Characteristics, Natural Course and Treatment of Intramuscular Capillary-type Haemangioma: A Systematic Literature Review.

Intramuscular capillary-type haemangiomas (ICTH) are rare vascular anomalies that can easily be misdiagnosed as other entities. A systematic review was performed of all cases of ICTH in the literature since its first description in 1972. An adjudication committee reviewed cases to include only ICTHs. Among 1,143 reports screened, 43 were included, involving 75 patients. The most frequent differential diagnosis was intramuscular venous malformations. The mean age of patients at diagnosis was 21.2 years. ICTH was mainly described as a gradually increasing mass (81.8%), painless (73.9%), that could occur anywhere in the body but most frequently on the head and neck (44.0%). Magnetic resonance imaging (MRI) was mainly used for diagnosis (69.1%) and displayed specific features. The most frequent treatment was complete surgical removal (73.9%), which could be preceded by embolization, and led to complete remission without recurrence in all but 1 case.

Spinal epidural capillary hemangioma: case report and review of the literature.

BACKGROUND: Almost all of the epidural hemangiomas reported are cavernous hemangiomas. Purely extradural spinal capillary hemangiomas are very rare. Capillary hemangiomas are hamartomatous malformations that result from proliferations of vascular endothelial cells. Only ten cases have been reported in the English literature, treated with surgical excision. CASE DESCRIPTION: A case of a dorsal extradural spinal capillary hemangioma is described. A total surgical removal has been performed after spinal angiography and embolization. Complete surgical removal should always be the goal in these lesions. Embolization did not show to reduce bleeding during the surgical procedure in this case.

[Deforming endonasal mass during pregnancy]. / Deformierende endonasale Raumforderung während der Schwangerschaft.

This article presents the case of a 33-year-old woman who consulted the authors' ENT clinic in the 39th week of pregnancy with recurrent epistaxis. A livid endonasal mass was found on the left side, subtotally displacing the nose and leading to deformation of the external nose. External biopsy provided no indications of malignancy. Postpartum CT of the paranasal sinuses revealed a mass destroying the cartilaginous nasal septum. Endoscopic resection of the finding was performed with preservation of the clinically sound nasal septal cartilage. Histopathological examination revealed a capillary hemangioma, which was classified as granuloma gravidarum due to its occurrence during pregnancy.

Management of Capillary Hemangioma of the Sphenoid Sinus.

Background and objectives: Capillary hemangiomas are rare, benign vascular tumors that mainly affect the skin and soft tissue, with scarce appearance within the nasal cavities and paranasal sinuses. Materials and methods: We present a case report of capillary hemangioma of the sphenoid sinus and a review of the literature in the last ten years. Results: Clinical and endoscopic examination of the nose, radiologic assessment and particular histologic features contribute to the correct diagnosis of capillary hemangioma of the nose and paranasal sinuses. Conclusions: Transnasal endoscopic resection of capillary hemangioma located in the nose and paranasal sinuses is a valuable treatment method with good outcomes.

Dual-energy CT lung perfusion characteristics in pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH): preliminary experience in 63 patients.

BACKGROUND: In the stratification of potential causes of PH, current guidelines recommend performing V/Q lung scintigraphy to screen for CTEPH. The recognition of CTEPH is based on the identification of lung segments or sub-segments without perfusion but preserved ventilation. The presence of mismatched perfusion defects has also been described in a small proportion of idiopathic pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH). Dual-energy CT lung perfusion changes have not been specifically investigated in these two entities. PURPOSE: To compare dual-energy CT (DECT) perfusion characteristics in PAH and PVOD/PCH, with specific interest in PE-type perfusion defects. MATERIALS AND METHODS: Sixty-three patients with idiopathic or heritable PAH (group A; n = 51) and PVOD/PCH (group B; n = 12) were investigated with DECT angiography with reconstruction of morphologic and perfusion images. RESULTS: The number of patients with abnormal perfusion did not differ between group A (35/51; 68.6%) and group B (6/12; 50%) (p = 0.31) nor did the mean number of segments with abnormal perfusion per patient (group A: 17.9 ± 4.9; group B: 18.3 ± 4.1; p = 0.91). The most frequent finding was the presence of patchy defects in group A (15/35; 42.9%) and a variable association of perfusion abnormalities in group B (4/6; 66.7%). The median percentage of segments with PE-type defects per patient was significantly higher in group B than in group A (p = 0.041). Two types of PE-type defects were depicted in 8 patients (group A: 5/51; 9.8%; group B: 3/12; 25%), superimposed on PH-related lung abnormalities (7/8) or normal lung (1/8). The iodine concentration was significantly lower in patients with abnormal perfusion (p < 0.001) but did not differ between groups. CONCLUSION: Perfusion abnormalities did not differ between the two groups at the exception of a higher median percentage of segments with PE-type defects in patients with PVOD/PCH. KEY POINTS: • Patchy perfusion defect was the most frequent pattern in PAH. • A variable association of perfusion abnormalities was seen in PVOD/PCH. • Lobular and PE-type perfusion defects larger than a sub-segment were depicted in both PAH and PVOD/PCH patients.

Fetal cardiac capillary hemangioma resulting in tachyarrhythmia.

We present the case of a fetus with cardiac capillary hemangioma in the right atrial cavity. The tumor showed dramatic growth between the 28th and 32nd week of gestation and resulted in tachyarrhythmia. The patient was born at the 33 weeks of gestation weighing 2430 g via urgent cesarean section because the rapidly growing cardiac tumor caused incessant tachyarrhythmia, pericardial effusion, and fetal circulatory incompetence. Coronary angiography revealed that the right coronary artery drained into the tumor. Due to hemodynamic deterioration, the patient underwent subtotal resection of the tumor on the 2nd day after birth. Histopathological examination revealed an undifferentiated capillary hemangioma. The patient was discharged at the age of 86 days, as the tachyarrhythmia and hemodynamic incompetence had subsided; however, bradycardia and intermittent atrioventricular conduction disturbance gradually developed. Capillary hemangioma, a rare primary cardiac space-occupying tumor in children, can invade the conduction system.

Jugular foramen capillary hemangioma masquerading as a paraganglioma.

Capillary hemangiomas are benign vascular lesions that are common in head and neck, but hemangiomas of jugular foramen and temporal bone are rare with only a few cases reported in the literature. We present a case report of this rare disease entity highlighting the subtle radiographic nuances that can benefit clinicians when encountered with similar unusual clinical scenario. Although radiographic features of capillary hemangioma can be distinctive, they are not specific. In this case, the lack of significant involvement of the jugular bulb and the absence of the typical pattern of osseous erosion of the jugular carotid spine led to the alternative diagnosis of a rare capillary hemangioma of the jugular foramen.

Conjunctival granulomatous capillary haemangioma in children: case report and review of the literature.

BACKGROUND: Granulomatous capillary haemangioma refers to a benign vascular tumour that commonly affects the skin, with occasional involvement of the mucosa. Reports of conjunctival granulomatous capillary haemangioma in children are uncommon. In this article, we present a case of granulomatous capillary haemangioma and a brief review of the relevant literature. CASE PRESENTATION: An 11-year-old girl presented with a conjunctival mass. An excision of the entire lesion was performed. Histopathology showed a granulomatous capillary haemangioma. CONCLUSIONS: The clinical manifestations of granulomatous capillary haemangioma lack specificity; pathological characteristics and immunohistochemistry are the main basis for diagnosis. We retrospectively analysed the diagnosis and treatment of a patient with conjunctival granulomatous capillary haemangioma to deepen the understanding and facilitate the diagnosis and treatment of this disease.

Value of Doppler ultrasound scans in deciding whether to treat infantile haemangioma with oral propranolol.

BACKGROUND: Oral propranolol (Pr) must be administered until the end of the proliferation phase of infantile haemangioma (IH). This phase may be difficult to assess, particularly where a deep component is involved. Doppler ultrasound scans (DUS), which identify vascular activity (VA), could assist the clinician in making the correct therapeutic decision (CTD). PATIENTS AND METHODS: All children with IH treated with Pr for at least 3 months and up to the age of 9 months, and who also underwent DUS, were enrolled in this retrospective, single-centre, observational study. The quality of DUS as a binary diagnostic test for IH proliferation was assessed, together with its value in deciding whether to discontinue Pr (at the end of the presumed proliferation phase) or resume this drug (in the case of suspected recurrence). RESULTS: A total of 29 children were enrolled and 45 DUS were performed. Thirty-nine (87%) DUS were of high quality (80% sensitivity, 95% specificity) and made a major, moderate, or minimal contribution to the CTD in respectively 20%, 60% and 7% of cases. DISCUSSION: DUS proved to be a high-value tool. They were essential in some cases of IH, mainly periocular and localised forms, and those involving deep components, in which the question of discontinuing Pr arose (age>1 year) and where clinical examination had not been sufficient to make the CTD. Furthermore, in the vast majority of cases, they provide a helpful examination and complement clinical findings in terms of patient follow-up and reaching a CTD. CONCLUSION: DUS is an effective and complementary tool to clinical investigation.

[Solitary pulmonary capillary hemangioma of adult: a series of 10 cases].

Objective: To examine the clinical features, diagnostic and therapeutic strategy of solitary pulmonary capillary hemangioma (SPCH). Methods: The data of 10 SPCH cases who underwent surgical operations from June 2017 to June 2020 in Shanghai Pulmonary Hospital, Tongji University were retrospectively reviewed. There were 4 males and 6 females, aged (49.8±13.6) years (range: 26 to 66 years). The clinical manifestations, imaging manifestations, treatment and pathological diagnosis were analyzed. Results: All patients were asymptomatic, and all nodules were detected by CT. The size of nodule was (14.9±5.8) mm (range: 8 to 30 mm). Seven of 10 cases showed the mixed ground-glass nodule appearance and 2 cases showed solid nodule and 1 case showed cystic solid nodule appearance in CT findings. The growth speed was very slow. The follow-up time was 4.5(21.5) months before surgery. Histologically, SPCH manifested as a solitary lesion composed of densely proliferating and dilated capillaries without cytologic atypia within the alveolar septa. Immunohistochemically, capillaries of SPCH uniformly expressed endothelial markers, such as CD31, CD34. The patients were followed up for 15.0(22.0) months after surgery and all recovered well. Conclusions: SPCH is probably an unrecognized benign capillary proliferative disease. SPCH lesions mimic early lung cancer on CT as mixed ground-glass nodule, may be misdiagnosed as other nonspecific benign lesions. With careful histologic examination, SPCH can be successfully diagnosed using CD34 or CD31 immunohistochemistry staining.

Progressively increasing density of the solid center of a ground-glass nodule in a solitary pulmonary capillary hemangioma: A case report.

Solitary pulmonary capillary hemangiomas (SPCHs) are recently recognized, rare benign lesions that form solitary nodules owing to capillary proliferation. These lesions are usually detected incidentally as small ground-glass nodules (GGNs) on computed tomography (CT), and progressively enlarge over time. The radiological distinction from peripheral lung cancers is particularly challenging. However, to date, there have been no reports on progressive changes in the central density of SPCH on CT. An asymptomatic 49-year-old man was referred to our hospital for an abnormal shadow that was detected on chest CT during medical check-up. He was subsequently followed-up with chest CT. The nodule increased in size, and the central area became progressively denser. He underwent surgery 5 years and 10 months after the first visit owing to suspicion of lung cancer. Despite the collapse of the surgical specimen by artifacts, histopathological examination revealed a diagnosis of SPCH; collagenous fibers were found in the walls of the intralesional capillaries. The patient is presently alive without any recurrence, 6 months after the operation. In this case, the SPCH demonstrated a GGN with progressively increasing density of the central solid area on the CT. This remarkable feature made the preoperative distinction from lung cancer particularly difficult.

Assessment of Oral Propranolol Administration for Infantile Hemangioma in Oral and Maxillofacial Region Aided by Ultrasonography.

BACKGROUND: Propranolol has become the first-line therapy for the treatment of complicated infantile hemangioma. However, there are still many queries regarding the hemangioma volume in relation to drug's dose and duration. OBJECTIVE: The aim of this study was to evaluate the therapeutic effect of oral propranolol for treating infantile hemangiomas in the oral and maxillofacial region aided by gray scale ultrasonography (GSU). MATERIALS AND METHODS: Twelve patients with infantile hemangioma, age ranged between 2 and 11 months, have been treated with oral propranolol for 6 months' period. They received a dose of 1 mg/kg per body weight per day, increased after 1 week to 2 mg/ kg per body weight per day maintenance for 24 weeks. The changes in tumor sizes were evaluated by ultrasonography (GSU) using 4-points scale system: excellent, good, fair, poor) RESULTS:: All infants less than 6 months of age showed more hemangiomas regression in size in comparison with of those aged >6 months (P value 0.042) as a rapid response. After the 24 week; 5 patients had excellent results, 4 patients had good results, 2 patients had fair results, and only 1 patient had poor results. None of the treated infants showed rebound phenomena after cessation of treatment. CONCLUSION: Oral propranolol at dose of 2 mg/kg/day in 2 divided doses for 24 weeks aided by GSU is shown to be a safe and effective treatment of infantile hemangioma during the proliferative phase.

Efficacy of propranolol treatment in infantile hepatic haemangioma.

AIM: The aim of this study was to assess the efficacy of propranolol treatment in multifocal and diffuse infantile hepatic haemangioma (IHH). METHODS: A retrospective study of symptomatic or potentially symptomatic IHH was performed in our hospital between 2011 and 2016. RESULTS: Thirteen patients were identified: 2 patients had diffuse lesions, and 11 patients had multifocal lesions, including 2 patients who had combined lesions that shared features of both multifocal and diffuse lesion patterns. Eleven (84.6%) patients had cutaneous infantile haemangioma. Hepatomegaly was the predominant clinical presentation. Hypothyroidism was identified in three patients, including one patient who had documented congestive heart failure (CHF). The median age at diagnosis and the median duration of treatment were 2.0 months (range 1.2-26.0) and 24.0 months (range 4.0-30.0). The median duration of follow-up was 30.0 months (range 3.0-48.0). For patients with hypothyroidism, the thyroid hormone level was normal after 4 weeks of propranolol and levothyroxine treatment. All but one patient responded well to propranolol treatment. The patient who failed to respond to treatment died of CHF and abdominal compartment syndrome induced by hepatomegaly. No significant side effects of propranolol were observed during follow-up. CONCLUSIONS: Most multifocal and diffuse IHH respond well to propranolol. However, progressive cases may be fatal despite aggressive treatments. Our data suggest that propranolol may be considered the first-line treatment for multifocal and diffuse IHH due to its efficacy.

Orbital infantile haemangioma: radiological features and treatment - case series and literature review.

AIM: The aim of this study is to report a retrospective case series on orbital infantile haemangiomas (OIH). Radiological features and treatment with oral propranolol (OP) are illustrated along with an updated literature review. METHODS: A retrospective chart review of six children, diagnosed with OIH from November 2015 to October 2016, was carried out. Only children with deep documented orbital involvement were included. All patients underwent magnetic resonance imaging (MRI) under general anaesthesia. OP was administered to the infants according to the Nottingham Children's Hospital guideline. As per the guideline, a preliminary paediatric assessment was performed and a 1 mg/kg test dose was administered, followed by definitive treatment at a dosage of 2 mg/kg in three divided doses. RESULTS: Average age at presentation was within the first 3 weeks of life. T1 hypointensity, T2 hyperintensity, avid enhancement with contrast, and the presence of flow-voids appear a fixed pattern of OIH on MRI. Response to treatment was noticed within 4 weeks in all children, and two of them (33.3%) responded within the first 7 days. In two children (33.3%), the haemangioma became clinically undetectable by the seventh month of treatment, while the other four (66.6%) experienced an almost complete regression of the OIH by the last follow-up. No complications were found. CONCLUSIONS: Our series strengthens the understanding that MRI is the preferred imaging modality in the investigation of OIH, showing vascular features, detailed orbital extension, and possible associated malformations. OP is the treatment of choice for OIH, and our study confirms its safety and effectiveness.

Giant congenital right atrial epithelioid-capillary hemangioma with prolonged QT interval: Case report and practical surgical treatment strategy for primary cardiac tumors in children based on 25-year review of 299 cases.

Cardiac hemangioma is very rare and accounts for 2%-3% of the primary cardiac tumors. Cardiac epitheloid-capillary hemangioma has not been reported in the pediatric population so far. We report the fatal outcome of a preterm neonate with a huge congenital right atrial epitheloid-capillary hemangioma and elevated serum alpha-fetoprotein, associated with prolonged QT interval. We describe the echocardiographic, computed tomographic (CT) imaging, microscopic and immunohistochemical features of the tumor. Complete resection of the tumor was done at operation necessitating extensive reconstruction of atrial walls. Intramural infiltration of this tumor into the surrounding myocardial walls is a challenging characteristic of cardiac hemangioma. The temptation to complete resection should be avoided in the setting of extensive intramural infiltration and entrapment of the tumoral cells into atrial walls, particularly in a preterm neonate. None of the current classifications for hemangioma was inclusive of our case. Based on a concise literature review of nine published classification systems from 1996 to 2017 , we discuss the shortcomings of the current classifications for hemangioma. We also performed a 25-year-review of 299 cases of primary cardiac tumors in neonates and children, from 1993 to May 2018. We suggest a stepwise surgical treatment strategy according to the characteristics of the patient and of the tumor, based on this review. The stepwise strategy includes watchful observation, partial resection, complete resection and cardiac transplantation.

Cutaneous metastases of infantile choriocarcinoma can mimic infantile hemangioma both clinically and radiographically.

Infantile metastatic choriocarcinoma is a rare tumor of placental origin that can be observed with or without maternal metastases. A single cutaneous mass may be the only clinically observed sign. Reports of imaging findings are scarce given the extreme rarity of the tumor, and the disease can be rapidly fatal in the absence of prompt diagnosis. In order to promote timely consideration for this malignancy as a differential consideration in the approach to skin lesions in infancy, we present the findings of this neoplasm in an infant. While imaging and clinical characteristics similar to infantile hemangioma were demonstrated at presentation, biopsy and further radiologic investigation revealed multifocal metastatic choriocarcinoma. This case also highlights important differences between these entities, as the T2 hyperintensity and contrast enhancement observed with this choriocarcinoma were predominantly peripheral in location.

Intralesional Bleomycin Injection for Propranolol-Resistant Hemangiomas.

Propranolol has been the first-line treatment for alarming hemangiomas. However, some hemangiomas are propranolol-resistant. The authors reported 1 propranolol-resistant hemangioma which was treated with intralesional bleomycin injections. Sixteen months after 3 injections, the lesion still remained stable. Its potential mechanism was clarified by ultrasonic monitoring. Intralesional bleomycin injection can be considered an ideal option in treating propranolol-resistant hemangiomas.

Purely Spinal Epidural Capillary Hemangiomas.

Capillary hemangiomas (CAs) are benign endothelial cell neoplasms that are often encountered superficially in the soft tissues of the head and neck region. Most of the reported purely spinal epidural hemangiomas have been of cavernous type, and the occurrence of purely spinal epidural CA is exceedingly rare, and there are only 12 reported cases of spinal epidural CAs in the English literature. Herein, the authors report the 13th case of purely spinal epidural CAs, and the clinical characteristics, histopathological features, and treatment were also investigated.

[Rare presentations of infantile hemangiomas: 4 cases]. / Présentations rares d'hémangiomes infantiles : 4 cas.

BACKGROUND: Infantile hemangioma (IH) is a common benign vascular tumor in children. In most cases, diagnosis is based entirely on clinical examination. When the diagnosis is uncertain, the first-line complementary examination is Doppler ultrasound. We report 4 cases of atypical infantile hemangiomas with delayed diagnosis and non-contributory imaging. PATIENTS AND METHODS: One child had congenital purple papules and nodules on the back of the foot, the second had inaugural ulceration of the buttocks, and the last two presented telangiectasia, either isolated or on an erythematous macula. In two cases, ultrasound showed no vascular lesions, and in the other two cases, the absence of hyperemia did not allow a diagnosis of IH to be made. For one patient, diagnosis was made on the basis of cutaneous biopsy, and for the other three, on the basis of clinical course. DISCUSSION: We report 4 rare forms of infantile hemangioma resulting in initial diagnostic error. The atypical nature of some IHs may direct the clinician and the radiologist toward other diagnoses that in some cases have no vascular contingent. It is important for the dermatologist to be aware of these rare forms of IH in order to reduce the time to diagnosis and allow early initiation of appropriate management.