ECP 2023 Abstracts

S174 Virchows Archiv (2023) 483 (Suppl 1):S1–S391 13 Results: An Intimal Sarcoma was excluded with Undifferentiated Pleo- morphic Sarcoma (UPS) being considered primarily. However, positive immunostaining for rhabdomyoblastic markers, focal glandular forma- tion and loss of H3K27me3 suggested a Malignant Triton Tumour (MMT), although it is now known that loss of H3K27me3 staining is non-specific for nerve sheath differentiation. Sequencing did not identify EWSR1 and FUS gene rearrangements, and FISH was negative for MDM2 amplification. The final favoured diagnostic opinion was an epithelioid rhabdomyosarcoma. Conclusion: In the two months after initial presentation, the patient suffered intestinal perforation complicated by burst abdomen as well as neurological deterioration. Neuroimaging suggested haemorrhagic metastases while pathological examination of the intestines confirmed involvement by sarcoma. The patient succumbed to the disease and complications four months following initial presentation. E-PS-03-009 Primary cardiac synovial sarcoma: a case report W. Majdoub*, K. Ben Lazreg, A. Baccouche, S. Mestiri, D. Benletaifa, F. Saidani, O. Belkacem, A. Bdioui, S. Hmissa *Pathology Department Sahloul Hospital, Tunisia Background & objectives: Synovial sarcoma is an aggressive type of soft tissue sarcoma that usually occurs in the extremities of young adults. Cardiac SS(CSS) is rare accounting less than 5% of all primary cardiac sarcomas. Herein we report another rare case of CSS. Methods: A 27-year-old men with no medical history, presented in the department of cardiovascular surgery for a left intra-atrial mass meas- uring 5 cm of major axis, originating from the septum and prolapsing into the mitral valve. The patient underwent a surgical biopsy. Results: Histological examination of the fragments showed a tumoral proliferation of diffuse pattern. Tumoral cells were large and round with distinctive cellular borders, abundant pale eosinophilic cytoplasm, and vesicular nuclei. Some cells were multinucleated. Low mitotic count (3 mitoses /10HPF) with atypical mitoses were observed. The tumour was highly vascularized with fine branching vessels. Tumoral necrosis was observed. According to immunohistochemistry staining, the tumoral cells were positive for cytokeratin, EMA, and AML. They were focally positive for desmin, PS100 and CD99. Tumoral cells were negative for H caldesmon, MDM2, CD34, MyoD1,Ber Ep4, calretinin. 60% of tumoral cells showed a nuclear expression of TLE1.Moreover, the tumoral cells were positive for the antibody SSX-SS18. Conclusion: The mean age of the few cases reported in literature of CSS is 33.8 years with a potential predilection to occur in males. The monophasic subtype is the most described in CSS. The true poorly differentiated SS is extremely rare. In this site, differential diagnosis can be challenging. The selection of epithelial, mesothelial, myogenic, and vascular-derived specific immunohistochemistry markers may be helpful in resolving the difficulties. SSx-SS18 antibody is useful for diagnosis reflecting the presence of the translocation t(X,18). E-PS-03-010 An unusual case of cardiac metastasis of neuroendocrine tumour A. Pestana Lopes*, B. Martins, J. Cassis, V. Costa Sousa *Hospital da Luz Lisboa, Portugal Background & objectives: Intracardiac neuroendocrine tumour metas- tases are rare and have been estimated to occur in <5% of patients with neuroendocrine tumours. They are generally asymptomatic and detected incidentally. We, hereby, present an unusual case of cardiac metastasis of a neuroendocrine tumour. Methods: A 52-year-old man with no significant past medical his- tory was referred to our Institution for further evaluation of a heart murmur. The patient reported palpitations and chest discomfort. Echocardiography and magnetic resonance imaging revealed a well- circumscribed mass extending from the right ventricular free wall, measuring 29x40 millimetres in the latter. The decision was to remove the cardiac mass surgically. Results: Histological examination of the resected mass was consist- ent with cardiac metastasis of well-differentiated (G2) neuroendocrine tumour, with a proliferation index Ki67 (MiB-1) estimated at 5% and mitotic rate of 3/mm2. On the immunohistochemical study, tumour cells were positive for CK (AE1/AE3), synaptophysin, chromogranin and CDX-2, making spread from a gastrointestinal primary likely. A gallium-68 DOTANOC PET/CT scan was performed, revealing a pri- mary somatostatin-avid tumour within the terminal ileum (SUVmax of 14.45). There was also evidence of somatostatin avid metastatic dis- ease in multiple lymph node groups, in the mesentery and in the liver (SUVmax ranging from 5.00 to 18.46, 28.51 and 17.10, respectively). Conclusion: Our case illustrates a neuroendocrine tumour presenting as a symptomatic cardiac metastasis. While intracardiac metastases from neuroendocrine tumour are exceedingly rare, when they are pre- sent, they are generally detected incidentally and cause no symptoms. Additionally, they are frequently observed in patients with late, wide- spread tumours as shown in our case, based on the findings of the gallium-68 DOTANOC PET/CT scan. E-PS-03-011 Anatomical cardiac changes in individuals with anorexia: could microcardia underlie sudden cardiac death? J. Westaby*, M.N. Sheppard *St George’s University of London, United Kingdom Background & objectives: Anorexia nervosa is an eating disorder char- acterized by low weight and food restriction. There is an increased risk of arrhythmia and premature sudden cardiac death (SCD). We aimed to characterise anatomical cardiac changes in those with anorexia. Methods: We identified 97 cases of anorexia with no other cause of death from 7702 cases of SCD. Anorexia was based upon a previous diagnosis or a body mass index of less than 17kg/m2 without alternate causes. Cases were age and sex matched to normal weight individuals with a BMI of 18.5-24.9kg/m2. Hearts were weighed and measured prospectively. Results: Age was 33±16years with 58 females and 39 males in both anorexia and controls. Weight was 48±8kg and BMI was 15±2kg/m2 in anorexia compared to 65±9kg and 22±2kg/m2 in controls. There was a significantly lower heart weight in individuals with anorexia (270±98g vs 324±74g, p<0.001). Right atrial (44±11mm vs 39±9mm, p=0.005) and left atrial (44±11mm vs 39±8mm, p=0.006) measurements were significantly smaller in anorexia. The left ventricular cavity diameter (28±10mm vs 31±7mm, p=0.006) and lateral muscle wall (12±2mm vs 13±2mm, p=0.037) were significantly smaller in anorexia. Conclusion: These findings raise the possibility that microcardia may underlie SCD in anorexic individuals. Genetic analysis and follow up of relatives will allow detection of genetic susceptibility to SCD or determine whether this is solely acquired. Correlation with phenotype in living patients and outcomes is required. Public health initiatives to address anorexia and good access to psychiatric healthcare may decrease SCD risk. The effect of the intervention on SCD risk is not established therefore prospective assessment is warranted. Funding: Cardiac Risk in the Young fund the cardiovascular pathology laboratories at St George’s University of London E-PS-04 | E-Posters Cytopathology E-PS-04-001 Drug defaulters in tubercular lymphadenitis: a cytopathological study S. Ahuja*, A. Kumari, S. Bajaj, S. Zaheer, S. Ranga *Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India