scholarly journals Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Qurratulain Chundriger ◽  
Muhammad Usman Tariq ◽  
Jamshid Abdul-Ghafar ◽  
Arsalan Ahmed ◽  
Nasir Ud Din
Keyword(s):  
Pediatric Population ◽  
Adult Population ◽  
Correct Diagnosis ◽  
Kaposi Sarcoma ◽  
Minor Component ◽  
Vascular Tumor ◽  
Giant Cells ◽  
Clinicopathological Characteristics ◽  
Multinucleated Giant Cells ◽  
Kaposiform Hemangioendothelioma

Abstract Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

scholarly journals Kaposiform hemangioendothelioma/Kasabach–Merritt syndrome. Сlinical and laboratory characteristics. Analysis of clinical cases

2021 ◽  
Vol 20 (3) ◽  
pp. 74-91
Author(s):  
L. A. Khachatryan ◽  
I. S. Kletskaya ◽  
A. N. Remizov ◽  
G. A. Novichkova ◽  
A. A. Maschan
Keyword(s):  
Heart Function ◽  
Kaposi Sarcoma ◽  
Volume Overload ◽  
Vascular Tumor ◽  
Congenital Defects ◽  
Consumption Coagulopathy ◽  
Kaposiform Hemangioendothelioma ◽  
Liver Size ◽  
Pediatric Hematology ◽  
National Medical

Kaposiform hemangioendothelioma (KHE) is a rare, usually congenital vascular tumor. It resembles Kaposi sarcoma histologically, but etiologically it is not associated with herpes simplex virus type 8. KHE refers to tumors of intermediate malignancy degree. The most severe complication is the addition of thrombocytopenia and consumption coagulopathy, i.e. development of the Kasabach–Merritt syndrome/phenomenon (KMS), which determines the high mortality rate (up to 30%) in this histological variant. The frequency of occurrence of KMS is unknown. Over Patients with KHE/KMS have clear clinical and laboratory characteristics, which in most cases allow make to diagnose without histological confirmation. Over 7-year follow-up period 32 patients with KHE were registered in our center; in 90.6% of cases it was complicated by the development of KMS. The study was approved by the Independent Ethics Committee and Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In the most of patients the tumor was detected from birth (84%), in half of the cases (52%) hematological complications were diagnosed simultaneously with the detection of the tumor. Сommon local complications include joint contractures, destruction of bone tissue, and invasion of neighboring organs. The half of the patients had changes in the heart function: from minor cardiac pathology to congenital defects. In addition, there were clinical and instrumental changes associated with volume overload: an increase in liver size, myocardial hypertrophy. Despite the presence of clear clinical and laboratory characteristics of KMS, some cases require differential diagnosis with other vascular anomalies accompanied by thrombocytopenia and consumption coagulopathy – with congenital hemangiomas (rapidly involuting congenital hemangioma), multifocal lymphangioendotheliomatosis with thrombocytopenia, kaposiform lymphangiomatosis, venous malformations. The parents of the patients agreed to use the information, including photos of children, in scientific research and publications.

scholarly journals Kaposiform Hemangioendothelioma of the GI Tract: An Exception to Occam’s Principle in an Adult with SBO

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Luis E. Aguirre ◽  
Robert A. Ali ◽  
Darcy A. Kerr ◽  
Mahsa Khanlari ◽  
Gilberto Lopes
Keyword(s):  
Small Bowel ◽  
Soft Tissues ◽  
Treatment Options ◽  
Muscularis Propria ◽  
Kaposi Sarcoma ◽  
Exploratory Laparotomy ◽  
Original Description ◽  
Vascular Tumor ◽  
Capillary Hemangioma ◽  
Kaposiform Hemangioendothelioma

Kaposiform hemangioendothelioma (KHE) is a rare and locally aggressive vascular tumor with histological features resembling Kaposi sarcoma and capillary hemangioma mainly occurring in children and adolescents. Approximately 200 cases have been reported since its original description in 1993, with the vast majority presenting at an early age as raised ill-defined lesions with a red-blue hue mainly involving the skin and soft tissues in the extremities. Cases in adults remain extremely rare. Herein, we describe the case of a 29 year-old man who presented with progressive abdominal pain for 4 months and signs of obstipation found to be consistent with small bowel volvulus. The patient underwent exploratory laparotomy and resection of 55 cm of necrotic small bowel followed by enteroenterostomy and anastomosis. Microscopic examination revealed KHE involving small intestinal mesentery, muscularis propria, and submucosa. His recovery was uneventful and he was discharged after stabilization, opting to manage him expectantly with abdominopelvic imaging and to monitor for development of Kasabach-Merritt phenomenon. To our knowledge, this represents the first reported case of this entity presenting as intestinal obstruction in an adult for which we also present a review of the existing literature and possible treatment options.

Kaposiform Hemangioendothelioma of the Thymus

2000 ◽  
Vol 124 (10) ◽  
pp. 1542-1544 ◽  
Author(s):  
Jennifer J. Wilken ◽  
Frederick A. Meier ◽  
Michael J. Kornstein
Keyword(s):  
Soft Tissues ◽  
Kaposi Sarcoma ◽  
Vascular Tumor ◽  
Capillary Hemangioma ◽  
Kaposiform Hemangioendothelioma ◽  
Recent Success ◽  
Formalin Fixed Paraffin ◽  
Formalin Fixed Paraffin Embedded ◽  
Feeding Vessels ◽  
Formalin Fixed

Abstract Kaposiform hemangioendothelioma is a rare pediatric neoplasm that presents most commonly in the soft tissues. We report the case of a 1-month-old infant who presented with stridor and was found to have a diffusely infiltrating tumor in the thymus that extended into the pericardium and up the carotid sheaths. Histologic examination revealed a vascular tumor infiltrating among the lobules of the lymphocyte-depleted thymus. The lesion had features of both a capillary hemangioma and Kaposi sarcoma. Immunoperoxidase studies on formalin-fixed, paraffin-embedded tissue demonstrated the neoplastic endothelial cells to be positive for vascular markers CD31 and CD34. Antibody to factor VIII-related antigen labeled feeding vessels, but failed to stain the lobules of tumor. Although these tumors have been treated in a fashion similar to capillary hemangiomas in the past, it may be important to differentiate Kaposiform hemangioendotheliomas because of their association with Kasabach-Merritt syndrome and recent success with more aggressive chemotherapy regimens.

CLINICAL AND MORPHOLOGICAL CORRELATIONS AND HISTOPATHOLOGY OF JOINT DAMAGE IN PATIENTS WITH DIFFUSE-TYPE TENOSYNOVIAL GIANT CELL TUMOR

2019 ◽  
Vol 72 (12) ◽  
Author(s):  
Olena O Dyadyk ◽  
Anastasiia Hryhorovska
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Cell Size ◽  
Giant Cells ◽  
Cell Tumor ◽  
Multinucleated Giant Cells ◽  
Diffuse Type ◽  
Association Coefficient ◽  
Mean Values ◽  
Tenosynovial Giant Cell Tumor

Introduction: Tenosynovial giant cell tumor (TSGCT) (synonym – pigmented villonodular synovitis) – is a rare benign proliferative lesion of the synovial sheath, localized in the joint capsule, bursa or tendon sheath and characterized by locally destructive growth. Depending on the prevalence within the joint elements, the presence of a capsule around the tumor, histophotographic features of cell structure and clinical behavior TSGCT can be divided to localized or diffuse type. The aim of the study was researching of histopathological properties of diffuse-type TSGCT, determine the parameters its morphological indicators and to find out the correlation between these morphological and clinical parameters. Materials and methods: The research material was used biopsy (resect) of pathological lesions from 50 patients who were diagnosed and histologically verified diffuse-type TSGCT. Microscopic examinations of the stained sections and their photo archiving were carried out with use of a Olympus-CX 41 light optical microscope. Group measurable parameters (mean values and Pearson tetrachoric index (association coefficient) were calculated in groups of comparison for morphological and clinical indices of TSGCT. The mean values were compared by Student’s test, P value of ≤0.1 was considered statistically significant. Results:Correlation analysis of indicators that accounted for the pairs of cases «clinic – morphology» revealed the relationships, that had the highest parameters of the association coefficient between such indicators: «presence of villous growths» - «severity of hemosiderosis» (if hypertrophied synovial villi available, with vascular injection and pronounced proliferation of synovial cells, there is also a significant accumulation of hemosiderin pigment); «presence of villous growths» - «type of predominant cellular proliferates» (if cells of TSGCT diffuse type consists of monotonous sheets of stromal cells, with uniform, oval to reniform nuclei, the proliferation of villi in synovial layer is non-distinctive); «presence of nodes» - «kind of stroma» (if nodes predominate, their histological structure is mainly represented by polymorphic clusters of synovitis cells in the form of cells, strands, chains, solid formations, among immature connective tissue with low hyalinosis); «cell size (area, cm²)» - «severity of haemosiderosis» and «cell size (area, cm²)» - «the number of multinucleated giant cells» (there is a pronounced deposition of pigment and accumulation of osteoclast-like multinucleated giant cells type, although usually their number is relatively small compared to the localized type of TSGCT). Conclusions: Morphological parameters, that we have identified, characterize pathological changes in the tissues of TSGCT; careful analysis of the frequency of their occurrence in the different comparison groups made it possible to establish intergroup differences and correlations between individual indicators, which were previously unknown or not obvious. Our study was determine to analyze of incidence rates and correlation relationships, revealed some previously unknown differences and dependencies that are important for understanding the pathogenesis, improvement of diagnosis and prognosis of diffuse-type TSGCT.

Juvenile Xanthogranuloma of the Sellar Region with a 5-Year Medical History: Case Report and Literature Review

2021 ◽  
pp. 1-8
Author(s):  
Dekui Cheng ◽  
Fengyu Yang ◽  
Ziji Li ◽  
Fan Qv ◽  
Wei Liu
Keyword(s):  
Medical History ◽  
Pediatric Population ◽  
Benign Lesion ◽  
Giant Cells ◽  
Juvenile Xanthogranuloma ◽  
Epithelial Tissue ◽  
Sellar Region ◽  
Optic Chiasma ◽  
Suprasellar Region ◽  
Field Disturbance

<b><i>Introduction:</i></b> Xanthogranuloma of the sellar region is a rare benign lesion, and there are few cases reported in children. Its histogenesis is controversial, and it is difficult to strictly differentiate it from craniopharyngioma (CP), Rathke’s cleft cyst, or pituitary adenoma. <b><i>Case Presentation:</i></b> A 16-year-old boy presented with a rare xanthogranuloma of the sellar region after complaining of retardation of growth 5 years previously. The ophthalmologic evaluation revealed no visual field disturbance. Endocrinological examination revealed hypopituitarism. Magnetic resonance imaging showed an intrasellar mass extending into the suprasellar region and compressing the optic chiasma, which appeared mixed signals on T1-weighted images. Endonasal transsphenoidal resection of the tumor was performed. Histological analysis of the tumor sections demonstrated granulomatous tissue with cholesterol clefts, hemosiderin deposits, fibrous tissues, multinucleated giant cells, and lymphocyte. Thus, the tumor was pathologically diagnosed as xanthogranuloma of the sellar region, which is different from adamantinomatous CP. There was no epithelial tissue in any part of the tumor including tumor capsule but have focal necrosis and calcification. His endocrinological dysfunction did not recover, so a hormonal replacement was continuously required. <b><i>Conclusion:</i></b> Xanthogranuloma of the sellar region is a rare entity but must be considered in the differential diagnosis of lesions of the sellar region, even in pediatric population. We should think about this disease when dealing with children with stunted growth accompanied by a long medical history. Our case demonstrates the natural progression of the disease, suggesting that xanthogranuloma of the sellar region without epithelial components may be an independent disease.

scholarly journals Factors Associated with Clinician Adherence to USPSTF Diabetes Screening Recommendations

2021 ◽  
Vol 12 ◽  
pp. 215013272110165
Author(s):  
Elaine Seaton Banerjee ◽  
Kyle Shaak ◽  
Nicole Burgess ◽  
Melanie Johnson ◽  
Beth Careyva
Keyword(s):  
Gestational Diabetes ◽  
Adult Population ◽  
Correct Diagnosis ◽  
Retrospective Chart Review ◽  
Diabetes Screening ◽  
Factors Associated ◽  
The Us ◽  
History Of ◽  
Missed Diagnosis ◽  
Significant Health

Introduction/Objectives: Diabetes and prediabetes impact nearly half of the US adult population and are associated with significant health risks but may be underdiagnosed. Effective screening may improve diagnosis and give patients opportunity to manage their disease. The purpose of this study was to determine screening rates, identify characteristics predictive of screening, and evaluate correct diagnosis of diabetes and prediabetes. Methods: Retrospective chart review of 71 433 patients eligible for diabetes screening, defined by completing A1c test within the 3-year study period. Results: A total of 31.3% of eligible patients received diabetes screening. Factors associated with screening include older age, female sex, non-white race, Hispanic ethnicity, Medicare or Medicaid insurance, higher BMI, and having a medical comorbidity. History of prediabetes or gestational diabetes were the strongest predictors for diabetes screening, but history of gestational diabetes was under-documented. Of those screened, 10.4% had a result consistent with diabetes and 51.8% had a result consistent with prediabetes. However, 52.9% of these patients had a missed diagnosis. Conclusions: Findings of this study indicate the need for uniform coverage for diabetes screening for all insurances, increased documentation of gestational diabetes to improve screening for patients with this history, and improving accurate diagnosis after screening is completed.

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