Benign Vascular Tumors Research Articles

A CASE REPORT ON INFANTILE HEMANGIOMA

Infantile hemangiomas are the most common benign vascular tumors in childhood. They usually appear as macular or raised bluish nodular swelling.It most commonly involves head and neck region which results in tissue damage, ulceration, bleeding, functional impairment and disfigurement. We present a case of 4-year-old patient with infantile hemangioma in the left side of face.

Prenatal diagnosis of cavernous hemangioma in a specialist hospital in Port Harcourt, South South Nigeria. A case report and review of the literature

Congenital cavernous hemangiomas are rare benign vascular tumors that are present at birth. They can occur in various organs of the body. Prenatal diagnosis can be done using ultrasound and magnetic resonance imaging. Very few cases have been described in the literature in Port Harcourt, south-south Nigeria. We present the case of a 33-year-old P3+1 mother who presented for a third-trimester fetal ultrasound scan at 35weeks gestation in a specialist hospital in Port Harcourt. The ultrasound scan showed a live fetus with a huge multiloculated soft tissue mass measuring ~ 11.4cmx8.7cm with areas of minimal flow located in the anterior aspect of the neck, chest, and abdomen extending to the ipsilateral arm and forearm sparing the fingers. No other gross abnormalities were noted. A magnetic resonance imaging (MRI) study was also done to further characterize the aforementioned mass. A diagnosis of congenital hemangioma of the neck, chest, and upper limb was made. The pregnancy progressed and a planned cesarean section was done at 38 weeks with the birth of a live 3.5kg female baby. An excision biopsy of the mass was done at the age of 2 months confirming the diagnosis of a congenital cavernous haemangioma.

Tumor de Masson en la mama: un reto diagnóstico

Papillary endothelial hyperplasia (PEH) or Masson's tumor is a rare benign vascular tumor that usually appears in the soft tissues of the head and neck, trunk and extremities, being extremely rare in the breast. Its diagnosis can be a challenge, especially in the follow-up of patients with previous disease of breast carcinoma. We present the case of a 65-year-old patient, with a history of bilateral breast cancer and reconstruction with implants, who presented a Masson's tumor during follow-up. An ultrasound scan was performed, showing a well-circumscribed mass in the left breast, located in the posterior contour of the implant. Subsequently, magnetic resonance imaging (MR) depicted an enhancing tumor, without infiltration of adjacent structures. Finally, the definitive anatomopathological diagnosis was obtained after surgical excision.

Infantile hemangiomas screening modalities for primary care physicians.

Infantile hemangiomas are the most common benign vascular tumors in children. They present a characteristic natural history of spontaneous involution after a phase of initial proliferation. A small but significant minority demonstrates incomplete regression or complications and requires prompt intervention. Prediction of the evolution of infantile hemangiomas is challenging because of their morphological and behavioral heterogeneity. The decision between referral for treatment and observation is sometimes difficult, especially among non-expert physicians, with the risk of missing the period for optimizing outcomes in case of delayed intervention. The aim of this review is to update our knowledge, especially of the primary care providers, regarding the ongoing difficulties of the early clinical evaluation of infantile hemangiomas, and to outline the importance of current practical scoring tools for the identification of the lesions which require expert consultation and referral.

Hemangioma of the Buccal Mucosa of the Cheek Presenting as a Dermoid Cyst: A Case Report

This case report presents a 73-year old Asian female with a presumed dermoid cyst in the buccal mucosa of the left cheek that was visible and embarrassing to her. Histopathology revealed the mass to be consistent with a cavernous hemangioma. Hemangiomas are benign vascular tumors of mesenchymal origin. It is usually present at birth but can develop later in life. It commonly occurs in the head and neck region, but rarely in the oral cavity.

Anterior Mediastinal Capillary Hemangioma: A Very Rare Finding

Hemangiomas are benign vascular tumors. Their location in the mediastinum represents a very rare finding. The definite diagnosis is sometimes difficult to make due to nonspecific features on single-phase contrast-enhanced computed tomography (CT) images. Our case shows a 53-year-old male that had presented with chest pain and dyspnea. On the dynamic CT study, there was a mediastinal mass that showed peripheral nodular enhancement on early phase images and progressive centripetal fill-in on delayed phase images. Hemangioma was preoperatively diagnosed on the basis of this characteristic CT appearance.

Bleeding cavernous hemangioma of lower lip: a case report.

A 67-year-old female presented with bleeding from the lower lip. The bleeding increased with palpation. A clinical diagnosis of hemangioma of the lower lip was made. It was difficult to localize with ultrasound. It was explored and excised successfully. Hemangioma can be superficial, deep, or mixed. Generally, hemangioma involute spontaneously. Bleeding hemangioma causing functional disturbances need to be treated, for which various modalities are available, including excision. Hemangioma of the lip is a benign tumor of vascular origin. In selected cases, excision can be performed.

Somatic genetic reversion of large duplication event in IL2RG in siblings

BackgroundDeleterious mutations in the common gamma chain (γC, CD132) are associated with X-linked severe combined immunodeficiency (X-SCID) characterized by a T-B+NK- phenotype. Hypomorphic variants in the IL2RG gene have also been associated with a milder or atypical SCID phenotype. Milder phenotypes can also occur when somatic genetic rescue occurs in the setting of severe IL2RG mutations. Spontaneous somatic variants particularly in hematologic disorders can occur particularly in genes with known selection pressure including IL2RG. ResultsHere, we report the clinical and molecular findings from two brothers with atypical X-SCID phenotype including hypogammaglobulinemia, elevated IgM, T cell lymphopenia and extensive verruca plana. One brother required splenectomy secondary to a benign vascular tumor and had recurrent pancreatitis consistent with primary sclerosing cholangitis. Both brothers developed recurrent suppurative pneumonia resulting in early bronchiectasis in their first decade. Next generation sequencing initially was negative, yet reanalysis found a large duplication event in exon 3 of IL2RG gene with somatic genetic rescue events that occurred twice in two brothers. Functional studies revealed a unique mosaic pattern in each affected sibling when stimulating peripheral blood mononuclear cells with IL-2 or IL-21 resulting in bimodal phospho-STAT5 or phospho-STAT3 respectively. The genetic analysis revealed the maternal allele was heterozygous as expected, although the ratio of these events and clonal analysis revealed cell type skewing. ConclusionOur data demonstrate an atypical SCID phenotype in two brothers secondary to the combination of a large duplication event in the IL-2RG gene partially rescued by somatic genetic rescue. Although previous somatic reversion cases have been reported involving the IL2RG genetic locus, our cases demonstrate a large duplication reversion event with recurrence. Our case highlights the unique selection pressure to amplify rare genetic events at the IL2RG locus.

Intraorbital Cavernous Hemangioma: Rare Cause of Exophthalmos

Nasopharyngeal angiofibroma refers to a locally aggressive benign vascular tumor composed of vasogenic and myofibroblastic elements, representing between 0.05% and 0.5% of all head and neck tumors. Here, we report a case of a 21-year-old patient with no particular personal or familial pathological history, who presented with recurrent epistaxis and anterior rhinorrhea and headaches for 1 year. Nasofibroscopy revealed a mass filling the entire left nasal cavity, streaked with blood. A face MRI revealed a highly vascularized and lytic nasopharyngeal tumor infiltrating the deep left lateral facial spaces with the beginning of endocranial infiltration, supplied by the homolateral maxillary artery, initially suggestive of a nasopharyngeal fibroma. Preoperative arteriography showed a voluminous vascular blush at the arterial time to the nasopharyngeal projection with branches arising from the left external and internal carotid arteries and multiple arterial shunts (between the left internal and maxillary carotid arteries), thus contradicting embolization. Early diagnosis is important as it is associated with a high risk of morbidity, but advances in imaging and surgical treatment methods have modified the sites associated with high morbidity risk.

Aggressive Tumor and Challenging Location: A Case Report of Nasopharyngeal Fibroma

Nasopharyngeal angiofibroma refers to a locally aggressive benign vascular tumor composed of vasogenic and myofibroblastic elements, representing between 0.05% and 0.5% of all head and neck tumors. Here, we report a case of a 21-year-old patient with no particular personal or familial pathological history, who presented with recurrent epistaxis and anterior rhinorrhea and headaches for 1 year. Nasofibroscopy revealed a mass filling the entire left nasal cavity, streaked with blood. A face MRI revealed a highly vascularized and lytic nasopharyngeal tumor infiltrating the deep left lateral facial spaces with the beginning of endocranial infiltration, supplied by the homolateral maxillary artery, initially suggestive of a nasopharyngeal fibroma. Preoperative arteriography showed a voluminous vascular blush at the arterial time to the nasopharyngeal projection with branches arising from the left external and internal carotid arteries and multiple arterial shunts (between the left internal and maxillary carotid arteries), thus contradicting embolization. Early diagnosis is important as it is associated with a high risk of morbidity, but advances in imaging and surgical treatment methods have modified the sites associated with high morbidity risk.

Our experience with propranolol for infantile hemangioma.

The most frequent benign vascular tumor in children is infantile hemangioma (IH). For severe IHs, propranolol has become the first-line Treatment. Despite the fact that several studies have comprehensive therapy regimens, including the best time to start Treatment, dosage, visit frequency, and treatment duration, there is still controversy about the best time to start and stop propranolol medication. Between January 2016 and February 2019, dermatologists experienced hemangioma treatment and recommended propranolol treatment for 232 IHs. A total of 90 patients completed the treatment process after undergoing a color Doppler ultrasound test. Propranolol uniquely affects each IH. Ninety patients were divided into two groups in this study: entire regression (n=40) and partial regression (n=50). The entire regression group's initial treatment period (4.3±2.97 months) was substantially shorter than the partial regression group's (5.2±4.57 months) (p<0.05). Between the entire regression group (23.4±12.8 months) and the partial regression group (24.5±16.6 months), there was no significant difference in time to reduce propranolol. The partial regression group (32.9±25.3month) had a lengthier treatment course than the entire regression group (23.4±13.7 months) (p<0.05). The partial regression group (22%), like the entire regression group, had a higher recurrence rate (5%). The overall proportion of hemangiomas on the face (particularly periocular hemangioma) in the regression group was greater than in the control group. The entire regression group's initial treatment time was significantly shorter than the partial regression group's. As a result, as soon as a hemangioma is discovered, it should be treated. To determine the appropriate time to reduce propranolol, we must evaluate the patient's age and the percentage of tumor regression. Periocular hemangioma may have a better prognosis than other types. Given the small number of patients in our study, we will need to do more research in the future to confirm our findings.

Analysis of cytokine profiles in sera of single and multiple infantile hemangioma.

Infantile hemangiomas (IH) are benign vascular tumors that are common in infancy. They vary in growth, size, location, and depth, and although most lesions are relatively small, approximately one fifth of patients have multiple lesions. Risk factors for IH include female sex, low birth weight, multiple gestation, preterm birth, progesterone therapy, and family history, but the mechanism that causes multiple lesions is unclear. We hypothesized that blood cytokines are involved as a cause of multiple IHs, and tried to prove this using sera and membrane arrays from patients with single and multiple IHs. Serum samples were obtained from five patients with multiple lesions and four patients with a single lesion, none of which had received any treatment. Serum levels of 20 cytokines were measured using human angiogenesis antibody membrane array. Four of the 20 cytokines (bFGF, IFN-γ, IGF-I, and TGF-β1) were higher in the patients with multiple lesions than in those with single lesion, with statistically significant difference (p < 0.05). Notably, signal for IFN-γ was evident in all cases with multiple IHs, but was absent in cases with single IH. Although not significant, there was mild correlation between IFN-γ and IGF-I (r=0.64, p=0.065), and between IGF-I and TGF-β1 (r=0.63, p=0.066). bFGF levels were strongly and significantly correlated with the number of lesions (r=0.88, p=0.0020). In conclusion, blood cytokines could act as a cause of multiple IHs. This is a pilot study with a small cohort, so further large-scale studies are necessary.

Development of a digital imaging analysis system to evaluate the treatment response in superficial infantile hemangiomas.

Superficial infantile hemangiomas (IH) are benign vascular tumors common in children characterized by bright red "strawberry" lesions on the skin. In order to optimize the treatment for this disease, there is a need to develop objective tools to assess treatment response. Since a color change in the lesion is a good indicator of treatment response, we have developed a digital imaging system to quantify the values of red, green, and blue (RGB) difference and RGB ratio between the tumor and normal tissue to take into account the variations in color between different skin types. The efficacy of the proposed system in assessing treatment response in superficial IH was evaluated in relation to established visual and biochemical tools used to grade hemangiomas. As the treatment progressed, the RGB ratio was almost 1, while the RGB difference was close to 0, which indicates a good response to treatment. There was a strong correlation between the RGB score and the other visual grading systems. However, the correlation between the RGB scoring system and the biochemical method was weak. These findings suggest that the system can be used clinically to objectively and accurately evaluate disease progression and treatment response in patients diagnosed with superficial IH.

Rectal Bleeding in a Child Revealing a Cavernous Hemangioma of the Sigmoid

Hemangiomas are the most common benign vascular tumors in the pediatric age group. They originate from embryonic remnants of unipotent angioblastic cells. Although hemangiomas may occur anywhere within the abdomen, including the solid organs, hollow viscera, ligaments, and abdominal wall, the liver is the most common site. Cavernous hemangiomas of the sigmoid colon are uncommon vascular malformations usually found in young adults with a long history of episodic and painless rectal bleeding. Imaging plays an essential role in diagnosis and post-therapeutic follow- up. The diagnosis of certainty remains histological and the treatment is essentially surgical. We report a case of cavernous hemangioma of the sigmoid diagnosed in a 5-year-old boy admitted to our hospital for investigation of repeated episodes of rectal bleeding. The diagnosis was confirmed after a histological examination.

Timolol For the Treatment of Infected Pyogenic Granuloma (pg) Over the Nose.

Pyogenic granuloma (PG) is a benign vascular neoplasm, which exhibits several clinical features suggestive of reactive neovascularization. It is common in children and young adults, with a slight predominance in women. PG may occur spontaneously or may result from local trauma. Histologically the lesion of pyogenic granuloma. Proliferating capillaries are often grouped into lobules by dense fibrous bands, hence the synonym lobular capillary hemangioma The lesion is often clutched by an epithelial. On dermoscopy Red homogenous areas intersected by whitish lines – most common in pyogenic granuloma. There are no standard guidelines for the management of PG So invasive or noninvasive approach. Key Message Topical timolol therapy may be the first line of treatment for infected pyogenic granulomas; no adverse systemic effects were reported, over delicate areas like the nose - face on young children, older people, where invasive methods are not preferred, topical timolol may be a therapy option. Objectives to determine whether topical timolol is safe and effective for treating Infected pyogenic granulomas.. Methods We report an A 15-year-old female patient came in with a history of a single, rapidly expanding red lesion on the left side of her nose that had been developing in size for more than a month and was accompanied by minor pain and itching. In addition to unilateral purulent discharge from inside the nose, there was a history of a mild bleeding episode a few days before to consultation. The patient disclosed that she had her nose pierced a month earlier. Suggesting that infected pyogenic granuloma (PG) may coexist. The patient was prescribed timolol twice daily and trimethoprim/polymyxin four times daily with a pyogenic granuloma as the presumptive diagnosis. Results Significant improvement was noted with topical timolol with reduction of the lesion size decrease of bleeding tendency and here symptoms had resolved. Limitations we observed Effects of treatment only in 8 weeks. Conclusion Infected pyogenic granulomas may benefit from topical timolol therapy Longer studies are needed to evaluate resolution and recurrence Rates.

Mortality predictive factors in congenital hepatic hemangioma: a case-control study.

CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality. • Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening. • Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients.

Cystic pancreatic lymphangioma: a case report

BackgroundLymphangiomas are benign vascular tumors arising from the lymphatic system. They commonly affect the head and neck regions. Pancreatic involvement is extremely rare. Even though they are rare, it should be considered as a differential diagnosis for patients who present with pancreatic mass.Case presentationWe report the case of a 6-year-old African male patient who presented with abdominal mass and dull aching pain of 6 months duration. He was examined and underwent excision, with biopsy showing pancreatic lymphangioma.ConclusionThese tumors are benign and slow growing and have potential to regress spontaneously. Thus, most literature agrees that surgical interventions should be reserved for symptomatic cases.

Recurrent FOSL1 rearrangements in desmoplastic fibroblastoma.

The FOS gene family has been implicated in tumourigenesis across several tumour types, particularly mesenchymal tumours. The rare fibrous tumour desmoplastic fibroblastoma is characterised by overexpression of FOSL1. However, previous studies using cytogenetic and molecular techniques did not identify an underlying somatic change involving the FOSL1 gene to explain this finding. Prompted by an unusual index case, we report the discovery of a novel FOSL1 rearrangement in desmoplastic fibroblastoma using whole-genome and targeted RNA sequencing. We investigated 15 desmoplastic fibroblastomas and 15 fibromas of tendon sheath using immunohistochemistry, in situ hybridisation and targeted RNA sequencing. Rearrangements in FOSL1 and FOS were identified in 10/15 and 2/15 desmoplastic fibroblastomas respectively, which mirrors the pattern of FOS rearrangements observed in benign bone and vascular tumours. Fibroma of tendon sheath, which shares histological features with desmoplastic fibroblastoma, harboured USP6 rearrangements in 9/15 cases and did not demonstrate rearrangements in any of the four FOS genes. The overall concordance between FOSL1 immunohistochemistry and RNA sequencing results was 90%. These findings illustrate that FOSL1 and FOS rearrangements are a recurrent event in desmoplastic fibroblastoma, establishing this finding as a useful diagnostic adjunct and expanding the spectrum of tumours driven by FOS gene family alterations. © 2022 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

Rhinoethmoidal mixt cavernous-capillary hemangioma – endoscopic transnasal resection. Case report

Hemangiomas are benign vascular tumors which originate in the skin, mucosae and deep structures such as bones, muscles or glands. Hemangiomas have capillary, cavernous, mixed, and hypertrophic subtypes. These neoplasms rarely arise in the nasal cavity, and when they appear here, they are predominantly capillary and are found attached to the nasal septum. Cavernous hemangiomas, on the other hand, are more likely to be found on the lateral wall of the nasal cavity. This article presents a case of a mixt cavernous-capillary hemangioma, arising from the mucosa of the posterior and superior nasal septum, in a 62-year-old female, successfully treated by the transnasal endoscopic excision technique.

Treatment of Symptomatic Focal Hepatic Hemangioma with Propranolol in Neonates: Is It Efficient?

Hepatic hemangiomas (HH) - classified into congenital hepatic hemangiomas (CHH) or infantile hepatic hemangiomas (IHH) - are benign vascular tumors that are mainly asymptomatic, but may cause clinical problems that require treatment. While focal, multifocal, and diffuse IHH are responsive to propranolol treatment, CHH is mainly focal and thought to be resistant to treatment with propranolol. The clinical and imaging distinctions between CHH and IHH in cases of focal lesions can be challenging, while histopathological distinction is mostly lacking in the clinical setting. We report 4 neonatal symptomatic cases of focal HH treated with propranolol, with partial or complete resolution of the tumor, and the positive hemodynamic effect of propranolol in one case. We believe that although clear differentiation cannot be achieved between CHH and IHH without histopathological examination in cases of focal HH in neonates, propranolol treatment should be attempted in symptomatic cases since its benefits outweigh the possible small risk of side effects of propranolol.