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Cutaneous follicle center cell lymphoma, follicular type.

This article discusses the clinicopathologic and molecular features of primary cutaneous follicle center cell lymphoma, follicular type. Synthesis of morphologic, immunohistochemical, and molecular studies have clearly characterized this peculiar morphological variant of the cutaneous B-cell lymphomas. Although local recurrences can be frequently observed, the overall prognosis of these patients is very good and extracutaneous dissemination is very rare.

Humans↗

Refluxing megaureter for the Mitrofanoff channel using continent extravesical detrusor tunneling procedure.

PURPOSE: We present a variation on the continent procedure using the refluxing megaureter for the Mitrofanoff channel, and its results. MATERIALS AND METHODS: The Mitrofanoff procedure using the refluxing megaureter was performed in 35 patients (valve bladder syndrome 15, neurogenic bladder 10, non-neurogenic bladder 10) between 1995 and 2001. Mean patient age was 5.9 years. In 5 patients the distal segment of the megaureter was used after nephrectomy, and in 30 patients the proximal segment of the megaureter was simultaneously reimplanted unilaterally. The distal segment of the megaureter was inserted under the detrusor close to the native hiatus, pulled through the tunnel between the unresected detrusor and the mucosa, and subsequently brought to the abdominal wall. The ureterovesical junction was left intact. RESULTS: In all of our patients we obtained sufficient length and good vascularization of both ureteral segments. Satisfactory tunnel length was achieved in 29 patients. In 6 cases the tunnel was elongated by dissection of the detrusor. Median followup was 37 months. Three patients had development of stenosis at the stoma level, which resolved with minimal revision at the ureter-skin level. Minimal leakage occurred in 3 patients, all of whom were successfully treated with anticholinergics. On routine followup no patient had signs of reflux recurrence in the reimplanted ureter. CONCLUSIONS: The results of our variant procedure expand the number of patients who may benefit from use of the ureter for the Mitrofanoff channel.

Adolescent↗

Multiple-time scale accelerated molecular dynamics: addressing the small-barrier problem.

We present a method for accelerated molecular-dynamics simulation in systems with rare-event dynamics that span a wide range of time scales. Using a variant of hyperdynamics, we detect, on the fly, groups of recurrent states connected by small energy barriers and we modify the potential-energy surface locally to consolidate them into large, coarse states. In this way, fast motion between recurrent states is treated within an equilibrium formalism and dynamics can be simulated over the longer time scale of the slow events. We apply the method to simulate cluster diffusion and the initial growth of Co on Cu(001),where time scales spanning more than 6 orders of magnitude are present, and show that the method correctly follows the slow events, so that much larger times can be simulated than with accelerated molecular dynamics alone.

Journal Article↗

Treatment of a large, ulcerating, granular-cell tumor by microscopically controlled excision.

Malignant granular-cell tumors with benign histologic appearance are normally identified only following nodal and metastatic spread. Local recurrence following conventional excision of both the benign and malignant variant is not uncommon. A patient with a large, rapidly growing, spontaneously ulcerating granular-cell tumor was treated by microscopically controlled (Mohs) surgery. The rationale for this treatment, as well as criteria for the differentiation of the benign from the malignant variant, is discussed.

Biopsy↗

"Clear cell" atypical fibroxanthoma.

An 87-year-old man with extensive solar damage presented with a 2-month history of a dome-shaped, crusted lesion on the dorsum of the left hand. Microscopically, the tumor consisted of fascicles of spindle cells with bizarre nuclei and clear, vacuolated cytoplasm. Histochemical, immunohistochemical, and ultrastructural features supported the diagnosis of atypical fibroxanthoma. There was no evidence of metastatic disease. The lesion was completely excised, and there has been no recurrence in 3 months of observation. This unusual "clear cell" variant of atypical fibroxanthoma must be distinguished from other clear cell tumors, such as metastatic renal cell carcinoma, clear cell eccrine carcinoma, and clear cell sarcoma (malignant melanoma) of soft parts. Although atypical fibroxanthoma is usually cured by complete surgical excision, metastases have been reported.

Aged↗

Stasis papillomatosis. Clinical features, etiopathogenesis and radiological findings.

Stasis papillomatosis can be divided into localized and widespread forms. Ten patients with this disease are discussed with emphasis on their clinical and lymphoradiological findings. Using isotope lymphography we were able to find an overall lymphostasis in only four patients. In all patients, local lymphatic disturbances were detected by means of indirect lymphography with intradermal lymph cysts as the most specific sign. Local dermal lymphostasis seems to be the common final pathogenesis in spite of different etiologies (ie, primary lymphedema, chronic venous insufficiency, trauma recurrent erysipelas, and local lymphangiodysplasia of unknown origin). A maximum variant was seen, following en-bloc resection of subcutaneous tissue in a patient suffering from congenital lymphedema.

Adult↗

Giant intravenous glomus tumor.

We report a painful intravenous glomus tumor located in the right forearm of a 79-year-old woman. The tumor originated from the wall of a vein, protruded into its lumen and was completely excised. The largest dimension of the tumor occluding the vein was 14 cm. Tumor cells were characterized immunohistochemically by the presence of vimentin, alpha-smooth-muscle actin, and collagen IV. Intravascular spread of the glomus tumor is rare and has been described in the stomach and subcutaneous tissue. An entirely intravenous glomus tumor has been reported only three times. However, a huge intravenous growth as in our case appears never to have been reported. A review of the intravascular cases showed that the average age of presentation is 61.5 years (range 40-79 years) and the most frequent location is the forearm. Despite this intravascular growth, there is no evidence of aggressive clinical behavior, recurrence or metastasis. The pathologist must be aware of this variant of glomus tumor to avoid misdiagnosis and unnecessary additional treatments.

Actins↗

Fourier-transform infrared spectroscopic analysis is a powerful tool for studying the dynamic changes in Staphylococcus aureus small-colony variants.

Infections due to small-colony variants (SCVs) of Staphylococcus aureus in patients with chronic and recurrent infections are an emerging problem; however, studies with this subpopulation are hampered by the fact that SCVs may exhibit unstable phenotypes, making them difficult to study, particularly in broth media. In this study, two S. aureus sets comprising the (i) normal and the (ii) SCV phenotype (clonal with normal phenotype) recovered from clinical specimens, as well as (iii) corresponding site-directed mutants displaying the SCV phenotype (knockout of hemB) and (iv) their complemented mutants were examined by Fourier-transform infrared (FTIR) spectroscopy. Phenotypes were defined on solid and in broth media. Using first-derivative infrared spectra to calculate spectral distances, hierarchical clustering based on spectral information resulted in a dendrogram with clear discrimination between SCV and normal phenotypes. The SCVs gave an FTIR fingerprint that was easily recognizable and that was much closer to other SCVs than to their parent strains. This technique offers for the first time a noninvasive approach to investigate dynamic processes of reversion of SCVs to the normal phenotype and vice versa. Thus, FTIR spectroscopy allowed a rapid and reproducible tool for the examination of different subpopulations of S. aureus on solid and in broth media for diagnostic and research purposes.

Bacterial Proteins↗

Episodic macroglossia as the sole manifestation of angiotensin-converting enzyme inhibitor-induced angioedema.

We describe a patient who had recurrent life-threatening episodes of isolated macroglossia due to the use of an angiotensin-converting enzyme (ACE) inhibitor. No associated facial, labial, pharyngeal, or laryngeal edema was noted. Aggressive treatment with epinephrine, steroids, and antihistamines resulted in rapid resolution of the tongue swelling and respiratory distress. Recurrent isolated angioedema of the tongue is an extremely rare variant of ACE inhibitor-related angioneurotic edema. The widespread use of ACE inhibitors mandates a special awareness by physicians of this potentially life-threatening yet treatable side effect.

Aged↗

Adenomatoid and calcifying epithelial odontogenic tumors.

The histologic diversity of the odontogenic epithelium's neoplasms is nicely illustrated by the adenomatoid and the calcifying epithelial odontogenic tumors. The former has a predilection for the maxilla, the latter for the mandible. Neither has a malignant variant, and each is controlled by conservative local removal. The recurrence rate of the adenomatoid odontogenic tumor is 0.2%, while the calcifying epithelial odontogenic tumor's rate is 14%.

Female↗

Vanishing White Matter Disease With EIF2B2 c.254 >A Variant: Mild Clinical and MRI Findings.

OBJECTIVES: Typical MRI findings of vanishing white matter disease (VWM) include diffuse white matter lesions with cystic degeneration. However, mild cases may lack these typical features, posing diagnostic challenges. METHODS: We describe 2 of 3 individuals carrying the homozygous c.254T >A variant in EIF2B2 identified at our hospital, excluding 1 previously reported case.1 Genetic analyses were performed using whole-genome sequence or whole-exome sequence analysis, and detected variants were confirmed by direct nucleotide sequence analysis. Brain MRI findings and clinical features were reviewed for the 2 individuals along with other cases in the literature with the same variant. RESULTS: A 69-year-old woman presented with recurrent transient dizziness and secondary amenorrhea. MRI of the brain revealed small T2-hyperintense lesions confined to the subcortical white matter with hyperintensities on diffusion-weighted images and mildly elevated apparent diffusion coefficient values. A 28-year-old woman presented with transient dizziness and secondary amenorrhea. MRI of the brain showed mild T2-hyperintense lesions in the cerebral white matter with frontal predominance. DISCUSSION: This report highlights the clinically mild cases of VWM with subtle abnormalities on brain MRI who had the homozygous c.254T >A in EIF2B2, further expanding the clinical spectrum of VWM and underscoring the importance of genetic assessments in the diagnosis of individuals with mild clinical and MRI findings.

Journal Article↗

Differential reactivity for galectin-3 in Hürthle cell adenomas and carcinomas.

Hürthle cell carcinomas behave as the most aggressive variant of differentiated thyroid carcinoma of follicular origin, with frequent recurrences and higher morbidity. Its differential diagnosis with Hürthle cell adenoma remains a problem for the clinician and for the pathologist. The vertebrate lectins, galectin-1 and galectin-3 have been implicated in the regulation of cellular growth, differentiation, and malignant transformation in thyroid neoplasms. Galectin-3, a beta-galactoside binding protein, has been recently found to be highly expressed in papillary and follicular carcinomas. The current study was undertaken to investigate immunohistochemical reactivity for galectin-3 of thyroid specimen tissues with Hürthle cell adenomas (n = 14) and carcinomas (n = 17), follicular (n = 14) and papillary (n = 11) carcinomas, colloid goiter (n = 30), Hashimoto's thyroiditis (n = 11), follicular adenoma (n = 9), and normal thyroid tissues (n = 18). Follicular (78.5%) and papillary (82.0%) carcinomas were frequently reactive for galectin-3, more often when some H rthle cells were present. There was no galectin-3 immunostaining in any of the specimens from Hashimoto's thyroiditis, colloid goiters or normal thyroid samples, whereas only one case of follicular adenoma was found positive (11.1%). By contrast, galectin-3 immunostaining in Hürthle cell carcinomas was significantly higher (59%) than in H rthle cell adenomas (7.1), p < 0.05). These results suggest that galectin-3 may potentially serve as a marker in difficult differential diagnosis cases involving Hürthle cell adenomas and Hürthle cell carcinomas.

Adenocarcinoma, Follicular↗

Pediatric Hematogenous Osteomyelitis: New Trends in Presentation, Diagnosis, and Treatment.

The character of acute hematogenous osteomyelitis (AHO) in North American children has changed significantly during the past several decades. Although the typical clinical picture of established acute osteomyelitis in children (illness, dehydration, and an acutely painful limb) is still seen, more subtle presentations appear more frequently. Children often present with subacute osteomyelitis. Less common variants include Brodie's abscess, subacute epiphyseal osteomyelitis, and chronic recurrent multifocal osteomyelitis. Some patients present with a bone lesion that may be confused with other disease entities, including neoplasms. Biopsy is often needed to clarify the diagnosis. With the trend toward more invasive procedures in the neonatal intensive care unit, neonatal osteomyelitis is also seen more frequently. Advances in imaging technology, particularly improvements in technetium bone scanning and the advent of magnetic resonance imaging, have contributed to more precise diagnosis and better management of AHO. With the increased concern about medical economics, the recent trend toward decreasing the duration of intravenous antibiotic treatment of these infections appears to be appropriate as long as certain criteria are met. Neither surgery nor antibiotics alone will be associated with successful treatment in all cases, and this fact may explain the rare but continued morbidity that is still seen in children with AHO.

Journal Article↗

[Mutations in the BRCA1 gene in young Spanish women with breast cancer].

BACKGROUND: Germline mutations in the BRCA1 gene have been associated with familial breast/ovarian cancer. Furthermore, women diagnosed of early-onset breast cancer have a higher probability of being carriers of BRCA1 mutations. Our aim was to know prevalence of BRCA1 mutations in women with breast cancer diagnosed before 40 years. PATIENTS AND METHODS: We analyzed genomic DNA samples of 159 women with early-onset breast cancer. Ten fragments of BRCA1 gene covering the 36% of cases with mutations described in the literature were screened. Analysis involved polymerase chain reaction (PCR), single-strand conformation polymorphisms (SSCP) and direct sequencing. RESULTS: Three germline BRCA1 mutations were identified, one of them not previously described. Two mutations were found in women with familial history of breast cancer. Five additional rare variants and polymorphisms were also detected. CONCLUSIONS: The absence of recurrent mutations or mutations detected in other countries, except for the 185delAG mutation, present in Ashkenazim population, shows the influence of ethnic and geographic origin of population studied, and illustrates the difficulties of establishing DNA-based screening tests for hereditary breast cancer.

Adolescent↗

Trisomy 21 is a recurrent secondary aberration in childhood acute lymphoblastic leukemia with TEL/AML1 gene fusion.

TEL/AML1 gene fusion is the most frequent genetic lesion in pediatric acute lymphoblastic leukemia (ALL). It occurs as a consequence of the cryptic chromosomal translocation t(12;21)(p13;q22). In a cohort of 50 RT-PCR-positive TEL/AML1 patients, karyotype examination by GTG banding and fluorescence in situ hybridization (FISH) allowed us to identify chromosome anomalies in addition to the already existing t(12;21). Secondary aberrations were found in 29 out of 41 patients (71%) at initial diagnosis and in all 9 patients with relapse. Structural rearrangements affected chromosome arms 2p, 2q, 5q, 9p, 12p (n = 2), 6q, 11p (n = 3), and 21q (n = 4). An extra chromosome 21 was found to be the most frequent anomaly. It was detected in 6 out of 41 patients at initial diagnosis (15%) and in 7 out of the 9 patients at relapse. No karyotype with trisomy 21 exceeded 47 chromosomes. Gain of chromosome 21 was the sole anomaly in GTG-banding analysis in 2/41 patients at initial diagnosis and in 4/9 at relapse. Notably, chromosome painting analysis performed in 11 out of the 13 patients with an extra chromosome 21 revealed duplication of the normal chromosome 21 in 8, and duplication of der(21)t(12;21) in 3 patients. Furthermore, gain of der(21)t(12;21) chromosome was confined exclusively to the relapse patients.

Child↗

[Duodeno-pyloroplasty by resected stomach in ulcer disease].

Organ-saving operation in ulcer disease--duodenopyloroplasty with resected stomach--is proposed. Unlike the other pylorus-saving operations, this variant is universal, because it allows to remove ulcer in different location and also to correct acidity. The operation was performed in 647 patients with complicated forms of ulcer disease. Postoperative lethality was 0.46% (3 patients). Stomach motor-evacuatory function disorders were revealed in 21 (3.40%) patients, 7 (1.08%) of them underwent repeated operation. Depending on acidity before operation, different variants of acidity correction were applied. There was no ulcer recurrence in patients with normacidic gastric ulcers. Patients with hyperacidic pyloroduodenal ulcers had the risk of recurrence. From 472 operated patients high acidity after operation was revealed in 33 (6.99%), in 16 (3.38%) of them the recurrence was observed. Reoperation was performed in 13 (2.75%) patients. In comparative assessment of long-term results, the sum of "penal" points in duodenopyloroplasty was 4 times smaller than in standard stomach resection.

Adult↗

CV706, a prostate cancer-specific adenovirus variant, in combination with radiotherapy produces synergistic antitumor efficacy without increasing toxicity.

Radiation is an effective means of treating localized prostate cancer. However, up to 40% of men with certain risk factors will develop biochemical failure 5 years after radiotherapy. CV706, a prostate cell-specific adenovirus variant, is currently in clinical trials for the treatment of recurrent organ-confined prostate cancer. We demonstrated previously that a single administration of CV706 at 5 x 10(8) particles/mm3 of tumor eliminated established tumors within 6 weeks in nude mouse xenografts (Rodriguez et al., Cancer Res. 57: 2559-2563, 1997). We now demonstrate that CV706-mediated cytotoxicity is synergistic with radiation. In vitro, addition of radiation to CV706 resulted in a synergistic increase of cytotoxicity toward the human prostate cancer cell line LNCaP and a significant increase of virus burst size, with no reduction in specificity of CV706-based cytopathogenicity for prostate cancer cells. In vivo, prostate-specific antigen (+) LNCaP xenografts of human prostate cancer were treated with CV706 (1 x 10(7) particles/mm3 of tumor), 10 Gy of single fraction local tumor radiation, or both. Tumor volumes of the group treated with CV706 or radiation was 97% or 120% of baseline 6 weeks after treatment. However, when the same dose of CV706 was followed 24 h later with the same dose of radiation, the tumor volume dropped to 4% of baseline at this time point and produced antitumor activity that was 6.7-fold greater than a predicted additive effect of CV706 and radiation. Histological analyses of tumors revealed that, compared with CV706 or radiation alone, combination treatment with two agents increased necrosis by 180% and 690%, apoptosis by 330% and 880%, and decreased blood vessel number by 1290% and 600%, respectively. Importantly, no increase in toxicity was observed after combined treatment when compared with CV706 or radiation alone. These data demonstrate that CV706 enhances the in vivo radioresponse of prostate tumors and support the clinical development of CV706 as a neoadjuvant agent with radiation for localized prostate cancer.

Adenoviridae↗

[Clinical classification of benign ENT tumor].

The proposed clinical classification of benign ENT tumors is based on division of anatomic regions (the ear, nose, pharynx, larynx) into sections and fragments and allows to assess the spread of any ENT tumor. Moreover, it agrees with TNM classification. The symbols "T" and R are used which denote four variants (T1-4) or R1-4 for the tumor and recurrence, respectively.

Ear Neoplasms↗