Sarcomas Arising in Paget Disease of Bone: A Clinicopathologic Analysis of 70 Cases

2007 ◽  
Vol 131 (6) ◽  
pp. 942-946 ◽  
Author(s):  
Andrea T. Deyrup ◽  
Anthony G. Montag ◽  
Carrie Y. Inwards ◽  
Zhiheng Xu ◽  
Ronald G. Swee ◽  
...  

Abstract Context.—Sarcomatous transformation is a rare complication of Paget disease of bone. Prognosis in patients with other types of sarcomas arising in bone has improved in the last several decades because of therapeutic advances. However, because of the rarity of Paget sarcoma, outcome studies in these patients are limited. Objective.—To determine whether prognosis for Paget sarcoma has improved. Design.—Seventy cases of sarcomas arising in the setting of Paget disease were collected, and the histologic and clinical findings were reviewed. Clinical follow-up was obtained in 67 cases. Results.—Sarcoma arising in Paget disease tended to arise in older men (46 men, 24 women; age range, 31–88 years; mean age, 66 years) and predominated in the axial skeleton (n = 37), especially in the pelvis. Thirty-three patients had a clinical history of Paget disease ranging in duration from 16 months to 30 years (mean, 15 years). No significant difference in incidence between monostotic (n = 33) and polyostotic (n = 36) disease was noted. Most tumors were osteosarcomas (88%). All tumors were high grade. Follow-up information was obtained in 67 of 70 cases (range of follow-up, 1–252 months). Survival ranged from 1 month to 20 years, with a 5-year survival rate of 10%. Conclusions.—Prognosis remains poor in patients with Paget sarcoma. There is no significant correlation between the number of bones involved with Paget disease or the duration of disease and development of Paget sarcoma. Poor prognosis in Paget sarcoma is unrelated to site or stage at presentation.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 11018-11018
Author(s):  
Brittany Siontis ◽  
Emily Roberts ◽  
Lili Zhao ◽  
Jonathan B. McHugh ◽  
Dawn Owen ◽  
...  

11018 Background: Osteosarcoma (osarc) can be a rare complication from radiation (rt) therapy. Radiation-associated osarc (RAO) is reported to have a worse prognosis than non rt-associated osarc with limited objective data comparing the two. We conducted a retrospective study comparing demographics, therapy and outcomes of sporadic osarc (SO) to RAO. This study was confined to adults. Methods: We identified patients (pts) > age 18 years (yr) with osarc treated at our institution between 1990 and 2016 using an institutional database. We categorized tumors as SO or RAO based on history of prior rt within field of osarc. We extracted data on demographics, treatment, and primary malignancy characteristics. Results: We identified 159 pts with osarc, 28 were RAO tumors. Results are in Table 1. Median follow-up was 2.8 yr (0.1-19.6 yr). For RAO, median time from rt to diagnosis was 11.5 yr (1.5-28 yr) with a median cumulative dose of 60 Gy (44-75.8 Gy). Median progression free survival (PFS) and overall survival (OS) were not significantly different in pts presenting with metastatic osarc; PFS 10.3 mo vs 4.8 mo (p=0.45) and OS 15.6 mo vs 6.1 mo (p=0.96) in SO vs RAO pts, respectively. For pts with localized osarc, median relapse-free survival (RFS) and OS were significantly different, not reached vs 12.2 mo (p<0.001) and not reached vs 27.6 mo (p=0.001) in SO vs RAO, respectively. Conclusions: In our series, there was a significant difference in age, size and location of RAO vs non rt-associated osarc. Overall, all osarc pts with metastatic disease at diagnosis fared poorly. Pts presenting with localized RAO had worse outcomes than patients with localized SO. This was not associated with a detectable difference in therapy rendered or treatment effect in resection specimens. [Table: see text]


PEDIATRICS ◽  
1965 ◽  
Vol 36 (2) ◽  
pp. 268-270
Author(s):  
WILLIAM D. COCHRAN ◽  
MICHAEL CORNFIELD ◽  
DAVID FRIEDBERG

Recent advances have made it possible to isolate rubella virus from affected persons and to propagate the virus in tissue culture. The virus may be identified by the unique cytopathogenic changes in human amnion tissue culture by interference with ECHO virus type 11 in monkey kidney tissue culture. Thus an even closer correlation between an active rubella infection in the mother in the first trimester of pregnancy and the clinical findings of the rubella syndrome is now feasible. The rubella epidemic of 1964 can be expected to produce significant numbers of infants with the congenital rubella syndrome. This paper reports the clinical history of two cases of rubella associated with thrombocytopenia in the newborn.


2021 ◽  
Vol 37 (1) ◽  
Author(s):  
Ahmed Gamal Khafagy ◽  
Mohamed El-Begermy ◽  
Marwa Mohamed El-Begermy ◽  
Pretty O. Afifi

Abstract Background This study aims to compare the graft uptake rate and hearing improvement of fat graft versus inlay butterfly tragal cartilage in the repair of perforations in chronic otitis media mucosal in adults. In this retrospective study, twenty-eight patients were included with small dry anteroinferior tympanic membrane perforations (less than 1/3 of the tympanic membrane). The age range was 18 to 44 years old. Myringoplasty was done under general anesthesia for 8 patients with a fat graft (FG) and 20 patients with inlay butterfly cartilage graft (IBCG). Six months postoperatively, a follow-up evaluation was done for successful graft uptake and hearing outcomes. Results The success rate of graft uptake in the first group (fat graft) was 6/8 cases (75%) while in the second group (IBCG) was 19/20 (95%) with no statistically significant difference (P = 0.0148). Also, there was no statistical difference between the two groups as regards postoperative ABG, improvement changes in ABG, and number of patients with improved hearing. Conclusions Inlay butterfly cartilage graft is a useful graft in repairing small tympanic membrane perforations as regard graft take and hearing outcomes.


2020 ◽  
Vol 1 (3) ◽  
pp. 154-159
Author(s):  
Suzan Amana Rattan ◽  
◽  
Mahir Kadhim Mutashar ◽  

AIM: To evaluate the effectivity of the combination of intracameral moxifloxacin 0.1% with subconjunctival triamcinolone acetonide 4 mg as prophylaxis of infection and inflammation after phacoemulsification in comparison with topical medication treated group. METHODS: A total one thousand patients with age range from 38 to 70 years old who scheduled for phacoemulsification were divided into 2 groups of no statistically significant differences in age, preoperative intraocular pressure (IOP) and central macular thickness (CMT), P=0.6, 0.9 and 0.8 respectively. The surgeries were done by 2 surgeons each one planned to use one method of prophylaxis at Eye Speciality Private hospital, Baghdad, Iraq. For the 1st group of patients (500) a topical moxifloxacin hydrochloride 0.5% and dexamethasone 0.1% eye drops were prescribed four times a day for 1mo postoperatively. For the 2nd group intracameral (IC) diluted moxifloxacin at 0.1% with subconjunctival (SC) triamcinolone 4mg in 0.4 cc were administered at the conclusion of the surgery. Follow up visits were on the first postoperative day, 1wk, 1mo, and 3mo postoperatively. Anterior chamber (AC) reaction was examined during the 4 visits while IOP was measured during the last 3 and CMT was measured only in the last one. RESULTS: The current clinical trial study compared 2 samples with 2 different prophylaxis methods. No endophthalmitis case reported in both group. By a 2-Sample t-test, the IC-treated group (group 2) had statistically significant lower AC cells at the 1st day postoperative visit than the other group while there were no statistically significant differences at 1wk, 1 mo and 3mo visits between the 2 groups. There was no statistically significant difference at 3mo visits in IOP and CMT between the two groups. A breakthrough inflammation rate with the topical medication was (9.6%) while in the other group (IC treated ) was 4.0%. A significant IOP elevation ≥10 mm Hg at 1mo in 2.4% within the topical medication group which was higher than the rate in the other group (0.8%). CONCLUSION: In addition to the safety and effectivity of the combination of intracameral moxifloxacin and subconjunctival triamcinolone in preventing infection and inflammation after cataract surgery. The majority (480) of our included patients didn’t require any topical postoperative medication that is cost saving for the patient, helped patients who were unable to administer topical medication, and decreased chance of complication related to patient poor adherence to postoperative medication.


PEDIATRICS ◽  
1984 ◽  
Vol 74 (1) ◽  
pp. 1-10
Author(s):  
Kenneth M. McConnochie ◽  
Klaus J. Roghmann ◽  
Suzanne J. Klein ◽  
Thomas K. Mclnery ◽  
James B. MacWhinney ◽  
...  

A historical cohort study was performed in order to assess the hypothesis that even mild bronchiolitis in infancy is a predictor of wheezing later in childhood. Subjects who had experienced bronchiolitis and a matched control group were compared in terms of reported wheezing 8 years later. A highly significant difference was found between the bronchiolitis group and the control group in terms of current wheezing (P &lt; .0001, relative risk 3.24). This difference was maintained after adjusting for many potentially confounding variables including family history of allergy and other allergic manifestations in the child. Results suggested that 13.6% of a normal practice population in the age range 6 to 9 years currently wheeze, but that 44.1% of children who experienced bronchiolitis currently wheeze. Based on the incidence of bronchiolitis (4.27/100 children in their first 2 years of life) and the relative odds for wheezing derived from a logistic regression model including variables that measured passive smoking, genetic tendency to wheeze, and bronchiolitis, calculations of attributable risk suggested that wheezing in 9.4% of the population of children who currently wheeze was attributable to bronchiolitis.


2001 ◽  
Vol 125 (8) ◽  
pp. 1047-1050 ◽  
Author(s):  
Yi Jun Yang ◽  
Linda K. Trapkin ◽  
Roberta K. Demoski ◽  
Jeannette Bellerdine ◽  
Celeste N. Powers

Abstract Context.—Several endometrial diseases, such as endometrial hyperplasia, endometrial carcinoma, and endometrial polyps, have been reported to be associated with tamoxifen administration. We recently observed a high incidence of distinctive small blue cells in Papanicolaou tests of women who had received tamoxifen treatment for breast carcinoma. Objectives.—To define the characteristics of these small blue cells, to identify the patient population in which they are found, and to determine the clinical significance and possible etiology of these findings. Design.—A total of 154 Papanicolaou tests from 60 patients with a clinical history of tamoxifen therapy were reviewed retrospectively. Results.—Small blue cells were found in 40% of Papanicolaou tests from patients who received tamoxifen therapy. Patients with small blue cells in their Papanicolaou tests were an average of 9 years older at the time tamoxifen therapy was initiated than those without. Among the available follow-up surgical biopsies, no malignant diagnoses were made. Conclusions.—We conclude that these distinctive small blue cells are found more frequently in older patients and most probably represent proliferative reserve cells of cervical/vaginal epithelium resulting from the estrogenic agonist effect of tamoxifen. More importantly, they are nonneoplastic in nature.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hae-Yong Lee

Purpose: It has been reported that serum level of immunoglobulin E (IgE) is increased in patients with Kawasaki disease (KD) after acute phase. However the exact mechanism of increasing IgE is yet to be revealed. We investigated whether the interleukin-21 (IL-21) could be related with the high IgE in KD. Instead of IL-4, IL-21 was focused in this study because it has been reported that its level is increased in various autoimmune vasculitis. Methods: From June 2008 to June 2010, 49 patients with KD admitted in Wonju Severance Christian Hospital and 13 controls with high fever due to unknown infection who had no history of KD were included in this study. The sera from patients and controls were collected and checked in terms of immunoglobulin E (Chemiluminescent method, Siemens, Munich, Germany) and IL-21 (ELISA, eBioscience, San Diego, USA). Results: The median age of patients with KD was 3 years of age (range: 0.4-10) and that of controls was 7 years of age (range: 1-12). The group of patients with KD was composed of 39 complete KD and 10 incomplete KD. Among patients with KD, 10 patients had coronary arterial dilatation (CAD) and 39 patients had no coronary complications. The median value of IL-21 in patients with KD was significantly increased as 466 pg/mL (range: 0-1544) while that value in controls was <62.5 pg/mL (range: 0- 825 pg/mL) ( P < 0.01). We could not find the significant correlation between the serum level of IgE and that of IL-21 in patients with KD (Spearman R=0.2, P = 0.08) though 30% of patients with KD showed increased IgE more than 100 IU/mL. In addition, our data showed no significant difference between CAD group and non CAD group in terms of serum IL-21. Conclusion: Our data showed firstly that IL-21 is increased in patients with KD. There was no significant correlation between high IgE and the level of IL-21.


2015 ◽  
Vol 81 (10) ◽  
pp. 955-960 ◽  
Author(s):  
Shawn Diamond ◽  
H. Gill Cryer

Grading systems developed by the Ventral Hernia Working Group (VHWG) for complex open abdominal wall reconstruction rely on limited outcomes: surgical site occurrence (SSO) and hernia recurrence. This does not account for the longitudinal restoration of a functional abdominal wall and the ability to correct complications. We performed a single-site, retrospective review of consecutive complex open abdominal wall reconstruction interventions with 24-month minimum follow-up to establish reoperation rates and compare long-term results to the VHWG. About 125 midline hernia repairs (>200 cm2) were studied. All had loss of functional domain and 47-month average follow-up. Demographics included: mean age 57 years, 47 per cent male, 63 per cent obese, and 34 per cent with contamination. Rates of SSO per VHWG grade were 9 per cent grade I, 45 per cent grade II, and 55 per cent grade III. Forty-three of 59 patients who developed complications were eventually successful after reoperation leading to an 87 per cent restoration rate. Select factors independently associated with reoperation included biological mesh and clinical history of infection. Although rates of SSO were higher than the VHWG published, we experienced high salvage rates except in patients who underwent biologic repair. We recommend restricted use of biologic mesh in contaminated and clean fields as well as modifications to the VHWG grading and recommendations.


2019 ◽  
Vol 128 (10) ◽  
pp. 693-698
Author(s):  
Sabine Dillenberger ◽  
Detlef K. Bartsch ◽  
Elisabeth Maurer ◽  
Peter Herbert Kann

Abstract Purpose It is assumed that primary hyperparathyroidism (pHPT) in Multiple Endocrine Neoplasia (MEN) and lithium-associated pHPT (LIHPT) are associated with multiple gland disease (MGD), persistence and recurrence. The studies purpose was to determine frequencies, clinical presentation and outcome of sporadic pHPT (spHPT), LIHPT and pHPT in MEN. Additional main outcome measures were the rates of MGD and persistence/recurrence. Methods Retrospective analysis of medical records of 682 patients with pHPT who had attended the University Hospital of Marburg between 01–01–2004 and 30–06–2013. All patients were sent a questionnaire asking about their history of lithium medication. Results Out of 682 patients, 557 underwent primary surgery (532 spHPT, 5 LIHPT, 20 MEN), 38 redo-surgery (31 spHPT, 7 MEN), 55 were in follow-up due to previous surgery (16 spHPT, 1 LIHPT, 38 MEN) and 37 were not operated (33 spHPT, 1 LIHPT, 3 MEN). Primary surgeries were successful in 97.4%, revealed singular adenomas in 92.4%, double adenomas in 2.9% and MGD in 3.4% of the cases. Rates of MGD in MEN1 (82.35%) were significantly higher than in spHPT (3.8%), while there was no significant difference between LIHPT (20%) and spHPT. Rates of persistence/recurrence did not significantly differ due to type of surgery (bilateral/unilateral) or type of HPT (spHPT/LIHPT/MEN). Conclusions History of lithium medication is rare among pHPT patients. While MGD is common in MEN1, rates of MGD, persistence or recurrence in LIHPT were not significantly higher than in spHPT.


2019 ◽  
Vol 5 (1) ◽  
pp. 205511691983185
Author(s):  
Lisa Graziano ◽  
Stefania Di Paco ◽  
Giancarlo Avallone ◽  
Paola Roccabanca

Case summary A 10-year-old male neutered domestic shorthair cat was presented with a 4 month history of slow, progressive swelling and intermittent multifocal bleeding of the distal right forepaw that progressively developed after cephalic vein catheterisation for anaesthesia. The procedure was performed for dental calculus removal. Initial clinical and biopsy (superficial biopsy) findings matched two differential diagnoses: progressive angiomatosis and acquired peripheral arteriovenous fistula (AVF). AVF was suspected based on Doppler ultrasound examination that identified multiple abnormal sinusoidal neoformed branching vessels with turbulent high-velocity flow spectrum at the confluence of the artery and vein, and high-velocity arterialised flow in the vein. Owing to rapid and severe progression of lesions after biopsy, the owner requested amputation of the cat’s limb. A definitive diagnosis of acquired iatrogenic peripheral AVF was based on deep-tissue microscopical evaluation. High numbers of arterioles and venules in superficial and deep dermis embedded in oedematous stroma were connected to dilated and tortuous vessels with irregularly thickened walls in deep dermis and panniculus. Vasculitis and vascular thrombosis and thickening (attributed to local hypertension) were common findings. No bone or muscular invasion were observed. The cat was doing well 3 years after surgery. Relevance and novel information This report describes a case of iatrogenic acquired peripheral AVF following venous catheterisation in a cat, fully documented for the first time by a combination of clinical findings, ultrasound imaging, complete histopathology and follow-up history.


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