ECP 2022 Abstract Book

Virchows Archiv (2022) 481 (Suppl 1):S1–S364 13 *Department of Pathology, Farhat Hached University Hospital, Tunisia Background & objectives: Mucosal melanoma of the nasal cavity(MMNC) is extremely rare. Nevertheless, it is the most common mucosal melanoma of the head and neck region, with an increasing incidence in many western countries. We describe 5 additional cases with a literature review. Methods: A retrospective study of 5 cases of MMNC, diagnosed and treated in our institution between 2000 and 2020. Clinical and pathological data were reviewed. Results: Our series comprised 2 male and 3 female patients. The mean age at diagnosis was 63.2 years (range: 51-86). The most common symptoms at presentation were recurrent epistaxis (4/5 cases) and unilateral nasal obstruction (1/5 cases). All patients were treated surgically. Tumours measured 1.5 to 5 cm. Histologi- cally, 2 tumours consisted exclusively of spindle cells. 3 others were mixed composed of both spindle and epithelioid cells. Surgi- cal margins were negative in only 2 cases. Tumour cells stained with HMB45 and Melan-A antibodies respectively in 5 and 4 cases. One patient developed bone metastasis and two others experienced local recurrence during a median follow-up of 26 months. Conclusion: MMNC is highly aggressive. Therefore, T1 and T2 stages were omitted in the 8th edition of the AJCC staging of these tumours. Prognosis is poor, with a 5-year survival rate less than 40% in most published series. The diagnosis is often delayed due to the “hidden” tumour site and the non-specific clinical presentation. No optimal treatment therapy has yet been defined. A better under- standing of the biology of this tumour will enable the identification of targetable oncogenic driver. E-PS-11-044 Persistent epistaxis – a neoplastic differential M. Rodrigues*, R. Ilgenfritz *Hospital Distrital de Santarém, Portugal Background & objectives: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade sarcoma with neural and myogenic differentiation. It was first described by Lewis et al. in 2012, and since then only about 100 cases have been described. Methods: We submit a case of a 59-year-old man that presents to the emergency department with persistent epistaxis from the left nasal cavity in the previous two days. Nasal endoscopy showed a voluminous neoformation on the left middle turbinate. Magnetic resonance imaging revealed a 40x25x74 mm well-delimited mass with gadolinium enhancement in the same topography. Endoscopic sinus surgery was then performed. Results: Several elastic fragments with dimensions between 10 and 45 mm were received. Microscopic examination revealed sec- tions of respiratory mucosa intersected by a well-differentiated mesenchymal proliferation with a rich vascular stroma, composed by monomorphic spindle cells with clear nuclei. No mitosis, necro- sis, perineural nor lymphovascular invasions were observed. Due to sample fragmentation, surgical margins were not assessed. The tumour cells exhibited focal staining for S100, SMA and β-catenin (both nuclear and cytoplasmic expression). CD34, Calponin, CK CAM5.2, CK AE1/AE3 and Desmin were negative. The lesion was diagnosed as BSNS. Conclusion: BSNS oncogenesis is related to the PAX3 gene which is a transcription factor involved in the development of both muscle and neural tissues of nasal structures. Hence the characteristic staining pattern described. The symptoms are non-specific. It predominantly affects women, and 30-50% of cases recur. No cases of metastases have been reported and only one case of death due to disease is described. The differential diagnosis ranges from benign (e.g. glomangiopericytoma) to aggressive neoplasms with metastatic potential (e.g. synovial sarcoma). E-PS-11-045 A review of adenoid cystic carcinoma patients in Ireland K. Kulakova*, R. O’Shea, C. Hegarty, C. Andrieu, S. Brennan, P. Casserly, S. Kennedy *Research Foundation, Royal Victoria Eye and Ear Hospital, Dub- lin, Ireland Background & objectives: Adenoid Cystic Carcinoma (ACC) of head and neck is a relentlessly progressive tumour. EU incidence is 13 cases per million annually. RVEEH files were analysed over a 20-year period (2002/2021) to establish the clinico-pathologic and molecular findings in Ireland. Methods: Patients with a diagnosis of ACC were retrieved from pathology files. All clinical information was reviewed, and material submitted for NGS-based WES including genes known to be asso- ciated with ACC i.e.. MYB, MYBL, NOTCH, SPEN. A targeted Oncomine NGS mutation panel was also performed on 2 cases. Results: From 15 identified cases, 4 were female and 11 male. The mean age was 45 (range: 20 to 72) at time of initial diagnosis. Primary tumour location; external auditory canal, nasal and oral cavity, lacrimal, parotid, and submandibular glands. All known histologic patterns were observed. Ten patients were treated by surgery and adjuvant radiotherapy (RT). Five patients were inoperable and were treated by RT. Two patients had a local recurrence and four patients developed systemic metastasis (follow-up period 42 to 153 months); sites included cervical and distant (pulmonary, renal, splenic). The average time to recurrence was 44 months. Six patients died on average 4 years post-diagnosis. Genetic analysis is underway. Conclusion: From our findings, the incidence and prevalence of ACCs in Ireland appears to be in line with European average. There is a similar age range. In this study molecular findings will be correlated with clinical pathological characteristics in order to identify potential actionable genetic mutations. E-PS-12 | E-Posters History of Pathology E-PS-12-001 The relic of the Blessed Maria Lorenza Longo founder of the “Hospital of the Incurables” in Naples. A paleopathological and paleoradiological study C. Bellevicine*, A. Brunetti, G. Troncone, L. Ventura, M. Traversari *Public Health Department, Università degli Studi di Napoli “Fed- erico II”, Naples, Italy Background & objectives: Maria Lorenza, born in Spain, followed the husband Joan Llonc (Longo in italian), the chancellor of King Fernando, in Naples. She founded the Hospital of the Incurables. This study aims to verify the historical accounts about Maria Longo. Methods: Maria Longo died in 1539 and was beatified in October 2021. She suffered from a disabling disease attributed either to poi- soning, syphilis or rheumatoid arthritis. Before the beatification, a scientific inspection of her relic was approved by the Curia. In particular, a 16-slice CT scanning with multiplanar reconstructions (MPR) and volumetric (3D) rendering was performed. Results: The relic is represented by a completely skeletonized calvarium and was examined through both visual and digital radi- ology inspection on different projections. The biological profile S291