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

Introduction: 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. Methods: 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. Results: 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. Conclusions: 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.

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.

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.

Epithelioid Hemangioma of the Nasal Cavity: a Diagnostic Challenge.

Epithelioid hemangioma also known as angio-lymphoid hyperplasia with eosinophilia is a rare benign vascular neoplasm of unknown etiology. It very rarely involves the nasal cavity. It always poses a diagnostic challenge for clinicians and is usually misdiagnosed as Kimura's, IgG4-related disease, or malignant vascular tumors. The present case report describes an extremely rare presentation of epithelioid hemangioma inside the left nasal cavity causing complete obstruction and nasal septal deviation towards the right side in a young male. Hematoxylin and eosin-stained sections depicted a lobular proliferation of small capillary-sized vessels lined by plump epithelioid endothelial cells surrounding central vessels and scattered around them. These cells had abundant eosinophilic to amphophilic cytoplasm and enlarged nuclei with fine chromatin and central nucleoli. On immunohistochemistry, CD34 and CD31 highlighted the vascular proliferation and epithelioid endothelial cells. Erythroblast transformation-specific (ETS)-related gene (ERG) showed strong nuclear positivity in neoplastic plump epithelioid endothelial cells. EH is a benign vascular proliferation, but a high chance of recurrence is seen if complete resection is not done. As a result, the treatment of choice is complete surgical excision with clear margins. The case highlights a non-conventional presentation of epithelioid hemangioma and the importance of histomorphological features in diagnosing this entity.

Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment.

Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications. Nevertheless, primary vascular bone tumors still represent a challenge for medical oncologists. Given their rarity and heterogeneity, in the last few years, there has been no significant progress in medical treatment options, so further research is needed. Here we present a review of the current knowledge regarding primary vascular tumors of the bone, correlating clinicopathologic features with tumor behavior and therapeutic approaches.

Multiple Eruptive Epithelioid Hemangiomas on the Right Upper Extremity in an Asian Man.

Epithelioid hemangioma is a rare benign vascular neoplasm. Recently, the term "multiple eruptive epithelioid hemangioma" has been proposed for epithelioid hemangioma with distinct features. It is different from usual epithelioid hemangioma because of the multifocal distributions in various body regions with an eruptive onset. In addition, the histopathology of multiple eruptive epithelioid hemangioma shows increased cellular proliferation, mitosis, and nuclear pleomorphism and positive findings for FOS-B compared to classic epithelioid hemangioma. Herein, we report the case of a 59-year-old man with unusual manifestations suitable for multiple eruptive epithelioid hemangioma. He had multiple erythematous to purple-red dome-shaped nodules on the right hand, arm, and shoulder. The initial lesion was a solitary erythematous nodule on the right hand that abruptly extended to the right arm and shoulder. Microscopically, the tumor was a well-demarcated dermal nodule and showed capillary sized vascular structures. Vascular structures had epithelioid endothelial cells with abundant eosinophilic cytoplasm and vesicular nuclei. The tumor cells showed mild nuclear pleomorphism and a few mitosis and feature of resembling cobble stone was observed. In immunohistochemistry, CD31 and CD34 were positive in the endothelial cells. The endothelial cells showed nuclear positivity in FOS-B. Based on the clinical and histopathological findings, the final diagnosis was multiple eruptive epithelioid hemangiomas. This is the first report of multiple eruptive epithelioid hemangiomas in an Asian man after the term had been introduced.

Epithelioid Hemangioma of Tongue a Case Report.

Epithelioid hemangioma is an uncommon benign vascular lesion which most commonly appears as a nodular lesion in the head and neck area. Here, we present a rare case of nodular swelling in the dorsal aspect of the tongue in a 30 year old male for the past 3 months. Excision of growth was done and histopathological examination revealed epithelioid hemangioma of the tongue. Patient is on regular follow up and there is no evidence of recurrence. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03969-x.

Subacute Brachial Plexopathy due to Intraneural Epithelioid Hemangioma: A Case Report.

Brachial plexopathy is a common consideration in the differential diagnosis of upper extremity sensory and motor deficits, and neoplasms signify one possible etiology of brachial plexopathy. Of the neoplastic brachial plexopathies, hemangiomas involving the brachial plexus are rare. Most reported cases describe extraneural brachial plexus hemangiomas that present as a palpable, tender neck mass associated with pain and sensory disturbance, with minimal motor deficits. Here we share the case of a 48 year-old man with intraneural epithelioid hemangioma of the brachial plexus who presented with prominent motor weakness and no palpable mass. The patient presented with subacute onset of left arm pain, numbness and progressive weakness. Neurologic exam revealed lower motor neuron signs and weakness spanning multiple nerve root and peripheral nerve distributions. Dedicated brachial plexus MRI showed two mass lesions involving the cords of the brachial plexus, with corresponding FDG-avidity on PET/CT. Biopsy revealed intraneural atypical epithelioid hemangioma. After nerve transfer surgery, he had moderate improvement in left arm strength. This case serves to: emphasize the importance of both clinical localization and dedicated brachial plexus imaging in the evaluation of brachial plexopathy; introduce to the literature a new clinical presentation of brachial plexus hemangiomas; encourage consideration of neoplastic brachial plexopathy even when faced with an illness script resembling Parsonage-Turner Syndrome, to avoid delays in diagnosis and treatment.

Epithelioid hemangioma (ALHE) on the tongue of an infant treated with oral corticosteroids: A case report and review of the literature.

Epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular lesion presenting as multiple nodules on the head and neck. Surgery had been considered to be the best treatment modality for ALHE. We report the case of a 6-month-old boy with ALHE on his tongue that was treated successfully with oral prednisolone.

[Penile epithelioid hemangioma]. / A hímvesszo epithelioid haemangiomája.

Epithelioid hemangioma is a benign vascular tumor. Complete surgical excision is curative, there is no tendency to recurrence or metastasize. Its penile occurrence is extremely rare, only 33 cases have been reported in English literature. A patient with epithelioid hemangioma of the deep dorsal vein of the penis is reported. To our knowledge, this is the first report of penile epithelioid hemangioma in Hungarian literature. The 64-year-old patient presented to our department with painful erection caused by a palpable penile mass. Physical examination revealed a mobile subcutaneous nodule on the dorsum of the penis. Penile ultrasound showed a 10 mm homogeneous, well circumscribed lesion superficial to the tunica albuginea of the corpora cavernosa without intralesional blood flow. Local excision was performed through a dorsal longitudinal penile incision. The deep dorsal vein was dissected circumferentially, then the lesion was removed following ligation of the vein proximal and distal to the mass. Histopathological examination revealed epithelioid hemangioma. At three months after surgery, the patient reported complete resolution of pain, his International Index of Erectile Function Score was 21. At four years after the operation, there were no signs of recurrence or metastasis. Successful treatment of epithelioid hemangioma of the penis requires in-depth knowledge of processes resulting in penile subcutaneous masses, therefore the differential diagnosis is detailed in discussion. Orv Hetil. 2023; 164(21): 836-840.

An Epithelioid Hemangioma Camouflaged As Radial Aneurysm.

Epithelioid hemangioma (EH) is an uncommon benign vascular lesion usually present as subcutaneous nodules in the head and neck area. Sometimes, these lesions can occur in the peripheral arteries, and when they do, they can be mistaken for aneurysmal dilatations of that respective vessel. We report a case of a 43-year-old male who underwent surgical recession of a radial aneurysm, which after anatomopathological examination, revealed an EH.

Immunohistochemistry for FOSB and FOS is a Useful Ancillary Tool in the Diagnosis of Epithelioid Hemangioma but There are Pitfalls in Interpretation Including Expression in Other Vascular Lesions.

Introduction. Epithelioid hemangioma is a benign vascular neoplasm associated with FOS and/or FOSB protein overexpression detected by immunohistochemistry (IHC). Methods. The aim of our study was to determine the co-expression or independent IHC expression of FOS and FOSB in a cohort of epithelioid hemangiomas. We also included two cohorts of other vascular lesions: papillary endothelial hyperplasia and lobular capillary hemangioma / pyogenic granuloma. Results. We identified 50 cases of epithelioid hemangioma, 84% of which were cutaneous and the remaining involved other anatomic locations. Over two thirds of all cases expressed FOSB (68%; 34/50) while FOS immunoreactivity was identified in 46% of all cases. Co-expression of FOSB and FOS occurred in 37% of cases while 76% of all cases stained for at least one of the antibodies. Fifty-eight percent (n = 14/24) and 33% (8/24) of all cases of papillary endothelial hyperplasia expressed FOS and FOSB, respectively. Thirty-two per cent of lobular capillary hemangiomas (n = 8/25) were positive for either FOS or FOSB. Conclusion. In summary, we present the largest cohort of epithelioid hemangiomas assessed with both FOS and FOSB and demonstrated that the use of both antibodies increases the detection rate of these proliferations by 10%. Nonetheless, the use of thresholds may not be appropriate, as only a subset of lesional endothelial cells label with FOS/FOSB. Over half of all cases of papillary endothelial hyperplasia and a third of lobular capillary hemangiomas also displayed immunoreactivity with FOS and/or FOSB.

Resection of Condylar Skull Base Tumor via Combined Far Lateral and Infrajugular Approaches with Single-Stage Occipitocervical Fusion.

Epithelioid hemangioma is a rare vascular mesenchymal tumor with a paucity of reports of cranial involvement. In particular, guidance on treatment for lateral skull base lesions is lacking, despite this being a highly technically challenging location. Nuances in the management decisions for this tumor type are discussed. Two major challenges with this location are proximity to critical neurovascular structures and managing secondary craniocervical instability. We present a patient with a lateral skull base epithelioid hemangioma treated with transcondylar resection, single-stage occipitocervical fusion, and adjuvant radiation and chemotherapy. The patient consented to both the procedure and the published report of her case including imaging. Obtaining tissue was necessary for diagnosis. Maximal safe resection, resection of a tumor such that the greatest clinical benefit is achieved with the minimum risk, was favored given the location and vascularity of the lesion. Occipitocervical fusion was recommended given ongoing bony destruction by the tumor and further expected iatrogenic instability upon resection. This was performed as a single stage given expected need for postoperative adjuvant radiation therapy and dynamic neck pain (Video 1). Surgical planning and decision making are detailed, including rationale and potential risks and benefits. We discuss positioning, equipment needs, and the importance of a multidisciplinary surgical team. Park bench positioning was used for part 1, left-sided extended far lateral and infratemporal fossa presigmoid approaches. For part 2, occipitocervical fusion, the patient was transitioned to prone position. The anatomy is highlighted in labeled pictures of the approach and dissection, and surgical video is presented for key surgical steps. Preoperative and postoperative imaging is analyzed. A desirable clinical outcome was obtained.

A Rare Case of Primary Epithelioid Hemangioma of Bone with WWTR1::FOSB Fusion: A Benign Lesion with the Potential to Mimic Malignancy.

Epithelioid hemangioma of bone is a rare benign, locally aggressive vascular tumor that can be particularly challenging to diagnose given its frequent multifocality, non-specific imaging findings, and wide range of morphologic appearances. Additionally, some epithelioid hemangiomas demonstrate atypical histologic features including increased cellularity, necrosis, and moderate cytologic atypia - characteristics that may raise concern for malignancy. Molecular studies can serve as a powerful, objective tool in the differential diagnosis of diagnostically challenging epithelioid vascular tumors. Importantly, FOS and FOSB gene rearrangements have been identified as the genetic hallmarks of osseous epithelioid hemangioma, present in greater than 70% of cases. FOSB-fusion-positive epithelioid hemangioma, in particular, may display atypical histologic features. While ZFP36 is the typical FOSB fusion partner in epithelioid hemangioma, we herein present a case of epithelioid hemangioma of bone with a rare WWTR1::FOSB fusion. This case demonstrates the diagnostic challenges associated with epithelioid hemangioma, especially in the setting of FOSB gene rearrangements, and the importance of genomic studies in the work up of these vascular tumors.

A Rare Case of Epithelioid Hemangioma Presenting as an Isolated Sacral Mass.

Epithelioid hemangioma (EH) is an uncommon benign vascular tumor of mesenchymal origin. It mainly presents as expanding nodules around the ear, the forehead, and long bones. Only a handful of cases have been found in cervical, thoracic, lumbar, and sacral vertebrae as lytic lesions with pain and neurological impairment. We present the case of a 36-year-old female with an incidental finding of a sacral mass along with inguinal lymphadenopathy on imaging. Initially, there were no symptoms. The mass gradually progressed and later showed an extraosseous extension with involvement of sacral neural foramina and nerve roots causing severe low back pain and weakness of the left lower extremity. Differential diagnoses initially included secondary metastases and chordoma. However, the biopsy of the mass revealed findings consistent with an EH. To our knowledge, this is the first case of EH presenting as an isolated mass in the sacrum and the third case of EH involving the sacrum in continuation with other vertebrae. EH should be in our differential diagnoses when there is a sacral mass.

A Case of Adult-Onset Eccrine Angiomatous Hamartoma-The Comparison with Epithelioid Hemangioma.

Eccrine angiomatous hamartoma (EAH) is a relatively rare benign skin disease characterized by the proliferation of eccrine sweat glands associated with capillary hemangioma and the proliferation of other skin elements such as adipose tissue, hair, and epidermis. The onset of the disease is usually at birth or in childhood and tends to occur in the extremities of females, but it occurred in an adult male in this case. The patient was a 72-year-old man with a 12 × 12 mm light brown, elastic, slightly firm skin nodule on the flexor aspect of his right forearm. A biopsy revealed enlargement of blood vessels, sweat glands, sweat ducts, and erector spongiosum with both lumen dilation and narrowing, leading to the diagnosis of EAH. The histopathological features of EAH include a marked proliferation of microvessels, epithelial-like changes in vascular endothelial cells (such as enlarged nuclei), and infiltration of inflammatory cells, mainly lymphocytes and plasma cells. In adult-onset cases, EAH can be clinically difficult to distinguish from epithelioid hemangioma (EH), which differs in the predominance of microvascular proliferation and the presence of eosinophils in the infiltrating inflammatory cells. It can also be distinguished from EAH by the negative results of S100 and anti-EMA in immunohistological staining. In the current cases, we were able to differentiate the two cases from characteristic findings on HE staining.

Categorization of cutaneous epithelioid angiomatous nodule as epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia: Clinicopathologic, immunohistochemical, and molecular analyses of seven lesions.

BACKGROUND: The status of cutaneous epithelioid angiomatous nodule (CEAN) as a distinct entity remains controversial. This study investigated the relationship between CEAN and epithelioid hemangioma/angiolymphoid hyperplasia with eosinophilia (ALHE). METHODS: Data of seven lesions with CEAN features from four cases (Cases 1-4: 61-year-old, 76-year-old, 53-year-old, and 21-year-old men, respectively) were investigated. RESULTS: Cases 1 and 2 showed multiple lesions in the head and neck region, but Cases 3 and 4 showed solitary lesions on the back and scalp, respectively. Moreover, the histopathologic findings of the lesions of Cases 1 and 2 were consistent with those of conventional epithelioid hemangioma or classic cutaneous ALHE. Diffuse immunoexpression of FOSB was observed in Cases 1 and 2, but FOSB split signals were absent in break-apart fluorescence in situ hybridization (FISH). In contrast, the histopathologic findings of the lesions of Cases 3 and 4 were consistent with those of cellular-type epithelioid hemangiomas. Diffuse immunoreactivity for c-FOS was observed in Cases 3 and 4, and split signals of FOS were present in break-apart FISH in Case 3. CONCLUSIONS: This study showed that the seven tumors with CEAN features could be reclassified under the epithelioid hemangioma/ALHE group, although the small sample size is a limitation.

Epithelioid hemangioma of the ascending aorta: A rare case.

Cardiac epithelioid hemangioma is extremely rare. Currently, there are only a few described cases of intraluminal hemangiomas of the aorta and the aortic valve and no described cases with extraluminal epithelioid hemangiomas of the ascending aorta. We now present a case of epithelioid hemangioma of the ascending aorta that was an incidental finding during the coronary artery bypass and successfully resected.