Congenital hemangioma is a rare benign vascular tumor that is present at birth and fully developed, and it differs from infantile hemangioma in both biology and behavior. Clinicians recognize three main subtypes based on natural behavior: rapidly involuting congenital hemangioma, which shrinks quickly after birth; non-involuting congenital hemangioma, which persists indefinitely; and partially involuting congenital hemangioma, which displays characteristics overlapping the other two. Growing genetic evidence indicates that most cases across all three subtypes arise from somatic activating variants in the genes GNAQ and its paralog GNA11, suggesting that they represent one disease category rather than three separate entities. These tumors can appear on the head and neck, trunk, or limbs, and large lesions may drive fetal high-output heart failure, hydrops fetalis, anemia, thrombocytopenia, and even death.

The fistula complicating this case belongs to a different family of vascular disease altogether. Vascular malformations arise from abnormal blood vessel morphogenesis rather than from proliferating tumor cells, and endothelial cell renewal within them remains normal. They are classified by the vessels involved and their flow characteristics: low-flow lesions involve capillaries, veins, lymphatic channels, or mixtures of these, while high-flow lesions involve arteries or direct arteriovenous connections. An arteriovenous malformation is a high-flow malformation composed of abnormal arteries, veins, and capillaries in which blood shunts directly from the arterial to the venous side without passing through a capillary bed. In the trunk and extremities, such lesions typically produce a pulsatile mass, pain, ulceration, bleeding, tissue necrosis, enlarged draining veins, venous hypertension, or heart failure. Because of this high-flow shunting, arteriovenous malformations are more invasive than other congenital vascular malformations and are often accompanied by life- or limb-threatening complications, which is why early and active treatment is generally recommended, even though options remain limited and the disease carries significant morbidity and mortality.

The prenatal course of this case illustrates how such lesions evolve under close surveillance. The fetus was transferred to West China Second University Hospital at 23 weeks of gestation with a progressively enlarging mass on the right forearm, and a multidisciplinary team evaluated the fetus comprehensively while tracking the lesion. At 25 weeks and 3 days, magnetic resonance imaging showed the mass measured approximately 3.48 by 2.02 by 3.55 centimeters, with a multistrip expanding tubular structure and a possibility of hemorrhage, raising suspicion of congenital hemangioma. By 38 weeks, color Doppler ultrasound of the right upper limb showed a mixed-echo mass measuring 6.1 by 7.4 by 8.2 centimeters, suggesting vascular malformation. The baby was delivered by cesarean section at 38 weeks and 2 days, weighing 3,010 grams with perfect Apgar scores of 10 at each of the three assessment points. Physical examination then revealed a 7.0 by 6.5 by 5.0 centimeter mass with clear boundaries, slightly high tension, bluish-purple overlying skin, partial skin breakdown with crusting, and mildly elevated local skin temperature. The authors note that the apparent size difference between prenatal ultrasound and postnatal caliper measurement reflects methodological differences between in-utero imaging through a confined acoustic window and surface measurement, not true tumor shrinkage.

Deterioration came quickly. Two days after birth, the infant developed progressive dyspnea and bleeding from the mass. Echocardiography disclosed severe pulmonary hypertension, with an estimated right ventricular systolic pressure of 52 millimeters of mercury derived from a tricuspid regurgitation peak velocity of 3.4 meters per second using the modified Bernoulli equation, accompanied by bidirectional shunting through the ductus arteriosus. Chest radiography ruled out neonatal respiratory distress syndrome, pneumonia, and pneumothorax, while computed tomography angiography confirmed a giant congenital hemangioma with an arteriovenous fistula. Serial laboratory monitoring revealed a distinctive pattern. On day one, before bleeding began, the platelet count was 182 × 10⁹ per liter, coagulation parameters were normal, and D-dimer was markedly elevated at 6.67 milligrams per liter against a reference value below 0.5, suggesting localized intravascular coagulation within the lesion. On day two, coinciding with active bleeding, platelets fell to 73 × 10⁹ per liter, clotting times showed only minimal prolongation, fibrinogen fell slightly to 2.3 grams per liter but remained normal, and D-dimer rose further to 8.15 milligrams per liter.

The team deliberately excluded Kasabach–Merritt phenomenon, a consumptive coagulopathy classically linked to kaposiform hemangioendothelioma and tufted angioma and only exceedingly rarely reported in congenital hemangioma. The platelet nadir of 73 × 10⁹ per liter never reached the profound thrombocytopenia below 50 × 10⁹ per liter typical of that syndrome; coagulation parameters stayed near-normal with preserved fibrinogen, and the marked D-dimer elevation with normal fibrinogen pointed to localized rather than systemic consumption; the bleeding was confined to the ulcerated skin rather than diffuse; and the underlying tumor type made the syndrome unlikely. Bleeding was therefore attributed primarily to local ulceration and high-flow shunting. Coagulation studies were not repeated after the procedure because clinical bleeding had ceased and the infant was hemodynamically stable, and serial hemoglobin levels remained stable throughout the acute phase without any need for transfusion.

With the team suspecting that the progressive dyspnea stemmed from pulmonary hypertension driven by the giant hemangioma and its fistula, arteriography was performed to confirm the lesion, and emergency combined embolization and sclerotherapy followed immediately. Via right femoral artery and vein puncture with 5-French sheaths placed after systemic heparinization, selective catheterization of the right subclavian artery confirmed a high-flow arteriovenous fistula with dilated, tortuous feeding arteries. A 4F Cobra catheter was advanced into the dilated draining vein, where two 8-millimeter Nester coils were deployed. The operators then punctured the lesional vessels at multiple points through the skin and placed eight additional coils—three 10-millimeter, one 8-millimeter, three 6-millimeter, and one 4-millimeter—for a total of ten coils ranging from 4 to 10 millimeters. Once all coils were in place, a mixture of 0.5 milliliters of iodized oil and 2 milliliters of absolute ethanol was injected, followed by 3 milligrams of bleomycin combined with contrast medium and iodized oil for sclerotherapy. Post-procedural angiography showed reduced lesion opacification and slowed venous drainage, and no immediate procedural complications occurred.

Pharmacotherapy accompanied the intervention. Oral propranolol was prescribed at 1 milligram per kilogram twice daily after contraindications were excluded, then gradually increased to 2 milligrams per kilogram per day. During titration and for 72 hours afterward, continuous telemetry was maintained, with heart rate checked every two hours, blood pressure every four hours, and capillary blood glucose every four hours for 48 hours and then every six hours; all values stayed within age-appropriate ranges, with no bradycardia, hypotension, or hypoglycemia documented. The clinical condition improved, and the mass gradually shrank during follow-up. Baseline echocardiography had shown moderate tricuspid regurgitation with a peak velocity of 3.5 meters per second and a peak gradient of 49 millimeters of mercury, yielding an estimated pulmonary artery systolic pressure of roughly 52 millimeters of mercury, plus trace pulmonary regurgitation from which mean pulmonary artery pressure was estimated at about 35 millimeters of mercury. There was also a 5.2-millimeter patent ductus arteriosus with predominantly right-to-left bidirectional shunting, bidirectional interatrial shunting through two communications of approximately 2 millimeters each, right ventricular wall thickening of 3.1 millimeters, and D-shaped septal motion consistent with right ventricular pressure overload. Follow-up echocardiography four weeks after baseline, fourteen days after embolization, showed normal left ventricular systolic function with an ejection fraction of 66 percent and fractional shortening of 34 percent, normal right heart dimensions, no measurable tricuspid regurgitation jet, no visible ductus, and only a small residual interatrial left-to-right shunt—documenting complete resolution of the pulmonary hypertension. The infant achieved all age-appropriate developmental milestones, with full range of motion, normal grip strength, and no residual edema or compression sequelae in the forearm and hand.

The report situates the case against a sparse literature. Giant congenital hemangiomas of the extremities combined with arteriovenous malformation have rarely been described, and previous accounts include a fetus with a giant congenital hemangioma and arteriovenous malformation that suffered fetal malnutrition, hydrops, and neonatal death, as well as an infant with a giant axillary hemangioma and subclavian arteriovenous fistula who developed heart failure at birth. Among patients with hepatic arteriovenous fistula, more than 50 percent develop heart dysfunction, and 50 to 90 percent die of severe heart failure within the first week of life. A summarized series of 20 congenital hemangioma cases found that 70 percent of affected fetuses survived to birth, with complications such as elevated cardiothoracic ratio, atrioventricular valve regurgitation, and fetal hydrops occurring in 40 percent, and larger lesions—67 percent with maximum diameter of at least 5 centimeters and all with volume of at least 40 cubic centimeters—more likely to cause trouble. In the present case, the authors justified immediate intervention over observation by pointing to active bleeding, a lesion substantially larger than the previously reported axillary case managed conservatively, severe pulmonary hypertension progressing despite respiratory support, and a trajectory of rapid deterioration rather than gradual improvement.

The authors acknowledge important limitations. The diagnosis rested solely on prenatal and postnatal imaging—MRI, Doppler ultrasound, computed tomography angiography, and angiography—because the infant’s unstable condition and the risk of provoking hemorrhage precluded biopsy, so neither histopathological examination nor molecular testing for the characteristic GNAQ and GNA11 mutations was performed. A primary high-flow arteriovenous malformation with secondary mass effect therefore cannot be definitively excluded, and the diagnostic conclusion should be interpreted with caution. The propranolol component also demands careful reading: congenital hemangioma is driven by GNAQ and GNA11 mutations rather than the biology of infantile hemangioma, and some researchers have criticized beta-blocker use for congenital hemangioma, noting that evidence-based support exists only for infantile hemangioma. In this case propranolol was given as an off-label adjunct after emergency embolization and sclerotherapy, based on a multidisciplinary risk–benefit assessment in a life-threatening situation, and the authors stress that a single favorable outcome does not establish efficacy. Their broader message is that clinicians should remain alert to congenital hemangioma with arteriovenous malformation as a cause of severe pulmonary hypertension and bleeding, and that prompt, integrated perinatal management by a multidisciplinary team—reserving intervention for cases with active hemorrhage, progressive hemodynamic compromise, or failed conservative care—can achieve favorable outcomes.

Subject of Research: A neonatal case report of giant congenital hemangioma with arteriovenous fistula treated by embolization, sclerotherapy, and propranolol

Article Title: Case Report: Fetal giant congenital hemangioma with arteriovenous malformation: to be or not to be

Article References: Yue, P., Ma, F., Ma, L., Wan, J., Liu, X., & Hu, F. (2026). Case Report: Fetal giant congenital hemangioma with arteriovenous malformation: to be or not to be. Frontiers in Medicine, 13, Article 1925509. https://doi.org/10.3389/fmed.2026.1925509

Image Credits: AI Generated

DOI: 10.3389/fmed.2026.1925509

Keywords: congenital hemangioma, arteriovenous fistula, arteriovenous malformation, pulmonary hypertension, coil embolization, sclerotherapy, propranolol, neonate, prenatal diagnosis, GNAQ mutation, vascular malformation, case report

To understand why this case matters, it helps to know how vascular tumors of infancy are classified. Hemangiomas are the most common vascular tumors of infancy, and clinicians broadly divide them into infantile hemangiomas, which typically appear after birth and grow during early infancy, and congenital hemangiomas, which are fully formed by the time a baby is born. Congenital hemangiomas are themselves split into three behavioral categories: rapidly involuting congenital hemangiomas, which regress quickly after birth; non-involuting congenital hemangiomas, which persist indefinitely; and partially involuting congenital hemangiomas, which shrink somewhat but never disappear entirely. This three-way classification, formalized in influential work by Mulliken and Enjolras, reflects the fact that these tumors behave in fundamentally different ways despite looking superficially similar under the microscope.

The genetic underpinnings of congenital hemangiomas have become clearer in the past decade. Researchers have identified somatic activating mutations in the GNAQ and GNA11 genes in these tumors — alterations that occur in the tumor tissue itself rather than being inherited, and that drive the abnormal proliferation of blood vessel cells. This molecular signature distinguishes congenital hemangiomas from infantile hemangiomas, which follow a different biological program altogether. One of the classic diagnostic markers separating the two is GLUT1, a glucose transporter protein that is abundantly expressed in infantile hemangiomas but characteristically absent in congenital ones. Immunohistochemical staining for GLUT1, first described as a diagnostic marker for juvenile hemangiomas in landmark work published in 2000, remains a cornerstone of daily practice in distinguishing cutaneous vascular anomalies.

Most congenital hemangiomas appear on the skin and soft tissues of the head and neck or around the joints of the extremities — the periarticular regions. A RICH on a scalp or a knee is unusual enough; a RICH inside the brain, in the suprasellar region, is a genuine medical rarity. The suprasellar region is one of the most crowded and consequential addresses in the human brain. It houses the pituitary gland, the optic chiasm, the hypothalamus, and the circle of Willis, the arterial ring that supplies blood to much of the cerebrum. Tumors in this location — whether congenital craniopharyngiomas, hypothalamic hamartomas, or other lesions — can threaten vision, hormone regulation, and core metabolic functions, and they often demand delicate neurosurgical intervention. The prospect of a vascular tumor growing there before birth is the kind of finding that sets off immediate alarm bells in any fetal imaging suite.

That is precisely what makes the new report so valuable. The lesion was first detected on prenatal ultrasound, the workhorse of fetal surveillance, and then further characterized with fetal MRI, which offers superior soft-tissue contrast and can help differentiate vascular lesions from other fetal brain tumors. Fetal brain tumors themselves are exceedingly rare, and imaging them well is a technical challenge; a dedicated review of fetal brain tumor imaging published in Pediatric Radiology in 2020 underscored how difficult it can be to pin down a precise prenatal diagnosis. In this case, the imaging features and clinical course pointed toward a RICH, and the diagnosis was confirmed by the tumor’s behavior after birth: serial postnatal ultrasound and MRI demonstrated rapid interval regression, the hallmark of the rapidly involuting subtype.

The natural history of RICH tumors is what gives this case its happy ending. These tumors are fully grown at birth — they do not exhibit the postnatal proliferation phase that defines infantile hemangiomas — and then they begin to shrink, often dramatically, during the first months of life. Many resolve completely, sometimes leaving behind only a subtle patch of atrophic skin or residual telangiectasias when they occur on the skin. The speed of involution can be striking, which is how the subtype earned its name. But the behavior of a RICH in the brain is far less well charted than its cutaneous counterpart, simply because so few intracranial cases have been reported. Prior reports have described RICH lesions of the skull and the hand, and one team documented prenatal diagnosis and postnatal follow-up of a RICH in this same journal more than a decade ago, but suprasellar examples remain vanishingly scarce.

The clinical stakes of such a diagnosis are considerable. Congenital hemangiomas are usually benign and self-limited, but they are not always harmless. Rare catastrophic cases have been described in which congenital hemangiomas caused severe coagulopathy — a consumption of clotting factors by the tumor’s high-flow vasculature — leading to fatal cardiac failure in newborns. High-flow vascular lesions in the brain carry additional risks, including hemorrhage and mass effect on critical structures. For families and clinicians facing a prenatal diagnosis of a suprasellar mass, the differential diagnosis includes lesions with far grimmer prognoses, and the temptation toward aggressive intervention can be strong. A documented case in which watchful waiting was rewarded by spontaneous, imaging-confirmed regression provides a crucial data point: not every prenatal brain mass is a surgical emergency, and accurate diagnosis can spare an infant invasive procedures.

That said, the report also implicitly acknowledges that not all congenital hemangiomas can be left alone. When these tumors cause serious complications, medical therapy has entered the picture in recent years. Sirolimus, an mTOR inhibitor better known as an immunosuppressant, has emerged as a treatment option for problematic vascular anomalies, and a 2026 report in Pediatric Blood & Cancer described the successful management of a refractory congenital hemangioma with sirolimus in a neonate. The decision matrix for a neonatal vascular tumor thus spans a spectrum from simple observation to pharmacotherapy to, in select cases, surgery — and the choice hinges on accurate subtype classification, which is exactly where detailed imaging documentation like that in this new case report earns its keep.

For the radiology community, the case adds a rare entry to the atlas of fetal and neonatal intracranial vascular tumors, complete with the kind of longitudinal imaging follow-up that textbooks crave. For the broader public, it is a reminder of how much the prenatal imaging revolution has changed medicine: a tumor invisible to any previous generation of doctors is now spotted on ultrasound before a baby takes its first breath, characterized by MRI in utero, and tracked noninvasively as it resolves. The researchers reported no competing interests, obtained written informed consent from the child’s parents, and noted that institutional review board approval was not required for this single case report under institutional policies. The authors — Noman Khan, Rafaela Minguetti, Alexandra J. Borst, Kimberly M. Hamilton, and Carolina V. Guimaraes — frame the contribution around the clinical course and imaging features of the lesion, offering colleagues worldwide a template for recognizing a diagnosis that most will encounter only once, if ever, in their careers.

Behind the technical narrative lies a genuinely moving human story: parents who learned before birth that their child had a mass in one of the most delicate regions of the brain, and who then watched, scan by scan, as that mass melted away on its own. Medicine’s growing ability to distinguish tumors that threaten from tumors that retreat — using imaging signatures, immunohistochemical markers like GLUT1, and now somatic genetics — is quietly transforming outcomes for infants like this one. In an era when the word tumor triggers instinctive dread, this suprasellar RICH stands as a counterexample: a lesion that arrived fully formed, announced itself on fetal MRI, and then, true to its name, vanished in a rush — leaving behind only images, data, and a rare lesson in the power of knowing when to do nothing at all.

Subject of Research: A rare case of rapidly involuting congenital hemangioma in the fetal suprasellar brain region documented by prenatal and postnatal imaging

Article Title: Rapidly involuting congenital hemangioma of the suprasellar region

Article References: Khan, N., Minguetti, R., Borst, A. J., Hamilton, K. M., & Guimaraes, C. V. (2026). Rapidly involuting congenital hemangioma of the suprasellar region. Pediatric Radiology. https://doi.org/10.1007/s00247-026-06780-2

Image Credits: AI Generated

DOI: 10.1007/s00247-026-06780-2

Keywords: congenital hemangioma, RICH, suprasellar region, fetal MRI, prenatal diagnosis, pediatric radiology, vascular tumors, GLUT1, GNAQ mutation, infantile hemangioma, spontaneous regression, neonatal imaging