Third Report of t(19q)(13.4) in Mesenchymal Hamartoma of Liver with Comments on Link to Embryonal Sarcoma

1998 ◽  
Vol 1 (5) ◽  
pp. 438-442 ◽  
Author(s):  
Kevin E. Bove ◽  
Ruth I. Blough ◽  
Shirley Soukup
Keyword(s):  
Developmental Disorders ◽  
Chromosome Abnormality ◽  
Post Treatment ◽  
Mesenchymal Hamartoma ◽  
Low Grade ◽  
Liver Lesions ◽  
Balanced Translocation ◽  
The Third ◽  
Karyotype Abnormality ◽  
Embryonal Sarcoma

We report the third known case of mesenchymal hamartoma of the liver (MHL) with a balanced translocation involving a common breakpoint, 19q13.4. A common clonal chromosome abnormality appears to characterize an important subset of MHL, some of which may be low-grade neoplasms. We found no consistent karyotype abnormality in a post-treatment sample of embryonal sarcoma of the liver (ESL). Reports of coexistent MHL and ESL in two patients and detection of 19q abnormalities in two ESLs appear to support Stocker's hypothesis of a histogenetic link between these two rare liver lesions. More data are needed to clarify this relationship. It is possible that MHLs are etiologically heterogenous and may be developmental disorders, disruptions, or neoplasms.

Low-Grade Mucosa-Associated Lymphoid Tissue Lymphoma Involving the Kidney: Report of 3 Cases and Review of the Literature

2006 ◽  
Vol 130 (1) ◽  
pp. 86-89 ◽  
Author(s):  
Libo Qiu ◽  
Pamela D. Unger ◽  
Robert W. Dillon ◽  
James A. Strauchen
Keyword(s):  
Lymphoid Tissue ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lymphoid Cells ◽  
Low Grade ◽  
The Third ◽  
First Case ◽  
Abundant Cytoplasm ◽  
History Of

Abstract Low-grade B-cell lymphoma of mucosa-associated lymphoid tissue involving the kidney is rare. We report a series of 3 cases. The first case occurred in an 83-year-old woman who presented with back pain. The second case was a 53-year-old man with a history of sarcoidosis who was found, in the course of evaluation of sarcoidosis, to have a right renal mass. The third case occurred in a 72-year-old man who had a history of periorbital mucosa–associated lymphoid tissue lymphoma and had been treated with surgery and radiation 1 year prior to this presentation. Histologically, all 3 patients showed infiltrate of uniform small-to-medium–sized lymphocytes with irregular nuclear contours and abundant cytoplasm resembling centrocytes or monocytoid lymphoid cells. The first patient received chemotherapy without complications. The second patient underwent a partial nephrectomy and was asymptomatic at the subsequent follow-up. The third patient developed a pulmonary embolism following nephrectomy, and further follow-up is not available.

Extending the multistage surgical strategy for recurrent initially low-grade gliomas: functional and oncological outcomes in 31 consecutive patients who underwent a third resection under awake mapping

2021 ◽  
pp. 1-10 ◽  
Author(s):  
Noor Hamdan ◽  
Hugues Duffau
Keyword(s):  
High Rate ◽  
Karnofsky Performance Scale ◽  
Consecutive Series ◽  
Low Grade ◽  
Oncological Treatment ◽  
The Third ◽  
Oncological Outcomes ◽  
Group 2 ◽  
Group 1

OBJECTIVE Maximal safe resection is the first treatment in diffuse low-grade glioma (DLGG). Due to frequent tumor recurrence, a second surgery has already been reported, with favorable results. This study assesses the feasibility and functional and oncological outcomes of a third surgery in recurrent DLGG. METHODS Patients with DLGG who underwent a third functional-based resection using awake mapping were consecutively selected. They were classified into group 1 in cases of slow tumor regrowth or group 2 if a radiological enhancement occurred during follow-up. All data regarding clinicoradiological features, histomolecular results, oncological treatment, and survival were collected. RESULTS Thirty-one patients were included, with a median age of 32 years. There were 20 astrocytomas and 11 oligodendrogliomas in these patients. Twenty-one patients had medical oncological treatment before the third surgery, consisting of chemotherapy in 19 cases and radiotherapy in 8 cases. No neurological deficit persisted after the third resection except mild missing words in 1 patient, with 84.6% of the patients returning to work. The median follow-up duration was 13.1 ± 3.4 years since diagnosis, and 3.1 ± 2.9 years since the third surgery. The survival rates at 7 and 10 years were 100% and 89.7%, respectively, with an estimated median overall survival of 17.8 years since diagnosis. A comparison between the groups showed that the Karnofsky Performance Scale score dropped below 80 earlier in group 2 (14.3 vs 17.1 years, p = 0.01). Median residual tumor volume at the third surgery was smaller (2.8 vs 14.4 cm3, p = 0.003) with a greater extent of resection (89% vs 70%, p = 0.003) in group 1. CONCLUSIONS This is the first consecutive series showing evidence that, in select patients with progressive DLGG, a third functional-based surgery can be achieved using awake mapping with low neurological risk and a high rate of total resection, especially when reoperation is performed before malignant transformation.

Low-Grade Fibromyxoid Sarcoma: A Brief Review

2006 ◽  
Vol 130 (9) ◽  
pp. 1358-1360 ◽  
Author(s):  
Stephen E. Vernon ◽  
Pablo A. Bejarano
Keyword(s):  
Spindle Cell ◽  
Fusion Gene ◽  
Cytogenetic Abnormality ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
Balanced Translocation ◽  
Linear Arrangement ◽  
Myxoid Stroma ◽  
Fibromyxoid Sarcoma ◽  
The Common

Abstract Low-grade fibromyxoid sarcomas are uncommon deep soft tissue neoplasms first described by Evans in 1987. They exhibit a deceptively benign appearance, with a whorled or linear arrangement of spindle-shaped cells showing few to absent mitoses. A characteristic, but not specific, feature is the presence of areas of myxoid stroma. Recurrences are common, and late metastases have been recorded. A closely related but morphologically distinct tumor, the so-called hyalinizing spindle cell tumor with giant rosettes, has also been described; both neoplasms share the same cytogenetic abnormality, a balanced translocation resulting in a FUS/CREB3L2 fusion gene. Because of similar clinical behavior and the common cytogenetic abnormality, some authors prefer to consider both lesions as a single entity within the spectrum of low-grade sarcomas.

Fibrinogen, Platelet and Red Cell Kinetics in Mice Bearing an Experimental Tumor

1975 ◽  
Author(s):  
A. Poggi ◽  
N. Polentarutti ◽  
M. B. Donati ◽  
G. de Gaetano ◽  
S. Garattini
Keyword(s):  
Cell Kinetics ◽  
Fibrinogen Level ◽  
Low Grade ◽  
Red Cell ◽  
Experimental Tumor ◽  
Tumor Implantation ◽  
The Third ◽  
Red Cell Survival ◽  
Observation Period

In view of the possible role of platelets and coagulation mechanisms in the growth and dissemination of solid tumors, a number of haematological parameters have been followed during development of an experimental syngeneic tumor in mice (Lewis Lung Carcinoma, 3LL). This tumor, when transplanted intramuscularly in C57,B1/6 mice, grows locally and gives spontaneous metastases to the lungs. The transplanted animals survive for about 4 weeks. Metastases are visible since the third week. A slight but constant increase in plasma fibrinogen level and a marked thrombocytopenia were observed starting during the second week after tumor implantation. No other significant changes in coagulation and fibrinolysis parameters were found. Moreover, the animals developed a marked haemolytic anaemia, possibly microangiopathic in origin. 125I-fibrinogen survival was decreased of about 20% during the second week after tumor implantation and was not further reduced later on. Fibrinogen turnover was accelerated since the second week and was further increased thereafter, being more than doubled at the end of the third week. Labelled fibrinogen accumulated in the primary tumor and in the lungs; its rats of disappearance from the tumor was much slower than from lungs or blood. These data suggest the occurrence of a low-grade, localized fibrinogen consumption (intravascular coagulation ?). 51Cr-platelet survival was not modified throughout the observation period, whereas platelet turnover was markedly reduced since the end of the second week, suggesting a defective platelet production. 51Cr-red cell survival was drastically reduced to about 30% of controls starting from the second week, whereas labelled red cell turnover was almost doubled. The pathogenetic relevance of the observed modifications in the processes of grwoth and dissemination of 3 LL remains to be established.(Supported by Grant NIH-PHRB-IRO1 CA 12764–01.

scholarly journals Development of a clinical algorithm for treating urethral strictures based on a large retrospective single-center cohort

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 2378 ◽  
Author(s):  
Yuri Tolkach ◽  
Thomas Herrmann ◽  
Axel Merseburger ◽  
Martin Burchardt ◽  
Mathias Wolters ◽  
...  
Keyword(s):  
Clinical Decision ◽  
Low Grade ◽  
Single Center ◽  
Recurrence Rates ◽  
Decision Algorithm ◽  
Clinical Algorithm ◽  
The Third ◽  
Urethral Strictures ◽  
Male Patients ◽  
Second Procedure

Aim To analyze clinical data from male patients treated with urethrotomy and to develop a clinical decision algorithm. Materials and methods Two large cohorts of male patients with urethral strictures were included in this retrospective study, historical (1985-1995, n=491) and modern cohorts (1996-2006, n=470). All patients were treated with repeated internal urethrotomies (up to 9 sessions). Clinical outcomes were analyzed and systemized as a clinical decision algorithm. Results The overall recurrence rates after the first urethrotomy were 32.4% and 23% in the historical and modern cohorts, respectively. In many patients, the second procedure was also effective with the third procedure also feasible in selected patients. The strictures with a length ≤ 2 cm should be treated according to the initial length. In patients with strictures ≤ 1 cm, the second session could be recommended in all patients, but not with penile strictures, strictures related to transurethral operations or for patients who were 31-50 years of age. The third session could be effective in selected cases of idiopathic bulbar strictures. For strictures with a length of 1-2 cm, a second operation is possible for the solitary low-grade bulbar strictures, given that the age is > 50 years and the etiology is not post-transurethral resection of the prostate. For penile strictures that are 1-2 cm, urethrotomy could be attempted in solitary but not in high-grade strictures. Conclusions We present data on the treatment of urethral strictures with urethrotomy from a single center. Based on the analysis, a clinical decision algorithm was suggested, which could be a reliable basis for everyday clinical practice.

Undifferentiated Embryonal Sarcoma of the Liver is Associated with Mesenchymal Hamartoma and Multiple Chromosomal Abnormalities: A Review of Eleven Cases

2011 ◽  
Vol 14 (2) ◽  
pp. 111-116 ◽  
Author(s):  
Bahig M. Shehata ◽  
Nitika A. Gupta ◽  
Howard M. Katzenstein ◽  
Charlotte K. Steelman ◽  
Mark L. Wulkan ◽  
...  
Keyword(s):  
Chromosomal Abnormalities ◽  
Mesenchymal Hamartoma ◽  
Embryonal Sarcoma

High-Grade Fetal Vascular Malperfusion Is Associated With Diffuse Chorionic Hemosiderosis

2021 ◽  
pp. 109352662110646
Author(s):  
Eoghan E. Mooney ◽  
Emma Crotty
Keyword(s):  
Perinatal Outcomes ◽  
Low Grade ◽  
High Grade ◽  
Third Trimester ◽  
The Third ◽  
Term Delivery ◽  
Venous Bleeding ◽  
Placental Membranes ◽  
Adverse Perinatal Outcomes ◽  
Cord Length

Introduction Diffuse chorionic hemosiderosis (DCH) is an abnormality of the placental membranes characterized by the deposition of iron pigment. It is usually secondary to recurrent venous bleeding in early pregnancy. In many papers, it is associated with pre-term delivery. Fetal vascular malperfusion (FVM) is an abnormality of the feto-placental circulation that may be seen at any stage of gestation, but most often in the third trimester. It may be graded as low grade (LGFVM) or high grade (HGFVM). No link has been identified in the placental literature between DCH and FVM, but we have noted the 2 co-existing in placentas submitted for analysis. This study explored a possible association of these 2 entities. Methods Laboratory records were searched for singleton cases coded as DCH based on diagnosis on H&E stain over a 6-year period. Of 4478 placentas reported, 66 cases were coded as DCH (1.5%). These were classified as showing HGFVM, LGFVM, or no FVM. Controls (n = 132) were gestational age-matched cases without DCH. Cord length, coiling, insertion, or other abnormalities were noted. Membranes were classified as normal or circumvallate. Results were analyzed using Graphpad. Results Gestation ranged between 16 and 41 weeks gestation. 14/66 (21%) cases of DCH showed HGFVM and 2/66 (3%) showed LGFVM. 16/132 (12%) controls showed HGFVM and 21/132 (15.9%) had LGFVM. Where FVM is present, high-grade FVM is significantly associated with DCH versus controls ( P < .0031 Fischer’s Test). Discussion HGFVM occurs significantly more often in placentas with DCH than in controls. Both FVM and DCH are associated with adverse perinatal outcomes, and a possible relationship between the 2 remains to be clarified.

scholarly journals Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

2020 ◽  
Vol 7 (2) ◽  
pp. HEP19 ◽  
Author(s):  
Sebastiao N Martins-Filho ◽  
Juan Putra
Keyword(s):  
Chromosomal Aberrations ◽  
Benign Lesion ◽  
Differential Diagnoses ◽  
Mesenchymal Hamartoma ◽  
Clinicopathologic Characteristics ◽  
Radiological Features ◽  
Histologic Evaluation ◽  
Embryonal Sarcoma

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

scholarly journals Endoscopic Mucosal Resection: Early Experience in British Columbia

2010 ◽  
Vol 24 (4) ◽  
pp. 239-244 ◽  
Author(s):  
Mayur Brahmania ◽  
Eric Lam ◽  
Jennifer Telford ◽  
Robert Enns
Keyword(s):  
British Columbia ◽  
Barrett’S Esophagus ◽  
Endoscopic Mucosal Resection ◽  
Barrett's Esophagus ◽  
Low Grade ◽  
Complication Rates ◽  
The Third ◽  
Mucosal Resection ◽  
The Mean

BACKGROUND: Endoscopic mucosal resection (EMR) has been proposed as a primary method of managing patients with dysplasia- or mucosal-based cancers of the esophagus.OBJECTIVES: To evaluate the use of EMR for the treatment of Barrett’s esophagus with dysplasia or early adenocarcinoma, assessing efficacy, complication rates and long-term outcomes.METHODS: All patients who underwent EMR at St Paul’s Hospital (Vancouver, British Columbia) were reviewed. Eligible patients were assessed with aggressive biopsy protocols. Detected cancers were staged with both endoscopic ultrasound imaging and computed tomography. Appropriate patients were offered EMR using a commercially available mucosectomy device. EMR was repeated at six- to eight-week intervals until complete. Patients with less than one year of follow-up or who were undergoing other ablative methods were excluded.RESULTS: Twenty-two patients (all men) with a mean (± SD) age of 67±10.6 years were identified. The mean duration of gastroesophageal reflux disease was 17 years (range four to 40 years) and all were receiving proton pump inhibitor therapy. The mean length of Barrett’s esophagus was 5.5±3.5 cm. One patient had no dysplasia (isolated nodule), three had low-grade dysplasia, 15 had high-grade dysplasia (HGD) and three had adenocarcinoma. A mean of 1.7±0.83 endoscopic sessions were performed, with a mean of 6±5.4 sections removed. Following EMR, three patients developed strictures; two of these patients had pre-existing strictures and the third required two dilations, which resolved his symptoms. There were no other complications. Three patients underwent esophagectomy. Two had adenocarcinoma or HGD in a pre-existing stricture. The third patient had an adenocarcinoma not amenable to EMR. One patient with a long segment of Barrett’s esophagus underwent radiofrequency ablation. At a median follow-up of two years (range one to three years), the remaining 18 patients (82%) had no evidence of HGD or cancer.CONCLUSION: Most patients with esophageal dysplasia can be managed with EMR. Individuals with pre-existing strictures require other endoscopic and/or surgical methods to manage their dysplasia or adenocarcinoma.

The stepped hybrid plate for carpal panarthrodesis

2009 ◽  
Vol 22 (05) ◽  
pp. 389-397 ◽  
Author(s):  
M. C. Díaz-Bertrana ◽  
F. Darnaculleta ◽  
J. Franch ◽  
J. L. Puchol ◽  
I. Durall
Keyword(s):  
Bone Tumour ◽  
Degenerative Joint Disease ◽  
Metacarpal Bone ◽  
Joint Disease ◽  
Incomplete Fusion ◽  
Carpal Bone ◽  
Low Grade ◽  
Screw Insertion ◽  
The Third ◽  
Metacarpal Bones

SummaryFifty-two carpal panarthrodeses (CP) were carried out in 44 dogs (eight bilateral), in a multicentre study using a single (n = 47) or double (n = 5) stepped hybrid CP plate. Of these 44 cases, 39 were between 20–55 kg in bodyweight , 26 were males , and the carpometacarpal was the most common joint involved. Falling and other impact trauma were the most common aetiology. Pain of unknown origin, carpal luxation, chronic accessory carpal bone fracture, distal comminuted intra-articular radial fracture, bone tumour, degenerative joint disease, canine erosive idiopathic polyarthritis, avascular necrosis of the radial carpal bone and fractures of several metacarpal bones were some of the pathologies reported. Fracture of the third metacarpal bone during screw insertion was the only intrasurgical complication. Malpositioning of the plate or screws and over-tightened screws were technical errors observed in seven of the procedures. The radial carpal bone was not fixed with a screw in two cases due to bone deformity. Concurrent plate breakage and bending in the same patient operated on bilaterally was observed during the follow-up period, which represented a major complication rate of 3.8% for all procedures. Minor complications were: low grade infection, lick granuloma, digit hyperextension, screw loosening or failure, incomplete fusion of some joints and a fracture of the third metacarpal bone at the distal screw hole of the plate; which represented a rate of 44.2% on all procedures. Complete carpal healing was observed radiographically in 94.2% of all procedures. Limb function was excellent to good, and all of the owners, except for one, were satisfied with the procedure.

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