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Signal transduction pathway analysis in fibromatosis: receptor and nonreceptor tyrosine kinases.

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Despite reports of receptor tyrosine kinase activation in desmoid-type fibromatosis, therapeutic benefits of kinase inhibitor therapy are unpredictable. Variability in signal transduction or cellular kinases heretofore unevaluated in desmoid tumors may be responsible for these inconsistent

Myxoma virus and Shope fibroma virus encode dual-specificity tyrosine/serine phosphatases which are essential for virus viability.

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Sequence analysis of the genomes of the Leporipoxviruses myxoma virus and Shope fibroma virus (SFV) led to the discovery of open reading frames homologous to the vaccinia H1L gene encoding a soluble protein phosphatase with dual tyrosine/serine specificity. These viral phosphatase genes were

Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors.

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OBJECTIVE Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype. The role of chemotherapy in AF is not well defined, but can be useful in some cases. We examined the response of a case to both imatinib and

The tyrosine phosphatase SHP2 is required for cell transformation by the receptor tyrosine kinase mutants FIP1L1-PDGFRα and PDGFRα D842V.

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Activated forms of the platelet derived growth factor receptor alpha (PDGFRα) have been described in various tumors, including FIP1L1-PDGFRα in patients with myeloproliferative diseases associated with hypereosinophilia and the PDGFRα(D842V) mutant in gastrointestinal stromal tumors and inflammatory

Proof of principle for bevacizumab activity in desmoid-type fibromatosis.

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BACKGROUND Desmoid-type fibromatosis (DF) is a rare disease, which often occurs in young adults. Medical treatment is an important option in the treatment algorithm of DF. Different chemotherapeutic regimens showed clinical activity in DF, but overall treatment tolerability remains poor for this

Vulvar fibromatosis: a clinical enigma.

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OBJECTIVE To present a case of recurrent vulvar fibromatosis in an adolescent, discuss the specific difficulties of treating adolescents, and review the literature on available treatment. METHODS We present a case of recurrent vulvar fibromatosis in a 14-year-old girl, requiring several treatment

Isolation of protein-tyrosine phosphatase-like member-a variant from cementum.

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Cementum has been shown to contain unique polypeptides that participate in cell recruitment and differentiation during cementum formation. We report the isolation of a cDNA variant for protein-tyrosine phosphatase-like (proline instead of catalytic arginine) member-a (PTPLA) from cementum. A

Desmoid-type fibromatosis involving the brachial plexus.

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Desmoid-type fibromatosis involving the brachial plexus is a rare and challenging disease. Due to involvement of crucial neurovascular structures, wide local excision of the associated fibromas is rarely feasible and recurrence is common. The authors describe their experience in four surgically

Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor).

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Aggressive fibromatosis (also called desmoid tumor) occurs as a sporadic lesion or as part of Familial Adenomatous Polyposis, which is caused by germ line mutations in the Adenomatous polyposis Coli (APC) gene. APC is involved in the regulation of the cellular level of beta-catenin, which is a

Control of aggressive fibromatosis by treatment with imatinib mesylate. A case report and review of the literature.

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OBJECTIVE There has been only one report available that focuses on the treatment with imatinib mesylate of two individual persons with aggressive fibromatosis. The authors concluded that after long-term treatment, for 9 and 11 months, with imatinib mesylate, both patients demonstrated radiographic

Receptor tyrosine kinases and their hormonal regulation in uterine leiomyoma.

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Uterine leiomyomas (fibroids, myomas) are benign tumors that develop from smooth muscle cells. Although the most common gynecologic tumor in premenopausal women, there is still little known of the etiology, the genetics and basic/molecular biology, or the influence of the environment on the

Cementum attachment protein/protein-tyrosine phosphotase-like member A is not expressed in teeth.

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Cementum is a highly specialized connective tissue that covers tooth roots. The only cementum-specific protein described to date is the cementum attachment protein (CAP). A putative sequence for CAP was established from a cDNA clone isolated from a human cementifying fibroma cDNA library. This

Extra-abdominal aggressive fibromatosis treated with meloxicam and sorafenib: An encouraging option

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Objective: Aggressive fibromatosis (AF), also called desmoid tumor, is an uncommon soft-tissue neoplasm. Characteristically, it expands locally without metastatic potential. However, its tendency of relapse after curative resections has

Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: a case report.

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Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which

Five years of local control of subscapularis aggressive fibromatosis managed by surgery and imatinib: a case report.

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BACKGROUND Imatinib, a tyrosine kinase inhibitor, is a major therapeutic option for the management of unresectable aggressive fibromatosis. Unfortunately, for most patients of low or very low average income countries, surgery often is the first treatment option. This is related to unavailability of

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