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Intramuscular haemangiomas (IH) are rare lesions, accounting for less than 1% of all haemangiomas. This article presents the case of a woman in her 40s with a swelling in her left cheek. CT revealed an intramuscular lesion within the masseter, suggestive of a venous malformation. Surgical treatment was carried out with intraoral access to the lesion, allowing for complete removal, resulting in temporary swelling and trismus. There was no facial paralysis. We discuss information on IH in the head and neck and their surgical approaches., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Gamma knife radiosurgery (GKRS) for orbital cavernous hemangioma (OCH) has emerged as a promising method due to its significant clinical improvement and low incidence of complications. This study aimed to evaluate the safety and efficacy of GKRS for the treatment of OCH., Methods: In accordance with the PRISMA framework, we searched PubMed, Cochrane Central, and Embase for studies reporting outcomes of GKRS for OCH. Studies reporting complications, visual improvement, proptosis, tumor reduction rate, and tumor progression rate for OCH following GKRS were included., Results: Six studies, out of 1856 search results, with 100 patients were included. Among them, only 5 minor complications were related to GKRS, including 3 with orbital pain and 2 with periorbital chemosis. Thus, the complication rate was 13% (95% CI, 7-25%). Visual acuity and visual field improvement rates after GKRS were 80% (95% CI, 63-96%) and 71% (95% CI, 47-95%) respectively. Proptosis improved in 94% of cases (95% CI, 83-100%). The tumor reduction rate was 77% after GKRS (95% CI, 69-85%)., Conclusion: GKRS for OCH appears to be a safe technique, as evidenced by the rate of clinical improvement and radiological improvement. However, studies are limited by an absence of a control group. Additional studies are needed to evaluate the relative efficacy of GKRS as compared with alternative surgical modalities for OCH., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

To explore the advantages and disadvantages of 3D Slicer reconstruction and 3D printing localization combined with transcranial neuroendoscope in the surgical treatment of deep cerebral micro cavernous hemangiomas. Method The clinical data of patients with deep cerebral micro cavernous hemangiomas treated by our hospital from June 2022 to February 2023 using 3D Slicer reconstruction and 3D printing localization technology combined with transcranial endoscopic surgery were retrospectively analyzed. A total of 5 cases with complete data were collected, including 2 males and 3 females, aged 9-59 years. All 5 patients had deep supratentorial cavernous hemangiomas with a diameter of less than 1.5 cm, and had clinical symptoms such as headache or epilepsy, and had been diagnosed by CT or MRI. Repeated bleeding from small cavernous hemangiomas in the deep brain can lead to clinical symptoms such as recurrent headache and epilepsy, and is required surgical treatment. However, cavernous hemangiomas often have smaller lesions and are difficult to locate in the deep part. Without neuronavigation, surgery can become extremely difficult. Our team's newly developed 3D Slicer reconstruction and 3D printing localization technology which could provide new options for surgical treatment of small cavernous hemangiomas or other small lesions in the deep brain, but its accuracy and safety still need to be verified by further clinical research., (© 2024. The Author(s).)

Competing Interests: AC is a consultant for ERBE; RM is a consultant for ERBE, Fujifilm, 3DMatrix and Boston Scientific; AR is a consultant for Medtronic, ERBE, Fujifilm and Olympus; Other authors nothing to declare.

Hemangioma is a common vascular neoplasm that arises in the head and neck regions but is rare in the petrous bone. We report the first case of a solitary cavernous hemangioma in the mastoid antrum. A 68-year-old woman visited our hospital with a complaint of tinnitus without any other symptoms. Tinnitus of the right ear occurred especially when the patient yawned or swallowed. Both tympanic membranes appeared normal on otoscopic examination. On pure-tone audiometry, mild hearing loss up to 25 dB was detected in the right ear. Temporal bone computed tomography revealed a 7.0 mm × 4.5 mm × 5 mm, solitary soft tissue mass in the aditus ad antrum. Excisional biopsy was performed under general anesthesia through the canal wall as in a mastoidectomy. The mass was completely removed without any bleeding or ossicular chain damage. The mass was confirmed as a cavernous hemangioma. During follow-up, the patient's tinnitus and right low-tone hearing loss improved. No solitary hemangioma of the mastoid antrum has been reported previously. Surgical excision of the lesion appears to be proper treatment to achieve pathologic confirmation along with resolution of symptoms.

Vascular conjunctival tumors are uncommon, usually benign lesions. Here, we report a 65-year-old male who presented with a nasal interpalpebral bulbar firm, oval, and dark-brown and non-mobile conjunctival mass. The systemic and ocular examinations including fundoscopy and gonioscopy were within normal limits. With a diagnosis of conjunctival malignant melanoma (CMM), the patient underwent excisional biopsy, partial sclerectomy, and cryotherapy. Microscopic examination revealed dilated vascular channels filled by red blood cells, separated by fibrous interstitium and no sign of malignancy. Therefore, a diagnosis of conjunctival cavernous hemangioma was made. Our case demonstrates that the conjunctival cavernous hemangioma can mimic malignant conjunctival lesions as well as extrascleral extension of uveal melanoma or uveal prolapse due to scleral necrosis., (© 2024. The Author(s).)

Extra-axial cavernous hemangiomas (ECHs) are complex vascular lesions mainly found in the spine and cavernous sinus. Their removal poses significant risk due to their vascularity and diffuse nature, and their genetic underpinnings remain incompletely understood. Our approach involved genetic analyses on 31 tissue samples of ECHs employing whole-exome sequencing and targeted deep sequencing. We explored downstream signaling pathways, gene expression changes, and resultant phenotypic shifts induced by these mutations, both in vitro and in vivo. In our cohort, 77.4% of samples had somatic missense variants in GNA14, GNAQ, or GJA4. Transcriptomic analysis highlighted significant pathway upregulation, with the GNAQ c.626A>G (p.Gln209Arg) mutation elevating PI3K-AKT-mTOR and angiogenesis-related pathways, while GNA14 c.614A>T (p.Gln205Leu) mutation led to MAPK and angiogenesis-related pathway upregulation. Using a mouse xenograft model, we observed enlarged vessels from these mutations. Additionally, we initiated rapamycin treatment in a 14-year-old individual harboring the GNAQ c.626A>G (p.Gln209Arg) variant, resulting in gradual regression of cutaneous cavernous hemangiomas and improved motor strength, with minimal side effects. Understanding these mutations and their pathways provides a foundation for developing therapies for ECHs resistant to current therapies. Indeed, the administration of rapamycin in an individual within this study highlights the promise of targeted treatments in treating these complex lesions., Competing Interests: Declaration of interests The authors declare no competing interests., (Copyright © 2024 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Hemangiomas account for only 0.4% to 0.6% of all parotid tumors, making them extremely rare in adults. Unlike pediatric parotid hemangiomas, those in adults typically present as asymptomatic swellings of the parotid, have no skin discoloration, and usually do not regress spontaneously. Therefore, an accurate diagnosis of parotid hemangiomas in adults before surgical excision is generally challenging. Herein, we present 3 cases of adult parotid hemangiomas. The patients all received parotidectomies with tumor resection. Histopathological analysis of the resected specimens revealed numerous dilated, thin- or thick-walled (small, large, or variably sized) vessels lined with flattened endothelial cells. A diagnosis of cavernous hemangioma of the parotid gland was established only after the histopathological analysis. Parotid cavernous hemangiomas in adults are rare and often misdiagnosed before surgical resection. Clinical presentation and imaging features on ultrasonography, computed tomography, magnetic resonance imaging, and angiography may support an accurate preoperative diagnosis. Surgical resection proved a suitable treatment approach for our 3 cases., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Competing Interests: The authors have no conflicts of interest to disclose.

Background: Cavernous sinus cavernous malformations (CSCMs) is a vascular malformation of the cavernous sinus. Nowadays, there is an increasing preference to withhold using the terms cavernoma or cavernous hemangioma in order to stop considering these lesions as vascular neoplasms. These lesions are highly vascularized making surgical resection a challenge, mainly in endoscopic approaches. We present a case of this tumor treated in our institution with an endoscopic endonasal approach and incomplete resection. Because of the strenuous resection through this approach, we systematically reviewed the reported endoscopic cases of CSCMs to determine their intraoperative complications, results and tumor features., Methods: Using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, one database (PubMed) and crossed references were queried for CSCMs from 1948 to 2020. Data regarding demographic features, clinical presentation, MRI features, surgical results and overall pathology features extracted., Results: Eighteen patients were selected (including our case). The mean age was 50.4 ± 14 years. Pituitary dysfunction and cavernous sinus nerve compression were the most reported symptoms. Only five cases (27%) reported a gross total resection (GTR) through endoscopic endonasal approach. Intraoperative bleeding was the most frequent intraoperative complication., Conclusion: We present a comprehensive analysis of every reported CSCM treated through endoscopic approach. Partial or subtotal resection are the most used techniques because of the intraoperative bleeding and the adherence to surrounding structures. Radiotherapy is a very good option for patients with incomplete resections.

A 37-year-old female patient was admitted to the hospital with a large liver mass, diagnosed as hepatic inflammatory myofibroblastic tumour (HIMT), characterized by unique radiographic features and predominantly occurring in adults. HIMT consists of myofibroblast spindle cells infiltrated by plasma cells and/or lymphocytes, with an unclear aetiology linked to factors like infection and immune response. Treatment typically involves surgical resection, with chemotherapy or targeted therapy options for cases of incomplete resection or metastasis, emphasizing the need for precise diagnosis and tailored treatment strategies., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

The cervix of the uterus is a rare site for cavernous hemangiomas. Cervical hemangiomas are slow-growing tumors with characteristic histological findings, including dilated vessels with increased endothelial cells. Although their pathophysiology remains unclear, hormones are believed to play an important role in the development of these vascular tumors. They may be asymptomatic due to their small size, but they can cause gynecological and obstetrical complications, including abnormal uterine bleeding and impaired fertility. Due to their small size, conservative treatment is the first line of management. Hysterectomy is considered for refractory cases or for patients who are not of childbearing age. In this study, firstly, we presented a case of a 60-year-old postmenopausal female without any gynecological-related signs or symptoms with a polypoid nodule hanging over the anterior cervical wall through its stalk. The surgical biopsy revealed no signs of neoplastic changes, with the only notable finding being a benign vascular lesion representing a cavernous hemangiomatous cervical polyp. The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy and she is currently healthy without any further abnormal findings. Additionally, we provided a comprehensive review of 137 cases in the literature since 1883, detailing their characteristics, signs and symptoms, and pathology., (© 2023 International Federation of Gynecology and Obstetrics.)

Objectives: Hemangioma is a common soft tissue tumor that can be categorized mainly into cavernous, capillary, and venous subtypes. It usually develops in the head and neck and rarely in the external auditory canal (EAC)., Methods: A systematic review of patients with capillary hemangioma of the EAC was performed. A computerized systematic search was conducted in PubMed, Scopus, and Web of Science to extract relevant studies for a qualitative review., Results: A total of 38 patients were included in the systematic review, and half of them were men. Hearing loss was the most common presenting feature (n = 19; 50%), followed by aural fullness (n = 15; 39.4%). Cavernous hemangioma was the predominant type (n = 22, 57.8%). Endaural and transcanal surgical approaches were the most frequent, with ten (26.3%) and nine (23.6%) cases, respectively. Most of the patients (n = 31; 81.5%) had no recurrence., Conclusions: Hemangioma of the EAC may not be uncommon as healthcare professionals may expect. Hearing loss, aural fullness, and tinnitus are possible symptoms, but many patients remain asymptomatic. Surgical excision is the main effective management option, and complete resolution is the most prevalent outcome. Computed tomography assists in the diagnosis, but histopathological examination after resection is mandatory for the definitive diagnosis., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Cavernomas are histologically benign vascular malformations found at different sites in the brain. A rare site for such cavernomas, however, is the anterior optic pathway, comprising the optic nerve, chiasma, and optic tract-called optochiasmatic cavernomas (OCC). These lesions usually present with sudden onset or progressive vision loss, headache, and features mimicking pituitary apoplexy. In this paper, we describe a case of OCC operated at our center. We carry out an updated review of literature depicting cases of OCC, their clinical presentation, management, and postoperative complications. We also propose a novel classification system based on lesion location and further analyze these cavernoma types with respect to the surgical approach used and visual outcome. A 30-year-old lady had presented with a 3-week history of progressive bilateral vision loss and headache. Based on imaging, she was suspected to have a cavernous angioma of the chiasma and left optic tract. Due to progressive vision deterioration, the lesion was surgically excised using pterional craniotomy. Postoperatively, her visual symptoms improved, but she developed diabetes insipidus. Clinical and radiological follow-up has been done for 18 months after surgery. A total of 81 cases have been described in the literature, including the present case. Chiasmal apoplexy is the most common presentation. Surgical excision is the standard of care. Our analysis based on lesion location shows the most appropriate surgical approach to be used for each cavernoma type. Visual outcome correlates with the preoperative visual status. Visual outcome is good in patients presenting with acute chiasmal apoplexy, and when complete surgical excision is performed. The endonasal endoscopic approach was found to provide the best visual outcome. In addition to preoperative visual status, complete surgical excision predicts favorable visual outcomes in OCC. Our proposed classification system guides the appropriate surgical approach required for a particular location of the cavernoma., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Extra-axial cavernous hemangiomas (ECHs) are sporadic and rare intracranial occupational lesions that usually occur within the cavernous sinus. The aetiology of ECHs remains unknown., Methods: Whole-exome sequencing was performed on ECH lesions from 12 patients (discovery cohort) and droplet digital polymerase-chain-reaction (ddPCR) was used to confirm the identified mutation in 46 additional cases (validation cohort). Laser capture microdissection (LCM) was carried out to capture and characterise subgroups of tissue cells. Mechanistic and functional investigations were carried out in human umbilical vein endothelial cells and a newly established mouse model., Results: We detected somatic GJA4 mutation (c.121G>T, p.G41C) in 5/12 patients with ECH in the discovery cohort and confirmed the finding in the validation cohort (16/46). LCM followed by ddPCR revealed that the mutation was enriched in lesional endothelium. In vitro experiments in endothelial cells demonstrated that the GJA4 mutation activated SGK-1 signalling that in turn upregulated key genes involved in cell hyperproliferation and the loss of arterial specification. Compared with wild-type littermates, mice overexpressing the GJA4 mutation developed ECH-like pathological morphological characteristics (dilated venous lumen and elevated vascular density) in the retinal superficial vascular plexus at the postnatal 3 weeks, which were reversed by an SGK1 inhibitor, EMD638683., Conclusions: We identified a somatic GJA4 mutation that presents in over one-third of ECH lesions and proposed that ECHs are vascular malformations due to GJA4 -induced activation of the SGK1 signalling pathway in brain endothelial cells., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Abstract: A 65-year-old woman was referred to our hospital because of a liver lesion revealed by ultrasound and CT in an outside institution. 18 F-FDG PET/CT images revealed an FDG-avid lesion in the left lateral lobe of the liver. Malignancy cannot be excluded. A subsequent resection was performed to remove the lesion. Postoperative pathology was hepatic cavernous hemangioma. This case suggests that PET/CT findings of FDG-avid hepatic cavernous hemangioma could mimic malignancy., Competing Interests: Conflicts of interest and sources of funding: The authors declare no conflict of interest. This work was supported by National High Level Hospital Clinical Research Funding (grant 2022-PUMCH-B-071)., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Aims: To determine the incidence and risk factors for poor postoperative visual outcome (PPVO) after removal of orbital cavernous venous malformations (OCVMs)., Methods: Retrospective case-note and imaging review for patients undergoing excision of OCVMs, with estimation of odds-ratios (ORs) and relative risks (RRs) for visual loss in relation to the position of the mass, surgical approach, and patient factors., Results: The 290 patients (179 female; 62%) presented at a mean age of 46.4 years: 243/287 (85%) OCVMs were intraconal, with 213/243 (88%) located freely in the posterior two-thirds of the orbit, and 30/243 (12%) wedged tightly in the apex. PPVO was observed in 6.9% (20/290) patients, solely after removal of intraconal lesions, Univariate analysis showed increased risk with preoperative relative afferent pupillary defect (RAPD) (14/107 [13%]; RR 2.9; P = 0.011), apical lesions (9/30 [30%]; RR 5.8; P < 0.001), situated below optic nerve (15/115 [13%]; RR 3.3; P = 0.007), fibrous masses (14/78 [18%]; RR 6.7; P = 0.005), or intraoperative diastolic blood pressure below 50mmHg (10/64 [16%]; RR 2.8; P = 0.007). Multivariate analysis found apical extension (OR 4.9; P = 0.036) and fibrous lesions (OR 10.0; P = 0.035) as strongest predictors for PPVO. The incidence of complete visual loss (no light perception) was 4.1% (12/290); half of these patients had preoperative acuity of counting fingers or worse, 8 (67%) had RAPD, 7 (58%) wedged apical lesions, and 8 (67%) were below the optic nerve., Conclusion: PPVO after excision of OCVMs can occur in up to 5% of "free" retrobulbar intraconal lesions and in approximately one-third of apical lesions., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Abstract: A 50-year-old woman underwent 18 F-FDG PET/CT to evaluate possible abdominal malignancy, which was revealed by CT. The images showed a large cystic-solid lesion with peripherally increased FDG activity in the left mid-abdomen. Histopathology of the excised lesion confirmed a jejunal cavernous hemangioma. We reported a rare case of jejunal cavernous hemangioma with FDG accumulation on PET/CT, mimicking malignancy., Competing Interests: Conflicts of interest and sources of funding: The authors have no conflicts of interest to report. This project was sponsored by the grant provided by Zhuhai People’s Hospital (2021KYQD-03)., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

A 12-year-old male neutered Australian Shepherd Dog was presented to Charlotte Animal Referral & Emergency with a history of a thoracic mass. On physical examination, physiological parameters were within the normal ranges, and a complete haemogram and serum biochemistry profile were unremarkable except for mild thrombocytopenia. A computed tomography scan revealed a 21 × 15 × 12.7 cm thoracic mass encompassing the cranial mediastinum and extending to the right caudal thorax. The mass was surgically removed and histopathological evaluation revealed that it comprised remnants of the thymus and a neoplasm composed of large blood-filled vascular spaces lined by a single layer of endothelial cells with mild anisocytosis and anisokaryosis. The neoplastic cells had diffuse strong immunolabeling for endothelial cell marker CD31. Multifocally, there were large cystic degenerated areas of thymic tissue lined by plump cytokeratin AE1/AE3-positive epithelial cells. Based on these findings, a diagnosis of thymic cavernous haemangioma (CH) was made. Thymic CH is rare in animals, with the only reported case in a cross bred cow. To our knowledge, this is the first case of a thymic CH in a dog., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objective: To investigate the histopathological classification of orbital space-occupying lesions. Methods: This is a retrospective case series study. The clinical and pathological data of 1 913 tissue specimens from 1 913 patients with space-occupying lesions of the orbit which were examined in the Second Affiliated Hospital, Zhejiang University School of Medicine from January 2000 to December 2021 were collected. The mass lesions were classified based on histogenesis, pathological nature and age. Results: There were 913 males (47.7%) and 1 000 females (52.3%). The lesions were benign in 1 489 patients (77.8%) and malignant in 424 patients (22.2%). Based on histogenesis, there were 521 vasculogenic lesions (27.2%), which rancked first, 407 cystoid lesions (21.3%), 277 lymphoproliferative lesions (14.5%), 182 lacrimal gland lesions (9.5%) and 121 inflammatory lesions (6.3%). By pathological nature, there were 1 489 benign lesions, including cavernous hemangioma (275, 14.4%), dermoid cyst (225, 11.8%), other hemangiomas (199, 10.4%), epidermoid cyst (136, 7.1%) and benign mixed tumor of the lacrimal gland (134, 7.0%), and 257 malignant lesions, including lymphoma (210, 11.0%) and sebaceous gland carcinoma (47, 2.5%). The age of all patients ranged from 0 to 90 years, while 247 lesions (12.9%) occurred in patients aged 0 to18 years, 1 270 lesions (66.4%) in patients aged 19 to 59 years, and 396 lesions (20.7%) in patients aged 60 to 90 years. Conclusions: In 22 years, almost 2/3 benign orbital lesions in the Second Affiliated Hospital, Zhejiang University School of Medicine occurred in young and middle-aged patients, and males were fewer than females. The most common benign orbital tumors was cavernous hemangioma, followed by dermoid cyst and epidermoid cyst. And the most common malignant orbital tumor was lymphoma, which occurred more frequently in older patients.

Aims: The aim of this study was to determine the incidence and recovery rate for pupillary abnormalities after excision of orbital cavernous venous malformations (OCVMs)., Methods: Retrospective case-note and imaging review for patients with OCVMs, with an estimation of odds ratios (ORs) in relation to the position of the mass and surgical approach., Results: The 287 included patients (176 female; 61%) presented at a mean age of 46.5 years: 243/287 (85%) OCVMs were intraconal, 253/287 (88%) located in the posterior two-thirds of the orbit, and 29/287 (11%) wedged tightly in the apex. Pupillary changes-varying from minor sectoral paresis (only evident on slit-lamp examination) to a marked mydriasis-were noted in 21% (60/287) of patients and were strongly associated with removal of intraconal (60/243 [25%]; p < 0.001), apical (14/29 [48%]; OR, 4.3; p < 0.001), inferior or inferotemporal (41/105 [39%]; OR, 5.5; p < 0.001) masses. Lateral approaches-either with (23/129; 18%) or without (30/71; 42%) osteotomy-were associated with 88% (53/60) of all abnormalities (OR, 4.1; p < 0.001). Where known, the abnormalities improved in 43/50 (86%), although 15/43 (35%) had a persistent tonic pupil., Conclusion: Pupillary abnormalities after excision of OCVMs can occur in up to a quarter of intraconal lesions-particularly those located inferotemporally or inferiorly-and in about a half of apical lesions. Lateral or inferolateral orbital approaches that disrupt the inferior intraconal fat appear to be associated with a higher risk. Most changes resolve or improve markedly, although a third of those affected may have a persistent tonic pupil., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Cardiac tumors are rare. They were found in only 0.001%-0.300% of cases in a relatively recently reported autopsy series. Among cardiac tumors, primary hemangioma accounted for approximately 2.8% of all primary resected tumors, indicating this is a particularly rare benign neoplasm. We present a patient with a 5×3×2 cm cavernous hemangioma, arising from the right atrial roof and occupying the atrial septum and inseparable from the aortic root. We successfully accomplished a complete surgical resection of a cardiac cavernous hemangioma and reconstructed the cardiac atrium by a bovine pericardial patch.

Purely calvarial or intradiploic cavernous haemangiomas (PICHs) are rare benign tumours accounting for 0.2% of all bone tumours and 10% of benign skull tumours. They are generally small, slow-growing and asymptomatic lesions. Here the authors described an immunocompromised patient with concomitant giant intradiploic ossified globular cavernous angioma and multifocal neurotoxoplasmosis that underwent a combined approach to treat both lesions with an en-bloc resection of the right parietal intradiploic lesion and biopsy of the left occipital subcortical lesion.Indeed, it is essential to exclude the presence of metastases by making a timely differential diagnosis. En-bloc surgical resection of purely intradiploic ossified cavernous angioma is the gold standard treatment and the prognosis after a complete excision is usually excellent with rarer recurrence rate., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Gaze-evoked amaurosis is a transient monocular vision loss elicited by eccentric gaze and has been reported in many orbital conditions but is most classically associated with intraconal tumors, such as cavernous hemangioma and optic nerve sheath meningioma. Here, the authors report a case of gaze-evoked amaurosis due to idiopathic orbital inflammation. The patient was a 35-year-old man who presented with vision loss only when abducting his left eye. He had a history of sclerosing idiopathic orbital inflammation with a left orbital intraconal mass diagnosed 15 months prior to the current presentation. The patient had difficulty with immunosuppressive therapy, which was stopped 5 months prior to presentation. Repeat imaging during the current presentation revealed enlargement of the mass. This case demonstrates that idiopathic orbital inflammation should be considered in the differential diagnosis for gaze-evoked amaurosis, which may be the first symptom of disease progression.

Primary intraosseous cavernous hemangioma (PICH) is a rare, benign tumor of vascular origin, typically arising in the vertebral body. Its presence in the skull is exceedingly rare, with only a few cases being reported worldwide. We carried out the first systematic review of the literature, covering the epidemiology, clinical and imaging features, management, and prognosis of cranial PICH. The literature search revealed 51 studies with 77 patients; the mean age of the patients was 32.7 years with a female predominance of 1.4:1. The majority of cranial PICHs were located in the calvarium, primarily in the frontal and parietal regions, with only a few located in the skull base. The most common initial clinical manifestation was local growth or swelling, followed by a headache. Radiographically, PICHs represented osteolytic, intradiploic masses, which in many cases displayed trabeculations, leading to the so-called "honeycomb" or "starburst" pattern. After contrast administration, PICHs typically enhance. Tumor removal, with craniectomy or en bloc resection and subsequent skull reconstruction, was selected for calvarial PICHs, whereas a transsphenoidal approach, with only partial resection, was applied for clival/sella PICHs. Preoperative embolization, aiming to minimize intraoperative blood loss, was performed in the case of large tumors. At a mean follow-up of 39 months, no patient experienced tumor recurrence, even after subtotal resection. Owing to the benign nature of the tumor, maximal safe resection is recommended as the treatment of choice for patients with cranial PICH., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Traditionally, external craniofacial approaches have been used for orbital tumor resection. Over the last 30 years, endoscopic sinonasal and skull base techniques have become widely used throughout the world. These experiences paved the way for the extension of transnasal endoscopic techniques to the intraconal orbit. Transnasal endoscopic intraconal surgery has several advantages regarding morbidity and outcome as compared with purely external approaches. However, the anatomical knowledge and experience of the surgeon is crucial for the success of the surgery. Endoscopic approaches for intraconal tumor removal are feasible for medial and inferior lesions as well as for lesions lateral to the optic nerve provided they remain inferior to the "plane of resectability" and no optic nerve retraction is required. As intraorbital tumors are rare, new international staging systems including CHEER (Cavernous Hemangioma Exclusively Endonasal Resection) and ORBIT (Orbital Resection by Intranasal Technique) help to standardize safety, efficacy, and outcome., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objectives: 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)., Method: 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., Results: 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., Conclusion: 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., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Cavernous haemangiomas are a very rare occurrence in the parapharyngeal space (PPS). Here, we present a case of a 58-year-old woman with an incidentally identified left PPS mass thought to be a pleomorphic adenoma that underwent excision by transoral robotic surgery (TORS). Intraoperative findings demonstrated no solid mass present and histological assessment of resected tissue confirmed a cavernous haemangioma. We discuss our experience in the management of a radiological and surgical mismatch of a PPS mass in what we believe to be the first cavernous haemangioma to be excised by TORS in this region.

This study aimed to analyze the role of the FcepsilonRI (FcεRI) signaling pathway in the pathogenesis of benign lymphoepithelial lesion of lacrimal gland (LGBLEL). Transcriptomic analysis was performed on LGBLEL and orbital cavernous hemangioma (CH) patients diagnosed via histopathology in Beijing Tongren Hospital, Capital Medical University, between July 2010 and October 2013. Four LGBLEL and three orbital CH patients, diagnosed between October 2018 and August 2019, were randomly selected as experimental and control groups, respectively. RT-PCR, immunohistochemical staining, and western blotting were used to verify genes and proteins related to the FcεRI signaling pathway. Transcriptomic analysis showed that the FcεRI signaling pathway was upregulated in the LGBLEL compared with the CH group. The mRNA expression levels of important genes including SYK, p38, JNK, PI3K, and ERK were significantly increased in the LGBLEL group (P = 0.0066, P = 0.0002, P = 0.0003, P < 0.0001, P < 0.0001, respectively). Immunohistochemical staining results showed that SYK, p38, and ERK were positively expressed in LGBLEL, while JNK and PI3K were not. The protein contents of P-SYK, P-p38, P-JNK, P-PI3K, and P-ERK were significantly higher in the LGBLEL than in the CH group (P = 0.0169, P = 0.0074, P = 0.0046, P = 0.0157, P = 0.0156, respectively). The FcεRI signaling pathway participates in the pathogenesis of LGBLEL., (© 2021. The Author(s).)

Epithelioid hemangioma is a rare benign vascular tumor that consists of capillary-sized vessels lined by epithelioid endothelial cells. Diffuse cavernous hemangioma is a congenital benign vascular neoplasm consisting of increased dilated vessels. We report a case of epithelioid hemangioma and diffuse cavernous hemangioma that co-occurred in the rectum. To our knowledge, this is the first report in which two rare vascular lesions coexisted. Because both epithelioid hemangioma and diffuse cavernous hemangioma are often clinically confounded by malignant tumors, differentiating these benign lesions from other possible malignant tumors is significant., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

Orbital (slow flow) cavernous venous hemangiomas (OCVH) are the most common benign orbital tumors in adults. The c-KIT is a tyrosine kinase receptor, which is expressed on several types of cells, is thought to play a key role in tumor pathogenesis. The purpose of this study was to evaluate the presence of the receptor c-KIT in OCVH. Our retrospective study examined 16 orbital cavernous venous hemangiomas from 16 cases operated on between 2006-2016 at Emek Medical Center. The mean tumor size was 18.4 mm. Symptoms appeared between 6 months and 22 years before operation. All specimens were analyzed for the c-KIT receptor through immunohistochemistry. The c-KIT was expressed by the endothelium in all 16 preparates. Staining was strong in two cases, moderate in six, and weak in eight cases, with no statistically significant correlation between staining and tumor size ( p = 0.69) or the symptom duration ( p = 0.15). We conclude that c-KIT may play an important role in the pathogenesis of OCVH. This pilot study is significant in that tumor-targeted therapy such as Imatinib Mesylate and Sunitinib may have a role in treating surgically complicated cases located in the orbital apex. A large multicenter collaborative study is necessary to examine the role of c-KIT in OCVH.