scholarly journals Repidly involuting congenital hemangioma

2021 ◽  
Vol 20 (1) ◽  
pp. 145-155
Author(s):  
L. A. Khachatryan ◽  
I. I. Chikvina ◽  
I. S. Kletskaya
Keyword(s):  
Complete Resolution ◽  
Infantile Hemangioma ◽  
Vascular Tumor ◽  
Pathological Process ◽  
Vascular Tumors ◽  
Consumption Coagulopathy ◽  
Kaposiform Hemangioendothelioma ◽  
Histological Characteristics ◽  
Congenital Hemangioma ◽  
Rapid Regression

Congenital hemangiomas are rare benign vascular tumors that develop in utero and are fully formed by the time of birth. Depending on the ability to involution, there are three subtypes: RICH (repidly involuting congenital hemangioma), NICH (non involuting congenital hemangioma), PICH (partially involuting congenital hemangioma). PICH may be accompanied by thrombocytopenia and consumption coagulopathy. Despite clearly defined clinical and histological characteristics, it can be difficult to make a differential diagnosis between congenital hemangiomas and other vascular tumors (infantile hemangioma, kaposiform hemangioendothelioma/“fascicular” angioma and others). The clinical case in the article of a vascular tumor in a newborn complicated by thrombocytopenia and consumption coagulopathy was regarded as Kazabach-Merritt syndrome, which is based on kaposiform hemangioendothelioma/“fascicular” angioma. Rapid regression of the tumor and recovery of hemogram and coagulogram parameters, as well as anamnesis of the disease and initial characteristics of the tumor forced to reconsider the diagnosis. Based on the histological picture, the diagnosis of congenital hemangioma, RICH, was confirmed. Verification of the diagnosis made it possible to change therapeutic tactics and avoid chemotherapy. A giant hemangioma, accompanied by thrombocytopenia and consumption coagulopathy, may have a very favorable outcome – a complete resolution of the pathological process inherent in its natural course. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications. 

scholarly journals New Approaches In The Diagnosis Of Oral Hemangiomas

2020 ◽  
Vol 2 (08) ◽  
pp. 50-57
Author(s):  
Karimov M.A. ◽  
Mamarasulova D.Z. ◽  
Sadykov R.R. ◽  
Nurmatova Kh.Kh.
Keyword(s):  
Comparison Group ◽  
Scientific Work ◽  
Infantile Hemangioma ◽  
Pyogenic Granuloma ◽  
Medical Institute ◽  
Vascular Tumors ◽  
City Hospital ◽  
Medical Academy ◽  
Congenital Hemangioma

Scientific work was carried out on the basis of the Tashkent Medical Academy, Department of Surgery (1st city hospital), and Andijan Medical Institute, Department of Oncology from 2015-2020.  Clinical material includes 175 patients.  The comparison group consisted of 100 patients who were treated in various hospitals in Tashkent and regions of Uzbekistan.  According to the classification of vascular anomalies, the majority of patients with benign vascular tumors (DBT) were diagnosed with infantile hemangioma (IG) - 82.0%, congenital hemangioma (VH) - 11.4%, pyogenic granuloma (PG) - 5.8%.  Average age of patients: children - 1.2 ± 0.4, adults - 27 ± 1.2 years.  The frequency of DSO in girls was 78%, in boys - 22%.  Localization area: lip - 57.6%, cheek 8%, tongue 26.3%.  Complications of DSO: anatomical disorders, edema, bleeding, pain, infection, respiratory disorders occurred in 83.7% of patients.

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.

Rapidly Involuting Congenital Hemangioma: Clinical and Histopathologic Features

2003 ◽  
Vol 6 (6) ◽  
pp. 495-510 ◽  
Author(s):  
Beatriz Berenguer ◽  
John B. Mulliken ◽  
Odile Enjolras ◽  
Lawrence M. Boon ◽  
Michel Wassef ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Glucose Transporter ◽  
Fibrous Tissue ◽  
Cyst Formation ◽  
Infantile Hemangioma ◽  
Vascular Tumors ◽  
Glucose Transporter 1 ◽  
Pathologic Features ◽  
Congenital Hemangioma ◽  
Rapidly Involuting Congenital Hemangioma

We define the histopathologic findings and review the clinical and radiologic characteristics of rapidly involuting congenital hemangioma (RICH). The features of RICH are compared to the equally uncommon noninvoluting congenital hemangioma (NICH) and common infantile hemangioma. RICH and NICH had many similarities, such as appearance, location, size, and sex distribution. The obvious differences in behavior served to differentiate RICH, NICH, and common infantile hemangioma. Magnetic resonance imaging (MRI) of the three tumors is quite similar, but some RICH also had areas of inhomogeneity and larger flow voids on MRI and arterial aneurysms on angiography. The histologic appearance of RICH differed from NICH and common infantile hemangioma, but some overlap was noted among the three lesions. RICH was composed of small-to-large lobules of capillaries with moderately plump endothelial cells and pericytes; the lobules were surrounded by abundant fibrous tissue. One-half of the specimens had a central involuting zone(s) characterized by lobular loss, fibrous tissue, and draining channels that were often large and abnormal. Ancillary features commonly found were hemosiderin, thrombosis, cyst formation, focal calcification, and extramedullary hematopoiesis. With one exception, endothelial cells in RICH (as in NICH) did not express glucose transporter-1 protein, as does common infantile hemangioma. One RICH exhibited 50% postnatal involution during the 1st year, stopped regressing, was resected at 18 months, and was histologically indistinguishable from NICH. In addition, several RICH, resected in early infancy, also had some histologic features suggestive of NICH. Furthermore, NICH removed early (2–4 years), showed some histologic findings of RICH or were indistinguishable from RICH. We conclude that RICH, NICH, and common infantile hemangioma have overlapping clinical and pathologic features. These observations support the hypothesis that these vascular tumors may be variations of a single entity ab initio. It is unknown whether the progenitor cell for these uncommon congenital vascular tumors is the same as for common infantile hemangioma.

scholarly journals Rapidly involuting congenital hemangioma associated with Kasabach-Merritt Syndrome

2021 ◽  
Vol 4 (1) ◽  
pp. 050-052
Author(s):  
Ballona Rosalia ◽  
Zevallos Jean ◽  
Núñez Jeanette
Keyword(s):  
Surgical Excision ◽  
Vascular Tumor ◽  
Vascular Tumors ◽  
Histopathological Study ◽  
First Year ◽  
Coagulation Disorder ◽  
Tufted Angioma ◽  
First Year Of Life ◽  
Congenital Hemangioma ◽  
Rapidly Involuting Congenital Hemangioma

Background: Rapidly involuting congenital hemangioma (RICH) is a rare vascular tumor that is present at birth and involutes during the first year of life. Kasabach-Merritt syndrome (KMS) is a complication of some vascular tumors such as kaposiform hemangioendothelioma and tufted angioma associated with thrombocytopenia and coagulopathy. Results: The case of a 2-month-old infant with a diagnosis of RICH with thrombocytopenia and coagulation disorder, successfully treated with surgical excision without complications or recurrence is presented. Conclusion: The association between RICH and KMS is rare. Histopathological study, immunohistochemistry and ultrasound findings are important for the diagnosis. Brief summary: This report covers the rare association between rapidly involuting congenital hemangioma and Kasabach-Merritt syndrome in a 2-months-old female infant.

Prenatal diagnosis of rapidly involuting congenital hemangioma: a case report and review of the literature

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Maria-Elisabeth Smet ◽  
Vanessa Pincham ◽  
Andrew McLennan
Keyword(s):  
Case Report ◽  
Vascular Tumor ◽  
Vascular Tumors ◽  
Review Of The Literature ◽  
Skin Excess ◽  
Threatening Condition ◽  
Life Threatening ◽  
Fetus And Neonate ◽  
Congenital Hemangioma ◽  
Rapidly Involuting Congenital Hemangioma

Abstract Congenital hemangiomata are rare benign vascular tumors, presenting as fully mature lesions at birth. Three types have been described; the “rapidly involuting congenital hemangioma” (RICH), the “non-involuting congenital hemangioma” (NICH)and the “partially involuting congenital hemangioma” (PICH). We herein report on a RICH type congenital hemangioma, identified during a fetal morphology ultrasound performed at 19 weeks’ gestation. Early diagnosis allowed close surveillance of the fetus and neonate and observation of the natural course of this lesion. Although being a potentially life-threatening condition, no intervention was required. It presented as a 4 cm vascular tumor on the forehead at time of the cesarean section and diminished rapidly over the first 4 neonatal months, resulting in some skin excess and discoloration which is expected to entirely resolve by 14 months of age.

Vascular Anomalies

2018 ◽  
Author(s):  
Javier A Couto ◽  
Arin K Greene
Keyword(s):  
Physical Examination ◽  
Imaging Study ◽  
Infantile Hemangioma ◽  
Pyogenic Granuloma ◽  
Vascular Anomalies ◽  
First Line ◽  
Kaposiform Hemangioendothelioma ◽  
Line Imaging ◽  
Almost All ◽  
Congenital Hemangioma

Vascular anomalies are common pediatric lesions affecting approximately 5% of the population. Lesions are broadly classified into tumors and malformations. The most common tumors are infantile hemangioma, congenital hemangioma, pyogenic granuloma, and kaposiform hemangioendothelioma. The major malformations are capillary, lymphatic, venous, and arteriovenous. Almost all lesions can be diagnosed by the history and physical examination. Ultrasonography is usually the first-line imaging study to confirm a diagnosis. Management of vascular anomalies is based on the type of lesion. Treatments include resection, systemic pharmacotherapy, intralesional drugs, laser, sclerotherapy, and/or embolization. Most patients are best managed in an interdisciplinary vascular anomalies center. This review contains 9 figures, 2 tables and 29 references Key words: arteriovenous, capillary, congenital, hemangioma, kaposiform, lymphatic, malformation, pyogenic, vascular, venous

scholarly journals LASER SURGERY FOR SKIN VASCULAR TUMORS IN INFANTS

2019 ◽  
Vol 22 (2) ◽  
pp. 99-105
Author(s):  
L. D. Shakina ◽  
Igor V. Ponomarev ◽  
I. E. Smirnov
Keyword(s):  
Laser Radiation ◽  
Radiation Exposure ◽  
Laser Treatment ◽  
Laser Surgery ◽  
High Efficiency ◽  
Laser System ◽  
Infantile Hemangioma ◽  
Vascular Tumor ◽  
Vascular Tumors ◽  
Vascular Bed

In young children, benign vascular tumors (VT) are most often manifested as congenital hemangioma and infantile hemangioma. There are considered clinical variants, pathogenesis, evaluation of severity and approaches to assessing the effectiveness of laser surgical treatment for VT in infants. For the first time, there are presented numerical criteria for selecting the optimal laser system to provide the relevant photo-obstruction of the pathological vascular bed, reducing the risk for the development of side effects in the early and remote period after the laser radiation exposure. Comparison of the values of the coefficients of complete absorption of oxyhemoglobin and hemoglobin at different stages of vascular tumor development shows the optimal laser treatment of vascular tumors can be realized by the laser radiation with a wavelength of 578 nm, since it is absorbed to the maximum extent by the main vascular photothermophores - oxyhemoglobin and hemoglobin. Taking into account the features of the angioarchitecture of the pathological vascular bed of the papillary dermis, to prevent the occurrence of petechial hemorrhages (purpura) immediately after the procedure, laser irradiation should be carried out in a pulsed mode with a pulse duration of 15-20 ns. To prevent thermal damage to the reticular layer of the dermis, it is advisable to use a laser pen with a diameter of 0.6-1 mm. The choice of optimal conditions for radiation exposure ensures high efficiency of the laser treatment of vascular tumors, which have been becoming one of the most promising directions in modern hospital-replacing technologies in pediatric laser surgery.

scholarly journals Safe and Effective Treatment of Intracranial Infantile Hemangiomas with Beta-Blockers

2021 ◽  
Vol 13 (3) ◽  
pp. 347-356
Author(s):  
Aoife Naughton ◽  
Ariel Yuhan Ong ◽  
Goran Darius Hildebrand
Keyword(s):  
Effective Treatment ◽  
Beta Blockers ◽  
Combined Treatment ◽  
Infantile Hemangioma ◽  
Intracranial Extension ◽  
Vascular Tumors ◽  
Complete Regression ◽  
Pharmacological Management ◽  
Excellent Outcome ◽  
The Right

Infantile hemangiomas are common benign vascular tumors but are rarely found in an intracranial location. Our literature review identified 41 reported cases. There is no general consensus on management of these rare lesions and until recently, treatment was limited to surgery or pharmacological management with steroids or interferon. Although beta-blockers have been widely prescribed in the treatment of cutaneous infantile hemangiomas since 2008, their use in the treatment of intracranial infantile hemangiomas has been minimal. We present a case of infantile hemangioma affecting the right orbit, associated with intracranial extension, causing intermittent right facial nerve palsy. The patient achieved an excellent outcome following combined treatment with oral propranolol and topical timolol maleate 0.5%, with complete regression of the lesion by 4 months. We conclude that beta-blockers are a safe and effective treatment of intracranial infantile hemangiomas and can be employed as first-line management of these lesions.

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.

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