Case E1. Female aged 63 years Right Nephrectomy Two separate tumours Section of each tumour

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1 Case E1 Female aged 63 years Right Nephrectomy Two separate tumours Section of each tumour

2 Tumour 1 Upper pole tumour 28mm macro diameter Circumscribed Friable cut surface

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5 Tumour 2 Middle pole Part solid and part cystic 30mm macro diameter Several small cortical cysts were also noted

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8 100 Answers returned Angiomyolipoma and Mutilocular Clear cell RCC 63 Angiomyolipoma and Clear cell RCC 19 Angiomyolipoma 5 Cystic RCC 3 Angiomyolipoma and cystic nephroma 1 Angiomyolipoma and epithelial cysts 2 Angiomyolipoma and cavernous haemangioma 1

9 Answers - continued RCC sarcomatoid type with cyst 2 Papillary RCC (type2?) and multilocular RCC 1 Papillary RCC with cysts 1 Pseudopapillary tumour with cystic lymphangioma 1 Blank answer 1 Five asked if the patient had known VHL or Tuberous Sclerosis

10 Diagnosis Angiomyolipoma and Multilocular cystic renal cell carcinoma (Grade 1, pt1) With further isolated cysts

11 Expert opinion As it was an unusual case it was referred to Professor Fleming in Dundee for his expert opinion. He agreed this diagnosis. Adding that finding of multiple tumour with cystic change raised the possibility of inherited syndrome of which tuberous sclerosis would be the most likely After liaising with the urologist it was established that there were no clinical or family history to suggest TS, so mutational analysis was not pursued

12 Case E2 Female 22 years Laparoscopic Splenectomy performed Spleen received piece-meal weighing 400g

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16 EQA opinions 100 participants submitted a response There was a wide range of diagnoses submitted: Congestion 22 (many with? Re Hx) Hereditary spherocyosis 22 (many? Needing Hx or saying haemolytic anaemia?hs) Hairy Cell Leukaemia 9 Lymphoma or Leukaemia - 5

17 Hypersplenism 5 Too much red pulp 9 (including?congested,?haemolytic anaemia,?lymphoma or leukaemia, extramedullary haemopoesis) Angiomatosis 2 Littoral Cell Angioma 5 Haemangioma - 2

18 Other offerings Normal 1 White pulp reaction post pneumococcal vaccination 1 Haemophagocytic syndrome 1 Splenic Castleman s disease 1 Enlarged spleen -?EBV 1 Bacillary angiomatosis 1 Histeocytic infiltrate 1 Do not do haematopathology 1 Blank answer (including some which said no idea ) - 10

19 Pathological features Splenic weight 400g (normal g) Expansion of red pulp due to congestion by abnormal red blood cells, including spherical forms Erythrophagocytosis Some haemosiderin deposition No extramedullar haemopoiesis

20 Diagnosis Hereditary Spherocytosis Clinical information given was Hereditary spherocytosis laparoscopic splenectomy Appearances entirely in keeping with this

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22 E3 M 68. Orchidectomy for 5cm testicular tumour.

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27 E 3 Granulosa cell tumour 40 Sex cord stromal tumour probably adult granulosa cell tumour 1? Sex Cord stromal tumour/germ-cell tumour/metastatic prostate cancer 1? Sertoli cell tumour immuno required 1 Sertoli cell tumour 11 Sex cord stromal tumour 2 Carcinoid tumour 6 Carcinoid or neuroendocrine tumour plus more poorly differentiated area exclude background teratoma 2 Strumal carcinoid (overgrowth in teratoma) or granulosa cell tumour or PNET 1 Malignant non-germ-cell tumour (IHC) (carcinoid, lymphoma, metastatic carcinoma) 1? Spermatocytic seminoma with sarcomatous dedifferentiation 1? Metastasis such as prostate? Granulosa cell tumour 2 Neuroendocrine tumour; exclude metastasis 1? Granulosa cell +/- neuroendocrine tumour 3? Sertoli Leydig cell tumour malignant. Rule out neuroendocrine tumour plus PNET 1 NSGCT with yolk sac elements 1 Metastatic poorly differentiated carcinoma 1 Neuroendocrine carcinoma with ITGCN/DDX solid YST with ITGCN 1 Neuroendocrine carcinoma -2. Neuroendocrine carcinoma vs PNET-1 Sertoli cell tumour/ spermatocytic seminoma/ immuno to exclude lymphoma -1 Sex cord ST/SRBCT/Sertoli/Myeloid sarcoma/mtu/ anaplastic seminoma -1 Embryonal carcinoma 3 Lymphoma 1

28 PLAP Oct3/4 CD30 AFP

29 AE1/3 ChromograninA Synaptophysin CD56

30 inhibin Inhibin WT1 calretinin CD99

31 Immunohistochemistry of SCST Leydig cell tumour Sertoli cell tumour Granulosa cell tumour Inhibin Calretinin Keratin Vimentin S100 SMA MelanA + + -/+ + -/ /- +/ /- -/ /+ + +/- -/+ - SCST, NOS +/- +/- -/ /-

32 Adult type granulosa cell tumour of testis

33 E4 Female 69. Emergency admission with abdominal pain. CT shows a caecal tumour with no distant spread. Section is from caecal lesion.

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39 Case E4 Desmoid fibromatosis 33 Favour fibromatosis; cannot exclude Gist needs immuno 5 Bland spindle cells with no mitoses, possibly fibromatosis 1 Fibroma or fibromatosis 1 Gist 22 Spindle cell tumour in muscularis/serosa? Gist? Other (ICC) 2? Gist/? Leiomyoma/? Leiomyosarcoma (ICC) 1 Probable Gant or Gist (ICC) 1 Gist versus leiomyosarcoma 1 Spindle cell lesion? Gist? Schwannoma? MPNST? Leiomyoma? Fibromatosis ( immuno) -1 Spindle cell lesion fibromatosis/gist/leiomyoma/scar from previous operation/neural e.g. schwannoma 1 PECOMA 1 Neurofibroma 1 Sclerosing Mesenteritis 1 Spindle cell neuroendocrine tumour 19? Solitary fibrous tumour 1 Spindle cell tumour? Smooth muscle? Gist? Neural ( ICC) 1 Carcinosarcoma? Other 1 Gist/leiomyoma/peritoneal fibromatosis/spindle cell tumour 1 Spindle cell tumour? nature 1 (Angio) leiomyoma arising from outer muscle layer 2 Endometriosis 1

40 Desmin Caldesmon CD117 dog1

41 Β catenin

42 Dx Intra-abdominal (colonic) desmoid tumour Sporadic In association with FAP Aetiology unknown Association with abdominal surgery, trauma and oestrogen therapy

43 Mutational analysis FAP associated cases germline mutation of APC gene (30% FAP patients develop desmoids) Sporadic colonic desmoid- mutation of β catenin gene (CTNNB1) causing accumulation of nuclear β catenin.

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