IL13RA2 promotes progression of infantile haemangioma by activating glycolysis and the Wnt/ß-catenin signaling pathway.

Background: Interleukin 13 receptor subunit alpha 2 (IL13RA2) plays an essential role in the progression of many cancers. However, the role of IL13RA2 in infantile haemangioma (IH) is still unknown. Materials and Methods: IL13RA2 expression in IH tissues was analyzed using western blot, qRT-PCR, and immunofluorescence. The role of IL13RA2 in haemangioma-derived endothelial cells (HemECs) was determined following knockdown or overexpression of IL13RA2 using CCK-8, colony formation, apoptosis, wound healing, tubule formation, Transwell, and western blot. Results: IL13RA2 expression was upregulated in IH tissues. IL13RA2 overexpression promoted proliferation, migration, and invasion of HemECs and induced glycolysis, which was confirmed with a glycolysis inhibitor. Specifically, IL13RA2 interacted with ß-catenin and activated the Wnt/ß-catenin pathway in HemECs, which were involved in the above-mentioned effects of IL13RA2. Conclusions: These findings revealed that targeting IL13RA2 is a potential therapeutic approach for IH.

Adrenal gland haemangioma, a rare entity difficult to differentiate from malignancy.

Adrenal haemangioma is a rare benign vascular lesion, which is usually asymptomatic and it is typically discovered incidentally on radiographic imaging. Differential diagnosis from other benign or malignant adrenal neoplasms may be challenging, and in many cases, the diagnosis is only possible after surgical resection. We present a case of a 39-year-old female with abdominal pain in the upper right quadrant, who was referred to our hospital after incidentally discovering a mass above the right kidney on abdominal ultrasonography. MRI revealed an adrenal mass, with features not indicative of adenoma and suggestive of adrenal haemangioma, without ruling out other possible diagnoses such us phaeochromocytoma and adrenal cortical carcinoma. Biochemical tests did not reveal any endocrine dysfunction. The patient underwent adrenalectomy, and histopathological analysis confirmed a venous haemangioma. Adrenal gland haemangioma is an unusual vascular lesion, typically diagnosed incidentally during abdominal imaging. Certain radiologic features may raise suspicion for malignancy, making it difficult to distinguish them from a primary adrenal cortical carcinoma. They may also grow large, compressing surrounding structures and causing abdominal pain, or may rupture, leading to retroperitoneal haemorrhage. For these reasons, some authors recommend excision of all suspected adrenal haemangiomas, and in many cases, the final diagnosis is made only after surgical removal.

A giant lobular capillary haemangioma of the caruncle.

Key Clinical Message: Caruncle lesions are uncommon, though it may be involved by a wide variety of lesions including tumors and cysts of the conjunctiva, skin, or lacrimal gland. Lobular capillary haemangioma of the caruncle is a rare occurrence but may mimic some primary or secondary malignant neoplasms. Excision and histopathological examination confirm the diagnosis. Abstract: The caruncle lesions are uncommon. Lobular capillary haemangioma involving caruncle is a rare occurrence. We present a case of large lobular capillary haemangioma of caruncle in a 10-year-old boy, was treated successfully by surgical excision.

Facial Segmental Infantile Haemangioma: A Cutaneous Mosaicism Quandary.

Mosaicism has long been considered the underlying mechanism of segmental infantile hemangiomas (SIH). This was a prospective pilot case-control study conducted with the objective to quantify the percentage overlap of silhouettes of facial SIH with those of Blaschko lines (the most well studied archetypical pattern of mosaicism on face) as compared to other mosaic disorders on face. Lesional silhouettes of 8 patients with SIH (Group A) and 6 patients with other facial dermatosis known to have blaschkoidal distribution (Group B), were overlapped on a standardized template with Blaschkoidal lines on the frontal view of face. The alignment was done via the auto align tool of Photoshop and the percentage of overlap was calculated with an online image comparison software (IMGonline.com.ua). There was a significant difference in mean overlap in Group A (72.92 ± 15.6 %) as compared to Group B (90.1 ± 4.3%; P=0.018). Hence, we concluded that facial SIH do not follow lines of Blaschko.

Discontinuous peripheral enhancement of focal liver lesions on CT and MRI: outside the box of typical cavernous hemangioma.

The discontinuous peripheral enhancement is a pattern of enhancement usually attributed to typical cavernous hemangioma, that is the most common benign solid lesion of the liver. The discontinuous peripheral enhancement, however, may be encountered in many other benign and malignant focal liver lesions as an atypical presentation or evolution, and hemangiomas with discontinuous peripheral hyperenhancement on hepatic arterial phase may not always have the typical post-contrast pattern on portal venous and delayed phases. Therefore, abdominal radiologists may be challenged in their practice by lesions with discontinuous peripheral enhancement. This pictorial essay aims to review the spectrum of benign and malignant focal liver lesions that may show discontinuous peripheral enhancement. A particular point of interest is the diagnostic tree pathway that may guide the radiologists in the differential diagnosis.

Cavernous Haemangioma of the Submandibular Triangle Eluding the Gland - A Case Report.

Rationale: Haemangiomas are benign vascular malformations. They are common neoplasms of infancy but seldom manifest at birth. Although common in the head & neck region, they are rare in the submandibular region. Despite being benign, surgical treatment is necessary if it causes significant functional or aesthetic compromise. Patient Concerns: The patient's primary complaint was swelling in the submandibular region. Diagnosis: Initial examination and imaging studies were suggestive of sialolithiasis due to the presence of multiple calcifications. Ultrasonographic and magnetic resonance imaging were suggestive of haemangioma with multiple phleboliths. Treatment: Excisional biopsy was done without compromising the submandibular gland. A histopathological examination was done post-operatively to confirm the diagnosis. Outcomes: Post-operative recovery was uneventful and 6-month follow-up showed no recurrence. Take-away Lesson: This case highlights the importance of considering haemangioma in the differential diagnosis of submandibular region masses and emphasises the preservation of the submandibular gland when the extent of the lesion permits.

Revision total en bloc spondylectomy for a recurrent aggressive vertebral haemangioma of the thoracic spine causing progressive thoracic myelopathy with segmental kyphosis.

Vertebral haemangiomas are common amongst primary benign tumours of the spine, usually asymptomatic and discovered incidentally, whereby symptomatic cases are rare. Aggressive vertebral haemangiomas, occurring even less frequently, are characterized by their expansion, resulting in pain and neural compression symptoms. Depending on presentation and severity, several treatment options exist, and when causing progressive neurological deficit, surgical decompression and resection is warranted. Despite local recurrence being rare, regular follow-ups to detect recurrence are advised. In case of recurrent aggressive vertebral haemangiomas, however, subsequent treatment strategy usually depends on a case-by-case consideration, whereby reports in the literature are lacking. We describe a case of a recurrent aggressive vertebral haemangiomas of the thoracic spine in a 20-year-old male causing progressive thoracic myelopathy with segmental kyphosis, treated with a revision total en bloc spondylectomy and a multilevel fixation with vertebral column reconstruction using radiolucent instrumentation.

Beta-blockers for the treatment of infantile haemangiomas in premature infants.

Beta-blockers have been established as a treatment of infantile haemangiomas (IH) since its serendipitous discovery for use in IH in 2008. However, data on the safety of these beta-blockers for use in IH in preterm infants are scarce. A retrospective study was performed to review the safety of oral propranolol and topical timolol in the treatment of IH in a cohort of preterm infants treated at our tertiary paediatric hospital. It was observed that there was an increased risk of adverse events amongst the preterm infants with chronic lung disease, retinopathy of prematurity and gastroesophageal reflux, when treated with oral propranolol.

Diagnostic approach to hepatic vascular lesions: a paediatric perspective.

The pathological evaluation of hepatic vascular lesions in children requires special consideration. Inconsistent terminology, rarity of pathology specimens and overlapping pathological features between various lesions may pose a serious diagnostic challenge. In this review, we highlight the importance of using the International Society for the Study of Vascular Anomalies (ISSVA) classification scheme to characterise these lesions. Selected entities are discussed, including hepatic vascular tumours exclusively seen in the paediatric age group, hepatic infantile haemangioma and hepatic congenital haemangioma. Vascular malformations, with emphasis on their syndromic associations (venous malformation in blue rubber bleb naevus syndrome) and complications (hepatocellular nodules in Abernethy malformation) are also covered.

Primary Renal Angiosarcoma: Rare Tumour with Lethal Outcomes.

Introduction: Renal haemangioma is a benign tumour, and due to its characteristics, it must be distinguished from malignant diseases. We present a clinical case of primary renal angiosarcoma initially mistaken for haemangioma due to their similarity. Case report: A 58-year-old man was admitted to the hospital with suspicion of pulmonary embolism. The patient complained of pain on the left side. An ultrasound and CT scan of the abdomen showed a tumour mass ~20.5 × 17.2 × 15.4 cm in size in the projection of the left kidney. On CT images, there were data for clear cell renal clear cell carcinoma (ccRCC). A left nephrectomy was performed. However, histological examination revealed renal haemangioma. Three months later, the patient presented to the hospital with abdominal and lumbar pain. A CT scan showed multiple small hypoechoic foci up to 2 cm in size in the liver, lungs, and intra-abdominally, with the most data for carcinosis. Histological re-verification of the left kidney showed a renal vascular tumour with pronounced signs of infarction and necrosis with the majority of the evidence supporting angiosarcoma. Despite treatment, the patient's outcome was fatal. Conclusions: Based on the clinical presentation, radiological images and histological examination data, the tumour was initially misdiagnosed as kidney haemangioma. Due to the rarity of this tumour, there are no established treatment protocols or clinical guidelines for managing primary kidney angiosarcoma.

Surgical management of a 25-cm giant hepatic haemangioma: First case report from Bangladesh with literature review.

INTRODUCTION AND IMPORTANCE: Giant hepatic haemangioma (GHH) is defined as a hepatic haemangioma (HH) of >10 cm in diameter. Its association with thrombocytopenia and consumption coagulopathy is quite rare. CASE PRESENTATION: Here, we present a case of a 39-year-old man with a rapidly enlarging 25-cm GHH arising from the entire left hemiliver. Laboratory findings suggested pancytopenia but normal liver and renal functions. He was diagnosed with Kasabach-Merritt syndrome (KMS). After three units of aphaeretic platelet transfusion, the patient underwent left hepatectomy. Postoperative recovery was uneventful, and his regular follow-up revealed no recurrence even after two years. CLINICAL DISCUSSION: HH predominantly affects females, but males can also be affected, as seen in this case. With observation, it can grow over time, particularly in patients under 50 years of age. Surgical management should be considered when HH causes symptoms or is larger than 10 cm. The evolving understanding of GHH and the critical role of surgery are important, particularly when they complicate haematological or coagulation profiles and lead to thrombocytopenia. CONCLUSION: Our case report highlights the significance of surgical intervention in GHH, and a disease-free outcome can be expected for patients with this condition in the future. To our knowledge, this is the first such case report from Bangladesh.

Association of Attention Deficit Hyperactivity Disorder with the Use of Oral Propranolol in Infantile Haemangiomas: An Ambispective Comparative Study.

Previous studies have raised concerns about the effects of oral propranolol on the central nervous system in infants, the exact measure and mechanism and the long-term follow-up of which is less well studied. This was an ambispective comparative study of children with infantile haemangioma (IH) followed by a repeat visit 4-10 years after completion of propranolol therapy. Parents were asked about psychologic functioning along with an initial screening examination. All patients were evaluated by a paediatric psychiatrist. After evaluation by the Strength and Difficulties Questionnaire, and subsequently by the paediatric psychiatrist, 2 of 12 patients (16.67%) showed features of attention deficit hyperactivity disorder in comparison to 0 of 40 subjects in the control group (0.0498; α = 0.05). These results indicate an increased risk of neuropsychiatric illnesses such as attention deficit hyperactivity disorder (ADHD) in patients given propranolol for IH, as supporting evidence to previous claims.

Utilization of harmonic scalpel for the bloodless surgical management of a vascular lesion of the tongue and review of the literature.

A constant search for methods to limit blood loss, especially the vascular lesions of the tongue, has led to the acceptance of ultrasonic harmonic scalpels.The harmonic scalpel's reliability exists in its ability for lasting hemostasis with minimal heat dispersion to the surrounding structures.Surgical removal of the vascular pathology in a restricted area of the oral cavity and oropharynx dictates a bloodless surgical field, further allowing increased visibility of the lesion and the surrounding anatomical structures, subsequently narrowing the surgical time frame.We report a rare case of haemangioma of the lateral border of the tongue managed with complete resection of the tumor achieved using the harmonic scalpel with reduced intraoperative blood loss, increased visibility, evidently reduced post-operative necrosis and highly preserved tissue for the histopathological examination.

An imaging and diagnostic conundrum-the adrenal haemangioma.

The adrenal haemangioma, a rare benign vascular tumour, is increasingly detected through abdominal imaging. Just over 70 surgical cases have been reported since 1955. Their potential large size and overlapping imaging features with adrenocortical carcinoma poses a diagnostic challenge. Adrenalectomy is often needed for a definitive diagnosis due to inconclusive imaging. We report the case of a 61-year-old female presenting with an incidental finding of a right-sided 9.5-cm adrenal mass on imaging. Due to the risk of adrenocortical carcinoma with inconclusive imaging findings, an open right adrenalectomy was performed. The patient was discharged after 6 days with no complications. Post-surgical histopathology confirmed a diagnosis of adrenal haemangioma with a secondary adrenal pseudocyst. The presence of an adrenal incidentaloma with discordant radiological features proves to be a diagnostic conundrum. Therefore, in the setting of contradictory radiology and concerning mass size, we recommend adrenalectomy for definitive diagnosis of an adrenal haemangioma.

Percutaneous Vertebroplasty as the Treatment of Choice for Multiple Adjacent Lumbar Atypical Haemangiomas: A Case Report.

Atypical vertebral haemangiomas (VHs) are uncommon lesions that cause lumbar pain and motor symptoms. Current management mainly relies on radiotherapy, surgical spine decompression, or percutaneous techniques. We describe a unique case of a patient with two adjacent lumbar VHs and an underlying lumbar fracture which was treated only by percutaneous vertebroplasty (PV). The non-invasive technique relieved the patient's pain and did not affect column stability. PV may be considered an amenable pain-relieving treatment for adjacent atypical VHs in selected patients.

Epicardial cavernous haemangioma; A case report of a unique incidental finding.

Background: Primary cardiac tumours are rare, accounting for only 0.002-0.03% at autopsy. Cardiac haemangiomas are benign vascular tumours and constitute for 0.28% of all primary cardiac tumours. Cavernous haemangiomas, capillary haemangiomas, and arteriovenous haemangiomas are three distinct types. Cardiac haemangiomas are often misdiagnosed as myxomas and must be differentiated from malignant angiosarcomas. Case summary: We present a 44-year-old Mediterranean male patient with a cavernous haemangioma in the inferior vena cava and right atrium, detected on transthoracic echocardiography. The patient experienced palpitations and dyspnoea on exertion. Computed tomography (CT) angiography revealed a 7.5 × 6 × 5 cm mass suspected to be perfused by the distal right coronary artery. A watch-and-wait approach was suggested, leading to a cardiac magnetic resonance imaging (MRI) with contrast 6 months later. T1 mapping exhibited a prolonged relaxation time and isointensity to the myocardium. T2 mapping revealed a homogenous hyperintense mass with heterogenous late enhancement. Surgical excision was performed using a bicaval cannulation technique on cardiopulmonary bypass. Intraoperatively, no connection to the coronaries was noted. At 1 year follow-up, the patient reported restored physical resilience, with no evidence of tumour recurrence. Discussion: Clinical symptoms of cardiac cavernous haemangiomas are unspecific and become evident once the tumour grows. To investigate the nature and vascular involvement of the tumour, a contrast-enhanced CT angiography or MRI can be performed. Cardiac haemangiomas are often misdiagnosed and must be differentiated from malignant angiosarcomas. Clear guidelines for the treatment of cardiac haemangiomas in adult patients are lacking. Primary cardiac tumours require thorough investigation, and surgical intervention should be tailored to the individual's case.

Paediatric hepatic angiosarcoma with consumptive hypothyroidism-an important diagnostic pitfall to avoid during evaluation of hepatic vascular tumours.

Hepatic angiosarcoma is an extremely rare primary malignant vascular tumour in children with very poor prognosis. Radiological diagnosis of hepatic angiosarcoma is challenging due to overlapping imaging features with other benign vascular hepatic tumours, particularly infantile hepatic haemangioma. Consumptive hypothyroidism is a condition that is almost exclusively associated with infantile hepatic haemangioma and has never been reported in angiosarcoma. We present a case of hepatic angiosarcoma in a 20-month-old girl, associated with consumptive hypothyroidism and, as a result, initially misdiagnosed as infantile hepatic haemangioma. Radiologists should be aware that consumptive hypothyroidism is not a reliable feature to use in excluding paediatric hepatic angiosarcoma. Biopsy should be performed in patients older than 1 year of age or with atypical imaging features.

Multidisciplinary management and surgical resection of a rare posterior mediastinal haemangioma.

Mediastinal haemangiomas pose diagnostic and therapeutic challenges owing to their rarity and complex anatomy. A 36-year-old man, with a history of smoking and drinking, presented with a posterior mediastinal mass with back pain. Initial investigations suggested a lymphangioma. However, owing to persistent symptoms and complex pathology, we performed surgical intervention involving open resection of the tumour, which was closely associated with the descending aorta and extended into the right posterior mediastinum. The surgical approach was influenced by the proximity of the tumour to vital structures, necessitating an open procedure. Postoperative complications included chylothorax, managed with a fat-free diet. The final pathological diagnosis was consistent with a benign vascular tumour with a low proliferative rate. Two months post-surgery, computed tomography revealed no complications, and the patient's pain had decreased. A multidisciplinary approach and surgical intervention played important roles in the diagnosis and treatment of this posterior mediastinal haemangioma.

Calvarial hemangiomas: Series of 6 cases and review of literature.

Calvarial haemangiomas are benign, vascular tumours of the skull involving parietal and frontal bones. Mostly these lesions remain asymptomatic, and present with cosmetic deformity, headache, uncommon neurological symptoms and reported as case reports and case series. The radiological appearance can range from sessile growing intradiploically to globular and the lesions may extend outwards or inwards after eroding the outer and inner tables of the skull. "Sunburst appearance" and "Wagon-wheel sign" are classical radiological findings but the lesions may present simply as a lytic expansile or even sclerotic calvarial mass. Because of varied clinical presentation and atypical radiological characteristics, the final diagnosis can be clinched by histology only. In selected cases where these lesions are not cosmetically acceptable, en bloc resection with tumour free margins followed by cranioplasty is the treatment of choice. Most reports of calvarial haemangiomas in literature are in the form of case reports.

Benign paediatric liver tumours: The radiological maze demystified.

The trajectory from the clinical identification of a benign liver mass to establishing a precise diagnosis is perplexing and arduous. Related contributory factors are that such lesions are encountered infrequently and that there is a nonavailability of dedicated paediatric radiologists in the developing world. The objective of this study was to review the spectrum of benign liver lesions in children and their typical imaging features. Cross-sectional imaging of all paediatric patients (< 18 years) with liver lesions (single and multiple) performed in the institute from 01 January 2018 to 01 January 2019 as well as those acquired at outside institutions and referred to the institute for management was included. Ultrasound was done as the first line of investigation in all the cases with suspicious liver masses and retrospectively performed in referral cases in whom CT or MRI was already done. Images were analysed by two senior radiologists. Most of the cases were diagnosed based on clinical, biochemical and imaging findings, and biopsy was only performed in equivocal cases. Most of the benign liver lesions in the paediatric age group were hepatic haemangioma and mesenchymal hamartomas. A simplified clinical-radiologic paradigm should be established for benign liver lesions in children to assist in reaching the correct diagnosis. Contribution: The article demonstrates the salient radiological findings of various benign liver lesions in the paediatric age group and the role of demographic, clinical and biochemical findings, which plays a substantial role in the diagnosis and avoids unnecessary biopsies.