Title: Rare angiosarcoma in the postauricular region - a diagnostic challenge and treatmentShort Title: Hemangiosarcoma of the mastoid regionAuthors: Katarzyna Żyżynska, MD1; Kamila Grycner, MD1; Dmitry Tretiakow, MD, PhD1;prof. Andrzej Skorek11Clinical Department of Otolaryngology of the Academy of Applied Medical and Social Sciences in Elblag, Hospital Nicolaus Copernicus in Gdansk, PolandCorresponding author: Katarzyna Żyżyńska, Clinical Department of Otolaryngology of the Academy of Applied Medical and Social Sciences in Elblag, Hospital Nicolaus Copernicus in Gdansk 80-152, Poland; tel. +48 587640834; email:katarzyna.zyzynska@gmail.comKeywords: hemangiosarcoma, head and neck sarcoma, cranial baseCompeting interests: None.Funding source: None.Acknowledgments: Not applicable.Title: Rare angiosarcoma in the postauricular region - a diagnostic challenge and treatmentAuthorship: Katarzyna Żyżynska,1,2,3,4; Kamila Grycner 1,2,3,4; Dmitry Tretiakow, 3,4,; Andrzej Skorek 3,4Acknowledgments: Not applicable.Conflict of Interest Statement: The authors declare no Conflicts of interst.INTRODUCTION:Sarcomas are rare malignancies of mesenchymal origin, accounting for 1% and 0.2% of all bone and soft tissue cancers, respectively. Hemangiosarcoma, a vascular neoplasm derived from endothelial cells, is particularly uncommon, comprising less than 2% of soft tissue sarcomas and fewer than 1% of head and neck malignancies. It predominantly affects the scalp and face of individuals over 60, with a higher incidence in males and Caucasians. [1,2]Treatment of hemangiosarcomas includes surgery, radiation therapy, and chemotherapy. These methods are often combined to address both local and systemic disease [3,5]. Targeted therapies and immunotherapy are also being explored to improve outcomes. Our patient was treated successfully with surgery and proton therapy. There is limited specific literature on proton therapy for angiosarcoma of the head; however, available case reports and general principles suggest that proton therapy could be a viable and safe option for treating angiosarcomas in sensitive areas like the head and neck [4].CASE REPORT:A 72-year-old white male patient presented to the hospital on an acute-care basis with a complex set of symptoms involving the left ear and surrounding area. The patient reported swelling in the retroauricular region, accompanied by auricular protrusion and hearing loss that had persisted for approximately two weeks. Additionally, he experienced purulent-bloody discharge from the left ear, though notably without associated pain. The patient denied any dizziness or nausea, and clinical examination revealed no signs of facial nerve paresis.To further investigate these symptoms, a head CT scan was performed. The imaging results revealed dense shadowing of the middle ear and mastoid process, with evidence of bony lysis in the mastoid process. Based on these findings, the lesion was initially described as a mastoid process/periauricular abscess. Interestingly, despite the concerning presentation, the patient reported no additional complaints beyond the aforementioned symptoms. Laboratory tests were conducted, showing no elevation in inflammatory parameters, which was somewhat unexpected given the clinical picture.Following an ENT consultation, the patient was admitted to the hospital and deemed suitable for surgical intervention. During the procedure, the surgical team encountered an inflammation-damaged mastoid filled with soft-tissue lesions. Upon further exploration, a periareolar lesion containing septic-pleural contents was excavated. The surgeons noted that the wall of the dura and sigmoid sinus appeared unaltered. However, they identified a fistula connecting to the external auditory canal, adding complexity to the case.Intraoperative examination and microbiological diagnosis were performed as part of the standard protocol. Surprisingly, the intraoperative examination did not confirm the presence of a malignant lesion. Instead, it revealed a fusiform granuloma with foci of myofibroblasts (SMA+) and macrophages (CD68+). This finding, while not indicative of malignancy, presented a diagnostic challenge given the unusual presentation and imaging results.Following consultation with the hospital microbiologist, targeted antibiotic therapy with ceftriaxone and gentamicin was initiated based on culture results showing Proteus mirabilis and Escherichia coli. This treatment led to local improvement.The initial histopathological examination revealed no malignancy. The predominant finding was droplet-rich granulation tissue with some endothelial cells (CD31+, FVIII+, CD56+) showing secondary atypia. The granuloma contained stimulated myofibroblasts (SMA+) and macrophages (CD68+). No epithelial (CK Pan-, p40, p63-) or melanocytic (S100-) cells were identified.Five months post-surgery, the patient returned with retroauricular pain, periodic dizziness, and an ulcerated nodule with complete wound dehiscence. Re-operation and subsequent histopathological examination confirmed hemangiosarcoma - angiosarcoma exulcerans (CD31+, CD34+, CKPan-, p63-).Expanded diagnostic imaging, including MRI, CT, and PET scans, revealed tumor penetration into the middle cranial fossa. The patient underwent proton therapy (70 Gy/55 fr) at the National Cancer Institute’s Proton Therapy Center.One year post-treatment, CT scans showed no evidence of pathology or local recurrence. However, two years after initial hospitalization, chest CT revealed a metastatic nodule in the left lung, confirmed by biopsy. The patient underwent uncomplicated lobe resection.Four years post-second surgery, the patient remains under close oncological and ENT surveillance. While a CT scan showed a concerning lesion in the left external auditory canal, biopsy revealed only inflammatory granulation tissue without malignancy. Given the high-grade nature of angiosarcomas, the patient continues to require regular follow-up and imaging.Discussion:Angiosarcoma of the temporal bone is an extremely rare malignancy that can mimic chronic otitis media. This case highlights the challenges in diagnosis, as initial symptoms and histopathology did not suggest malignancy. The atypical postoperative course, including abnormal healing and rapid lesion regrowth, raised oncological concerns.This case underscores the importance of multidisciplinary collaboration among ENT specialists, radiologists, and histopathologists in managing such rare and aggressive tumors. The successful use of proton therapy in achieving remission is noteworthy, though long-term follow-up is crucial due to the high risk of recurrence and metastasis associated with angiosarcomas.Conclusion:This case report illustrates the diagnostic challenges and management complexities of temporal bone hemangiosarcoma. It emphasizes the need for vigilant follow-up and a high index of suspicion in cases with atypical presentations or postoperative courses. The successful use of proton therapy in this case offers a promising treatment option for similar cases, though long-term outcomes require further study.References:Lydiatt WM, Shaha AR, Shah JP. Angiosarcoma of the head and neck. Am J Surg . 1994;168(5):451-454. doi:10.1016/s0002-9610(05)80097-22. Fury MG, Antonescu CR, Van Zee KJ, Brennan MF, Maki RG. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy [published correction appears in Cancer J. 2005 Jul-Aug;11(4):354]. Cancer J . 2005;11(3):241-247. doi:10.1097/00130404-200505000-000113. Chen JX, Kozin ED, O’Malley J, et al. Otopathology in Angiosarcoma of the Temporal Bone. Laryngoscope . 2019;129(3):737-742. doi:10.1002/lary.272814. Kalavrezos N, Sinha D. Head and neck sarcomas in adulthood: current trends and evolving management concepts. Br J Oral Maxillofac Surg . 2020;58(8):890-897. doi:10.1016/j.bjoms.2020.05.0155.Vitolo V, Barcellini A, Mirandola A, et al. Is Proton Beam Radiotherapy Worthwhile in the Management of Angiosarcoma of the Scalp?. Anticancer Res. 2020 Mar;40(3):1645-1649. doi: 10.21873/anticanres.14114.Figure I.Computed tomografy scan A. before treatment B. after first surgery C. after second surgery D. after proton therapy