Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neurothekeoma is a rare benign dermal lesion that is commonly seen in children and teenagers. Despite its name, the true nature of this lesion is uncertain and controversial, particularly after the emergence of the cellular (spindle/epithelioid) variant. We describe the histological and immunohistochemical findings of a right thigh skin lesion in an 11-year-old girl. It consists of a dermal ill-defined plexiform mass composed of nests and fascicles of spindle cells with pale eosinophilic cytoplasm that lie within a sclerotic stroma. The immunohistochemistry shows diffuse reactivity to CD68, matrix metalloproteinase-II, CD10 and PGP9.5 with focal reactivity to CD57 and CD34. The lesion is negative for S100, factor XIIIa, smooth muscle markers and melanocytic markers. The features are compatible with a cellular variant of neurothekeoma with plexiform pattern that also exhibits an unusual pattern of fibrohistiocytic phenotype. Although such a lesion is benign, it has a wide but important differential diagnoses that are reviewed briefly together with a brief discussion about the origin of this rare entity.
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PMID:Cellular neurothekeoma with a plexiform morphology: a case report with a discussion of the plexiform lesions of the skin. 1730 11

This report describes the clinicopathologic findings in 176 patients who presented with 178 tumors currently referred to as neurothekeomas. Our study group included 64 males and 112 females, ranging from 20 months to 85 years old at the time of their first surgical procedure (median age: 17 y). Twenty-four percent of patients were <or=10 years of age and only 20% of patients were >or=30 years of age at initial diagnosis. The patients typically presented with a solitary, superficial, slow-growing, and relatively asymptomatic mass in the 0.3 to 2.0 cm size range. One patient had multiple tumors. More than 75% of the lesions involved the head (n=63), upper extremities (n=44), and shoulder girdle (n=27) regions. The tumors were evident a few weeks to 4 years (median duration: approximately 7 mo) before surgical resection was sought. Histologically, the lesions involved the dermis and/or subcutis, and they formed multinodular masses with varying amounts of myxoid matrix and peripheral fibrosis. On the basis of the amount of myxoid matrix, the tumors were subclassified as cellular (n=63), mixed (n=67), or myxoid (n=48). All cases had spindled and epithelioid mononuclear neoplastic cells with relatively abundant cytoplasm and indistinct cell borders. The majority of cases also had occasional multinucleated tumor cells. The lesional cells had a strong tendency for whorled growth, and oftentimes, focal fascicular growth was also present. Nuclear atypia was minimal in 62 cases, mild in 73 cases, at least focally moderate in 41 cases, and focally marked in 2 cases. Mitotic activity ranged from 0 to 124 mitotic figures/25 wide-field high power fields (WHPFs) (median mitotic count: 4 mitotic figures/25WHPFs). Twenty-five lesions had >10 mitotic figures/25WHPFs. A total of 16 cases (9%) had atypical mitotic figures. Osteoclastlike giant cells were detected in 39% of cases. Immunoreactivity was typically present for vimentin, NKI/C3, CD10, microphthalmia transcription factor, and PGP9.5, and focal reactivity was sometimes noted for smooth muscle actin and CD68. All tumors tested were negative for S100 protein, glial fibrillary acidic protein, and Melan A. The overwhelming majority of cases had involvement of the tissue margins. A complete follow-up record is available for 71 patients (40.3%) with follow-up intervals ranging from 3 years 2 months to 34 years 9 months (median: 17 y 9 mo). Limited or incomplete follow-up information is also available for an additional 14 patients with follow-up intervals ranging from weeks to approximately 10 years (median: 5 mo). Regrowth of tumor after biopsy or local excision was reported in 13 patients, one of whom had 2 recurrences. However, because of the nature of our consultation practice and a tendency for clinicians to specifically send us cases with a complex clinical course, this is believed an overestimation of the true recurrence rate. Neurothekeomas are morphologically and immunohistochemically distinct from true nerve sheath myxomas. An origin from fibroblastic cells with the ability to differentiate into myofibroblasts and a tendency to recruit histiocytic cells is postulated.
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PMID:Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information. 1759 78

Substance P has a stimulating effect on fibroblast proliferation, collagen organization, and angiogenesis in ruptured and subsequently sutured rat Achilles tendon. This effect is also reflected in the biomechanical properties of the tendon. The aim of this study was to substantiate the effect of exogenous substance P on endogenous substance P, NK-1 receptor, and nerve ingrowth in an in vivo tendon-healing setting. Ninety-six male Sprague-Dawley rats were randomly assigned to one of four groups and injected with saline, substance P (10(-6) micromol/kg BW and 10(-8) micromol/kg BW) associated with neutral endopeptidase inhibitors, or neutral endopeptidase inhibitors alone into the paratendinous region of the ruptured and subsequently sutured Achilles tendons from the second until the sixth day postoperatively. Substance P, NK-1 receptor, and nerve ingrowth (PGP 9.5) were analysed using immunofluorescence at four different time points: one, two, four and six weeks postoperatively. In all groups substance P was predominantly expressed in the extracellular matrix during the first two weeks, corresponding to fibroblast proliferation, and first disappeared from the saline group in the proliferative phase. In contrast, substance P was not expressed in the blood vessel wall during the first two weeks, when angiogenesis was most pronounced. NK-1 receptor was almost always expressed in the blood vessel wall and in the extracellular matrix during this period and disappeared progressively afterwards. No nerve ingrowth was identified. Exogenously administered substance P in sutured rat Achilles tendon rupture does not stimulate sensory nerve ingrowth, but seems to have a booster effect on endogenous substance P for fibroblast proliferation via autocrine/paracrine stimulation.
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PMID:Qualitative analysis of substance P, NK1-receptor and nerve ingrowth in substance P-treated ruptured rat Achilles tendon. 2069 62

We report an unusual case of a fibrolipomatous hamartoma that arose in a nuchal nerve. Typically, fibrolipomatous hamartoma, otherwise known as a neural fibrolipoma or lipomatosis of nerve, arises in the median nerve, brachial plexus, cranial nerves, or plantar nerves. The differential diagnosis is broad and includes benign and malignant spindle cell lesions, such as spindle cell lipoma, perineurioma, and myxoid liposarcoma. We were able to identify the lesion based on the typical histology, including triphasic composition with spindle cell, neural, and adipocytic components and whorled architecture. Because of the atypical location in the neck, detailed immunohistochemical staining was performed. The lesional spindle cells were negative for SMA, CD10, CD68, EMA, S100, PGP9.5, CD34, CD56, and beta-catenin. Colloidal iron stain highlighted marked intralesional mucin deposition. This detailed immunohistochemical profile is a useful diagnostic aid and to our knowledge has not been previously described.
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PMID:Fibrolipomatous Hamartoma of the Nerve Arising in the Neck: A Case Report With Review of the Literature and Differential Diagnosis. 2503 11

Ectomesenchymal chondromyxoid tumor (ECT) is a rare benign tumor of uncertain lineage, which almost exclusively affects the anterior tongue. Herein, we report 2 cases of ECT occurring in 58- and 56-year-old males on the right and on the left side of the dorsum of the anterior tongue, measuring 18 mm and 10 mm, respectively. Despite positive resection margin in one case, none of the tumors recurred during follow-up of 6 and 5 years. Microscopically, both tumors had lobular architecture with a mixture of solid, microcystic, and chondromyxoid areas. The tumor cells were polygonal or elongated and showed mild atypia in one case. Immunohistochemically, both tumors showed diffuse expression of vimentin and focal positivity of CD10 and of smooth muscle actin. Regarding neural tissue-related markers, there was nearly diffuse expression of CD56 and neuron-specific enolase and focal positivity of PGP 9.5 in both cases and variable expression of CD57, synaptophysin, glial fibrillary acidic protein, and S-100 protein. Interestingly, we observed diffuse expression of SOX10 in one case. In both tumors, diffuse strong nuclear expression of cyclin D1 was present, without CCND1/IGH translocation or CCND1 amplification. The EWSR1 gene rearrangement was not detected. To the best of our knowledge, expression of SOX10, which may support neural crest origin of this peculiar lesion, has not been reported in ECT. The significance of strong cyclin D1 expression remains to be further investigated.
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PMID:Cyclin D1 Expression in Ectomesenchymal Chondromyxoid Tumor of the Anterior Tongue. 2724 Aug 62