Cases reported "Teratoma"

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1/240. carcinoid heart disease from ovarian primary presenting with acute pericarditis and biventricular failure.

    A case is described of a 54 year old woman who had acute pericarditis with large exudative effusion accompanied by severe right and left ventricular failure. The patient was finally diagnosed with carcinoid heart disease from an ovarian carcinoid teratoma. She was treated with octreotide--a somatostatin analogue--followed by radical surgical resection of the neoplasm. At one year follow up only mild carcinoid tricuspid regurgitation remained. Only 16 cases of carcinoid heart disease from an ovarian primary have been described in literature. Moreover clinically manifest acute, nonmetastatic pericarditis and left heart failure are not considered as possible presentations of carcinoid heart disease, whatever the origin. In a recent series a small pericardial effusion was considered an infrequent and unexpected echocardiographic finding in carcinoid heart patients. One case of "carcinoid pericarditis" has previously been described as a consequence of pericardial metastasis. Left sided heart involvement is usually caused by bronchial carcinoids or patency of foramen ovale; both were excluded in the case presented.
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2/240. Atypical teratoid/rhabdoid tumor of the CNS: cytopathology and immunohistochemistry of insulin-like growth factor-II, insulin-like growth factor receptor type 1, cathepsin d, and Ki-67.

    insulin-like growth factor (IGF)-II is a potent growth factor, normally controlled by a number of other factors, including IGF binding proteins and IGF binding protein proteases. In general, the latter increase the bioavailability of IGF by cleaving IGF binding proteins. cathepsin d (an IGF binding protein protease) was also implicated in tumor invasion. Although IGF-II was implicated in the pathogeneses of various childhood neoplasms, its significance in the pathogenesis of atypical teratoid/rhabdoid tumor of the central nervous system (ATRT-CNS) was not studied to date. We present clinicopathologic features of two cases of ATRT-CNS. In addition, formalin-fixed, paraffin-embedded tissue sections were stained immunohistochemically for IGF-II, IGF receptor type 1, cathepsin d, and Ki-67. Both tumors demonstrated diffuse strong cytoplasmic positivity for IGF-II, diffuse cytoplasmic and focal membranous positivity for IGF receptor type I, and diffuse cytoplasmic positivity for cathepsin d. The Ki-67 labeling indices were 10.0% and 1.4%. We conclude that ATRT-CNS cells express both IGF-II and IGF receptor type 1, supporting the hypothesis that autocrine/paracrine stimulation of cell growth by IGF-II might be one mechanism involved in ATRT-CNS tumorigenesis. cathepsin d expressed by the tumor cells might also be involved in both tumor cell invasion and growth. The exact pathogenesis of ATRT-CNS remains to be elucidated.
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3/240. Dysembryoplastic neuroepithelial tumor (DNT): an ultrastructural study of six cases.

    We report six cases od DNT with a detailed ultrastructural characteristics. The patient age ranged from 7 to 16 years (mean 12), the location was temporal in three cases and frontal, temporooccipital and parietooccipital in each of one remaining cases. The predominant clinical feature in each case was history of episodes of intractable seizures. Histopathologically, the neoplasms were multinodular, each nodule was well-circumscribed and was composed of glioneuronal elements embedded in the variable amount of myxoid matrix. The oligodendroglial-like cells (OLC) predominated in the nodules with some accompanying mature neurons. The nodules were frequently surrounded by small calcifications which could be found also within the tumors. OLCs were immunoreactive for S-100 protein and neurons had the expression of synaptophysin and neurofilament proteins. Ultrastructurally, each tumor consisted of three major elements: neoplastic cells (OLC), elongated processes forming neuropil-like structure and expanded "mucoid" extracellular space: the latter gave an impression of cellular elements floating within it. Neoplastic cells had round, oval or elongated nuclei, no discernible nucleoli and a relatively narrow rim of the cytoplasm. Some nuclei were irregular and invaginated and pseudoinclusions were observed; a part of cytoplasm sequestered within pseudoinclusions often appeared degenerated with large blabs and electron-lucent vesicles, some of these contained in turn semicircular profiles of unknown significance. The second element consisted of innumerable cellular processes. Some of these were elongated and formed stacks connected by symmetrical symmetric or asymmetric adhesive plaque junctions. The others had shorter "neck" containing microtubules, these extended into bullous extensions. Dense-cored vesicles were occasionally observed, in both cytoplasm of neoplastic cells and within processes. In one cell, cross-sectioned annulate lamellae were found. In cytoplasm of a few cells, unusual inclusions reminiscent ribosome-lamellae complexes were observed. These were cylindrical resembling "laboratory tubes" with a cone-like endings. At higher power, walls of the "tubes" resolved into layered structures composed of several laminae; between laminae, ribosome-like structures were visible.
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4/240. Pleuropulmonary blastoma and ovarian teratoma.

    We report a patient with pleuropulmonary blastoma who had a benign teratoma as a second primary tumor. The radiology, clinical course, and the biological importance of this rare neoplasm are discussed.
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5/240. Blastomatous tumor with teratoid features of nasal cavity: report of a case and review of the literature.

    A case of blastomatous tumor with teratoid features is presented. The polypoid mass was observed in the left nasal cavity of a 72-year-old man. Histologically, the lesion was composed of neuroepithelial cells with blastomatous appearance, cystic squamous nests filled with keratin materials, many mucous glands, complex tubular and glandular structures with edematous fibroblastic stroma. Sinonasal neoplasms including teratoid components and immature neuroepithelium are exceedingly rare. We suggest that the term 'immature teratoma' is more suitable than blastoma or blastomatous tumor when there is no carcinomatous or sarcomatous component besides the immature neuroepithelium and teratoid elements.
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6/240. Gliomatosis peritonei as a complication of a ventriculoperitoneal shunt: case report and review of the literature.

    Gliomatosis peritonei, the implantation of neuroglial tissue upon the peritoneal surfaces, is a rare event most often associated with solid or immature teratomas of the ovary in young girls. The authors report a case of a 10-month-old girl with a ventriculoperitoneal shunt (VPS) who presented with bilateral inguinal hernias. herniorrhaphy was uneventful. Microscopic examination of the hernia sacs showed exuberant mesothelial hyperplasia containing multiple nests of differentiated glial tissue. Subsequent computed tomography and laparoscopy disclosed normal ovaries with no evidence of intraabdominal or pelvic abnormalities. Gliomatosis peritonei in this case was attributed to transport of glial tissue from the cerebrospinal fluid into the peritoneal cavity via the shunt. With the exclusion of an ovarian germ cell neoplasm and in the presence of a VPS, the clinical course with regard to the glial implants in these children is uneventful. If it is appreciated that gliomatosis peritonei may be a complication of a VPS, an extensive clinical evaluation generally is unnecessary.
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7/240. A case of klinefelter syndrome with retroperitoneal teratoma.

    klinefelter syndrome (KS) is often associated with various neoplasms, especially germ cell tumors. mediastinum is the most favored site of extragonadal germ cell tumors with KS, which is somewhat different from those without KS. The retroperitoneal germ cell tumor in KS is very rare. A five-month-old boy with an abdominal mass was found to have a retroperitoneal tumor. After surgical removal, he was diagnosed to have mature cystic teratoma. Cytogenetic study of his peripheral lymphocytes revealed that his karyotype was consistent with KS. This case suggests that patients with KS might be at risk of having germ cell tumors in sites other than mediastinum. It also suggests that all cases with these tumors should be screened for the presence of karyotypic abnormalities, and it might help to assess the exact correlation between germ cell tumors and KS, and to treat them accordingly.
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8/240. tricuspid valve metastasis from testicular carcinoma: a case report and review of the literature.

    A 20-year-old man with Stage II nonseminomatous germ cell tumor underwent chemotherapy and multiple surgical resections of recurrent abdominal and supradiaphragmatic mature teratomas. Evaluation of a new heart murmur led to the diagnosis of tricuspid valve teratoma, which required complete valve excision and replacement. We present our experience with the first discrete tricuspid valve metastasis from testicular carcinoma and review the literature regarding cardiac metastases from germ cell neoplasms.
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9/240. Mature teratoma of the nasal vestibule: a case report.

    Teratomas are rare congenital neoplasms that arise from pluripotent cells. They consist of tissue from all three embryonic germ layers. According to most studies, teratomas of the head and neck account for only 2 to 9% of all cases. In this article, we report the first case of a teratoma of the nasal vestibule in a newborn. We also discuss methods of diagnosis and treatment of upper airway teratomas, and we include a brief review of the literature.
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10/240. Alpha-fetoprotein production by pancreatic tumors exhibiting acinar cell differentiation: study of five cases, one arising in a mediastinal teratoma.

    Five cases of pancreatic neoplasms accompanied by production of alpha-fetoprotein (AFP) and serum elevation of this marker are presented, and the better-documented cases of this phenomenon from the literature are reviewed. Four of the cases originated in the orthotopic pancreas, whereas the fifth arose in the pancreatic component of a mediastinal teratoma. The patients were children or young adults. AFP production in pancreatic tumors is closely linked to acinar differentiation, most cases representing either pancreatoblastoma or acinar cell carcinoma. The distinction between these 2 tumors may be difficult because of the many morphologic and immunohistochemical features they share.
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