Hepatic Hemangiomatosis Research Articles

Hemangiomatosis hepática de inicio neonatal

Infantile hemangioma is the most frequent pediatric benign tumor. The involvement is usually cutaneous, but it can be visceral too. Some presentations pose a diagnostic challenge and can even be life-threatening. We present two cases that consulted to our center. The first is a 13-day-old female newborn, who presents with an increase in the number and size of erythematous micromacules-papules, which she already had. She is diagnosed with infantile hemangiomatosis. In the extension study, the abdominal ultrasound shows multiple hepatic hemangiomas. The evolution is favorable without treatment, disappearing almost all hemangiomas within the first year. The second case is a 37-day-old infant, who presents with propulsive vomiting and abdominal distention. The physical exam is remarkable for cutaneous jaundice and 4cm hepatomegaly. Blood tests show cholestatic hepatitis without liver failure and hypothyroidism. Abdominal ultrasound shows diffuse hepatic hemangiomatosis, which is confirmed by MRI. Oral Propanolol and Levothyroxine are started. The evolution is favorable, with resolution of the hemangiomatosis. Hemangiomas are usually cutaneous but visceral involvement must be studied in the presence of more than 5 cutaneous hemangiomas or if they are large. Hepatic hemangiomas are the most common. They can be congenital (focal), which can involute spontaneously, or infantile (multifocal or diffuse), which can produce symptoms (cholestasis, hepatomegaly, heart failure, hypothyroidism…). The treatment is Propranolol and, in case of hypothyroidism, Levothyroxine. To conclude, we emphasize the importance of early diagnosis of hepatic hemangiomatosis because it has the possibility of specific and effective treatment.

Hepatic hemangiomatosis: a case report from Morocco

Introduction: Hepatic hemangiomas are benign liver tumors comprising clusters of blood-filled cavities, lined by endothelial cells, and supplied by the hepatic artery. The vast majority of hepatic hemangiomas (HH) are asymptomatic, often discovered incidentally during imaging studies for unrelated pathologies. Case Report: We present the clinical case of a 72-year-old patient admitted to our department due to a suspicious mass that emerged in the right hypochondrium a few months earlier. Laboratory studies were within normal limits, and diagnostic findings supported hepatic hemangiomatosis. Therapeutic abstention (with symptomatic treatment and close monitoring) was recommended. Discussion: Diffuse hepatic hemangiomatosis (DHH) is rare with an unclear etiology. In many cases, HH are small and asymptomatic at diagnosis with a stable progression. Asymptomatic patients typically undergo bservation with long-term follow-up, and imaging is generally unnecessary for lesions smaller than 5 cm, unless there is rapid growth or diagnostic uncertainty. Surgical resection may be considered for symptomatic patients or those with hemangiomas causing mass effects, after excluding other causes of pain. Conclusion: Ensuring an accurate diagnosis of hepatic hemangiomas is essential to differentiate them from other focal hepatic lesions, considering the possibility of coexisting diagnoses. While the majority of cases are asymptomatic and non-progressive, a subset may exhibit rapid growth or complications, requiring specific therapeutic approaches.

A rare case of hemangioma complicated with hepatic hemangiomatosis

A rare case of hemangioma complicated with hepatic hemangiomatosis

Gd-EOB-DTPA Enhanced MRI Features of Liver Hemangiomatosis Coexistent with GCH.

This study aimed to clarify features of giant cavernous hemangioma (GCH) and liver hemangiomatosis, existing simultaneously on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI). A total of 17 patients with reported hepatic hemangiomatosis between 2015 and 2017 were identified retrospectively. All our patients underwent pre-contrast MRI, triphasic (atrial, portal, venous) Gd-EOB-DTPA dynamic enhancement and hepatobiliary phase (20 minutes delayed). The location, size, morphology and signal characteristics on T1-weighted (T1WI) and T2-weighted images (T2WI), and Gd-EOB-DTPA-enhanced MRI of liver hemangiomatosis were evaluated. Hemangiomatosis involved the liver adjacent to the edge of the GCH with no normal liver tissue found in 13 cases; in the other 4 patients, a small area of normal liver tissue separated GCH from hemangiomatosis was seen. On non-contrast MRI images, hemangionmatosis presented as numerous microcystic lesions, with low signal intensity on T1WI and high signal intensity on T2WI, compared with unaffected liver. After administration of Gd-EOB-DTPA, heterogeneous enhancement was presented in the arterial phase, during portal and venous phase imaging, becoming more homogeneous. 11 cases showed hypointensity in the hepatobiliary phase (6 cases with intratumor necrosis), and 6 cases showed hyper-intensity in the hepatobiliary phase with a remaining unfilled portion. Hemangiomatosis is extremely rare in the liver adjacent to a GCH. MRI is of great diagnostic and clinical value for this kind of tumor according to the configuration, size, signal, and style of enhancement, but the final diagnosis depends on pathology. Gd-EOB-DTPA-enhanced MRI may help in diagnosing hemangiomatosis coexistent with GCH.

Adult diffuse hepatic hemangiomatosis associated with focal segmental glomerulosclerosis

Adult diffuse hepatic hemangiomatosis associated with focal segmental glomerulosclerosis

Un caso raro de hemangioma complicado con hemangiomatosis hepática

Un caso raro de hemangioma complicado con hemangiomatosis hepática

Adult diffuse hepatic hemangiomatosis

Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.

A rare case of cavernous hemangioma accompanied with diffuse hepatic hemangiomatosis

BackgroundHepatic cavernous hemangioma (CH) is the most common hepatic benign tumor. Most cases are solitary, asymptomatic, and found incidentally. In symptomatic cases with rapidly growing tumors and coagulopathy, surgical treatment is considered. In rare cases, diffuse hepatic hemangiomatosis (DHH) is reported as a comorbidity. The etiology of DHH is unknown.Case presentationA 29-year-old female patient had a history of endometriosis treated with oral contraceptives. Hepatic CH was incidentally detected in the segment IVa of the liver according to the Couinaud classification. Follow-up computed tomography (CT) and ultrasound sonography showed the growth of the lesion and formation of multiple new lesions near the first. Enhanced CT and magnetic resonance imaging (MRI) revealed that the new lesions were different from CH. Although oral contraceptives were stopped, all lesions grew in size. Malignancy and possibility of rupture of these tumors were considered due to the clinical course, and we opted for surgical removal of the tumors. Left liver lobectomy and cholecystectomy were performed. Surgical findings were small red spot spreading and a mass in segment IV of the liver. Pathological examination revealed a circumscribed sponge-like tumor with diffuse irregular extension to the adjacent area. Both of the lesions consisted of blood-filled dilated vascular spaces lined by flat endothelium without atypia. The diagnosis was hepatic CH with DHH. The patient was discharged on postoperative day 12 uneventfully.ConclusionWe report the successful resection of CH with DHH. The case findings suggest a relationship between oral contraceptive use and enlargement of CH and DHH. Although DHH has been poorly understood, a few previously published cases reported DHH occurrence in patients using oral contraceptives. In such cases, the decision to perform surgical resection should be made after careful examination.

2388 Peliosis Hepatis Progressing to Acute Liver Failure

INTRODUCTION: Peliosis hepatis is characterized as a vascular condition in which blood-filled cystic cavities are distributed diffusely throughout the liver parenchyma. The condition is rare, with the pathogenesis poorly described in the literature. It is believed that this disease is secondary to an underlying malignancy, immune disorder, infection, or a drug/toxin mediated effect. We present a case of a patient diagnosed with peliosis hepatis complicated by the development of acute liver failure. CASE DESCRIPTION/METHODS: A 71-year-old man with a past medical history of coronary artery disease, hypertension, hyperlipidemia, and prostate cancer treated with radiosurgery was admitted to our hospital with symptoms of fatigue, dysphagia and anorexia. He was in his usual state of health until four weeks prior to admission, when he began experiencing early satiety with poor oral intake, nausea, diarrhea, right upper quadrant abdominal pain, and weight loss. He was evaluated by his primary care provider who prescribed him a five day course of levofloxacin with no resolution of symptoms. Upon admission, labs were notable for leukocytosis and a mild transaminitis. Imaging revealed hepatomegaly with hepatic hemangiomatosis and splenic hemangiomas. A liver MRI was performed which returned consistent with diffuse hepatic peliosis with splenic peliosis. He underwent an intensive hematologic/oncologic, rheumatologic, and infectious workup that was significant for a positive quantiferon TB gold and he was subsequently started on empiric TB therapy. Synthetic liver function continued to worsen consistent with acute liver injury, which progressed to acute liver failure. The patient continued to decline and ultimately passed from multi-organ system failure. DISCUSSION: The majority of patients with peliosis hepatis will present asymptomatically and are found incidentally, while a subset of patients will present with abdominal pain or stigmata of liver disease. A liver biopsy is useful for diagnosis and is used in the context of the clinical picture, laboratory data, and imaging findings. Once the underlying offending etiology is recognized and appropriately treated, symptoms can improve and regression of the hepatic lesions can be seen. Our patient is unique in that his presentation was atypical and rapid, and a true causal etiology was never concretely identified, leading to progression to acute hepatic failure.

An Unexpected Cause of Coagulopathy

Hepatic hemangiomas are generally asymptomatic and rarely cause symptoms of consumptive coagulopathy and thrombocytopenia. We present a case of Kasabach-Merritt Syndrome in a patient found to have extensive hepatic hemangiomatosis. A 37-year-old male with a medical history of an abdominal hernia repair and an essential tremor presented with worsening fatigue, increased bruising, and abdominal distention over a 4-month period. Labs were significant for a hemoglobin of 8.4 g/dL, total bilirubin of 1.5 mg/dL, platelet count of 70,000/uL, INR 2.3, and fibrinogen level of less than 60 mg/dL. Abdominal magnetic resonance imaging (MRI) revealed variably sized lesions representing hepatic hemangiomas that replaced a majority of his hepatic parenchyma (Figure 1). Based on abnormal imaging and laboratory data revealing consumptive coagulopathy and hemolysis, a diagnosis of Kasabach-Merritt syndrome (KMS) was made. He was started on high dose methylprednisolone and transfused aggressively with packed red blood cells, cryoprecipitate, prothrombin complex concentrate, and fibrinogen concentrate with minimal improvement in his consumptive coagulopathy. Given his extensive hepatic dysfunction, he was listed for liver transplantation. He remained clinically stable with no signs of active bleeding and was discharged home. Two months after initial diagnosis, he underwent a successful liver transplant. His liver explant weighed 6.7 kg and measured 28cm in the right lobe and 30 cm in the left lobe. Kasabach-Merritt Syndrome is a rare but serious complication defined by the triad of hemangioma, consumptive coagulopathy, and thrombocytopenia. The incidence of this syndrome in adults is unknown and the majority of cases reported in children. Due to its rarity, there are no clinical guidelines for the treatment of this syndrome and the general approach is the correction of the hematologic derangements. Other cases reported the successful use of corticosteroids, antiplatelet therapy, and chemotherapeutic agents. Primary treatments of hepatic hemangiomas consist of embolization, enucleation, and resection with surgery reserved for symptomatic or complicated cases. Among previous case reports, the majority of giant liver hemangiomas (diameter greater than 20cm) underwent transplant, with one undergoing enucleation, and proved curative. Given his extensive hemangiomatosis, liver size, and coagulopathy refractory to medical therapy, a liver transplant was performed and proved curative.2414 Figure 1. Magnetic resonance imaging revealing multiple hepatic lesions without evidence of cirrhosis.

Hemangiomatoza cutanată infantilă asociată cu hemangiomatoză hepatică multifocală – prezentare de caz

Hemangiomatoza cutanată infantilă asociată cu hemangiomatoză hepatică multifocală – prezentare de caz

Portal hypertension secondary to multiple hepatic hemangiomatosis

Portal hypertension secondary to multiple hepatic hemangiomatosis

Diffuse Hepatic Hemangiomatosis of rapid growth after Bariatric Surgery

Diffuse Hepatic Hemangiomatosis of rapid growth after Bariatric Surgery

Approach to haemangiomatosis causing congestive heart failure.

Haemangiomas represent the most common tumour of infancy. Although most cases are cutaneous benign lesions, multiple skin haemangiomas are associated with visceral involvement, especially of the liver. Hepatic haemangiomatosis may be complicated by high-output cardiac failure due to high-flow arteriovenous connections within the lesions. Different therapeutic strategies for treating haemangiomatosis causing heart failure include medical, surgical and interventional modalities. This study aimed to review the treatment options, discuss their benefits and flaws and propose a practical therapeutic approach for this medical situation. Our approach incorporates heart failure medications, dietary support and propranolol as first-line treatment, while corticosteroids, vincristine, percutaneous intervention and surgery are reserved for refractory cases.

Giant Hemangioma Causing Budd-Chiari Syndrome

Triphasic CT scan of abdomen showed diffuse, large heterogeneous area involving left lobe and anterior segments of right lobe of liver with multiple similar character lesions in posterior segment with centripetal filling suggestive of liver hemangiomatosis (Figure 1A, B and C). The giant hemangioma was seen causing mass effect in form of compression of right hepatic vein and non-visualization of middle and left hepatic veins with chinking of intrahepatic inferior vena cava consistent with Budd-Chiari syndrome (Figure 1B). The diagnosis was confirmed with magnetic resonance imaging and venography (2D). Free fluid was also present in the abdomen. Patient improved on conservative measures (salt restriction and diuretics) and after 6 months had total disappearance of ascites. Figure 1 Nodular peripheral enhancement of the lesions in arterial phase (A) with centripetal filling in the venous phase (B) which are isodense in delayed phase with one lesion showing centripetal enhancement in delayed phase (C) (arrows). There is chinking of ... Liver hemangiomas are common incidental finding reflecting a high overall prevalence in the population. Approximately 80% of hemangiomas are of the cavernous type. These can, occasionally, grow to reach large size and may become symptomatic.1 Giant hemangiomas are defined as those measuring 5 cm or more in diameter. These may cause symptoms, including abdominal pain and right upper quadrant fullness. Acute pain may also occur if thrombosis or hemorrhage occurs within the hemangioma. The majority of hepatic hemangiomas remain stable over time though few studies have described significant growth, with occurrence of symptoms requiring surgical resection of lesions.1 Weimann et al reported size increase in 11 (10.6%) of 103 patients with liver hemangiomas over a median follow up of 32 months.2 Hemangiomatosis of the liver, with multiple hemangiomas scattered in the hepatic parenchyma, is uncommon in adulthood and has been reported only occasionally.3 Secondary Budd-Chiari syndrome is an uncommon clinical disorder characterized by hepatic venous outflow obstruction due to compression or invasion by a lesion originating outside the hepatic veins. There are only two previous reports of hepatic hemangioma causing Budd-Chiari syndrome. In one of the earlier reports, a 52-year old male was incidentally found to have hepatic hemangioma with cystic degeneration, causing symptoms of Budd-Chiari syndrome, which improved with cyst excision.4 In another report, a 45-year old woman symptomatic with pain in right hypochondrium had a giant hepatic hemangioma compressing the retrohepatic vena cava which improved with hepatic segmentectomy.5 Our patient had a giant hemangioma completely replacing the left lobe of liver and parts of right lobe with multiple other small hemangiomas consistent with hepatic hemangiomatosis causing compression of hepatic veins and the intrahepatic inferior vena cava resulting in Budd-Chiari syndrome. Although the therapeutic strategy for hemangiomas is usually non-operative, symptomatic lesions need operative intervention in form of hepatic resection or enucleation of hemangiomas. Surgical intervention was not considered in our patient because of the diffuse involvement of liver with multiple lesions. Our patient improved with conservative measures and remains asymptomatic on follow-up.

Ukrainian

ukrainian

Ovarian thecal metaplasia of the adrenal gland in association with Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome associated with increased risk to develop malignancies including adrenocortical carcinoma. Ovarian thecal metaplasia of the adrenal gland is a rare tumor-like mesenchymal lesion in BWS patients that lacks detailed radiological description. We report a 17-year-old female patient with BWS, associated with bilateral Wilms tumor, hepatic hemangiomatosis, pancreatic neuroendocrine tumor, and a phyllodes tumor of the right breast. Surveillance abdominal ultrasound identified a right adrenal mass that was further characterized by computed tomography and magnetic resonance imaging. Radiologically, this mass displayed features that overlap with adrenocortical carcinoma and pheochromocytoma but after pathological examination this proved to be an ovarian thecal metaplasia of the adrenal gland. Adrenal masses in BWS should raise the suspicion for adrenocortical carcinoma though other adrenal tumors including ovarian thecal metaplasia can be seen in these patients.

Adult Diffuse Hepatic Hemangiomatosis: A Rare Cause of Dilated Cardiomyopathy and Sudden Cardiac Arrest

A 53-year-old man presented with two months of fatigue and dyspnea on exertion, and was found to have hepatomegaly and hypotension (71/30 mmHg). Initial tests showed lactic acidosis with normal cardiac enzymes. Computed tomography of the abdomen revealed diffuse, hypodense, hepatic lesions without ischemic bowels. Shortly after admission, he developed cardiopulmonary arrest with ventricular fibrillation and expired despite aggressive medical support. Postmortem examination demonstrated dilated cardiomyopathy without coronary artherosclerosis (heart weight 785 g, normal 365 ± 71 g) and hepatomegaly with diffuse hemangiomatosis (liver weight 4,455 g, normal 1,677 ± 396 g, Fig. 1).1 Figure 1. Multiple irregular, well-circumscribed, dark brown lesions (arrow) were seen in the liver parenchyma. The lesions ranged from 0.5-10 cm in greatest dimension. Diffuse hepatic hemangiomatosis is extremely rare.2 The histologic characteristics include dilated vascular channels lined with flat endothelial cells (Fig. 2). The etiologies are poorly understood, though its association with the use of estrogen and metoclopramide has been described.3,4 The two most common causes of death are cardiomyopathy (from massive hepatic arteriovenous shunting) and cirrhosis.5 Surgical resection is an option in selected cases with small lesions without cirrhosis.5,6 Most cases are inoperable because of poor delineation of the tumor border and bleeding risk. Other anecdotal therapies include hepatic artery ligation and radiation.6 Prognosis depends on the area of hepatic involvement and cardiac function. Figure 2. Microscopic examination revealed cavernous vascular spaces (asterisk) separated by thin fibrous septae lined by endothelial cells (arrow). These findings confirmed the diagnosis of hepatic hemangiomatosis.

The role of red blood cell scintigraphy in the multiple-modality imaging diagnosis of a rare case of diffuse hepatic hemangiomatosis in an adult

Introduction: Angiomas are one of the most common primary tumors of the liver. Diffuse hepatic angiomatosis, however, is quite rare and usually observed in pediatric patients. We report a rare case of diffuse hepatic hemangiomatosis in a 33-year-old woman. Case report: The patient presented with abdominal pain and a palpable upper abdominal mass. Abdominal CT and magnetic resonance imaging (MRI) findings suggested diffuse hepatic hemangiomatosis, but this finding was not confirmed by subsequent contrast-enhanced abdominal ultrasonography (US). The patient then underwent single photon emission computed tomography (SPECT)/CT scintigraphy with Tc-99m-labeling of red blood cells (RBC). This examination revealed increased uptake of labeled erythrocytes in several of the hepatic lesions corresponding to CT and RM findings, thereby confirming the clinical hypothesis of diffuse hepatic hemangiomatosis. Discussion: RBC scintigraphy with SPECT/CT can facilitate the comparison of other crosssectional imaging methods such as CT and MRI. This case highlights the importance of a multiple-modality approach in the imaging diagnosis of this condition.

An unusual presentation of hemangiomatosis of the liver

Diffuse hemangiomatosis of the liver is a rare condition of infancy characterized by extensive hemangiomas in the liver. It is extremely rare in adults. The imaging findings are similar to hemangioma showing centripetal enhancement with stasis of contrast. We present a case of hemangiomatosis of the liver and spleen in a 52 year old male patient. Following splenectomy there was enhanced growth of the hepatic hemangiomatosis within a period of four months and died soon after due to liver failure. We could not find a similar rapid progression of hepatic hemangiomatosis in our literature search.