Benign metastasizing leiomyoma (BML): A retrospective case series analysis of 8 patients (pts).

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e22525-e22525 ◽  
Author(s):  
Vittoria Colia ◽  
Angelo Paolo Dei Tos ◽  
Elena Fumagalli ◽  
Rossella Bertulli ◽  
Domenica Lorusso ◽  
...  

e22525 Background: BML is a rare entity marked by the presence of lung lesions in women with a history of surgery for a benign leiomyoma of the uterus. Optimal treatment strategy for BML is poorly defined. We report on the activity of systemic therapy in a retrospective series of pts with BML. Methods: Cases diagnosed with BML from June 1993 to January 2017 at Istituto Nazionale Tumori, Milan, were reviewed. Results: Eight pts were identified, with a median age of 43 yrs. Estrogen and progesteron receptors were positive in all cases. All pts underwent surgery (3 hysterectomy, 2 myomectomy, 2 hysteroannessiectomy and 1 left ovariectomy) for suspected uterine leiomyoma (1 leg; 1 thigh); 2 pts had concomitant lung disease. 8 pts developed lung metastases and 2 had also limb metastases. 2 pts underwent lung metastasectomy, followed by watchful waiting with CT every 6 mos and were disease-free at their last follow up after 132 mos and 84 mos from diagnosis. 6 pts received systemic therapy for progressing advanced disease (1-6 lines). Among 6 pts treated, 2 were in fertility age and underwent ovary-sparing hysterectomy, receiving GnRH agonist with 1 PR lasting 96 mos and 1 SD lasting 38 mos; 2 pts received an aromatase inhibitor with 1 PR lasting 24 mos and 1 SD lasting 12 mos; 2 pts received oral estrogens with 1 PR lasting 39 mos and 1 SD lasting 2 mos; 1 pt received oral progestins with a PR lasting 12 mos; 3 patients received antracyclin +/- ifosfamide obtaining 2 PR after 3 cycles (cys) and 1 SD after 3 cys lasting 6 mos; 1 pt received high-dose ifosfamide with a PR after 5 cys; 1 pt received ifosfamide+dacarbazine obtaining a CR after 6 cys; 2 pts received gemcitabine with 1 PR after 3 cys and 1 SD after 2 cys lasting 6 mos; 1 pt received oral etoposide with a PR lasting 21 mos; 1 pt received sorafenib with a SD lasting 6 mos; 1 pt received everolimus with a PR lasting 57 mos. In this case, everolimus was discontinued due to lung toxicity. No pts progressed during treatment. At a median follow-up of 55 mos, 6 pts are alive, while 2 are dead of disease. Conclusions: In a series of 8 pts, we confirm the activity of hormonal treatment in BML. mTOR inihibitors or chemotherapy also show to be active.

2019 ◽  
Vol 44 (2) ◽  
pp. 60
Author(s):  
Theresia Yinski ◽  
Syntia Nusanti

Introduction : Methanol-induced toxic optic neuropathy (TON) is defined as a visual impairment due to optic nerve damage by methanol poisoning. Not only is this disease entity underdiagnosed at times, this sudden blindness is also often diagnosed at a stage where recovery of vision is no longer possible. Materials and Methods : A literature search was conducted using PubMed, ClinicalKey, Google Scholar and ScienceDirect by combining the keywords ‘methanol’ or ‘methyl alcohol’, ‘intoxication’ or ‘poisoning’, ‘toxic optic neuropathy’, and ‘visual acuity’ with ‘high-dose steroid’. Results : The total amount of subjects in each article varied from 2 to 37, with mean age distribution of 26.34 to 55 years old, where most patients were male. The follow up duration varied from 1 week to 1 year. Four articles do not mention high-dose steroids treatment as therapy while the other four mention use of 1000 mg of intravenous methylprednisolone per day with divided doses of either 2x500 mg or 4x250 mg. Improvement percentages show 100% improvement in all studies that used high-dose steroids, while in the non-high-dose steroids studies the improvement percentages range from 33.33% to 90%. Summary : High-dose steroids are showing efficacy in improving visual acuity and reducing the inflammation in methanol-induced TON. The period of how fast the therapy takes effect is inconclusive, as the mean follow-up time differs widely per study. However, because most of the reviewed studies here are retrospective case series, a larger, more comprehensive study is required to acknowledge more of the efficacy profile.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1298-1298
Author(s):  
Andrew Hodson ◽  
Claire Harrison ◽  
Melanie Percy ◽  
Frank Jones ◽  
Mary Frances McMullin

Abstract Idiopathic Erythrocytosis (IE) is a diagnosis given to patients who have an absolute erythrocytosis (red cell mass more than 25% above their mean normal predicted value) but who do not have a known form of primary or secondary erythrocytosis (BCSH guideline, 2005). We report here the results of a follow-up study of 80 patients (44 male and 36 female) diagnosed with IE from the United Kingdom and the Republic of Ireland over a 10 year period. Baseline information was initially collected when investigating for molecular causes of erythrocytosis in this group. The diagnosis of IE was made on the basis of a raised red cell mass >25% above mean normal predicted value, absence of Polycythaemia Vera (PV) based on the criteria of Pearson and Messinezy (1996), and the exclusion of secondary erythrocytosis (oxygen saturation >92% on pulse oximetry, no history of sleep apnoea, no renal or hepatic pathology, and a normal oxygen dissociation curve (if indicated). The average age at diagnosis of erythrocytosis was 34.5 (2–74 years). Erythropoietin levels were available for 77/80 of the patients and were low in 18 (23%) and normal or high in 59 (74%). Ultrasound imaging was carried out in 67 patients (84%) at time of diagnosis and no significant abnormalities found. Fourteen patients had a family history of erythrocytosis. These patients have now been followed up for an average of 9.4 years (range 1–39). Out of 80 patients 56 patients can still be classified as having IE, of whom 52 are living (cause of death in the other 4 - lung cancer, RTA, sepsis, unknown). Thirty-five of these patients are regularly venesected, 3 take hydroxyurea (one also venesected), 11 receive no treatment while treatment is unknown in 2. Twenty take aspirin, 1 warfarin and 31 no thromboprophylaxis. Four of these patients had suffered thromboembolic complications (3 with CVA/TIAs and 1 with recurrent DVT) at or before their original diagnosis. Since diagnosis 8 patients have had 9 thrombotic events of which 7 were arterial (1 CVA, 3 TIAs, 1 MI, 2 PVD) and 2 venous (DVT/PE). Twenty take aspirin, 1 dipyridamole, 1 warfarin and 30 take no thromboprophylaxis. Out of the 24 patients who now have a diagnosis other than IE, 8 have been diagnosed with myelo-proliferative disease. Thirteen patients have a molecular abnormality which is likely to account for their erythrocytosis (11 VHL, 1 PHD-2, 1 EPO-receptor mutations). Three patients have secondary erythrocytosis. Older case studies identified a heterogenous group of patients, some of whom probably had apparent erythrocytosis and some who had either primary polycythaemia or secondary causes later identified (Modan and Modan, Najean et al). More recent reviews have identified a more homogenous group with low rates of transformation to myelofibrosis/acute leukaemia and low rates of thrombosis of around 1% patient-year. Follow up of our initial patient group does indeed reveal a heterogeneous group of patients with 10% now diagnosed with an MPD, although when analysis is confined to those patients who continue to fulfil the criteria for IE, the clinical course has been more stable. There has been no progression to MDS or leukaemia in this group (one patient with PV progressed to AML). The rate of thrombosis is 1.6% patient-years which is lower than the rate seen in PV and is consistent with the rate identified in other series. Molecular defects continue to be identified in this group and future investigation is likely to reveal further abnormalities.


2021 ◽  
pp. 000348942199015
Author(s):  
Tiffany P. Hwa ◽  
Qasim Husain ◽  
Jason A. Brant ◽  
Anil K. Lalwani

Objective: Jugular bulb abnormalities (JBA) such as high riding jugular bulb and jugular bulb diverticulum can extend or erode into the middle and inner ear. In this report, we report on a series of 6 patients with jugular bulb anomalies involving the internal auditory canal (IAC). Methods: A retrospective case series. Results: Six females, ages 6 to 63 presenting with myriad of otologic symptoms including hearing loss, tinnitus, balance disturbance, fullness, and otalgia were discovered to have JB eroding into IAC. Computerized tomography, but not Magnetic Resonance Imaging, was able to identify IAC erosion by a significantly enlarged JB. Conclusion: A significantly enlarged JB eroding into the IAC maybe congenital or acquired. It can present with a variety of common otologic symptoms. Long term follow-up is needed to determine the natural history of JB anomalies involving the IAC and need for intervention.


2020 ◽  
Vol 133 (5) ◽  
pp. 1473-1477 ◽  
Author(s):  
Aravind G. Kalluri ◽  
Madhav Sukumaran ◽  
Pouya Nazari ◽  
Pedram Golnari ◽  
Sameer A. Ansari ◽  
...  

OBJECTIVEThe carotid cave is a unique intradural region located along the medial aspect of the internal carotid artery. Small carotid cave aneurysms confined within this space are bound by the carotid sulcus of the sphenoid bone and are thought to have a low risk of rupture or growth. However, there is a lack of data on the natural history of this subset of aneurysms.METHODSThe authors present a retrospective case series of 290 small (≤ 4 mm) carotid cave aneurysms evaluated and managed at their institution between January 2000 and June 2017.RESULTSNo patient presented with a subarachnoid hemorrhage attributable to a carotid cave aneurysm, and there were no instances of aneurysm rupture or growth during 911.0 aneurysm-years of clinical follow-up or 726.3 aneurysm-years of imaging follow-up, respectively.CONCLUSIONSThis series demonstrates the benign nature of small carotid cave aneurysms.


Author(s):  
Benjamin K. Hendricks ◽  
Joseph D. DiDomenico ◽  
Igor J. Barani ◽  
F. David Barranco

<b><i>Introduction:</i></b> The ZAP-X Gyroscopic Radiosurgery system (ZAP Surgical Systems, Inc., San Carlos, CA, USA) is a novel high-dose targeted stereotactic radiosurgery platform for outpatient use that includes self-shielding, X-ray image guidance, and the capacity to aim the radiation beam gyroscopically at an intracranial lesion using 5 independent degrees of freedom. The ZAP-X Gyroscopic Radiosurgery system accomplishes these actions while meeting widely accepted standards for dose gradient and accuracy. This retrospective study examined data of patients treated with gyroscopic radiosurgery (GRS) to document clinical outcomes. <b><i>Methods:</i></b> Medical records of all outpatients treated with GRS over a 20-month period from January 2019 to August 2020 were searched to extract relevant details, including follow-up data until August 2021 (32-month study interval). Patients with &#x3c;6 months of radiographical follow-up data were excluded unless death occurred. Data collection included pretreatment clinical history, pathological diagnosis, radiographical features, treatment parameters, and long-term clinical and radiographical follow-up. <b><i>Results:</i></b> Sixty-eight patients received outpatient treatment with GRS during the 20-month treatment interval, with 59 patients remaining after exclusion for the minimum follow-up threshold, with a mean (standard deviation [SD]) fractionation of 1.85 (1.63). Eighty-two lesions were treated across a very heterogeneous patient population, including meningiomas (42.4%), metastases (39.0%), gliomas (6.8%), schwannomas (1.7%), and pituitary tumor (1.7%). Mean (SD) radiographical follow-up data (14.7 [6.60] months) were available for 56 patients. During that interval, 13 treated lesions in 13 patients (15.9%) demonstrated progression, 9 of which were stable during the initial posttreatment imaging surveillance period. Mean lesion volume was stable from pretreatment (2.54 cm<sup>3</sup> [4.37 cm<sup>3</sup>]) to most recent follow-up (2.80 cm<sup>3</sup> [8.20 cm<sup>3</sup>]) (<i>t</i> [79] = −0.310; <i>p</i> = 0.76). Minor adverse clinical events were noted in 3 (5.1%) of the 59 patients during the posttreatment phase that may have been related to the treatment. Ten (16.9%) patients died within the 32-month study interval. <b><i>Discussion/Conclusion:</i></b> This preliminary assessment of the first series of patients treated with the Zap-X Gyroscopic Radiosurgery system documents its overall feasibility in clinical applications. Although the duration of follow-up was brief, GRS appeared to be both safe and effective. Additional analysis, with an ongoing prospective registry, is underway.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 388.2-389
Author(s):  
A. Rubbert-Roth ◽  
P. K. Bode ◽  
T. Langenegger ◽  
C. Pfofe ◽  
T. Neumann ◽  
...  

Background:Giant cell arteritis (GCA) may affect the aorta and the large aortic branches and lead to dissections and aortic aneurysms. Tocilizumab (TCZ) treatment has the capacity to control aortic inflammation as has been demonstrated by CRP normalization and imaging data. However, limited data are available on the histopathological findings obtained from patients who underwent surgery because of aortic complications during TCZ treatment.Objectives:We report on 5 patients with aortitis who were treated with TCZ and developed aortic complications.Methods:We describe a retrospective case series of patients with GCA treated with TCZ, who presented in our clinic between 2011 and 2019. Three patients underwent surgery. Histopathologic examination was performed in specimen from all of them.Results:Five female patients were diagnosed with GCA (4/5) or Takaysu arteritis (1/5) involving the aorta, all them diagnosed by MR angiography and/or FDG PET CT scan. Three patients (one with aortic aneurysm, one with dissection) underwent surgery after having been treated with TCZ for seven weeks, nine months and four years, respectively. Imaging before surgery showed remission on MRI and/or PET-CT in all cases. At the time of surgery, all patients showed normalized CRP and ESR values. Histopathological evaluation of the aortic wall revealed infiltrates, consisting predominantly of CD3+CD4+ T cells. Enlargement of pre-existing aneuryms was observed in the other two patients 10 weeks and 4 months after discontinuation of TCZ, respectively. Both patients were not eligible for surgical intervention and died during follow-up.Conclusion:Our case series suggests that during treatment with TCZ, regular imaging is necessary in this patient population to detect development of structural changes such as aneurysms or dissections. Despite treatment, residual inflammation might persist which could contribute to eventual aortic complications.Disclosure of Interests:Andrea Rubbert-Roth Consultant of: Abbvie, BMS, Chugai, Pfizer, Roche, Janssen, Lilly, Sanofi, Amgen, Novartis, Peter Karl Bode: None declared, Thomas Langenegger: None declared, Claudia Pfofe: None declared, Thomas Neumann: None declared, Olaf Chan-Hi Kim: None declared, Johannes von Kempis Consultant of: Roche


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii324-iii324
Author(s):  
Derek Hanson ◽  
Nicolas Andre ◽  
Susan Chi ◽  
Mariella Filbin ◽  
Michael Fisher ◽  
...  

Abstract Embryonal tumors with multilayer rosettes (ETMR) are rare and highly-aggressive central nervous system (CNS) neoplasms which occur primarily in young children and carry a dismal prognosis. To date, no large clinical investigations have been conducted to determine the optimal therapy for ETMR. Data from retrospective case series suggest that our most aggressive standard therapies are not sufficient for cure in the majority of cases. New treatment approaches incorporating pre-clinical data and the known biology of ETMR are therefore urgently needed. A German drug screen using the patient-derived ETMR BT183 cell line and its xenograft revealed anti-tumor activity of topotecan, doxorubicin, and actinomycin D; three agents used infrequently for treating infant CNS tumors. Additional results from a small series of ETMR patients suggest that optimization of induction chemotherapy using these active agents may improve response and survival outcomes. In 2019, an international panel of pediatric neuro-oncology experts convened to advance therapy for ETMR. A consensus protocol was developed incorporating maximal safe surgical resection, induction chemotherapy with active pre-clinical agents, intrathecal chemotherapy, radiotherapy, and high-dose chemotherapy. This international consensus protocol represents the first prospective clinical investigation specific to ETMR and will be available through a treatment registry globally and as a clinical trial at select centers. The study aims to improve survival by providing aggressive, optimized therapy for ETMR and will serve as a platform to explore new biologically-promising agents. The investigation will also provide valuable prospective outcome data and correlative biological studies to serve as baseline comparators for future clinical trials.


Author(s):  
Gabriele Colo’ ◽  
Mattia Alessio Mazzola ◽  
Giulio Pilone ◽  
Giacomo Dagnino ◽  
Lamberto Felli

Abstract The aim of this study is to evaluate the results of patients underwent lateral open wedge calcaneus osteotomy with bony allograft augmentation combined with tibialis posterior and tibialis anterior tenodesis. Twenty-two patients underwent adult-acquired flatfoot deformity were retrospectively evaluated with a minimum 2-year follow-up. Radiographic preoperative and final comparison of tibio-calcaneal angle, talo–first metatarsal and calcaneal pitch angles have been performed. The Visual Analog Scale, American Orthopedic Foot and Ankle Score, the Foot and Ankle Disability Index and the Foot and Ankle Ability Measure were used for subjective and functional assessment. The instrumental range of motion has been also assessed at latest follow-up evaluation and compared with preoperative value. There was a significant improvement of final mean values of clinical scores (p < 0.001). Nineteen out of 22 (86.4%) patients resulted very satisfied or satisfied for the clinical result. There was a significant improvement of the radiographic parameters (p < 0.001). There were no differences between preoperative and final values of range of motion. One failure occurred 7 years after surgery. Adult-acquired flatfoot deformity correction demonstrated good mid-term results and low recurrence and complications rate. Level of evidence Level 4, retrospective case series.


2021 ◽  
pp. 1-9
Author(s):  
Biljana Kuzmanović Elabjer ◽  
Mladen Bušić ◽  
Andrej Pleše ◽  
Mirjana Bjeloš ◽  
Daliborka Miletić ◽  
...  

<b><i>Introduction:</i></b> Ultrasound biomicroscopy (UBM) is the only widely used method for the evaluation of anterior uveal melanoma (AUM). <b><i>Objective:</i></b> Documentation of regression of AUM treated with ruthenium-106 (Ru-106) plaque types CCB and CCC using UBM. <b><i>Methods:</i></b> This single institution-based retrospective case series involved 10 Caucasian patients with AUM followed after brachytherapy with UBM from January 2014 until February 2019. The largest prominence of the tumor perpendicular to the sclera or the cornea (including scleral/corneal thickness) (<i>D</i>) and the largest basal dimension (<i>B</i>) were measured in millimeters with UBM for all patients prior to the brachytherapy and at 4-month interval follow-up. Tumor regression was calculated as a percentage of decrease in the initial <i>D</i> and <i>B</i> values. <b><i>Results:</i></b> The study involved 10 patients with a mean age of 64.4 years (yr) (range 46–80 yr). <i>D</i> ranged from 1.82 to 5.5 mm (median 2.99 mm) and <i>B</i> from 2.32 to 12.38 mm (median 4.18 mm). The apical radiation dose in all patients was 100 Gy. The median follow-up was 42.02 months. Regression for <i>D</i> was 21.11 ± 13.66%, 31.09 ± 14.66%, and 34.92 ± 19.86% at 1st, 2nd, and 3rd year of the follow-up, respectively, while for <i>B</i> it was 21.58 ± 16.05%, 28.98 ± 17.71%, and 32.06 ± 18.96%, respectively. Tumor recurrence was documented in 2/10 patients. <b><i>Conclusion:</i></b> The major regression of AUM, treated with Ru-106 plaque types CCB and CCC, was documented in the first 2 years after brachytherapy in our study group. In the following years, only minimal regression was documented that warns of the need for close monitoring and active search for local recurrences.


2021 ◽  
pp. 112067212110071
Author(s):  
Vijitha S Vempuluru ◽  
Monalisha Pattnaik ◽  
Neha Ghose ◽  
Swathi Kaliki

Purpose: To describe the risk factors, clinical presentation, management, and outcomes of patients with bilateral ocular surface squamous neoplasia (OSSN). Methods: Retrospective case series. Results: Of the 25 patients with bilateral OSSN, the mean age at diagnosis of OSSN was 31 years (median, 24 years; range, 2–60 years). Risk factors for bilateral OSSN included xeroderma pigmentosum ( n = 15, 60%), human immunodeficiency virus infection ( n = 3, 12%), conjunctival xerosis ( n = 1, 4%), and topical steroid use ( n = 1, 4%). There were no identifiable ocular or systemic risk factors in 7 (28%) patients. Presentation was synchronous in 14 (56%) and metachronous in 11 (44%) patients. Tumor morphology was bilaterally similar in 12 (48%) patients. Histopathological examination ( n = 36) revealed conjunctival intraepithelial neoplasia (CIN) grade 1 in 4 (8%); grade 2 in 7 (14%); carcinoma in situ in 5 (10%), and invasive carcinoma in 20 (40%). Primary management of OSSN ( n = 49) included excisional biopsy ( n = 31, 62%), topical immunotherapy (IFN α2B) ( n = 11; 22%), topical Mitomycin C (MMC) ( n = 3, 6%), enucleation ( n = 1, 2%), orbital exenteration ( n = 2, 4%), and plaque brachytherapy (PBT) ( n = 1, 2%). One patient was lost to follow-up after detection of tumor in the second eye. Recurrent tumors were noted in 16 (32%) eyes and binocular globe salvage was achieved in 16 (64%) patients at a mean follow up of 41 months (median 30 months; range, 1–164 months). Conclusion: OSSN occurrence can be synchronous or metachronous. Meticulous examination of the fellow eye is important for an early diagnosis of OSSN.


Sign in / Sign up

Export Citation Format

Share Document