Abstract
Epithelioid haemangioma is a rare benign vasoproliferative lesion that commonly affects the head and neck region but is exceptionally uncommon within the nasal cavity, particularly in children. We report a 5-year-old previously healthy child presenting with recurrent left-sided epistaxis associated with progressive anemia. Nasal endoscopy demonstrated a vascular mass arising from the inferior turbinate, while computed tomography revealed a localized polypoid lesion without bony destruction. The patient underwent endoscopic excision with partial inferior turbinectomy, and histopathological examination confirmed epithelioid hemangioma with CD31 positivity. The postoperative course was uneventful, with complete resolution of symptoms and no recurrence during 1 year of follow-up. This case highlights the importance of considering rare vascular tumors in children presenting with persistent unilateral epistaxis.
Introduction
Epithelioid haemangioma (EH) is a rare benign vascular neoplasm that typically presents as painless vascular nodules within the dermal and subcutaneous tissues of the head and neck region. It was first described by Wells and Whimster in 1969 under the term angiolymphoid hyperplasia with eosinophilia [1].
Due to its rarity, EH may be clinically mistaken for a range of benign inflammatory and vascular lesions, including Kimura disease, IgG4-related disease, bacillary angiomatosis, and cutaneous epithelioid angiomatous nodule. It may also mimic malignant vascular neoplasms, including epithelioid haemangioendothelioma and epithelioid angiosarcoma [2].
Historically, EH has overlapped morphologically and terminologically with angiolymphoid hyperplasia with eosinophilia (ALHE). However, contemporary classification recognizes EH as a distinct vascular neoplasm. Recent molecular studies have identified recurrent Fos proto-oncogene, AP-1 transcription factor and FosB proto-oncogene, AP-1 transcription factor gene rearrangements in a subset of EH, whereas ALHE generally lacks these alterations, supporting the distinction between these entities.
Complete surgical excision is generally regarded as the treatment of choice for localized EH. Other treatment modalities, including laser therapy, cryotherapy, intralesional corticosteroid injections, and radiotherapy, have been described in selected cases, with variable success [3].
Only a limited number of EH cases involving the nasal cavity have been reported, most of which occurred in adults and involved sites other than the inferior turbinate. To our knowledge, pediatric intranasal EH arising from the inferior turbinate has not been clearly documented in the available English-language literature. However, given the historical overlap between EH and ALHE terminology, this case should be considered an exceptionally rare presentation rather than a definitive first reported case. We report a 5-year-old previously healthy child who presented with a 10-day history of recurrent left-sided epistaxis.
Case presentation
We report a 5-year-old previously healthy child presenting with a 10-day history of recurrent left-sided epistaxis, progressively increasing in frequency and severity, resulting in multiple emergency department visits. Clinical evaluation revealed pallor and ongoing anterior nasal bleeding. Laboratory investigations demonstrated progressive anemia, with hemoglobin declining from 8.4 g/dL to 7.4 g/dL. The patient had no significant past medical or surgical history and was not taking any medications. There was no history of trauma, bleeding disorders, anticoagulant use, allergic disease, or relevant family history. No significant psychosocial or environmental contributing factors were identified.
On examination, nasal endoscopy demonstrated a smooth, pinkish, polypoid mass arising from the anterior aspect of the inferior turbinate. The lesion appears well-circumscribed with a lobulated surface, partially occupying the nasal cavity and causing narrowing of the adjacent nasal passage (Fig. 1).
Endoscopic view of the nasal cavity showing a smooth, reddish, well-circumscribed vascular mass arising from the inferior turbinate, representing an epithelioid hemangioma.
“A non-contrast computed tomography of the paranasal sinuses identified a 3.0 ‘0.7 cm polypoid soft tissue lesion arising from the left inferior turbinate without evidence of bony destruction”. Before surgical intervention, conservative measures, including anterior nasal compression and topical vasoconstrictive therapy, were attempted without sustained control of bleeding. Due to persistent recurrent epistaxis and progressive anemia, nasal packing was required for temporary hemostasis before definitive surgical management.
The patient underwent endoscopic excision of the lesion with partial inferior turbinectomy. Concurrent adenoidectomy was performed because of symptoms suggestive of obstructive sleep apnea. Surrounding nasal structures were preserved as much as possible. Hemostasis was achieved intraoperatively, and the patient tolerated the procedure well without perioperative complications.
Histopathological examination confirmed epithelioid hemangioma, supported by immunohistochemical positivity for CD31 and smooth muscle actin.
The postoperative course was uneventful, with complete resolution of epistaxis and no evidence of recurrence or postoperative complications on 2 weeks, 6 months, and 1-year follow-up. The patient’s parents reported significant concern regarding the recurrent bleeding episodes and repeated emergency visits before diagnosis. Following surgical treatment, they expressed satisfaction with the clinical outcome and resolution of symptoms.
Discussion
Epithelioid haemangioma is a rare benign vascular neoplasm that typically presents as painless nodules in the head and neck region. It is not commonly included in the differential diagnosis of a nasal cavity mass in routine clinical practice, particularly in pediatric patients. When present within the sinonasal tract, symptoms may vary according to lesion size and location and may include epistaxis, nasal obstruction, facial pressure, or incidental discovery during examination [3].
The pathogenesis of EH remains poorly understood, although a reactive origin has been widely supported in the literature. The presence of lymphocytic infiltrates in older lesions, together with a history of local trauma or vascular injury, provides evidence for this theory [4]. However, alternative aetiological mechanisms should also be considered.
Radiological findings in epithelioid haemangioma are often non-specific. Imaging is primarily valuable for determining lesion size, site of origin, extent, and the presence of adjacent structural involvement, thereby assisting operative planning [5]. In our case, computed tomography demonstrated a polypoid soft tissue lesion arising from the left inferior turbinate without evidence of bony destruction, supporting a localized benign process.
Histologically, EH is characterized by proliferating small blood vessels lined by epithelioid endothelial cells, accompanied by a variable inflammatory infiltrate rich in eosinophils and lymphocytes. Immunohistochemical staining is useful for confirming the vascular nature of the lesion, with endothelial markers such as CD31 typically positive [4]. In our case, the diagnosis was supported by positivity for CD31 and smooth muscle actin, molecular testing for FOS/FOSB rearrangements was not available; therefore, the diagnosis was based on the histopathological findings and immunohistochemical profile.
Epithelioid haemangioma is an exceptionally rare lesion, with only a limited number of nasal cases reported in the literature. Most reported cases have occurred in adults and have involved various anatomical sites, including the nasal tip, middle turbinate, nasal vestibule, and the skin overlying the nose [6, 7]. To our knowledge, this is the first reported case arising from the inferior turbinate. In the pediatric population, only two cases have been previously reported, involving patients aged 12 and 13 years; both were cutaneous in origin, affecting the nostril and the left side of the nose, respectively, rather than the intranasal EH [8, 9].
Our case is particularly unusual because the lesion originated from the inferior turbinate in a 5-year-old child and presented with recurrent unilateral epistaxis severe enough to result in progressive anemia. Diagnostic evaluation in this case was challenging because sinonasal epithelioid hemangioma is exceptionally rare in the pediatric population and may clinically mimic more common inflammatory, vascular, or neoplastic lesions of the nasal cavity. The nonspecific radiologic appearance further complicated preoperative diagnosis, underscoring the need for histopathological examination for definitive diagnosis. A limitation of this report is that molecular testing for FOS/FOSB rearrangements was not available. Although the diagnosis was supported by the histopathological features and endothelial marker positivity, molecular analysis could have provided additional support for distinguishing EH from morphologically overlapping lesions, particularly ALHE.
This case highlights the importance of considering rare vascular tumors in children presenting with persistent unilateral nasal bleeding, especially when accompanied by declining hemoglobin levels.
Conclusion
This case highlights the importance of considering rare vascular tumors in children presenting with recurrent unilateral epistaxis and declining hemoglobin levels. Early recognition and surgical management are essential to prevent complications.
Conflicts of interest
None declared.
Funding
None declared.
