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.

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 RARE-31. TREATMENT OUTCOME OF CHILDREN WITH PLEOMORPHIC XANTHOASTROCYTOMA: RETROSPECTIVE ANALYSIS – A SINGLE INSTITUTION EXPERIENCE

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi228-vi228
Author(s):  
Ossama Maher ◽  
Toba Niazi ◽  
Ziad Khatib ◽  
John Ragheb
Keyword(s):  
Local Recurrence ◽  
Retrospective Analysis ◽  
Malignant Transformation ◽  
Cranial Nerve ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Biological Behavior ◽  
High Local Recurrence Rate ◽  
Cranial Nerve Palsies

Abstract BACKGROUND Pleomorphic xanthoastrocytoma (PXA) and anaplastic pleomorphic xanthoastrocytoma (APXA) are two types of rare astrocytomas in pediatrics. There is limited literature reporting their outcomes. METHODS A retrospective analysis of pediatric patients with diagnosis of PXA and APXA treated at Nicklaus Children’s Hospital was conducted using descriptive measures. RESULTS A chart review of patients with brain tumors from 2001 to 2019 revealed that 13 patients (median age 6 years, range 2–17 years) were diagnosed with non-metastatic PXA (n=12) and APXA (n=1). Six patients (46%) were male. Clinical presentation included seizures (n=8), headaches (n=2) cranial nerve palsies (n=2). Diagnostic imaging showed tumor in the temporal lobe (n=5), parietal lobe (n=2), temporoparietal lobe (n=2), frontal lobe (n=1), occipital lobe (n=1), others (n=2). BRAF V600E mutation were identified in three of five analyzed tumors including PXA (n=4) and APXA (n=1). Surgical intervention consisted of gross total resection (n=8) and near/subtotal resection (n=5). Ten patients (76%) required a second surgery (median 2; range 1–4) due to local recurrence. The median time to recurrence was 3 years (range 6 months - 7 years). Three patients (23%) had malignant transformation of PXA, which occurred three to eight years from the initial surgery; one of them also had extensive spinal metastasis. Focal radiation was given to six patients (46%) due to multiple recurrence (n=2), malignant transformation (n=3), APXA (n=1). At a median follow up of 8 years (range 3 to 20 years), eight patients remain alive, three patients had lost to follow up, two patients died from progressive disease. Long-term sequelae varied as follows; seizures (n=5), motor weakness (n=4), cranial nerve palsies (n=2), and learning disability (n=1). CONCLUSION PXA is associated with high local recurrence rate and uncommonly malignant transformation to APXA in pediatrics, which requires close follow-up due to unpredictable biological behavior of these tumors.

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.

GIANT CELL TUMOURS OF TENDON SHEATH OF HAND: CAUSES AND STRATEGIES TO PREVENT RECURRENCE

Hand Surgery ◽  
2011 ◽  
Vol 16 (02) ◽  
pp. 149-154 ◽  
Author(s):  
Azal Jalgaonkar ◽  
Baljinder Dhinsa ◽  
Howard Cottam ◽  
Ganapathyraman Mani
Keyword(s):  
Risk Factors ◽  
Local Recurrence ◽  
Giant Cell ◽  
Recurrence Rate ◽  
Case Series ◽  
Tendon Sheath ◽  
Giant Cell Tumours ◽  
High Incidence ◽  
The Mean

Giant cell tumours of tendon sheath of hand present a surgical dilemma due to their high incidence of local recurrence. We present a case series of 46 patients with 47 histologically confirmed giant cell tumours of tendon sheath over a ten-year period from 1998 to 2008. The mean follow-up was 47 months (range 25–124 months). We identified tumours with bony erosions and piecemeal resections as predictors of recurrence. Our recurrence rate of 9% was at the lower end of spectrum of previously published reports (range 7%–44%). We recommend "en-masse" excision of these tumours. All the patients with suspicion of these tumours should have preoperative radiographs to identify erosions. A thorough curettage of the bone should be done in cases with osseous erosion to prevent recurrence. Patients with these risk factors should be followed up annually for five years and be warned about recurrence.

scholarly journals Absent vagina a description of cases with varied ages, presentations and reconstructions

2019 ◽  
Vol 10 (3) ◽  
pp. 1741-1745
Author(s):  
Ali Adwal Ali ◽  
Khalida M. Amin ◽  
Emil N. AZZO ◽  
Ayla K. Kahya
Keyword(s):  
Adolescent Girls ◽  
Reproductive Tract ◽  
Case Series ◽  
The Other ◽  
Vaginal Agenesis ◽  
Young Females ◽  
The Third ◽  
Case Series Study ◽  
Series Study

The aim is to describe cases of vaginal agenesis of varied ages presentations and managements and report the outcome of interventions regarding restoration of continuity of reproductive tract and normal sexual activity. A prospective descriptive case series study.  Five female patients with vaginal agenesis were recruited from November 2005 to November 2016. Three were adolescents and had a functioning uterus. Three patients underwent vaginoplasty using McIndoe method, one had vagino-vaginal anastomosis, and the other had hysterectomy to remove source of menstruation. Age ranged from 12 to 24 years. The outcome of vaginoplasties to the 2 young females and to one of the adolescent girls was adequate vagina, whereas the other 2 adolescent girls had failed redo vaginoplasty and ended with a patent narrowed vagina in one and a closed vagina in the other which required a hysterectomy. On Follow up (range 5 - 42 months) 2 of the adolescent’s girls had regular menstruation with no pain and pelvic collection, while the third one had amenorrhoea following a hysterectomy. The 3 patients who had successful vaginoplasty got married and enjoyed satisfactory intercourse. Patients with absent vagina presents at variable ages depending on whether a functioning uterus is present or not. The success of vaginoplasty is less when it has to be done in adolescence than when it is done in adulthood when patient is grown-up. Best outcomes require accumulative experience, ideally with the aid of doctors of other specialties.

scholarly journals Does methotrexate influence COVID-19 infection? Case series and mechanistic data

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Fabian Schälter ◽  
Kerstin Dürholz ◽  
Laura Bucci ◽  
Gerd Burmester ◽  
Roberto Caporali ◽  
...  
Keyword(s):  
Family Member ◽  
Case Series ◽  
The Other ◽  
Protective Effects ◽  
Susceptibility To Infection ◽  
The Third ◽  
Experimental Part ◽  
Mrna And Protein Expression ◽  
Methotrexate Treatment

Abstract Background To investigate whether methotrexate treatment may affect the susceptibility to infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Methods Clinical assessment of symptoms, SARS-CoV-2 RNA, and anti-SARS-CoV-2 IgG in an initial case series of four families and confirmatory case series of seven families, within which one family member developed coronavirus disease 19 (COVID-19) and exposed another family member receiving methotrexate treatment; experimental part with methotrexate treatment of mice and organoids followed by the assessment of mRNA and protein expression of the SARS-CoV-2 receptor angiotensin-converting enzyme (ACE)-2. Results In the initial case series, three of four women on a joint ski trip developed COVID-19, while the fourth woman, under treatment with methotrexate, remained virus-free. Two of the three diseased women infected their husbands, while the third husband treated with methotrexate remained virus-free. In addition, 7 other families were identified in a follow-up case series, in which one member developed COVID-19, while the other, receiving methotrexate, remained healthy. Experimentally, when mice were treated with methotrexate, ACE2 expression significantly decreased in the lung, in the intestinal epithelium, and in intestinal organoids. Conclusion These clinical and experimental data indicate that methotrexate has certain protective effects on SARS-CoV-2 infection via downregulating ACE2.

scholarly journals Topographical Irregularity and Keratoconic Findings in Five Siblings and their Parents

2014 ◽  
Vol 3 (3) ◽  
pp. 130-135
Author(s):  
Jose M González-Méijome ◽  
Antonio Queiros ◽  
Alberto Diaz-Rey ◽  
José Salgado-Borges ◽  
Sofia C Peixoto-de-Matos ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Refractive Error ◽  
Case Series ◽  
Advanced Case ◽  
The Third ◽  
History Of ◽  
Ophthalmologic Examination ◽  
Degrees Of Severity ◽  
Irregular Astigmatism

ABSTRACT Purpose To report the clinical features of five siblings, four of them with positive diagnostic of keratoconus in different degrees of severity as well as their parents findings. Materials and methods Seven elements of the same family, five young siblings (2 males, 3 females), and their parents were screened for potential keratoconic signs. Topographic data from 60 normal eyes are also reported for comparison purposes. Complete ophthalmologic examination including biomicroscopic, topographic and refractive examination. Main outcome measures included biomicroscopic findings, refractive error and visual acuity, simulated keratometry, corneal eccentricity, indices of asymmetry and regularity. Results The more advanced case was present in the left eye of a male member, needing a rigid gas permeable lens to correct irregular astigmatism. Another brother also presented bilateral mild to moderate keratoconus with no apparent vision complaints. The third case diagnosed was one sister with history of monthly disposable soft toric lens to compensate a presumed initially regular astigmatism two years before. The remaining two cases being the older sister and one of the younger sisters presented the less noticeable signs, with confirmation of the pathology only in one of them and warranting a close follow- up of both due to the asymmetric corneal topography between both eyes as well as between the flatter superior and steeper inferior corneal areas. Conclusion This is the first reported case series involving diagnosis of keratoconus or atypical corneal topographies in several young siblings and their parents. Further evaluation of this and other cases with similar presentations might help to gain a deeper understanding on the potential genetic paths of keratoconus. How to cite this article González-Méijome JM, Peixoto-de- Matos SC, Soares A, Queirós A, Díaz-Rey A, Salgado-Borges J. Topographical Irregularity and Keratoconic Findings in Five Siblings and their Parents. Int J Kerat Ect Cor Dis 2014;3(3):130-135.

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.

Littoral Cell Angioma of the Spleen: An Additional Report of Four Cases with Emphasis on the Association with Visceral Organ Cancers

1998 ◽  
Vol 84 (5) ◽  
pp. 595-599 ◽  
Author(s):  
Michele Bisceglia ◽  
Joshua Z. Sickel ◽  
Felice Giangaspero ◽  
Vito Gomes ◽  
Mostafà Amini ◽  
...  
Keyword(s):  
Vascular Tumor ◽  
Clinical Syndrome ◽  
Vascular Tumors ◽  
Littoral Cell ◽  
Littoral Cell Angioma ◽  
Tentorial Meningioma ◽  
Clinical Observations ◽  
Littoral Cells ◽  
Tumors Of The Liver

Aims and background Littoral cell angioma (LCA) is an uncommon vascular tumor of the spleen recently described and interpreted as the tumoral counterpart of the normally present littoral cells lining the splenic sinus channels of red pulp. The diagnosis of LCA is suggested by a quite characteristic morphology and confirmed by the demonstration of a hybrid endothelial/histiocytic phenotype. Methods Four original and previously unreported cases of LCA are presented. All four splenic vascular tumors were investigated by light microscopy and immunohistochemistry for endothelial and histiocytic markers. Results All four cases were associated with visceral epithelial malignancies (colorectal adenocarcinoma in two cases, renal and pancreatic adenocarcinoma in one case each). One case was also associated with an intracranial tentorial meningioma. Conclusions We consider our findings as a novelty and signal the possible existence of a clinical syndrome. Five of a total of 21 previously reported cases in the literature were also described as being associated with other cancers (non-Hodgkin's lymphoma in two cases, two not further specified tumors of the liver and brain, an epithelial ovarian cancer, and a non-small cell lung cancer in one case each). Close follow-up and careful investigation in search of a second visceral neoplasm are strongly recommended in cases of LCA, but further clinical observations and more in-depth genetic and molecular studies are needed before any valid conclusions can be drawn.

Autologous, Noncultured Epidermal Cell Suspension Grafting in the Management of Mechanically and Chemically Induced Leukodermic Scars

2015 ◽  
Vol 19 (5) ◽  
pp. 488-493 ◽  
Author(s):  
Nikki S. Vyas ◽  
Kelsey L. Lawrence ◽  
James L. Griffith ◽  
Iltefat H. Hamzavi
Keyword(s):  
Cell Suspension ◽  
Epidermal Cell ◽  
Case Series ◽  
Prognostic Indicator ◽  
The Third ◽  
Small Case Series ◽  
Chemically Induced ◽  
Transplant Procedure ◽  
Skin Phototype

Background: Melanocyte-keratinocyte transplant procedure (MKTP) successfully repigments postburn leukodermic scars. Objective: To further investigate the efficacy and limitations of MKTP for treatment of mechanically and chemically induced leukodermic scars. Methods: Ten patients with mechanically or chemically induced, depigmented or hypopigmented scars were preoperatively evaluated with Wood’s light examination, treated with MKTP, and followed for at least 6 months, with monitoring of repigmentation and colour matching. Results: Nine patients attended at least 6 months of follow-up. Six patients showed no fluorescence of scars under Wood’s lamp. All patients whose lesions didn’t fluoresce displayed less than 50% repigmentation, with 5 of 6 attaining 0% to 24%. Of the 3 patients displaying bright or some fluorescence, more than 95% repigmentation was achieved in 2 patients (skin phototypes V and VI), while less than 24% was attained for the third (skin phototype II). Conclusions: In this small case series, lack of fluorescence in leukodermic scars may be a useful negative prognostic indicator for MKTP, but additional trials are needed to verify that this is not due to melanocompetency.

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