Benign Vascular Tumors Research Articles

Everolimus for the treatment of epithelioid hemangioma: a case report

Epithelioid hemangioma is a rare benign vascular tumor manifested as slow-growing subcutaneous or dermal nodules. A previously therapeutic strategy involved surgical excision and sclerotherapy. However, no standard treatment has been established. Here, we reported an atypical case of epithelioid hemangioma that had a locally aggressive behavior and was successfully treated with everolimus, a rapamycin analog used for cancer therapy. A 1-year-old boy presented with an ulcerated lump in the left palmar region. The imaging test results, confirmed through histopathologic examination, suggested a diagnosis of epithelioid hemangioma. Targeted therapy with everolimus was administered orally because of a minimal response to initial transarterial sclerotherapy. The patient achieved a satisfactory response with a significantly reduced lesion size and improved hand function after a 2-year follow-up. This finding showed that patients with locally aggressive forms of epithelioid hemangioma may significantly benefit from everolimus as a potential targeted therapy.

CYSTIC LYMPHANGIOMA A CASE REPORT

Cystic lymphangioma of the pancreas is a rare benign vascular tumor. Its histogenesis is still hypothetical and its preoperative diagnosis difficult to establish. We report a case in a 26-year-old boy who presented with abdominal pain with the notion of a constipation-type transit disorder. Radiological investigations showed an intraperitoneal mesenteric cystic tumor. A complete resection of the mass was performed. Pathological examination of the operative specimen confirmed the presence of cystic formations, the wall of no need keep it.to the end which was lined by a squamous, endothelial epithelium with a fibrous wall dotted with a few lymphoid clusters in favor of cystic lymphangioma.

Intraosseous Hemangioma of the Zygoma: A Case Report

Intraosseous hemangioma is a rare, benign vascular tumor of endothelial origin. It accounts for fewer than 1% of all hemangiomas, and very rarely occurs in the face. Intraosseous hemangioma usually presents as an asymptomatic lesion, but symptoms can occur due to the mass effect. The authors describe a case of intraosseous hemangioma of the zygoma with a review of the relevant literature. A 44-year-old man presented with a chief complaint of painless swelling on the left zygomatic region that had been slowly growing for the past year. On physical examination, a hard, non-movable mass in a deep layer was palpated. On computed tomography performed to evaluate its layers and extent, trabeculation was found inside the mass, but the lack of destruction of the surrounding bone suggested that the mass was benign. Complete surgical excision was performed under local anesthesia. After complete excision of the mass, slight erosions remained on the cortical bone of the zygoma, but because it was small enough not to cause a facial deformity such as depression or asymmetry, no additional reconstructive procedure was performed. There were no symptoms or recurrence during a 8-month follow-up period. Key Words: Intraosseus, Hemangioma, Zygoma

Modern treatment of infantile hemangioma

Infantile hemangioma (IH) is the most common benign vascular tumor in children of the first year, which is based on abnormal proliferation of endothelial cells under the influence of the main pro-angiogenic factors: vascular endothelial growth factor (VEGF) and fibroblast growth factors (FGF). It develops in the first weeks after birth, forming over 3–9 months with regression in the next 3–7 years. Three-quarters of infantile hemangiomas are nodular and are not accompanied by malformations. At the same time, segmental IH is most often associated with syndromic forms. Despite spontaneous regression (in 90% of cases), some forms and localization of IH can lead to the development of complications, local and endangering vital functions. In most cases, the diagnosis is based on anamnesis, characteristic features of the tumor, and clinical course. Additional studies (ultrasound DG, MRI/CT, biopsy) are necessary in complicated forms and in doubtful clinical cases.

Cavernous Hemangioma of the Temporal Muscle

Haemangiomas are benign vascular neoplasms characterized by an abnormal proliferation of blood vessels. These tumours are common in infancy and childhood and commonly involve subcutaneous or mucosal tissues. Fewer than 1% are intramuscular hemangiomas occurring within skeletal muscle, they occur more often in trunk and extremity muscles, whereas involvement of the temporal muscle is extremely rare.

Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.

Congenital haemangioma (CH) is a rare benign vascular tumour presenting at birth with excellent prognosis. Usually, CH regresses without treatment within the first few months of life. Kaposiform Haemangioendothelioma (KHE) is another type of vascular tumours that has been described as benign with locally aggressive potential. Although the diagnosis of vascular tumours is usually straightforward based on typical clinical presentation, yet some confusing similarities may exist with congenital sarcomas.Conclusion: Data of cases managed at the vascular anomaly clinic during the period 2015 through 2019 were retrospectively analysed. The study included three groups of patients: cases diagnosed as congenital haemangioma (9 cases), cases of Kaposiform Haemangioendothelioma who presented in the neonatal period (7 cases), as well as cases of congenital fibrosarcoma (4 cases) that were referred to the vascular anomaly clinic because of apparent similarity with vascular tumours. The hallmark of the study was to compare clinical and imaging features in the three groups to facilitate differentiation and remove diagnostic confusion when managing these rare cases in the future. What is Known: • Congenital haemangioma is a rare benign vascular tumour presenting at birth. • Kaposiform Haemangioendothelioma is another type of vascular tumours that has been described as benign with locally aggressive potential. What is New: • Confusing similarities may exist between vascular tumours and congenital sarcomas.

Pyogenic granuloma under nail: A rare localization

Pyogenic granuloma (PG) – also known as lobular capillary hemangioma – is a benign vascular tumour that occurs on the skin and mucous membranes, it’s can arise spontaneously, in sites of injury, or within capillary malformations [1]. Because malignant tumors, such as amelanotic melanoma, can be misdiagnosed as PG, histopathology is mandatory for the correct diagnosis, even though dermoscopy can play a primary role in the prompt recognition of PG, showing highly repetitive patterns that can support the diagnosis [2]. In particular, the presence of reddish homogeneous area, white rail lines are strongly suggestive of PG; even more so when associated together. On the other hand, with dermoscopy, it is not uncommon to detect ulceration and vascular structures that do not rule out malignancies [3]. Vascular structures are observed in 45% of cases, they can be linear-irregular or polymorphic and atypical [2]. Despite its benign nature, GP can have a significant negative impact on patients’ quality of life, and therefore appropriate therapy is needed. This is a 35-year-old patient with a history of trauma to his left hand dating back 3 months before his admission. He presents with a nodule under his left thumb, which has been evolving for 2 months, fixed in depth and sometimes-bleeding (Fig. 1). The nodule was slightly painful. X-rays of the thumb were normal. On dermoscopy (Fig. 2), we noted the presence of vascular lagoons with fibrous septal and linear vessels. We suspected a botryomyoma or achromic melanoma. Surgical excision was performed (Fig. 3) with an anatomopathological study in favour of a pyogenic granuloma.

A Huge Adrenal Incidentaloma With Suspicious Features and a Complete Cure!

Introduction: Adrenal hemangioma is an extremely rare benign vascular tumor occurring in adrenal glands (~0.01%). Its clinical presentation is usually vague and is frequently discovered as an incidentaloma. These tumors are mostly encountered at 40–70 years of age and are twice as common in women. We report a case of a nonfunctioning large adrenal incidentaloma with suspicious features on imaging. This in fact was an adrenal hemangioma diagnosed postoperatively with histopathology. Case Description: Our patient was 76 year old Hispanic male, active smoker, with medical history significant for well controlled Type 2 diabetes, hyperlipidemia and hypertension. He was seen for evaluation and management of an adrenal incidentaloma discovered on iv contrast enhanced CT performed to investigate recurrent hematuria. Imaging of the abdomen and pelvis revealed a posterior bladder mass measuring up to 2.6 cm and 20.1 cm x 17.1 cm x 20.5 cm left adrenal mass with few punctuate calcifications displacing the left kidney and spleen- findings concerning for adrenal cortical carcinoma. The right adrenal gland unremarkable. Given the size of mass and suspicious features hormonal workup was initiated in preparation for surgery. Workup was unremarkable for Pheochromocytoma {24 hour urine metanephrine 124 (nl: 0–300), normetanephrine 440 (nl: 0–400)}, Cushing syndrome {(negative 1 mg overnight dexamethasone suppression test), ACTH 22 (nl: 7—63)} and hyperaldosteronism (Aldosterone 5, renin 0.7). Patient underwent an uncomplicated total radical left adrenalectomy, he had no evidence of invasive disease. Pathology showed a large entirely encapsulated heterogenous adrenal mass weighing 2300 grams with largely solid (80%) and cystic components. There was hemorrhage and extensive infarcts- findings consistent with a Hemangioma. He recovered well. No evidence of adrenal insufficiency was noted on subsequent follow-ups. He had a normal surveillance CT scan at 6 months. Discussion: Adrenal hemangiomas are difficult to differentiate clinically and radiographically from adrenal cortical cancer. They must be evaluated to exclude hormonal hypersecretion and malignancy. Previous case reports hypothesized that adrenal hemangiomas may have functional hypersecretion due to mass effect on the adrenal parenchyma. There was no evidence of this in our patient. These tumors increase in size overtime. Surgical resection is often necessary to rule out any malignant potential and alleviate symptoms secondary to mass effect. Adrenal hemangiomas though rare and must be considered in the differential diagnosis.

GNA14, GNA11, and GNAQ Mutations Are Frequent in Benign but Not Malignant Cutaneous Vascular Tumors.

Cutaneous vascular tumors consist of a heterogeneous group of benign proliferations, including a range of hemangiomas and vascular malformations, as well as heterogeneous groups of both borderline and malignant neoplasms such as Kaposi’s sarcoma and angiosarcomas. The genetics of these tumors have been assessed independently in smaller individual cohorts making comparisons difficult. In our study, we analyzed a representative cohort of benign vascular proliferations observed in a clinical routine setting as well as a selection of malignant vascular proliferations. Our cohort of 104 vascular proliferations including hemangiomas, malformations, angiosarcomas and Kaposi’s sarcoma were screened by targeted next-generation sequencing for activating genetic mutations known or assumed to be potentially relevant in vascular proliferations. An association analysis was performed for mutation status and clinico-pathological parameters. Frequent activating hotspot mutations in GNA genes, including GNA14 Q205, GNA11 and GNAQ Q209 were identified in 16 of 64 benign vascular tumors (25%). GNA gene mutations were particularly frequent (52%) in cherry (senile) hemangiomas (13 of 25). In angiosarcomas, activating RAS mutations (HRAS and NRAS) were identified in three samples (16%). No activating GNA or RAS gene mutations were identified in Kaposi’s sarcomas. Our study identifies GNA14 Q205, GNA11 and GNAQ Q209 mutations as being the most common and mutually exclusive mutations in benign hemangiomas. These mutations were not identified in malignant vascular tumors, which could be of potential diagnostic value in distinguishing these entities.

Littoral cell angioma of the spleen in a child with Cornelia De Lange syndrome

Littoral Cell Angioma (LCA) is a benign and rare vascular tumor of the spleen. It is uncommon in children, and a comprehensive literature review has yielded only 12 published pediatric cases of LCA. Here we present a seven year old, female who was a known case of Cornelia De Lange syndrome who presented with splenic cysts and underwent splenectomy. Histopathological review revealed LCA.

Repidly involuting congenital hemangioma

Congenital hemangiomas are rare benign vascular tumors that develop in utero and are fully formed by the time of birth. Depending on the ability to involution, there are three subtypes: RICH (repidly involuting congenital hemangioma), NICH (non involuting congenital hemangioma), PICH (partially involuting congenital hemangioma). PICH may be accompanied by thrombocytopenia and consumption coagulopathy. Despite clearly defined clinical and histological characteristics, it can be difficult to make a differential diagnosis between congenital hemangiomas and other vascular tumors (infantile hemangioma, kaposiform hemangioendothelioma/“fascicular” angioma and others). The clinical case in the article of a vascular tumor in a newborn complicated by thrombocytopenia and consumption coagulopathy was regarded as Kazabach-Merritt syndrome, which is based on kaposiform hemangioendothelioma/“fascicular” angioma. Rapid regression of the tumor and recovery of hemogram and coagulogram parameters, as well as anamnesis of the disease and initial characteristics of the tumor forced to reconsider the diagnosis. Based on the histological picture, the diagnosis of congenital hemangioma, RICH, was confirmed. Verification of the diagnosis made it possible to change therapeutic tactics and avoid chemotherapy. A giant hemangioma, accompanied by thrombocytopenia and consumption coagulopathy, may have a very favorable outcome – a complete resolution of the pathological process inherent in its natural course. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

Infantile hemangioma explained in simple terms

Infantile hemangioma (IH) it is the most common benign vascular tumor in children of the first year, which is based on abnormal proliferation of endothelial cells under the influence of the main pro-angiogenic factors VEGF and FGF. It develops in the first weeks after birth, forming over 3–9 months with regression in the next 3–7 years. Three-quarters of infantile hemangiomas are nodular and are not accompanied by malformations. At the same time, segmental IH is most often associated with syndromic forms. Despite spontaneous regression (in 90% of cases), some forms and localization of IH can lead to the development of complications, local and endangering vital functions. In most cases, the diagnosis is based on anamnesis, characteristic features of the tumor, and clinical course. Additional studies (ultrasound DG, MRI/CT) are necessary in complicated forms and in doubtful clinical cases. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

Aggressive Diffuse Intraosseous Hemangioma: Case Report.

Vertebral hemangioma is a benign vascular tumor that is usually asymptomatic and is discovered incidentally on imaging. When symptomatic, the most frequent presentation occurs in the form of vague back pain of insidious onset and, in rare cases, may be associated with root or spinal compression, causing sensory and motor deficits. The authors report the case of a 33-year-old man, previously healthy, with a diagnosis of thoracic spine hemangioma at multiple levels, in the sternum, in the scapula and in the costal arches; all lesions were symptomatic, and surgical intervention was required; one of the lesions at the thoracic spine level evolved with spinal compression and acute neurological deficit, requiring urgent surgical intervention. Intraosseous hemangiomas represent < 1% of all bone tumors, having few reports of multifocal presentation in the axial and appendicular skeleton. In the literature review, no other case of aggressive multifocal intraosseous hemangioma with this presentation was found, including associated neurological symptoms in the same case.

β-elemene affects angiogenesis of infantile hemangioma by regulating angiotensin-converting enzyme 2 and hypoxia-inducible factor-1 alpha.

Infantile hemangioma (IH) is the most common benign vascular tumor resulting from the hyper-proliferation of vascular endothelial cells. In treatment of various tumors including IH, β-elemene, a compound extracted from Rhizoma zedoariae, has been reported to have anti-tumor effect. However, the underlying mechanisms of β-elemene in hemangioma have remained uninvestigated. In this presented study, functional analysis showed that low concentrations of β-elemene promoted the proliferation, migration and tube formation of human hemangioma endothelial cells (HemECs), while high concentrations of β-elemene produced inhibitory effects. Further, we also found that angiotensin-converting enzyme 2 (ACE2) expression was down-regulated at both mRNA and protein levels, while hypoxia-inducible factor-1 alpha (HIF-1-α) was up-regulated in infantile hemangiomas tissues and HemECs at both mRNA and protein levels. This result suggested that ACE2 and HIF-1-α play roles in IH. ACE2 expression was down-regulated with the treatment of β-elemene at different dosage point. Interestingly, the expression of Vascularendothelialgrowthfactor-A (VEGFA) increased with treatment of low concentrations of β-elemene in HemECs, in contrary, the expression of VEGFA expression decreased with treatment of high concentrations of β-elemene. Moreover, if the concentration of β-elemene reached 40μg/ml or higher, the expression of HIF-1-α decreased. Taken together, our data indicated that the different effects of β-elemene on the proliferation, migration and angiogenesis of hemangioma at different concentrations: The ACE2 signaling pathway dominates with treatment of low concentrations of β-elemene, stimulating the expression of downstream VEGFA to promote the angiogenesis of hemangioma; under the condition of high concentrations of β-elemene, the HIF-1-α signaling pathway inhibits the expression of VEGFA and further inhibits the angiogenesis of hemangioma.

A Comparision of Nitroglycerine and Dexmedetomidine for Controlled Hypotension in Endoscopic Resection of Juvenile Nasopharyngeal Angiofibroma

Background: Juvenile nasopharyngeal angiofibroma(JNA) is a locally invasive benign vascular tumor, controlled hypotension is used to facilitate endoscopic resection. Nitroglycerine and Dexmedetomidine were compared as hypotensive agents in this study. Methods : Ethics Committee has approved the study which was conducted at Government ENT hospital, Hyderabad during 2016 – 2018.Study included 40 patients divided into two groups, Group D (Dexmedetomidine) n=20,1µg/kg over 15 min followed by a maintenance infusion at 0.5µg/kg/hr,GroupN(Nitroglycerine) n=20- Nitroglycerine @ 0.5µg/kg/min and titrated in doses 0.5-5µg/kg/min for target blood pressure respectively. Results : A statistically significant difference was observed in the study in mean pulse rate between two groups Group D-66.09±2.83, Group N-86.59±4.24, p value 0.0001. Blood loss was lower in Group D-310.71±140.58 compared to Group N 482.61±141.42 , and is statistically significant (p value 0.0004). SBP, DBPand MAP were comparatively lower in Group D throughout the surgery. Conclusions: Dexmedetomidine is a better hypotensive agent with favourablehemodynamics and significantly less blood loss when compared to Nitroglycerine.

Intestinal intussusception and progressive anemia due to pyogenic granuloma of the ileum: a case report

BackgroundPyogenic granuloma is a benign vascular tumor, usually occurring on the skin or in the oral cavity. Small intestinal pyogenic granuloma is extremely rare, but intestinal intussusception due to the tumor is even rarer. Only 3 cases have been reported in the English literature at this writing.Case presentationAn 86-year-old woman presented with abdominal pain and vomiting. Laboratory data discovered anemia. Contrast-enhanced computed tomography revealed small bowel obstruction due to intestinal intussusception. After decompression by long tube for 1 week, the obstruction did not improve and the anemia got worse. Therefore, laparoscopic assisted small bowel resection was performed as a diagnostic therapy. Pathology confirmed the diagnosis of pyogenic granuloma. The postoperative course was uneventful and the patient was discharged 10 days after surgery.ConclusionsWe experienced a case of intestinal intussusception and progressive anemia due to pyogenic granuloma of the ileum. Although the condition is extremely rare, surgeons must take into consideration the tumor in similar cases, and complete surgical resection is required.

Paediatrics: how to manage infantile haemangioma.

Infantile haemangiomas (IHs) are relatively common benign vascular tumours found in the paediatric population. They have varying sizes and involve different depths in the skin leading to various colours, shapes and textures. Although considered harmless in most cases, they may lead to life-threatening complications or cause permanent disfigurations and organ dysfunction. For problematic IHs, the treatment options include oral and topical beta-blockers, systemic corticosteroids, laser treatment, and surgery. In this narrative review, the treatment options for problematic IH are compared and delivered concisely to facilitate the clinical decisions from practitioners, including those in primary care settings. Oral propranolol is currently considered the first-line intervention for problematic IHs. For superficial lesions, there is robust evidence for the use of topical timolol maleate. Systemic corticosteroids are sometimes used in specific situations such as resistance or contraindications to beta-blockers. Surgical excision can be considered in cases requiring urgent intervention such as airway obstruction; this can be done alongside laser therapies for the removal of residual tissue or when reconstructing areas of deformity. The combination of multiple treatment modalities may lead to a more rapid clinical response.

Solid vs. cystic predominance in posterior fossa hemangioblastomas: implications for cerebrovascular risks and patient outcome

Hemangioblastomas (HGBs) are highly vascular benign tumors, commonly located in the posterior fossa, and 80% of them are sporadic. Patients usually present with features of raised intracranial pressure and cerebellar symptoms. HGB can be classified as either mostly cystic or solids. Although the solid component is highly vascularized, aneurysm or hemorrhagic presentation is rarely described, having catastrophic results. We identified 32 consecutive patients with posterior fossa HBG who underwent surgery from 2008 through 2020 at our medical center. Tumors were classified as predominantly cystic or solid according to radiological features. Resection was defined as gross total (GTR) or subtotal (STR). During the study period, 32 posterior fossa HGBs were resected. There were 26 cerebellar lesions and 4 medullar lesions, and in 2 patients, both structures were affected. Predominant cystic tumors were seen in 15 patients and solids in 17. Preoperative digital subtraction angiography (DSA) was performed in 8 patients with solid tumors, and 4 showed tumor-related aneurysms. Embolization of the tumors was performed in 6 patients, including the four tumor-related aneurysms. GTR was achieved in 29 tumors (91%), and subtotal resection in 3 (9%). Three patients had postoperative lower cranial nerve palsy. Functional status was stable in 5 patients (16%), improved in 24 (75%), and 3 patients (9%) deteriorated. One patient died 2 months after the surgery. Two tumors recurred and underwent a second surgery achieving GTR. The mean follow-up was 42.7 months (SD ± 51.0 months). Predominant cystic HGB is usually easily treated as the surgery is straightforward. Those with a solid predominance present a more complex challenge sharing features similar to arteriovenous malformations. Given the important vascular association of solid predominance HGB with these added risk factors, the preoperative assessment should include DSA, as in arteriovenous malformations, and endovascular intervention should be considered before surgery.

Morphological, genetic and clinical correlations in infantile hemangiomas and their mimics.

Infantile hemangiomas (IHs) are the most frequent pediatric benign vascular tumors, with a reported incidence of 5% to 10%. They have self-limiting evolution pattern divided into a growth phase in the first 12 months and a regression one, that may take up to 10 years. Occasionally, hemangiomas might lead to local or systemic complications, depending on their morphological characteristics. The first line of treatment is β-blockers, such as Propranolol, Timolol, Nadolol, administered either locally or systemically. Newer therapeutic strategies involving laser therapy and angiotensin-converting enzyme inhibitors are being studied, while older treatment modalities like corticosteroids, Imiquimod, Vincristine, Bleomycin and Interferon-α have become second line therapy options. Before establishing the appropriate treatment, clinical, histological, and imaging investigations are required.

Capillary Hemangioma of the External Auditory Canal Extending to Middle Ear and Mastoid Cavity: A Rare Case Report with Review of Literature.

Capillary hemangioma involving external auditory canal, middle ear and mastoid cavity is a very rare entity. Due to infrequent incidence but multiple overlapping clinical and radiological features amongst some common middle ear pathologies this benign vascular tumour often can be misdiagnosed. Histopathological report helps us to get definitive diagnosis. 40years old female presented with diminished hearing and mass in right ear cavity for last 3years. The otoscopic examination of right ear showed a pinkish polypoidal mass at the external auditory canal and tympanic membrane could not be visualized. Patient had a history of cortical mastoidectomy 2years back for the similar problem. HRCT temporal bone revealed a single cavity with soft tissue density content in right mastoid cavity which was extending into right middle ear and external ear. Contrast Enhanced MRI showed a long polypoidal heterogeneously but strong enhancing T2 and STIR hyperintense lesion within mastoid antrum extending into middle ear cavity and external auditory canal forming an aural polyp. Excision of the mass was done by canal wall down mastoidectomy and also type IIIc tympanoplasty performed in same sitting. There is no recurrence noted after 6months of follow up and hearing improvement noticed. Capillary haemangioma of mastoid antrum extending to middle ear and external auditory canal requires surgical excision as preferred treatment modality, pre-operative imaging guide us to choose the preferred surgical approaches.