Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0178874 (tumor progression)
 40,807 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary orbital intraosseous hemangiomas are rare--only 16 cases have been reported previously. The authors describe five patients with this benign tumor. Patients ranged in age from 49 to 77 years, and each had a painless or mildly tender nodular lesion. Ophthalmic symptoms were minimal, although nasolacrimal obstruction was the original sign in one patient. Four of five patients responded well to complete tumor excision (two patients) or partial resection of the lesion (two patients). The fifth patient required a second operation for tumor progression after partial excision of the hemangioma. The 16 previously reported cases of orbital intraosseous hemangioma are reviewed, and the natural history and pathology of this rare vascular lesion are discussed.
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PMID:Primary orbital intraosseous hemangioma. 205 11

Six cases of strawberry hemangioma have been presented in which some form of intervention in tumor progression was indicated. The argon laser was used. All six cases showed loss of surface vascularity. Growth was clearly arrested in four of the six. Premature regression could be definitely claimed in only one. The strong impression is presented that argon laser therapy, although often helpful in the more developed strawberry hemangiomas, as seen in this series, could be most effective if used when the lesion is first noted, before it has time to grow, especially in areas of high complication potential. Where feasible, further exploration into the use of the argon laser in very early strawberry hemangiomas is urged.
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PMID:Further evaluation of the potential of the argon laser in the treatment of strawberry hemangiomas. 668 10

Between July 1987 and June 1994 we operated upon 30 consecutive children suffering from endophytic intra-axial tumors located in the pons and/or medulla oblongata. The 25 children operated on between July 1987 and October 1993 whose postoperative course could be assessed for a minimum of 2 years after operation were included in this study. Operability of a brain stem tumor was shown to be independent of its size. A gross tumor resection between 80% and 100% could be performed in half these cases, and subtotal or partial resection in the other half. The radicality of resection was not influenced by tumor histopathology, but was dependent on intraoperative findings relating to its consistency, infiltration, and visibility. On follow up, 15 of the 25 children were found to have died within the period of 2 years. Two children died in the immediate postoperative period (at 2 days and 2 weeks after surgery), of acute brain stem swelling and an unsuspected bleeding disorder, respectively. The other 13 of these 15 children died of tumor progression between 1 and 19 months after operation, with a median survival time of 9 months. In the group of the surviving 10 children the histopathology was grade I astrocystoma in 6 cases, angioma in 2 cases, and grade II oligodendroglioma and grade II ependymoma in 1 case each. Postoperatively, most of the children showed some increase in their preoperative deficits, but recovered after 2-3 months. After 2 years, 10 of the 25 children who were followed up are alive and 9 of them attend regular school or kindergarten.
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PMID:Intra-axial endophytic tumors in the pons and/or medulla oblongata. II. Intraoperative findings, postoperative results, and 2-year follow up in 25 children. 913 55

We report a rare malignant endovascular papillary angioendothelioma (Dabska tumor) arising within the deep periosteal soft tissue of the ulna of an 8-year-old girl. To our knowledge, this is the first report of a Dabska tumor of deep soft tissue that did not extend down from the dermis. This Dabska tumor appears to be a focal change within a larger, well-defined, cavernous hemangioma of deep muscle. We suggest that this represents secondary neoplastic progression of an existing benign lesion, a common phenomenon among vascular tumors. A review of the family of "hobnailed" endothelial cell lesions to which Dabska tumor belongs is presented.
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PMID:Malignant endovascular papillary angioendothelioma (Dabska tumor) arising within a deep intramuscular hemangioma. 930 35

Xeroderma pigmentosum is an inheritable autosomal recessive DNA repair deficient syndrome characterized by a high predisposition to skin cancers. An elevated proportion of tumors from xeroderma pigmentosum patients harbor ultraviolet-induced mutations (CC:GG > TT:AA tandem transitions) of the p53 and/or the INK4a-ARF genes. Here, we report the clinical and molecular features of a 12 y old xeroderma pigmentosum patient who, in addition to severe cutaneous clinical symptoms, also had three unusual tumors, a mediastinal lymphoblastic lymphoma, an atypical fibroxanthoma, and an epithelioid hemangioma. Single strand conformation polymorphism and sequencing analysis of the p53 and INK4a-ARF genes were carried out in DNA from normal skin and different tumors (four actinic keratosis, two microinvasive squamous cell carcinomas, one basal cell carcinoma, and one atypical fibroxanthoma) from the patient. After characterization of the xeroderma pigmentosum C complementation group, we found unexpectedly that this patient also carried a germline mutation of the INK4a-ARF locus affecting the p16INK4A reading frame. Three different somatic mutations that all harbor the signature of ultraviolet light (two of p16INK4A and one of p53) were also detected in the basal cell carcinoma. We hypothesize that the germline mutation of p16INK4A, in association with the nucleotide excision repair defect, could explain the patient's unusual phenotype. Furthermore, this study confirms that concomitant somatic mutations of INK4a-ARF and p53 occur in some xeroderma pigmentosum associated tumors, and seem to accumulate during tumor progression rather than the initiation step.
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PMID:Germline and somatic mutations of the INK4a-ARF gene in a xeroderma pigmentosum group C patient. 1248 39

Gap junctions (GJs) have been shown to play a role in tumor progression including a variety of keratinocyte-derived and non-keratinocyte-derived skin tumors. Here we show that the synthesis of the GJ proteins connexin 26 and connexin 30 (Cx26 and Cx30) is induced in keratinocyte-derived epithelial skin tumors whereas there is either no change or a downregulation of Cx43. Cx26, Cx30, and Cx43 are absent in non-epithelial skin tumors. Further, Cx26 and Cx30 are induced in the epidermis adjacent to malignant melanoma but absent in the epidermis adjacent to benign non-epithelial skin lesions (melanocytic nevi and angioma). The keratinocyte-derived skin tumors are very heterogeneous regarding the Cx26/Cx30 pattern in the epidermis at the periphery of the tumors. We did not observe any difference in the localization of the very similar proteins Cx26 and Cx30 but a variation in intensity of immunoreactivity. As the staining patterns of Cx26 and Cx30 antibodies are not identical to those of CK6, a marker for hyperproliferation, and CK17, a marker for trauma, we discuss that the induction of these gap junctional proteins exceeds a reflection of reactive hyperproliferative or traumatized epidermis. We further discuss the putative roles of these gap junctional proteins in tumor progression.
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PMID:Differential induction of connexins 26 and 30 in skin tumors and their adjacent epidermis. 1604 68

Epithelioid hemangioendothelioma is a very rare tumor of vascular origin. It can develop in different tissues such as soft tissue, lung, or liver. Hepatic epithelioid hemangioendothelioma (HEH) mostly affects females. The malignant potential of HEH often remains unclear in the individual patient. It can range from benign hemangioma to malignant hemangioendotheliosarcoma. Here we present our experience with five patients with primary HEH, who were treated with curative intention in our department. All patients in our series with confirmed histological HEH did not show extrahepatic extension and consequently underwent surgical treatment. In three patients, liver transplantation (LTx) was performed (two cadaveric and one living related). In one patient, a right-sided hemihepatectomy with partial resection of the diaphragm was performed. One patient died while she was on the waiting list for LTx due to rapid tumor progression. Postoperative follow-up ranged from 1 to 13 years. No adjuvant chemotherapy was applied. Until now, no recurrence of local tumor or distant metastases could be observed during follow-up in our series. Early detection and surgical intervention in case of HEH can potentially offer curative treatment. The treatment of first choice appears to be radical liver resection. In our view, LTx represents a potentially important option for patients with a nonresectable tumor. Despite the long waiting time, its often unclear dignity, and a proven progressive growth pattern, living related LTx also plays a potentially important role. The 5-year overall survival rate of patients with HEH in the literature varies from 43% to 55%. Long-term survival of patients with HEH is significantly higher compared to other hepatic malignancies. The role of adjuvant therapy currently remains unclear.
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PMID:Surgical treatment of primary hepatic epithelioid hemangioendothelioma. 1628 86

A preterm male infant developed a rapidly growing, treatment-resistant orbital hemangioma. Despite aggressive management, the patient required enucleation of his right eye and image-guided ethanol sclerosis to limit tumor progression intracranially.
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PMID:Orbital hemangioma requiring enucleation in a premature infant. 1716 74

Vascular tumours are common lesions of the skin and subcutaneous tissue, but also occur in many other tissues and internal organs. The well-differentiated tumours consist of irregular anastomosing, blood-filled vascular channels that are lined by variably atypical endothelial cells. The less differentiated tumours may show solid strands and sheets, resembling carcinoma or lymphoma. Several growth factors, including basic fibroblast growth factor, transforming growth factors and vascular endothelial growth factor, play a role in tumour angiogenesis. Growth hormone (GH) is mitogenic for a variety of vascular tissue cells, including smooth muscle cells, fibroblasts and endothelial cells and exerts its regulatory functions in controlling metabolism, balanced growth and differentiated cell expression by acting on specific membrane-bound receptors, which trigger a phosphorylation cascade resulting in the modulation of numerous signalling pathways and of gene expression. Essential to the initiation of a cellular response to GH, the presence of receptors for this hormone may predict the adaptation of tumour cells resulting from GH exposure. To address the site/mode of action through which GH exerts its effects, a well characterized monoclonal antibody, obtained by hybridoma technology from Balb/c mice immunized with purified rabbit and rat liver GH-receptor (GHR) and directed against the hormone binding site of the receptor, was applied, using the ABC technique to determine GHR expression in a panel of vascular tumours. The GHR was cloned from a rabbit liver cDNA library with the aid of an oligonucleotide probe based on a 19 residue tryptic peptide sequence derived from 5900 fold purified rabbit liver receptor. A total of 64 benign and malignant vascular tumours were obtained from different human organ sites, including the chest wall, skin, axillary contents, duodenum, female breast, abdomen, stomach, colon, lymph node, bladder, body flank and neck regions. The tumours were of the following pathological entities: Haemangioma (n = 12); haemangioendothelioma (n = 10); Castleman's disease (n = 3), haemangiopericytoma (n = 4); angiosarcoma, (n = 11), Kaposi's sarcoma with focal infiltration by lymphoma, HIV +ve (n = 7), Kaposi's sarcoma (n = 17). The endothelial cell marker CD-31 was used to establish endothelial cell characteristics and microvascular density. To delineate tumour cell growth, immunohistochemical analysis of cycling nuclear protein and of proliferating cell nuclear antigen, using Ki-67 and PCNA polyclonal antibodies respectively, was used to demonstrate proliferative indexes. Results show that, compared to their normal tissue counterparts, nuclear and cytoplasmic expression of GHR consistently result in strong receptor immunoreactivity in the highly malignant angiosarcomas and Kaposi's sarcomas and was localized in the cell membranes and cytoplasm, but strong nuclear immunoreactivity was also identified. The presence of intracellular GHR is the result of endoplasmic reticulum and Golgi localization. Nuclear localization is due to identical nuclear GHR-binding protein. Furthermore, there was a positive correlation of GHR immunoreactivity with neoplastic cellular proliferation and cycling, as measured by Ki-67 and PCNA. In conclusion, this study shows that GHR expression in vascular tumours is a function of malignancy and cancer progression. Malignant cells, which are highly expressive of the receptor, have a greater proliferation rate and thereby also higher survival rate compared to tumours expressing lower or minimal receptor level. The presence of GHR in endothelial cells of vascular neoplasm indicates that they are target cells and GH is of importance in the proliferation of vascular tumour angiogenesis. GH is necessary not only for differentiation of progenitor cells, but also for their subsequent clonal expansion and maintenance. The results support the hypothesis that GH is involved in the paracrine-autocrine mechanism, acting locally in regulating vascular tumour growth and will be useful for site-specific studies of the evolution of vascular cancers. The use of anti-GHR antibodies to block tumour progression is an intriguing possibility.
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PMID:Growth hormone in vascular pathology: neovascularization and expression of receptors is associated with cellular proliferation. 1822 92

In order to determine possible differences in serum gangliosides content and composition before and after surgical removal of tumor, gangliosides isolated from preoperative and postoperative sera of patients with brain tumors were analyzed. Serum samples were collected from patients with glioblastoma, meningioma, acoustic neurinoma, haemangioma, oligodendroglioma and astrocytoma, one week before and one week after surgical removal of the tumor. Serum gangliosides were qualitatively and quantitatively analyzed by high performance thin layer chromatography and laser densitometry. Results showed changes of total gangliosides concentrations in analyzed postoperative sera comparing to preoperative sera. There was not a significant difference in ganglioside pattern of preoperative vs. preoperative sera. However, a postoperative decreased proportion of ganglioside GD3 was observed in sera derived from patients with complete tumor removal. The results of this study indicate that comparative quantitative and compositional analysis of both preoperative and postoperative serum gangliosides may provide useful information concerning tumor progression, surgical success and prognosis.
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PMID:Serum gangliosides in patients with brain tumors. 1840 78


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