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.

scholarly journals Melanotic Neuroectodermal Tumor of Infancy: Presentation of a Case Affecting the Mandible

2019 ◽  
Author(s):  
Souhir Khemiri ◽  
Jihene Feki ◽  
Afef Khanfir ◽  
Mohamed Abdelmoula ◽  
Mounir Frikha
Keyword(s):  
Neoadjuvant Chemotherapy ◽  
Surgical Excision ◽  
Neuroectodermal Tumor ◽  
Early Infancy ◽  
High Rate ◽  
Diagnosis And Treatment ◽  
First Year ◽  
Treatment Methods ◽  
First Year Of Life ◽  
Anterior Mandible

Abstract- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, and other rare locations. Common treatment methods’ include surgical excision and resection of the tumor. The aim of this article was to show the diagnosis and treatment of a 7-month-old patient with melanotic neuroectodermal tumor occurred in the anterior mandible and to demonstrate the effectiveness of the neoadjuvant chemotherapy.

Successful Use of Propranolol in a Symptomatic Giant Congenital Hepatic Hemangioma

2020 ◽  
Vol 34 (1-2) ◽  
pp. 99-102
Author(s):  
Luis S. Florian-Tutaya ◽  
Lizet Cuba-Valencia ◽  
Sandra F. Bustamante-Encinas ◽  
Fernando M. Vela-Alfaro
Keyword(s):  
Corticosteroid Therapy ◽  
Full Term ◽  
Vascular Tumors ◽  
First Year ◽  
Hepatic Hemangioma ◽  
Tumor Characteristics ◽  
Term Newborn ◽  
First Year Of Life ◽  
Complications And Mortality ◽  
Hepatic Hemangiomas

Congenital hepatic hemangiomas are rare benign vascular tumors present at birth. They often are asymptomatic but can have significant complications and mortality that directly correlate with the tumor characteristics and size. Congenital hemangiomas generally regress during the first year of life and are managed medically or surgically depending on its course. We present a case of a full-term newborn with a symptomatic giant congenital hepatic hemangioma that received propranolol and corticosteroid therapy.

Lymphangiomas of the Larynx in Infants and Children

1986 ◽  
Vol 95 (6_suppl) ◽  
pp. 1-20 ◽  
Author(s):  
Seymour R. Cohen ◽  
Jerome W. Thompson
Keyword(s):  
Radical Surgery ◽  
Surgical Excision ◽  
Cystic Hygroma ◽  
The Body ◽  
First Year ◽  
Body Structure ◽  
Rare Occurrence ◽  
First Year Of Life ◽  
Extensive Involvement ◽  
In Infants And Children

Lymphangiomas are uncommon benign congenital lymphatic tumors of childhood. They are found in all parts of the body structure, but rarely occur in the larynx. This paper reviews charts of 160 patients with cystic hygroma (lymphangiomata) of whom ten had extensive involvement of the larynx. A benign but nevertheless challenging tumor, its treatment demands the patience and skill of the surgeon. The lesion is more common in the white patient, and 40% appear in the newborn. Fifty percent present by the end of the first year of life, and 75% by the end of the second. The onset is uncommon in the older child and a rare occurrence in the adult. The treatment of lymphangiomata is surgical excision, and is more difficult when in the larynx. Laser surgery has been most helpful when the lesion is in the laryngeal complex. Considering the extreme difficulties which lymphangioma present to the child and to the physician, the ultimate results of all forms of conservative surgical therapy can be rewarding. Excessive or radical surgery will not necessarily guarantee complete elimination of disease and may be harmful.

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 Pulmonary Artery Sarcoma Diagnosed by Transbronchial Endosonographic (EBUS-TBNA) Approach

2020 ◽  
pp. 1-4
Author(s):  
Grigoris Sratakos ◽  
Konstantina Frangia-Tsivou ◽  
Abir Alsaid ◽  
Eleni Karetsi ◽  
Evangelia Koukaki ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Clinical Manifestations ◽  
Surgical Excision ◽  
Vascular Tumor ◽  
Endobronchial Ultrasound ◽  
Vascular Tumors ◽  
Surgical Biopsy ◽  
Transbronchial Needle Aspiration ◽  
Curative Intent ◽  
Pulmonary Artery Sarcoma

Background: Pulmonary Artery Sarcomas are the rarest and the most aggressive vascular tumors. Their accurate diagnosis is challenging due to the lack of specific symptoms and clinical manifestations and they are often initially misdiagnosed and managed as chronic pulmonary thromboembolic disease until an alternate diagnosis is suspected based on the lack of response to anticoagulants. Proper management is thus often delayed until histological confirmation. Moreover, the lack of consensus regarding their diagnosis and treatment further contributes to their reported high mortality [1]. Case Presentation: We present the case of a 34-year-old male, nonsmoker, who presented with hemoptysis, fatigue and dyspnea on exertion 3 months prior to admission. His chest x-ray showed enlargement of the left hilum and chest CT revealed a large intraluminal low attenuation filling defect at the level of the left PA. Chest MRI confirmed the presence of a large endovascular tumor of the left PA. Before referring the patient for surgical biopsy, he underwent bronchoscopy during which the vascular tumor was successfully located, observed and sampled by endobronchial ultrasound (EBUS) transbronchial needle aspiration (TBNA). Tissue biopsy through EBUS-TBNA revealed an intimal sarcoma of the PA. The patient was subsequently referred for surgical excision with curative intent. Conclusion: Bronchoscopy and EBUS-TBNA may safely and accurately diagnose even rare pathologies of the mediastinal vasculature adjacent to the airways through transbronchial approach.

Congenital Infantile Fibrosarcoma of the Thigh in a Newborn

2001 ◽  
Vol 87 (6) ◽  
pp. 436-438 ◽  
Author(s):  
Bilgehan Yalçin ◽  
Gürsel Leblebicioğlu ◽  
Elif Güler ◽  
Gökhan Gedikoğlu ◽  
M Tezer Kutluk
Keyword(s):  
Case Report ◽  
Adjuvant Treatment ◽  
Standard Treatment ◽  
Clinical Course ◽  
Surgical Excision ◽  
Left Thigh ◽  
Wide Excision ◽  
First Year ◽  
Infantile Fibrosarcoma ◽  
First Year Of Life

Congenital/infantile fibrosarcoma occurs frequently in the first year of life and differs from fibrosarcoma in adulthood. The clinical course of congenital/infantile fibrosarcoma is more favorable and metastatic spread is rare. While adult fibrosarcomas are common in the thigh, congenital/infantile fibrosarcomas affect chiefly the distal portions of the extremities. Standard treatment is primarily wide surgical excision. Chemotherapy may be given as neoadjuvant or adjuvant treatment in order to avoid the morbidity associated with wide excision. In this case report we present our experience of a newborn affected by congenital/infantile fibrosarcoma of the left thigh.

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. 

The Development of Swallowing Respiratory Coordination

2009 ◽  
Vol 18 (1) ◽  
pp. 19-24
Author(s):  
Maggie-Lee Huckabee
Keyword(s):  
Cortical Activation ◽  
First Year ◽  
Healthy Children ◽  
Nasal Airflow ◽  
Single Session ◽  
Primary Finding ◽  
Patterns Of Behavior ◽  
Cortical Modulation ◽  
First Year Of Life ◽  
Swallowing Apnea

Abstract Research exists that evaluates the mechanics of swallowing respiratory coordination in healthy children and adults as well and individuals with swallowing impairment. The research program summarized in this article represents a systematic examination of swallowing respiratory coordination across the lifespan as a means of behaviorally investigating mechanisms of cortical modulation. Using time-locked recordings of submental surface electromyography, nasal airflow, and thyroid acoustics, three conditions of swallowing were evaluated in 20 adults in a single session and 10 infants in 10 sessions across the first year of life. The three swallowing conditions were selected to represent a continuum of volitional through nonvolitional swallowing control on the basis of a decreasing level of cortical activation. Our primary finding is that, across the lifespan, brainstem control strongly dictates the duration of swallowing apnea and is heavily involved in organizing the integration of swallowing and respiration, even in very early infancy. However, there is evidence that cortical modulation increases across the first 12 months of life to approximate more adult-like patterns of behavior. This modulation influences primarily conditions of volitional swallowing; sleep and naïve swallows appear to not be easily adapted by cortical regulation. Thus, it is attention, not arousal that engages cortical mechanisms.

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