Pediatric Auricular Angiosarcoma: A Rare Case Report and Review of the Literature

Abstract

Introduction

Limited evidence exists regarding the clinical characteristics of auricular angiosarcoma in the pediatric population. To our knowledge, this represents the inaugural case report of auricular angiosarcoma in early childhood. This report aims to delineate the unique clinical features and findings associated with pediatric auricular angiosarcoma.

Case Presentation

We report a 7-year-old girl presented with a 2-year history of an enlarging, intermittently painful purplish-red mass in the left ear canal and undergo surgery with adjuvant chemotherapy.

Conclusion

Although a rarity, auricular angiosarcoma can manifest in pediatric patients, necessitating vigilant consideration for diagnosis in this population. The evaluation and management of auricular angiosarcoma in pediatric cases demands heightened caution due to the patients’ young age and the high risk of recurrence and metastases following primary treatments.

Introduction

Auricular angiosarcoma is an exceedingly rare vascular malignancy that predominantly affects the external ear. Angiosarcoma comprises only 1% of all soft tissue sarcomas [1], while angiosarcomas of the head and neck region account for only less than 2% of all types of angiosarcoma [2]. While there were several previous reports describing auricular angiosarcoma in adult patients; to this date, clinical evidence on auricular angiosarcoma in children is very scarce. In fact, angiosarcomas in the pediatric population are even more infrequent than those in adults, constituting only less than 1% of all pediatric soft tissue sarcomas [2]. Given the limited existing literature and the distinct demographic and clinical characteristics of pediatric patients, it is imperative to provide a comprehensive description of this malignancy in this population, thus enabling clinicians to perform early recognition and accurate diagnosis of pediatric auricular angiosarcoma and to optimize therapeutic interventions in these patients. Therefore, this case report aims to shed light on the unique clinical presentation, radiological and histopathological features, and therapeutic challenges posed by auricular angiosarcoma in a pediatric patient.

Case Presentation

A seven-year-old girl presented to the Ear, Nose, and Throat (ENT) outpatient department with 2-year history of an enlarging, intermittently painful purplish lump in the left ear canal. There was a history of cracking and pus-filled sores around 1.5 years ago, of which the patient was given antibiotics and painkillers before being referred to our center. Physical examinations revealed a purplish-red mass with a size of 3.5 × 2.5 cm, an irregular surface, and a fragile appearance with bleeding diathesis (Fig. 1a). From the contrast-enhanced computed tomography (CT) scan of the mastoids, we identified a lobulated mass within the cutis and subcutis layers of the left auricle, involving the left external acoustic canal and extending into the left parotid space and bilateral multiple level II-IV neck lymphadenopathy with the largest size measuring 0.6 cm at the level IVA of the left neck lymph node (Fig. 1b). In addition, the carotid CT angiography examination showed an increased vascularization of the mass which received its vascular supply from the branches of the left superficial temporal artery and left posterior auricular artery. Biopsy of the mass yielded atypical cell results, suggesting the possibility of a malignant tumor with granulomatous inflammation. Hence, a diagnosis of auricular malignant tumor without clear histopathological diagnosis was made prior to surgery.

Fig. 1.

Preoperative findings of physical examinations (a) and contrast-enhanced computed tomography (CT) scan of the mastoids (b).

Preoperative evaluation revealed that the tumor not only in the auricular and external ear but also had infiltrated into the left parotid gland and lymph node. Therefore, the patient was undergo auriculectomy and lateral temporal bone resection. We also performed superficial parotidectomy and neck dissection to radically eradicate the malignant cells. Prior to the surgery, the patient’s left temporal artery, left posterior auricular artery, and the left internal maxillary artery were embolized to limit blood loss. The postoperative wound was closed with fat-filled temporal muscle flap and split-thickness skin graft.

Postoperative histopathology and immunohistochemical staining of the resected mass was positive for CD34, ETS-related gene (ERG), vimentin, and anticytokeratin monoclonal antibodies (A1 and A3), and was negative for desmin, myogenin, Sal-like protein 4 (SALL4), and beta-human chorionic gonadotropin (beta-hCG; Fig. 2a–d). These findings suggested a working diagnosis of angiosarcoma. As soon as the patient was diagnosed with Angiosarcoma, our team decided to started on our sarcoma protocol chemotherapy consisting of vincristine and doxorubicin to prevent any metastasis or recurrence.

Fig. 2.

Histopathological features and immunohistochemical staining of the resected mass postoperatively showing histopathological appearance (a), positive staining for CD34 (b), positive staining for anticytokeratin monoclonal antibodies (A1 and A3; c), and positive staining for Vimentin (d).

The recovery following surgery and chemotherapy was uneventful. At 2 months after the surgery, the postoperative wound had healed and there was no peripheral facial nerve palsy noted (Fig. 3a). Contrast-enhanced axial magnetic resonance imaging of the head showed no residual lesions (Fig. 3b).

Fig. 3.

Results of 2-month postoperative follow-up visit showing clinical findings (a) and contrast-enhanced axial magnetic resonance imaging of the head (b).

The patient underwent a thorax CT scan 4 months postoperatively and after chemotherapy, revealing multiple lesions in both the lungs suggestive of metastases. Despite receiving a full chemotherapy regimen, the patient showed no response, and the lung masses became increasingly aggressive. Unfortunately, the patient succumbed to pulmonary failure due to lung metastases 2 months after the diagnosis of metastatic disease.

The CARE Checklist has been completed by the authors for this case report and is attached as online supplementary material at https://doi.org/10.1159/000543993.

Discussion

Cases of angiosarcoma in the head and neck region have rarely been documented in literature. To the best of our knowledge, this report marks the first discussion of auricular angiosarcoma in individuals under the age of 18. In our review, we identified 11 previous reports detailing cases of auricular angiosarcoma [3–12], with a majority of male patients (six out of eleven, 54.5%). Notably, none of these cases involved pediatric patients, with reported ages ranging from 18 to 90 years [3–12]. The exceptional rarity of this disease in the pediatric population underscores the importance of a comprehensive characterization of this patient’s disease.

The diagnosis of auricular angiosarcoma in patients with an enlarging ear mass can be challenging as they often exhibit no complaints or only mild pain. The typical presentation of these patients is usually a friable multinodular mass with irregular borders and purplish-red color. In more advanced stages of the disease, the tumor is prone to bleeding and ulceration, which can lead to anemia, skin and soft tissue infection and tumor progression [12, 13]. In our case, the patient initially presented with an enlarging mass accompanied by intermittent pain, which easily bled upon palpation, thus alarming us on the possibilities of malignancy.

In the diagnosis of auricular angiosarcoma, biopsy examinations remain the gold standard. Histopathological features of angiosarcoma may include epithelioid and spindle areas and vasoformative architecture with hobnailed appearance, whereas the tumor cells usually have atypical nuclei and ill-defined eosinophilic cytoplasm. In addition, immunohistochemical staining of the tumor is typically positive for vascular and endothelial markers (e.g., CD31, CD34, CD144, and factor VIII-related antigen), friend leukemia virus integration (FLI-1), vimentin, and BNH9 and cytokeratin antibodies, as well as negative for S100 protein, epithelial membrane antigen, HMB-45, and human herpesvirus 8. In addition, Ki-67 and p53 immunoreactivity, indicative of a highly proliferative disease, may also be observed in angiosarcoma [13]. This is indeed the case in our patient where immunoreactivity to CD34, vimentin, cytokeratin antibodies (i.e., AE1/AE3), and ERG, a marker closely linked to the ETS transcription factor FLI-1, was observed. Positive marker for ERG and CD34 enabled us to establish the diagnosis of auricular angiosarcoma in this patient. In cases of suspected auricular sarcoma, rhabdomyosarcoma is a critical differential diagnosis. Positive Immunohistochemical staining for Desmin, MyoD1, or myogenin is essential for confirming diagnosis of rhabdomyosarcoma [14]. Furthermore, the negative immunohistochemistry staining for myogenin, beta-hCG, and desmin and SALL4 in our case reduces the likelihood of the disease being rhabdomyosarcoma, choriocarcinoma, and germ cell tumor, respectively [15].

The treatment of resectable pediatric angiosarcoma primarily centers on complete local excision, whereas more advanced cases typically require radiotherapy and anti-angiogenic drugs. Radiotherapy is a common approach in angiosarcoma due to the high local recurrence rate. However, it is important to consider the risks associated with administering radiotherapy to children, as well as its effectiveness primarily in early stage disease. Meanwhile, anti-angiogenic drugs should be part of the treatment consideration for these patients, given the endothelial origin of the disease. For instance, Paclitaxel may inhibit both tumoral and endothelial cell mitosis, thereby impeding tumor growth. Evidence regarding the benefits of chemotherapy in managing pediatric angiosarcoma is still limited and requires further investigation [13]. Nevertheless, adjuvant chemotherapies are known to be valuable in preventing distant metastases and recurrence [16]. In our patient’s case, the multidisciplinary team decided to initiate anti-angiogenic drugs, specifically vincristine and doxorubicin, following surgery, rather than radiotherapy, due to the patient’s young age. This treatment regimen resulted in disease-free status during the latest follow-up visit 2-months after surgery. However, longer-term follow-up is necessary to fully assess the efficacy of these regimens in preventing disease recurrence. Patient eventually had lung metastases that lead to pulmonary failure and mortality. Key prognostic factors believed to influence treatment success in pediatric angiosarcoma include small tumor size (diameter <5 cm), well-differentiated neoplasms, low mitotic count, and a better performance status [13]. Although these prognostic features, except for the well-defined differentiated neoplasms, are seen in our patient, it should be noted that most pediatric angiosarcoma are considered high-grade [13], thus warranting intensive follow-up visits to comprehensively evaluate the patient’s condition and to prevent disease recurrence.

Conclusion

Our case report adds to the body of evidence suggesting that auricular angiosarcoma may also be found in pediatric patients, and that the diagnosis of auricular angiosarcoma in these populations should not be overlooked. The evaluation and management of auricular angiosarcoma in these patients require extra caution considering the young age and the high rate of recurrence and metastases following primary treatments.

Statement of Ethics

This manuscript has received approval from the Ethics Committee (Faculty of Medicine Universitas Indonesia Protocol number 23-11-1790). Written informed consent for publication of this case report and any accompanying information was obtained from the patient’s parents.

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

This study was not supported by any sponsor or funder.

Author Contributions

I.D.M. and A.A.S. conceptualized the study. I.D.M., A.A.S., N.A.C., M.S., and I.S. performed assessments and care of the patient according to each specialty. I.D.M., A.A.S., A.R., N.A.C., M.S., and I.S. provided resources for patient information, clinical examinations, and ancillary investigations. I.D.M. and A.A.S. drafted the manuscript, with critical input from M.A. and H.P.

Funding Statement

This study was not supported by any sponsor or funder.

Data Availability Statement

All data generated or analyzed during this study are included in this article. Further inquiries can be directed to the corresponding author.