dna aberrations in the epithelial cell component of adamantinoma of long bones.

    adamantinoma of long bones is a rare malignant tumor composed of cells with epithelial characteristics in various differentiation patterns surrounded by fibrous cells. Evidence as to whether this neoplasm should be designated as an epithelial bone tumor or a biphasic sarcoma with both epithelial and mesenchymal features is lacking. In this study the nature of the mesenchymal and epithelial components of adamantinoma was investigated by dna flow cytometry, dna image cytometry, p53 immunohistochemistry, and polymerase chain reaction-based loss of heterozygosity detection at the p53 locus. Specimens from 6 of 15 patients (40%) analyzed by flow cytometry had an aneuploid dna index. image cytometry analysis of Feulgen-stained paraffin sections of 6 aneuploid and 2 diploid tumors revealed that aneuploid nuclei were detected in cells with an epithelial phenotype only, whereas all fibrous cells were diploid. immunohistochemistry for p53 on specimens from 25 patients revealed moderate or strong immunoreactivity in 12 tumors (48%) restricted to the epithelial cells. loss of heterozygosity at the p53 locus could be confirmed in the epithelial component of an immunohistochemically p53-positive tumor. Additionally, sections of 7 lung metastases were studied histologically. Only keratin-positive epithelial cells, predominantly in the spindle cell pattern, were present in these metastases, whereas the osteofibrous tissue present in the primary tumors was not detected. These results suggest that either adamantinoma consists of a malignant epithelial part with a reactive osteofibrous stroma or that the malignant epithelial cells develop next to a proliferating benign fibrous component. Additional analysis of common genetic abnormalities in the fibrous and epithelial cells of adamantinoma is therefore indicated.
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15/23. Thyroid spindle epithelial tumor with thymus-like differentiation (the "SETTLE" tumor). An immunohistochemical and electron microscopic study.

    An intrathyroid primary epithelial spindle-cell tumor with mucous cysts is described in a 9-year-old child. Histologically, this well-circumscribed tumor exhibited a nodular pattern, a prominent spindle cell component with minimal pleomorphism, and well-differentiated mucinous glands within fibrous bands. The spindle cells demonstrated diffuse immunopositivity for cytokeratin and vimentin. Electron microscopy of tissue sections demonstrated that cells contained bundles of cytoplasmic tonofilaments and numerous desmosomes. The light and electron microscopic features and immunohistochemical profile of this tumor were similar to those of recently described thyroid tumors that have been called "SETTLE" tumors (i.e., spindle epithelial tumor with thymus-like differentiation). These uncommon tumors can be considered intrathyroid thymoblastomas and must be regarded as potentially malignant lesions.
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ranking = 5.7243081746463
keywords = spindle, spindle cell
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16/23. adamantinoma of the long bones. A clinicopathological study of thirty-two patients with emphasis on histological subtype, precursor lesion, and biological behavior.

    The records of thirty-two patients who had had an adamantinoma of the long bones were examined to investigate the relationship between the clinical presentation, the histological subtype, and the method of treatment, and the clinical result. All histological patterns of differentiation that are characteristic of adamantinoma were observed, including the basaloid, spindle-cell, tubular, squamous, and osteofibrous dysplasia-like subtypes. Follow-up data were available for twenty-eight (88 per cent) of the thirty-two patients. These patients were followed for a mean duration of 122 months (range, eleven months to twenty-nine years and two months). Nine patients (32 per cent), all of whom had been managed with an intralesional or marginal procedure, had a local recurrence of the tumor after a mean disease developed in three of the nine patients. In five other patients, metastasis developed without having been preceded by a local recurrence. Thus, the over-all rate of metastasis was 29 per cent (eight patients). The mean duration of survival for the patients who had metastasis was twelve years and eight months. Statistical analysis of various clinicopathological variables revealed intralesional or marginal excision to be the most significant risk factor for a local recurrence or metastasis (p < 0.001). Two patients who had had a presumed osteofibrous dysplasia-like adamantinoma, which contained few isolated keratin-positive epithelial cells within the stroma at the time of presentation, had a full-blown adamantinoma at the time of the local recurrence. Although the clinical course that was observed may be the result of a sampling error, it poses questions as to the regressive nature of osteofibrous dysplasia-like adamantinoma. On the basis of our findings and the data in the literature, we believe that an osteofibrous dysplasia-like adamantinoma may be a precursor lesion of the classic type of adamantinoma.
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ranking = 0.62071802910771
keywords = spindle
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17/23. Intraneural biphasic synovial sarcoma: an alternative "glandular" tumor of peripheral nerve.

    A primary intraneural biphasic synovial sarcoma that arose within the common digital nerve of the hand is reported. The patient, a 16-year-old girl, presented with a small soft tissue mass that clinically and intraoperatively resembled a nerve sheath tumor. The diagnosis of synovial sarcoma was established on the basis of a typical light microscopic appearance and immunohistochemical staining profile. Intraneural biphasic synovial sarcoma resembles "glandular" peripheral nerve sheath tumor, because both are composed of a mixture of spindled mesenchymal cells with admixed glandular epithelial elements. The features allowing differentiation of these two unusual tumors of peripheral nerve are discussed.
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ranking = 0.62071802910771
keywords = spindle
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18/23. Spindle epithelial tumor with thymus-like differentiation: a case report with cytologic, histologic, immunohistologic, and ultrastructural findings.

    Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare and distinctive low-grade neoplasm of thymic or related branchial pouch differentiation. The tumor usually presents in the thyroid or lateral neck of children and adolescents and could mimic spindle-cell carcinoma, synovial sarcoma, or malignant teratoma. We report the clinical, cytologic, histologic, immunohistochemical, and ultrastructural features of a SETTLE present for 10 years in a 15-year-old boy. The fine-needle aspirate, initially interpreted as synovial sarcoma, contained numerous clusters of bland spindle cells, with a few detached sheets of columnar mucous cells in a homogeneous background of dissociated spindle cells. Mitoses, necrosis, and atypia were not present. The excised tumor was a well-circumscribed, white-tan mass, with occasional microcysts. Microscopically, the mass consisted of a lobulated, highly cellular, spindle-cell neoplasm arranged in intersecting, whorled, and storiform fascicles separated by fibrous bands. Entrapped within the fibrous bands were squamous-lined cysts and benign-appearing glands lined by columnar epithelium with goblet cells or ciliated pseudostratified epithelium. Immunohistochemically, the spindle cells showed diffuse reactivity for cytokeratins, smooth muscle actin, muscle-specific actin, and MIC-2, and they were negative for epithelial membrane antigen, calcitonin, and thyroglobulin. Ultrastructurally, numerous perinuclear tonofilaments, some aligned with mature desmosomes, were identified in the spindle cells. Occasional cells showed thin filaments with fusiform dense bodies occupying the peripheral cytoplasm. These findings distinguish SETTLE from ectopic thymoma, synovial sarcoma, medullary carcinoma, and teratoma, and they support a thymic epithelial origin for SETTLE, possibly with myoepithelial differentiation.
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ranking = 5.2414360582154
keywords = spindle, spindle cell
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19/23. Management decisions for unusual periampullary tumors.

    The pessimism associated with the treatment of pancreatic cancer may result in inappropriate management in certain patients thought to have that disease. We analyzed the recent UCLA experience with a variety of periampullary tumors in which various issues concerning management were unusual. The records of nine patients (age 15-75 years) with pancreatic or periampullary tumors were reviewed retrospectively. The tumor was evident on CT scan in all patients. The diameter of the mass was greater than 5 cm in five cases. Eight of the tumors appeared to arise from the pancreas, but at exploration, two were found to originate from other structures (duodenum and retroperitoneum). One patient with an apparent gastric lesion on CT scan was found to have a mass of pancreatic origin at operation. Operative procedures included: pancreaticoduodenectomy (four), distal pancreatectomy (three), total pancreatectomy (one), and retroperitoneal tumor resection (one). Pathological diagnoses included: solid and papillary epithelial neoplasm (two), mucinous cystic neoplasm (two), serous microcystic adenoma (two), myositis ossificans (one), degenerative neurilemoma (one), spindle cell tumor (one), and intraductal papillary carcinoma (one). We conclude that patients with large or unusual-appearing pancreatic or periampullary tumors should be managed aggressively. Major resections can be done safely with the achievement of an excellent quality of life in individuals at the extremes of age. Unlike the usual pancreatic ductal adenocarcinoma, the prognosis for many of these neoplasms is excellent.
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20/23. Malignant peripheral nerve sheath tumor with glandular differentiation. Report of a case with emphasis to the usefulness of immunohistochemistry in the differential diagnosis.

    A case of malignant nerve sheath tumor with glandular differentiation in a patient with neurofibromatosis is presented. In the spindle celled areas the tumor cells reacted strongly with vimentin and S-100 protein, while the glandular epithelia reacted with EMA, cytokeratins (peptides 8, 19, and 20) and CEA, as well as a few of them also with chromogranin. Based on the results of immunohistochemical profile of the tumor cells, the differential diagnosis of this rare soft tissue tumor from biphasic synovial sarcoma with glands, as well as its distinction from any other spindle cell sarcoma with entrapped skin appendages could be greatly facilitated.
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