Pulmonary Infantile Hemangioma: Clinical and Histopathological Review of Eight Cases

Introduction Infantile hemangioma (IH) is the most common benign vascular tumor of childhood. It typically appears as a single cutaneous mass in the head, neck, and trunk area. IH that does not arise in the skin most commonly presents in the liver. The lesion emerges shortly after birth, rapidly enlarges within the first six months of life, and then spontaneously involutes by 5-10 years of age. Risk factors associated with IH complications include lesional size, location, and growth characteristics. Pulmonary IH is rare with limited reports of clinical presentations and outcomes. Methods An IRB-approved, retrospective review of pediatric patients with a diagnosis of pulmonary IH was conducted. Cases were identified within the Department of Pathology at Boston Children's Hospital from surgical or autoptic specimens evaluated between 1918 and 2021. Analysis of histopathological slides confirmed pulmonary IH in eight infants. We describe the diagnostic workup, radiological, and histopathological findings of these eight patients. Results All patients presented with symptoms of respiratory distress, including tachypnea, subcostal retractions, and hypoxia. The median age at initial symptoms was 1.5 months (range, birth to 12 months). Five patients had a single pulmonary hemangioma ranging in size from 0.2 to 8.0 cm; three patients had multiple pulmonary hemangiomas. Four patients had co-occurrence of multifocal hepatic IH. The median age at histologic diagnosis of pulmonary IH was 6.5 months (range, 5 weeks to 16 months). Glucose transporter-1 (GLUT-1) immunostaining was positive in seven cases. Chest radiography demonstrated nonhomogeneous, mass-like consolidative opacities or rounded nodules. Treatment was primarily supportive. Three patients received medical therapy; two were treated with interferon, and one received propranolol. The infant treated with propranolol responded well with decreased lesional size and resolution of respiratory symptoms. Half of the patients in the cohort died; causes of death included cardiac failure from hepatic involvement, sepsis, hemorrhage, and liver failure. Conclusions Although IH is a common childhood tumor, IH of the lung is rare. Most (80%) IHs are focal, with hepatic co-involvement in 50-60% cases (Zavras et al. Eur J Pediatr, 2020; Hinen et al. Front Pediatr, 2020). Given that all patients who had concomitant hepatic hemangiomas died-albeit before the widespread availability of medical therapy-the presence of hepatic hemangiomas may confer high risk of complications. Patients with hepatic hemangiomas presented with pulmonary symptoms. Biopsy may be necessary to confirm the diagnosis of pulmonary IH and inform treatment. In this series, only treatment with propranolol or surgical resection was curative. Pulmonary IH should be considered in the differential diagnosis of infants with unexplained pulmonary masses, especially when accompanied by hepatic IH. Early recognition is critical for patients to receive proper and potentially life-saving treatment. Disclosures No relevant conflicts of interest to declare.

DIFFUSE HEMANGIOMATOSIS OF THE SPLEEN - CASE REPORT

Diffuse spleen hemangiomatosis is a rare benign vascular condition that is often seen in systemic angiomatosis (associations with Turner, Beckwith-Wiedemanand Klippel Trenaunay syndromes) and very rarely unique splenic localization [1.4]. They often present a latent clinical picture of incidental discovery sometimes may be accompanied by severe hypersplenism and other complications, splenic hemangiomas exhibit imagingcharacteristics similar to hepatic hemangiomas. We present the case of a diffuse hemangiomatosis discovered accidentally in an asymptomatic patient.

Hepatic hemangioma: A case of spontaneous involution

Hepatic hemangiomas are benign, well-circumscribed tumors often found incidentally on imaging. They are estimated to be prevalent in 0.4-20% of the population. While usually solitary lesions, these tumors tend to not cause any issues for patients. However, when they grow to be larger than 5 cm, there can be associated symptoms including pain, nausea, or early satiety from compression on adjacent structures. There can be more rare, serious complications including hemobilia, rupture, coagulopathy, or high output heart failure seen in tumors that are typically larger than 10 cm. While small hepatic hemangiomas (<5 cm) that do not cause symptoms do not need to be followed, ones that are greater than 5 cm should be followed at least once to ensure that they are stable in size, or more regularly if they are growing. Symptomatic large hepatic hemangiomas can be intervened on with a number of procedures if needed. These include hepatectomy, enucleation, transcatheter arterial embolization, radiofrequency ablation, and in rare cases, liver transplant.

Complications of Radiofrequency Ablation for Hepatic Hemangioma: A Multicenter Retrospective Analysis on 291 Cases

PurposeTo report the complications of radiofrequency ablation (RFA) for hepatic hemangioma.Patients and MethodsInvestigators from six centers performed RFA for hepatic hemangioma and used a standardized follow-up protocol. Data were collected from 291 patients, including 253 patients with hepatic hemangioma 5 to 9.9 cm in diameter (group A) and 38 with hepatic hemangioma ≥ 10 cm (group B). Technical success, complete ablation, and complications attributed to the RFA procedure were reported. Analysis of variance was used to determine whether the major complication rate was related to tumor size or clinical experience.ResultsA total of 304 lesions were treated in 291 patients. Technical success was achieved without adverse events in all cases. A total of 301 lesions were completely ablated, including 265 of 265 (100%) lesions in group A, and 36 of 39 (92.31%) in group B. The rate of technology-related complications was similar in groups A and B (5.14% (13/253) and 13.16% (5/38), respectively; P = 0.121). Moreover, all technology-related complications occurred during the early learning curve period. The rate of hemolysis-related complications in two groups were 83.40% (211/253) and 100% (38/38) (P =0.007) and the systemic inflammatory response syndrome-related complications in two groups were 33.99% (86/253) and 86.84% (33/38) (P&lt;0.001). There were no delayed complications in either group.ConclusionRFA is minimally invasive, safe, and effective for hepatic hemangiomas 5 to 9.9 cm in diameter. More clinical data are needed to confirm the safety of RFA for hepatic hemangiomas ≥ 10 cm.

Imaging Characteristics and Management of Infected Hepatic Hemangioma: Case-in Discussion

Hepatic hemangiomas are the most common type of benign liver tumors. We present a case of an infected hepatic hemangioma, which posed a diagnostic challenge. A 43-year-old female presented with right upper quadrant pain after blunt force trauma and intentional weight loss. CT and MRI were done, showing a posterior right lobe liver mass. Imaging characteristics were thought to be suggestive of metastatic disease, with a differential diagnosis of abscess. Drainage and biopsy of the mass revealed Streptococcus sp. infection, and pathology showed a cavernous hemangioma with inflammation. Patients presenting with systemic symptoms and an indeterminate liver mass on imaging should be evaluated for infected hepatic hemangiomas. Biopsy may be needed to rule out cancer. Management may include surgical resection; however, antibiotics and percutaneous drainage can suffice, as in this case.