scholarly journals Cutaneous Vascular Neoplasms of Uncertain Biological Behavior

Biology ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 1160
Author(s):  
Kasey J. McCollum ◽  
Rami N. Al-Rohil
Keyword(s):  
Case Series ◽  
Epithelioid Hemangioendothelioma ◽  
Biological Behavior ◽  
Surgical Strategies ◽  
Pseudomyogenic Hemangioendothelioma ◽  
Vascular Neoplasms ◽  
Indeterminate Lesion ◽  
Comprehensive Framework ◽  
National Databases

Neoplasms of uncertain biological behavior present physicians with a genuine conundrum in practice. Cutaneous vascular neoplasms within this category are exceedingly rare, possessing significant gaps and uncertainty in many facets of clinical practice. Firstly, lesions were selected for review based on their categorization as indeterminate behavior, indicating the potential for local recurrence and rarely metastasize. After identification of the target lesions, a comprehensive review of the literature using national databases produced several landmark studies and case series regarding these neoplasms. Limiting the review to only cutaneous limited tumors narrowed the pool of studies; however, quite a large sum of papers remained. Examination of each paper yielded beneficial results on diagnosing, effective treatments, follow-up findings, and prognosis for each indeterminate lesion discussed. Overall, the literature search combined the molecular, histologic, immunohistochemical, surgical strategies to develop an up-to-date and comprehensive framework to guide physicians when encountering such lesions. The tumors reviewed include: kaposiform hemangioendothelioma, endovascular papillary angioendothelioma, pseudomyogenic hemangioendothelioma, retiform hemangioendothelioma, epithelioid hemangioendothelioma, and composite hemangioendothelioma.

scholarly journals Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Nasir Ud Din ◽  
Shabina Rahim ◽  
Tamana Asghari ◽  
Jamshid Abdul-Ghafar ◽  
Zubair Ahmad
Keyword(s):  
Surgical Resection ◽  
Case Series ◽  
Liver Function Tests ◽  
Vascular Tumor ◽  
Metastatic Carcinoma ◽  
Epithelioid Hemangioendothelioma ◽  
Clinicopathological Features ◽  
University Hospital ◽  
Detailed Review

Abstract Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.

scholarly journals Role of endoscopic transnasal surgery for skull base chondrosarcoma: a retrospective analysis of 19 cases at a single institution

2018 ◽  
Vol 128 (5) ◽  
pp. 1438-1447 ◽  
Author(s):  
Hirotaka Hasegawa ◽  
Masahiro Shin ◽  
Kenji Kondo ◽  
Shunya Hanakita ◽  
Akitake Mukasa ◽  
...  
Keyword(s):  
Skull Base ◽  
Cranial Nerves ◽  
Case Series ◽  
Neurological Complications ◽  
Biological Behavior ◽  
Tumor Control ◽  
Surgical Removal ◽  
Maximal Diameter ◽  
Transnasal Surgery

OBJECTIVESkull base chondrosarcoma is one of the most intractable tumors because of its aggressive biological behavior and involvement of the internal carotid artery and cranial nerves (CNs). One of the most accepted treatment strategies for skull base chondrosarcoma has been surgical removal of the tumor in conjunction with proactive extensive radiation therapy (RT) to the original tumor bed. However, the optimal strategy has not been determined. The goal of this study was to evaluate the early results of endoscopic transnasal surgery (ETS).METHODSThe authors retrospectively analyzed 19 consecutive patients who underwent ETS at their institution since 2010. Adjuvant stereotactic radiosurgery (SRS) was performed only for the small residual tumors that were not resected to avoid critical neurological complications. Histological confirmation and evaluation of the MIB-1 index was performed in all cases. The Kaplan-Meier method was used to determine the actuarial rate of tumor-free survival.RESULTSThe median tumor volume and maximal diameter were 14.5 cm3 (range 1.4–88.4 cm3) and 3.8 cm (range 1.5–6.7 cm), respectively. Nine patients (47%) had intradural extension of the tumor. Gross-total resection was achieved in 15 (78.9%) of the 19 patients, without any disabling complications. In 4 patients, the surgery resulted in subtotal (n = 2, 11%) or partial (n = 2, 11%) resection because the tumors involved critical structures, including the basilar artery or the lower CNs. These 4 patients were additionally treated with SRS. The median follow-up duration was 47, 28, and 27 months after the diagnosis, ETS, and SRS, respectively. In 1 patient with an anterior skull base chondrosarcoma, the tumor relapsed in the optic canal 1 year later and was treated with a second ETS. Favorable tumor control was achieved in all other patients. The actuarial tumor control rate was 93% at 5 years. At the final follow-up, all patients were alive and able to perform independent activities of daily living without continuous neurological sequelae.CONCLUSIONSThese preliminary results suggest that ETS can achieve sufficient radical tumor removal, resulting in comparative resection rates with fewer neurological complications to those in previous reports. Although the follow-up periods of these cases were relatively short, elective SRS to the small tumor remnant may be rational, achieving successful tumor control in some cases, instead of using proactive extensive RT. Thus, the addition of RT should be discussed with each patient, after due consideration of histological grading and biological behavior. To determine the efficacy of this strategy, a larger case series with a longer follow-up period is essential. However, this strategy may be able to establish evidence in the management of skull base chondrosarcoma, providing less-invasive and effective options as an initial step of treatment.

scholarly journals RARE-23. DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR: A CASE SERIES

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i45-i45
Author(s):  
Ashley Aaroe ◽  
Alexander Ou ◽  
Susan McGovern ◽  
Arnold Paulino ◽  
Jason Johnson ◽  
...  
Keyword(s):  
Obstructive Hydrocephalus ◽  
Prognostic Significance ◽  
Case Series ◽  
Biological Behavior ◽  
Glioneuronal Tumor ◽  
Low Grade ◽  
Mri Findings ◽  
Molecular Features ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Abstract Introduction Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare diagnosis first incorporated into the WHO Classification of Tumors of the Central Nervous System in 2016. Though historically considered indolent, emerging evidence suggests that the biological behavior of these tumors may be further classified by molecular features of prognostic significance. Methods A retrospective review was conducted in accordance with IRB approval of patients with the histologic diagnosis of DLGNT. Demographic, clinical, and molecular data where abstracted from the medical record when available. Results 10 patients were identified (M = 8, F = 2). Median age at diagnosis was 6 years (range 0.3–21 years), and the most common symptoms at diagnosis were related to obstructive hydrocephalus, for which 3 patients required CSF diversion. MRI findings included diffuse leptomeningeal thickening, nodularity, or coating of the subarachnoid or ependymal surfaces. Histologically, these tumors expressed variable features of neuronal and/or glial differentiation. Four patients (40%) were treated with radiation therapy (all craniospinal), which was upfront for 2 patients. Chemotherapy regimens used included temozolomide, carboplatin and vincristine and vinblastine. NTRK or BRAF-targeted therapy were used upon progression. At follow-up, 6/10 had stable disease (4/6 of whom were on second line therapy), 1 had partial response, 1 passed away from sepsis and 2 were lost to follow-up. The median progression-free survival for the four patients who developed disease progression was 26 months (range 12–34 months). Next generation sequencing of the tumor tissue performed using a high-multiplex PCR-based NGS panel detected BRAF-KIAA1549 (4 patients) and NTRK (1 patient) fusions. Conclusions DLGNT are rare tumors with scarce data about imaging characteristic and standard of care treatment. Our case series reinforces current literature that although these tumors appear low-grade, they can be clinically aggressive. Further study is needed regarding molecular diagnosis and profiling treatment strategies.

scholarly journals Retiform hemangioendothelioma: a case series and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Qurratulain Chundriger ◽  
Muhammad Usman Tariq ◽  
Shabina Rahim ◽  
Jamshid Abdul-Ghafar ◽  
Nasir Ud Din
Keyword(s):  
Local Recurrence ◽  
Case Series ◽  
Vascular Tumor ◽  
Biological Behavior ◽  
Vascular Tumors ◽  
Multiple Tumor ◽  
The Third ◽  
Immunohistochemical Markers ◽  
Dabska Tumor

Abstract Background Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. Therefore, complete surgical resection with negative margins is essential for accurate diagnosis and local control. Results In our series, two of the three Pakistani cases were in females, with an age range between 18 and 50 years. Our first patient presented with symptoms of cardiac compromise and pulmonary hypertension. Her computed tomography scan showed multiple tumor masses within the mediastinum. The second patient presented with an ulcerated lesion on his scalp, at right temple. The third patient presented with a hard growth on her left 4th toe which was amputated. Histologically, all cases exhibited retiform arborizing vascular spaces lined by bland endothelial cells with hobnail nuclei, characteristic of retiform hemangioendothelioma. Immunohistochemical markers CD31, CD34 and ERG confirmed the vascular nature of the tumor. The first and the second patient are alive and healthy at 4 and 7 months follow up respectively, while the third patient is lost to follow up. Conclusion Owing to the rate of local recurrence, RH should always be considered in the differential diagnosis of vascular tumors showing arborizing blood vessels, as it may have an atypical presentation and it should be carefully differentiated from Dabska tumor and an angiosarcoma.

Effect of Isolated Medial Patellofemoral Ligament Reconstruction in Patellofemoral Instability Regardless of Predisposing Factors

2020 ◽  
Author(s):  
Jae Ik Lee ◽  
Mohd Shahrul Azuan Jaffar ◽  
Han Gyeol Choi ◽  
Tae Woo Kim ◽  
Yong Seuk Lee
Keyword(s):  
Medial Patellofemoral Ligament ◽  
Trochlear Dysplasia ◽  
Case Series ◽  
Patellofemoral Instability ◽  
International Knee Documentation Committee ◽  
Predisposing Factors ◽  
Mpfl Reconstruction ◽  
Patellar Tilt ◽  
Case Series Study

AbstractThe purpose of this study was to evaluate the outcomes of isolated medial patellofemoral ligament (MPFL) reconstruction, regardless of the presence of predisposing factors. A total of 21 knees that underwent isolated MPFL reconstruction from March 2014 to August 2017 were included in this retrospective series. Radiographs of the series of the knee at flexion angles of 20, 40, and 60 degrees were acquired. The patellar position was evaluated using the patellar tilt angle, sulcus angle, congruence angle (CA), and Caton-Deschamps and Blackburne-Peel ratios. To evaluate the clinical outcome, the preoperative and postoperative International Knee Documentation Committee (IKDC) and Lysholm knee scoring scales were analyzed. To evaluate the postoperative outcomes based on the predisposing factors, the results were separately analyzed for each group. Regarding radiologic outcomes, 20-degree CA was significantly reduced from 10.37 ± 5.96° preoperatively to −0.94 ± 4.11° postoperatively (p = 0.001). In addition, regardless of the predisposing factors, delta values of pre- and postoperation of 20-degree CA were not significantly different in both groups. The IKDC score improved from 53.71 (range: 18–74) preoperatively to 94.71 (range: 86–100) at the last follow-up (p = 0.004), and the Lysholm score improved from 54.28 (range: 10–81) preoperatively to 94.14 (range: 86–100) at the last follow-up (p = 0.010). Isolated MPFL reconstruction provides a safe and effective treatment for patellofemoral instability, even in the presence of mild predisposing factors, such as trochlear dysplasia, increased patella height, increased TT–TG distance, or valgus alignment. This is a Level 4, case series study.

A case series of metastatic pheochromocytomas and paragangliomas: patients characteristics, therapeutic approach and follow-up

2019 ◽  
Author(s):  
Anna Angelousi ◽  
Eva Kassi ◽  
Melpomeni Peppa ◽  
Alexandra Chrysoulidou ◽  
George Zografos ◽  
...  
Keyword(s):  
Therapeutic Approach ◽  
Case Series

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

Mechanical thrombectomy in pediatric stroke: systematic review, individual patient data meta-analysis, and case series

2019 ◽  
Vol 24 (5) ◽  
pp. 558-571 ◽  
Author(s):  
Kartik Bhatia ◽  
Hans Kortman ◽  
Christopher Blair ◽  
Geoffrey Parker ◽  
David Brunacci ◽  
...  
Keyword(s):  
Systematic Review ◽  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Mechanical Thrombectomy ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Neurological Outcomes

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.

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