Angiolymphoid Hyperplasia With Eosinophilia Research Articles

Clinicopathological features of epithelioid hemangioma of 43 patients with long-term follow-up: A two-center retrospective study.

Epithelioid hemangioma (EH) is a rare angioproliferative disorder for which histopathology is the main approach to diagnosis. The tendency to recur is of concern to clinicians and patients. The factors associated with recurrence have been sporadically reported and a large-scale cohort study has been lacking. This study aims to investigate the clinicopathological characteristics and long-term clinical outcomes of EH patients from two tertiary care hospitals. A two-center retrospective cohort study of 43 patients with a diagnosis of EH between 2013 and 2023 was evaluated at follow-up. A comprehensive and detailed review of clinical and pathological data with long-term follow-up was performed. Information on prognosis was available for 43, and 8 (8/43, 18.6%) experienced local recurrence. Facial (Cramer's V = 0.405, P = 0.029) and multiple (relative risk [RR] 4.306, 95% confidence interval [CI] 1.213, 15.282, Phi = 0.369, P = 0.016) lesions, subcutaneous involvement (RR 5.063, 95% CI 1.157, 22.151, Phi = 0.374, P = 0.014), and the presence of lymphoid follicles (RR 9.750, 95% CI 3.853, 24.671, Phi = 0.670, P < 0.001) were associated with higher recurrence rates. According to the presence or absence of well-differentiated angiogenesis, EH can be pathologically classified into vascular, cellular, and intermediate types, while the depth, degree, and pattern of inflammation, tissue eosinophilia, eosinophilic microabscesses, and hobnail endothelial cells differed significantly between cellular and vascular types. The characteristics of EH are distinguished by different pathological subtypes. This study provides insight into the clinicopathological features and outcome of EH to assist clinicians in the diagnosis and management of this rare condition.

Epithelioid (Histiocytoid) hemangioma of the testis: A case report and literature review of a rare benign tumor.

Testicular hemangioma is a rare benign vascular neoplasm originating from the inner layer of the tunica albuginea, predominantly affecting the pediatric population and often associated with hemangiomas in other organs. Histopathologically can be categorized into four types, including capillary, cavernous, papillary endothelial hyperplasia, and epithelioid (histiocytoid). We report a case of a 24-year-old presenting with left inguinal pain, diagnosed with an epithelioid (histiocytoid) hemangioma of the testis. Diagnostic evaluation included scrotal doppler ultrasonography, which revealed a small, hypoechoic formation with evident vascularization. Serum tumor markers were within normal limits. Surgical excision via testis-sparing surgery (TSS) was performed based on frozen section analysis. A literature review underscores the rarity of testicular histiocytoid hemangiomas. Advancements in diagnostic technique have improved the accuracy of diagnosis and allowed for more conservative surgical approaches, preserving fertility and minimizing long-term adverse effects. In conclusion, while testicular hemangiomas may present characteristic features on ultrasound, frozen section examination remains crucial in guiding surgical decision-making. TSS offers significant advantages over traditional excisional surgery, including reduced morbidity and preservation of cancer control, making it an essential option in the management of testicular hemangiomas, particularly in young patients concerned about fertility preservation.

Molecular Testing Prevents Overdiagnosis of Epithelioid Hemangioma

Abstract Introduction/Objective Epithelioid hemangiomas (EH) are mostly solitary benign vascular tumors commonly involving the dermal and subcutaneous areas of the head and neck. However, reports of multifocality are increasing, specially in same anatomic region. Some reported cases of EH show morphologic features that may mimic malignant vascular tumors such as hemangioendotheliomas or angiosarcomas. However, there are no reported overlaps in the molecular characteristics of these tumors. We describe a multifocal EH with atypical features, suspicious for angiosarcoma, which needed molecular testing to resolve the diagnosis. Methods/Case Report The patient is a previously healthy 42-year-old male with a 5-month history of multiple growing painless penile nodules. Biopsy of one of the nodules was done. Histologic examination showed multifocal dermal- based ill-circumscribed nodules with interspersed inflammatory infiltrates and focal areas of necrosis. Tumor periphery shows vasoformative growth with intraluminal and extravasated red blood cells. Neoplastic cells were large and ovoid with abundant eosinophilic cytoplasm, large vesicular pleomorphic nuclei and prominent nucleoli. Immunohistochemical stains showed strong expression of ERG, FLI-1, and CD31 and negativity for AE1/AE3, CD34, HHV8 and EMA. The morphologic and immunohistochemical features of the specimen showed significant overlap of two primary differential diagnoses - epithelioid angiosarcoma and epithelioid hemangioma. Ultimately, molecular testing revealed ZFP36-FOSB fusion, confirming the diagnosis of EH. Results (if a Case Study enter NA) NA Conclusion Differentiating benign and malignant epithelioid vascular neoplasms can sometimes be challenging. There can be overlap in terms of architectural variety, presence of necrosis, vasoformation, inflammatory cell components and cytologic features, especially in EH with atypical features and epithelioid angiosarcoma (EA). Both of these tumors show positive immunoreactivity for CD31, CD34, FLI1 and ERG, and negativity for most keratins. Currently, the most reliable way to distinguish each tumor is molecular testing, with EH having FOS/FOSB rearrangements and EA with MYC or FLT4 amplification. Benign and malignant epithelioid vascular tumors can be indistinguishable morphologically and immunohistochemically. Molecular tests can help differentiate these neoplasms to better tailor patient management.

Characterization and Management of Epithelioid Hemangioma on the Lip Mucosa: A Case Report

Epithelioid Hemangioma (EH) is a rare benign vascular tumor characterized by well-formed blood vessels and epithelioid endothelial cells. Known as Angiolymphoid Hyperplasia with Eosinophilia (ALHE), the World Health Organization does not recommend the use of this term since 2020. Despite its common occurrence in subcutaneous soft tissues of head and neck, cases involving oral mucosa and other extra cutaneous areas are infrequent. This article presents a case of EH in an unusual location and discusses the differential diagnosis with other entities, including Kimura´s Disease (KD). We report the clinical case of a 26 year old man with no significant medical history who presented with a painless increase in volume on the right upper lip, subsequently diagnosed as EH through biopsy and histopathology. The lesion exhibited typical clinical features and a complex histological pattern including epithelioid endothelial cells and an inflammatory infiltrate rich in eosinophils. Diagnosis of EH is challenging due to morphological variability and the need to differentiate it from similar conditions such as KD. Differential diagnosis is crucial, especially in atypical locations. This case shows the importance of histopathology and complementary examinations in diagnosis and the need to consider KD in the differential diagnosis due to its therapeutic and prognostic implications. Therefore, it is concluded that EH, although benign, requires a rigorous diagnostic approach to differentiate it from similar diseases and manage it appropriately. Continuous control is essential due to the potential for recurrence. This case shows the importance of careful differential diagnosis, especially in atypical locations, to optimize management and improve patient outcomes.

Ear lobule reduction using a sub-antitragal groove technique in patients with angiolymphoid hyperplasia with eosinophilia on the earlobe: a case report and literature review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition characterized by nodular lesions predominantly in the head and neck region, often causing discomfort or pain. Treatment remains challenging because of its rarity and the lack of established guidelines. This report presents a case of ALHE affecting the earlobes that was successfully managed using ear lobule reduction surgery and subsequent intralesional steroid injections. A 31-year-old woman with a history of recurrent earlobe masses underwent a partial excision to avoid the loss of the earlobe. Histopathological examination confirmed Kimura disease, a variant of ALHE. Subsequent local methylprednisolone injections effectively controlled the remaining lesions, resulting in significant size reduction without notching. Various treatment modalities have been attempted for this condition; however, recurrence rates remain high. Surgical resection combined with intralesional corticosteroid injections is the preferred approach. In this case, a sub-antitragal groove technique for earlobe reduction was employed to preserve the lateral edge of the ear lobule, minimize the risk of deformity, and achieve a predictable outcome. The sub-antitragal groove technique offers an approach to reduce earlobe size without compromising aesthetics. Further research is required to elucidate the pathogenesis of ALHE and establish standardized treatment protocols for this rare condition.

Epithelioid Hemangioma of the Spine: A Case Series and Treatment Flow Chart-Experience from a Single Centre.

Epithelioid hemangioma is recognized by the World Health Organization as a distinct benign neoplasm; however, it is characterized by locally aggressive and rarely metastasizing behavior. Epithelioid vascular tumors are rare bony vascular lesions with varying degrees of malignant potential that remain controversial because of their rarity, unusual morphological features, and unpredictable biological behavior. The application of new molecular tools, such as massive parallel sequencing technologies, have provided new diagnostic markers and an opportunity to further refine the classification of bone vascular neoplasms. Very few cases of EH of the spine have been reported in the literature; therefore, it is difficult to make evidence-based therapeutic decisions for these patients. We report herein our experience with eleven patients suffering from EH of the spine. The study population included three males and eight females treated in our center from 2016 to the present; the average age was 44.8 years (range 14-75 years). The surgical, clinical, and radiographic data were retrospectively analyzed. The mean follow-up was 34.8 months. All patients presented lytic vertebral body lesions, six of them with pathological fracture. The majority of patients (80%) presented myelo-radicular compression. All patients were surgically treated, and preoperative embolization was performed in all cases. In light of the literature review and the clinical experience of our center, we can consider EH a locally aggressive tumor that requires surgical treatment in case of symptoms. Here, we propose a treatment algorithm that could be useful in the management of patients with this rare disease.

CT, MRI, and PET/CT imaging features of thoracic spine epithelioid hemangioma: a retrospective observational study.

Epithelioid hemangioma (EH) is an intermediate locally aggressive tumor that consists of epithelioid cells and endothelial cell differentiation, which can occur at any age, but is most common between the ages of 30 and 40 years. EH in the thoracic spine is rare, and accurate diagnosis is critical to treatment planning. Our aim was to explore the imaging and clinical data of thoracic spine EH to improve the understanding of this rare disease. From January 1, 2018 to June 30, 2023, a database of thoracic spine masses was retrospectively reviewed. Five patients with histologically proven thoracic spine EH and complete imaging available were identified and analyzed. Computed tomography (CT) and magnetic resonance imaging (MRI) findings were evaluated separately by two radiologists with more than 10 years of experience. Positron emission tomography (PET)/CT was conducted by two nuclear medicine diagnostic technologists with at least 5 years of experience. The patients included three male and two female patients aged 23 to 56 years (mean age was 38.4 ± 14.3 years). All patients underwent CT, MRI, and 18F-FDG PET/CT examination before treatment. Four patients were limited to one vertebral involvement, only one patient had multiple vertebral involvement, and all tumors involved the accessories, including one involving the posterior ribs. The maximum diameter of the tumor ranged from 2.7 to 4.3. CT, MRI, and 18F-FDG PET/CT findings of thoracic spine EH have certain characteristics, and understanding these imaging findings will help to obtain accurate diagnosis before surgery.

Soft tissue vascular tumor-like lesions in adults: imaging and pathological analysis pitfalls per ISSVA classification

ObjectivesTo compare the magnetic resonance imaging (MRI) and Doppler ultrasound (DUS) findings with the pathological findings of soft tissue vascular tumors (STVTs) according to the 2018 ISSVA (International Society for the Study of Vascular Anomalies) classification to differentiate vascular tumors from vascular malformations.MethodsThis retrospective study included patients with STVTs who underwent contrast-enhanced MRI and pathological analysis at our hospital between 2010 and 2020. The presumptive diagnosis based on the on-site imaging and histological analysis was compared with imaging and histological analysis conducted off-site utilizing the ISSVA criteria.ResultsThis study included 31 patients with 31 vascular tumors located in the head and neck (n = 3), trunk (n = 2), and extremities (n = 26). The off-site pathological analysis confirmed benign vascular tumors in 54.8% of cases (non-involuting congenital hemangioma: 35.5%; epithelioid hemangioma: 13%; pyogenic granuloma: 3%; and spindle cell hemangioma: 3%). Based on the off-site histological analysis, 25.8% were reclassified as having a vascular malformation whereas three had other benign lesions. Only phleboliths were associated with a vascular malformation (p = 0.03). The concordance between off-site MRI and pathological findings was fair (k = 0.3902 (0.0531–0.7274)), whereas that between on-site and off-site pathological analyses was poor (k = −0.0949 (−0.4661 to 0.2763)).ConclusionBenign vascular tumors have non-specific imaging features on imaging with some overlap with atypical vascular malformations. Therefore, histological analysis is recommended. Imaging and pathological analyses should be performed in accordance with the ISSVA classification to minimize inter-observer discrepancies.Critical relevance statementImaging features of benign vascular tumors on MRI are non-specific, leading to discrepancies with pathological findings and potential overlap with atypical vascular malformations. Imaging and histological analyses should be performed in accordance with ISSVA guidelines to improve patient management.Key PointsThe imaging features of benign vascular tumors are non-specific.Histological analysis is recommended for soft tissue vascular tumors in adults.Analyses of soft tissue vascular tumors should be performed in accordance with ISSVA guidelines.Graphical

Epithelioid Hemangioma: Report of a Case Series of 4 Patients

Epithelioid hemangioma or Angiolymphoid Hyperplasia with Eosinophilia (ALHE) is a rare benign vascular disease, characterized by solitary or multiple angiomatous papules and nodules, which are usually located on the skin of scalp and face (mainly in the preauricular zone); these have characteristic histological and immunohistochemical patterns, in particular characterized by an important lymphocytic inflammatory infiltrated with many eosinophils and vascular proliferation of capillaries with endothelial cellular changes. In this paper, four cases of this disease are reported, in the first series of Ecuadorian cases described in the literature.

Epithelioid hemangioma of the acromion causing shoulder pain: a case report.

Epithelioid hemangioma of bone is a rare benign vascular tumor. Because of the locally destructive clinical presentation and the presence of atypical histologic features with increased mitotic activity and necrosis, it is often misdiagnosed as low-grade malignant epithelioid hemangioendothelioma or high-grade malignant epithelioid angiosarcoma. Correct diagnosis through imaging studies and histopathological examination is mandatory to determine the appropriate course of treatment, as the prognosis differs from that of other malignant hemangioma tumors. A 69-year-old male who presented with intractable shoulder pain caused by epithelioid hemangioma in the acromion of the scapula was treated with tumor curettage. This paper reports a good result with a review of the relevant literature.

The "great imitator": IgG4-related disease of the oral cavity. Two case reports and scoping review.

This study aimed to review the lesser-known intraoral manifestations of immunoglobulin G4-related disease (IgG4-RD). In this paper we report an unprecedented case of oral IgG4-RD mimicking angiolymphoid hyperplasia with eosinophilia (ALHE), and another case presenting as plasma cell gingivitis. We then performed a scoping review of published cases of IgG4-RD involving the oral cavity. The following data were collected for each case: age, sex, intraoral site(s) involved, clinical appearance, imaging features, serum IgG4 values, histopathology, treatment, and follow-up duration. Fifty-one cases of oral IgG4-RD were published in literature. The hard palate and jaw bones were the two main locations reported, while the histological identification of a IgG4/IgG plasma cells ratio ≥40% was fundamental for diagnosis. Conversely, the pathological features of storiform fibrosis and obliterative phlebitis were not common. Future reports regarding oral IgG4-RD should report clear adherence to the recognized international diagnostic criteria of the disease.

Immune Checkpoint Inhibition in Pediatric Oncology Patients: A Single-Institution Experience

Immunotherapy has emerged as a promising treatment approach in oncology, as it is specifically designed to boost the strength and accuracy of the immune system, allowing it to target tumor cells but spare non-tumor tissue. This treatment not only demonstrates potential for improved clinical outcomes but may also be associated with fewer adverse effects compared to traditional therapies. Despite its early success, the application of immunotherapy has largely been limited to adult cancer patients, with slow adoption noted in the treatment of pediatric cancer patients. Our objective is to demonstrate a single institution’s experience with immunotherapy in pediatric cancer patients and to discuss the use of these treatment modalities in this unique patient population. We performed a retrospective chart review and identified patients who received immune checkpoint inhibitors (ICIs) and/or underwent immunohistochemistry (IHC) testing for programmed death ligand 1 (PD-L1), quantification of tumor mutational burden (TMB), and classification of microsatellite instability (MSI) status. In total, we identified seven pediatric cancer patients who received therapy with ICIs. Four of these patients demonstrated positive PD-L1 expression, high TMB, and/or MSI-high status. These patients were treated with nivolumab alone or in combination with ipilimumab or brentuximab. The diagnoses included: multifocal epithelioid and spindle cell hemangioma (n = 1); metastatic melanoma (n = 2); histiocytic sarcoma (n = 1); rectal adenocarcinoma in the setting of constitutional mismatch repair deficiency syndrome (CMMRD) (n = 1); and Hodgkin lymphoma (n = 2). The patients received between four and nineteen cycles of immunotherapy. Immunotherapy-related adverse events included: mild allergic reaction; prodromal symptoms; anemia; neutropenia; transaminitis; endocrinopathies; and self-limiting neuritis. Of the seven patients, three are still being treated with immunotherapy (the patients with rectal adenocarcinoma, metastatic melanoma, and multifocal epithelioid and spindle cell hemangioma) with positive treatment responses observed on imaging, one is being treated with other modalities (the patient with Hodgkin lymphoma), two have achieved remission (the patients with metastatic melanoma and Hodgkin lymphoma), and one has relapsed (the patient with histiocytic sarcoma). The three patients who completed their immunotherapy regimens have been followed for 1 month, 4 months, and 10 months, respectively. This report of a single-institution experience with immunotherapy in pediatric cancer patients highlights the positive impact immunotherapy can have, especially when utilized to treat relapsed/refractory malignancies, as tumor regression or stabilization of disease burden was achieved in six of the patients described (CR = 2; PR = 4). Further research is needed to accurately identify pediatric oncology patients who could benefit from immunotherapy.

Epithelioid Hemangioma Masquerading as Traumatic Lesion: A Rare Case Report with Diagnostic Workup

Abstract Wells and Whemser first identified epithelioid hemangioma (EH) as angiolymphoid hyperplasia in 1969. It is a rare type of vascular tumor that primarily affects men in their middle ages. Typically, it appears as a single or few reddish-to-brown nodules. EHs of the oral cavity have only seldom been reported up to this point. Histologically, it is distinguished by lobular vessel configuration, tombstone-like epithelioid cells lining the vessel wall, and a persistent inflammatory infiltration predominately made up of eosinophils. Although the terms EH and epithelioid hemangioendothelioma are sometimes confused, the two are distinct based on histopathology and a number of immunohistochemistry (IHC) markers, including high Ki67 index and ERG positivity observed later. Local trauma to a nearby vessel may be one of the etiopathogeneses for this entity. This is a rare case report of EH that was identified with the use of a panel of IHC markers and hematoxylin and eosin histology.

Vascular Neoplasms With NFATC1/C2 Gene Alterations : Expanding the Clinicopathologic and Molecular Characteristics of a Distinct Entity.

Despite significant advances in their molecular pathogenesis, skeletal vascular tumors remain diagnostically challenging due to their aggressive radiologic appearance and significant morphologic overlap. Within the epithelioid category and at the benign end of the spectrum, recurrent FOS/FOSB fusions have defined most epithelioid hemangiomas, distinguishing them from epithelioid hemangioendothelioma and angiosarcoma. More recently, the presence of EWSR1/FUS :: NFATC1/2 fusions emerged as the genetic hallmark of a novel group of unusual vascular proliferations, often displaying epithelioid morphology, with alternating vasoformative and solid growth, variable atypia, reminiscent of composite hemangioendothelioma. In this study, we further our understanding and morphologic spectrum of NFATC -fusion positive vascular neoplasms by describing 9 new cases, including soft tissue locations and novel fusion partners. Combining with the initial cohort of 5 cases, a total of 14 patients were analyzed, showing slight female predilection and an age range of 10 to 66 (mean 42y). Twelve patients had solitary lesions, while 2 had multifocal polyostotic (pelvic bones) disease. Overall, 12 lesions were intra-osseous and 2 in soft tissue. By targeted RNA Fusion panels or FISH, there were 6 cases of EWSR1::NFATC1 , 4 EWSR1::NFATC2 , 2 FUS::NFATC2 , 1 EWSR1 rearrangement, and 1 with a novel FABP4::NFATC2 fusion. Follow-up was available in 4 patients. One patient experienced 2 local recurrences, 11 and 15 years postdiagnosis, and one patient experienced progressive disease despite multimodality treatment (curettings, embolization, radiation) over 3 years. In summary, our extended investigation confirms that NFATC -related fusions define a distinct group of vascular neoplasms with variable architecture, epithelioid phenotype, and cytologic atypia, commonly located in the bone, occasionally multifocal and with potential for local recurrence and aggressive behavior but no metastatic potential. Molecular analysis is recommended in diagnostically challenging cases with atypical histology to exclude malignancy.

Angiolymphoid hyperplasia with eosinophilia in the eyelid: A case series and literature review

Abstract: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular disorder characterized by the proliferation of blood vessels and lymphoid tissue. While it can occur in various regions of the body, ALHE presenting in the eyelid is relatively uncommon, leading to diagnostic and management challenges. In this case series from South India, we present three unique cases of ALHE specifically affecting the eyelids. Each case illustrates different clinical manifestations, diagnostic workups, and treatment approaches. Tissue biopsies typically reveal nodules comprising immature and mature vascular structures, including capillaries, arterioles, and venules, which are lined by plump endothelial cells with an epithelioid appearance. The surrounding stroma contains a dense infiltrate of inflammatory cells, predominantly eosinophils, histiocytes, lymphocytes, and mast cells. Increased awareness of ALHE in the eyelid will aid ophthalmologists in early recognition, accurate diagnosis, and appropriate management, resulting in improved patient outcomes.

Intraoral epithelioid and spindle cell hemangioma associated with pseudoepitheliomatous hyperplasia: An immunohistochemical study.

Intraoral epithelioid and spindle cell hemangioma associated with pseudoepitheliomatous hyperplasia: An immunohistochemical study.

Epithelioid Hemangioma of the Nasal Cavity with Atypical Features: a Case Report and Review of the Literature

Epithelioid Hemangioma of the Nasal Cavity with Atypical Features: a Case Report and Review of the Literature

An Unusual Presentation of Angiolymphoid Hyperplasia with Eosinophilia as Postauricular Mass: A case Report.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, reactive vaso-proliferative condition in the dermal and subcutaneous tissues of the head and neck. A 28-year-old female presented with slow-growing painless swelling behind her left ear. FNAC revealed benign soft tissue neoplasm and histopathological examination after surgical excision revealed angiolymphoid hyperplasia with eosinophilia. ALHE origin has been variously attributed to prior trauma, hyperestrogenemia, infectious agents, atopy, reactive hyperplasia, and benign neoplasia. Retroauricular ALHE has been rarely reported. However, on the basis of our case report, it should be a viable differential diagnosis when large subcutaneous tumors of the head and neck are encountered. When big subcutaneous tumors of the head and neck are present, especially in females, a valid differential diagnosis for angiolymphoid hyperplasia with eosinophilia, a rare condition marked by dermal or subcutaneous endothelial cell proliferation, should also be considered.

Fusion transcriptome profiling defines the monoclonal origin of multifocal epithelioid haemangioma of bone.

Epithelioid haemangioma (EH) of bone remains a highly controversial entity. Indeed, the WHO classifies EHs of soft tissues as benign tumours, whereas bone EHs are considered intermediate-locally aggressive tumours due to common multifocal presentation and local destructive growth. To gain insights into theclinical behaviour and biology of EH of bone we retrospectively analysed 42 patients treated in a single institution from 1978 to 2021. Multifocal presentation was detected in 17 of 42 patients (40%) primarily as synchronous lesions. Patients were treated with curettage (57%), resection (29%) or biopsy, followed by radiotherapy or embolisation (14%). Follow-up (minimum 24 months) was available for 38 patients, with only five local recurrences (13%) and no death of disease. To clarify whether the synchronous bone lesions in multifocal EH represent multicentric disease or clonal dissemination, four cases were profiled by RNA-sequencing. Separate lesions from the same patient, which showed a similar transcriptional profile, expressed the same fusion transcript (involving FOS or FOSB) with identical gene breakpoints. These results indicate that, in EH of bone, multifocal lesions are clonally related and therefore represent the spread of a same neoplastic clone rather than simultaneous independent tumours. This finding is in apparent contradiction with the benign clinical course of the disease, and suggests that tumour dissemination in bone EH probably reflects a phenomenon of passive spreading, with tumour cells colonising distal sites while maintaining their benign biological nature.

Angiolymphoid Hyperplasia With Temporal Artery Eosinophilia: A Case Report.

Angiolymphoid Hyperplasia with Eosinophilia (ALHE) is a benign vascular proliferative disorder with uncertain etiology and pathogenesis. The aim of this paper is to report a case of ALHE in the temporal artery and discuss the general aspects of this pathology. A 29-year-old female black patient sought the Vascular Surgery Outpatient Service, complaining of bulging in the right temporal region, associated with pain and local discomfort. Physical examination revealed pulsatile bulging in the right temporal region measuring approximately 2.5 × 1.5cm. Nuclear Magnetic Resonance showed an expansive fusiform lesion in the superficial soft parts of the right temporal region, measuring 2.9cm in the longest longitudinal axis. Surgical excision proved to be the best therapeutic option for the patient in this case. Histopathological sections showed the proliferation of vessels of different sizes, covered by swollen endothelium, prominent inflammatory infiltrate composed of lymphocytes, plasma cells, eosinophils, and scarce histiocytes. Immunohistochemical analysis of the lesion showed positivity for CD31, corroborating the diagnosis of ALHE.