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Infantile Hepatic Hemangioendothelioma
Benign Infantile Hemangioendothelioma of Liver

Presentation

Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. [ncbi.nlm.nih.gov]

Infantile hepatic hemangioendothelioma: clinical presentation and treatment. Turk J Gastroenterol 2007; 18 (3) 182-187 23 Bosemani T, Puttgen KB, Huisman TA, Tekes A. [thieme-connect.com]

Case presentation A five-month-old girl was referred for hepatomegaly, which was incidentally found. [em-consulte.com]

Respiratoric

  • Persistent Cough

    The patient still, however, had a persistent cough, dyspnea, and fever after discharge. [journals.lww.com]

Cardiovascular

  • Systolic Murmur

    There was cardiomegaly, a grade 3/6 ejection systolic murmur in the tricuspid area and a left ventricular S 3. There were fine crackles over both lungs at the basal region. [ncbi.nlm.nih.gov]

    The heart rhythm was normal, with a 2/6 systolic murmur and the second heart sound was enhanced. Abdominal distention was noted. The liver was palpable 4.5 cm below the subcostal margin. The laboratory examinations are summarized in Table 1. [journals.lww.com]

Liver, Gall & Pancreas

  • Liver Mass

    Pathologic examination of these liver masses revealed mesenchymal hamartoma combined with infantile hepatic hemangioendothelioma. [ncbi.nlm.nih.gov]

    A hepatic hemangioma is a liver mass made of widened (dilated) blood vessels. It is not cancerous. A hepatic hemangioma is the most common type of liver mass that is not caused by cancer. It may be a birth defect. [medlineplus.gov]

    Definition A hepatic hemangioma is a liver mass made of widened (dilated) blood vessels. It is not cancerous. [ufhealth.org]

    Liver hemangioma; Hemangioma of the liver; Cavernous hepatic hemangioma; Infantile hemangioendothelioma; Multinodular hepatic hemangiomatosis A hepatic hemangioma is a liver mass made of widened (dilated) blood vessels. It is not cancerous. [leehealth.org]

Urogenital

  • Oliguria

    One month ago, these symptoms were aggravated and complicated by oliguria and fever. The infant was diagnosed with severe pulmonary hypertension, cardiac insufficiency, and bronchitis at the People's Hospital of Lhasa. [journals.lww.com]

Workup

Recent discoveries concerning hemangioma pathogenesis provide both an improved understanding and more optimal approach to workup and management. [pediatrics.aappublications.org]

The serum tumor marker, AFP, is obtained during the workup; more than 90% of patients with HB have elevated AFP levels. [emedicine.medscape.com]

Signs or symptoms suggestive of hepatic hemangiomas that would indicate the need for further workup include a history of poor growth or feeding, tachypnea, cardiac murmur, abdominal distention, or hepatomegaly. [jamanetwork.com]

An appropriate diagnostic workup for suspected vascular birthmarks. Cleve Clin J Med. 2004; 71 :505–510. [ PubMed ] [ Google Scholar ] 21. Sari N, Yalçin B, Akyüz C, Haliloglu M, Büyükpamukçu M. [ncbi.nlm.nih.gov]

X-Ray

  • Atelectasis

    In addition, because of cardiac enlargement and pulmonary artery dilatation, the bronchus and lung tissue were oppressed, and infants were subjected to external pressure resulting in local lung tissue overinflation or atelectasis. [journals.lww.com]

Hypertrophy

  • Biventricular Hypertrophy

    An electrocardiogram showed biventricular hypertrophy. [journals.lww.com]

Treatment

AIM: This study aims to analyze computed tomography (CT) and magnetic resonance (MR) imaging features of infantile hepatic hemangioendotheliomas before and after treatment. [ncbi.nlm.nih.gov]

She was resistant to, first line, steroid treatment but responded well to oral propranolol. The literature on treatment of hepatic hemangioendothelioma is scarce, and this case emphasizes the importance of propranolol treatment in IHH. [jcnonweb.com]

Neonatal hepatic haemangioendothelioma: treatment options and dilemmas. Pediatr Radiol 2009;39:277–281. 23. Touloukian RJ. Hepatic hemangioendothelioma during infancy: pathology, diagnosis and treatment with prednisone. Pediatrics 1970;45:71–76. 24. [synapse.koreamed.org]

The use of propranolol for the treatment of severe cutaneous hemangiomas was initially described by Léauté-Labréze et al. in 2008 [ 8 ]. [em-consulte.com]

Prognosis

All of the IHE cases in our study, except for the aborted fetuses, were associated with a good prognosis. [wjgnet.com]

It is the most common vascular tumour of the liver in infants. [1] General Favourable prognosis. [1] Often can be separated from hepatoblastoma based on clinical features. [2] Microscopic Images IHH - type 1 lesions (nih.gov). [3] IHH - type 2 lesions [librepathology.org]

The overall prognosis is much more favourable than other hepatic malignancies 4. Radical surgical resection or orthoptic liver transplantation are considered the treatments of choice 1,4. [radiopaedia.org]

Hepatic angiosarcoma in a child is extremely rare and associated with a poor prognosis. [cancerjournal.net]

Etiology

The book emphasizes not only the pathology seen in biopsy and surgical material, but also the most pertinent clinical and laboratory findings including epidemiology, etiologic and pathophysiologic concepts, and the differential diagnoses. [books.google.com]

The etiology is unknown, but it has been associated with Beckwith-Weidemann syndrome, familial adenomatosis polypi, and low birth weight. [scienceopen.com]

The etiology of hepatic vascular neoplasms in children is unknown, although the regression of most such tumors within months of the child's birth suggests that the maternal milieu plays some role. [cancerjournal.net]

The etiology of increased serum AFP in IHHE is unclear, but it may be secreted by morphologically normal hepatic cells in response to the tumor 5, 22). [ncbi.nlm.nih.gov]

Epidemiology

W. : Relation between cancer and congenital defects: an epidemiologic evaluation. J. Natl. Cancer Inst., 40: 1079 – 1085, 1968. Google Scholar | Medline 13. Pages, A., Marty, Ch. : Les tumeurs du foie chez l'enfant. Gaz. Med. [journals.sagepub.com]

The book emphasizes not only the pathology seen in biopsy and surgical material, but also the most pertinent clinical and laboratory findings including epidemiology, etiologic and pathophysiologic concepts, and the differential diagnoses. [books.google.com]

[…] novel tyrosine kinase inhibitor of angiogenesis, has shown some benefit in clinical trials and has been approved for HCC in adults by the US Food and Drug Administration (FDA). [28] The overall survival rate remains poor, with a recent Surveillance, Epidemiology [emedicine.medscape.com]

J Clin Res Pediatr Endocrinol 2:126–130 PubMed PubMedCentral CrossRef Google Scholar Chabalko JJ, Fraumeni JF (1975) Blood-vessel neoplasms in children: epidemiological aspects. [link.springer.com]

Pathophysiology

The book emphasizes not only the pathology seen in biopsy and surgical material, but also the most pertinent clinical and laboratory findings including epidemiology, etiologic and pathophysiologic concepts, and the differential diagnoses. [books.google.com]

It also addresses pathophysiology, diagnosis, management, and multidisciplinary team care. No Reference information available - sign in for access. No Citation information available - sign in for access. No Supplementary Data. [ingentaconnect.com]

It brings together the pathophysiology of disease with detailed clinical guidance on diagnosis and management for the full range of childhood cancers, including aspects important in optimal supportive care. [books.google.ro]

The pathophysiology has been attributed to genetic mutations in angiogenesis-related protein expression [2]. [romj.org]

Prevention

New modalities, such as oral propranolol, provide the caregiver with better therapeutic options, which can prevent or minimize medical risk or scarring, but the side effect profile and risk-benefit ratio of such interventions must always be evaluated [pediatrics.aappublications.org]

Understanding the management of TSH levels has prevented this complication. [novanews.org]

Antifibrinolytics help prevent the breakdown of fibrin. Observation Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. [northshore.org]

During infantile hemangioma proliferation and stimulated by angiotensin II (ATII), the mesenchymal stem cells (MSC) produce VEGF, which stimulates the EPCs to grow, and osteoprotegerin (OPG), which prevents the apoptosis of the hemogenic endothelium, [emedicine.medscape.com]

One of the leading causes of preventable blindness in children is stimulus-deprivation amblyopia caused by hemangioma obstruction. [cancer.gov]

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