EDUCATIONAL CASES E1 & E2 Natasha Inglis 20/03/15
CASE E1 79 year old female Rectum. Altemeier operation
Histology Superficial erosions and mucosal congestion volcano lesion and pseudomembrane formation Serration of glands No dysplasia or malignancy
Dx: RECTAL PROLAPSE
Discussion Symptoms: Obvious rectal protrusion, faecal incontinence, constipation, rectal bleeding Causes: Repeated childbirth, heavy lifting, prior hysterectomy, increasing age. Unsurprisingly, most patients are female
Treatment Altemeier operation avoids an anterior resection by utilising a perineal approach to resect prolapsed portion of rectum and then ends are anastomosed together. Delorme procedure is similar, but only the mucosa is resected.
Pathology Superficial erosion/ulceration mucosa Pseudomembrane formation Capillary congestion and thrombosis Thickened muscularis mucosae with smooth muscle seen around crypts Crypts may show cystic dilatation, villous hyperplasia or serration
Beware Displaced submucosal glands +/- mucus extravasation localised colitis cystica profunda Can be mis-diagnosed as invasive adenocarcinoma as in the following case from ~20 years ago
Biopsy and resection reported as probable rectal prolapse but a well-differentiated adenocarcinoma cannot be excluded For past 17 years patient has believed that she had a resection for cancer!
CASE E2 71 year old female Laparoscopic distal pancreaticosplenectomy for cystadenoma of pancreas Macro: distal pancreas and spleen with thin-walled fluid filled unilocular cyst just anterior to pancreas
Histology Normal pancreas Thin walled cyst lined by attenuated single layer of cells Lymphoid tissue present in wall
Immunohistochemistry Cyst lining positive for CD31 AE1/3, Calretinin, CK7 and CD34 all negative
Dx: Cystic Lymphangioma of Pancreas
Discussion Rare, usually occur in head and neck region Females > males Usually occur body and tail Aetiology unclear:? Occur during embryological development,? Secondary to trauma/ surgery/ inflammation / XRT 3 types: capillary, cavernous and cystic
Discussion cntd Most are symptomatic and present with pain and / or abdominal mass Not really possible to diagnose radiologically Require radical excision as can recur Can behave aggressively locally, necessitating resection adjacent organs Very very rare malignant transformation
ED4 58/F No clinical history given Nephrectomy specimen with a circumscribed brown tumour 78x49mm.No necrosis, renal sinus and renal vein clear. The capsule was intact
Chromophobe RCC/oncocytoma Unusual RCC Unusual renal epithelial tumour with intracytoplasmic eosinophilic inclusions HCC mets, collecting duct carcinoma, No atypical mitoses or necrosis No evidence of Mucin Hales colloidal iron was very weak On ICC Positive Cam 5.2 Negative CK7,Vimentin,RCC, CD10 Electron microscopy- unusual. Cells contained abundant mitochondria with normal to many abnormal forms. There was enlargement of the mitochondria but with preserved cristae.in extreme forms no cristae were noted except for some myelin figures. Molecular genetic studies showed a loss of succinate dehydrogenase subunit B gene
Succinate dehydrogenase complex,subunit B,iron sulphur(ip) A,B,C,D Subunits SDH enzyme critical respiratory enzyme in the Krebs cycle needed to convert Succinate to Fumarate >150 mutations have been identified Hereditary Paraganglioma-pheochromocytoma Cowden syndrome GIST Pituitary adenoma SDH deficient Renal carcinoma: A morphologically distinct entity. A clinicopathological series of 36 tumours from 27 patients Anthony J Gill et al ( Am J Surg Pathol. 2014;38
Rare tumours incidence 0.05% to 0.2% Slight male preponderance 1.3:1 All cases of SDH RCC have shown mutations in one of the subunits Loss of ICC staining for SDHB 15% associated with synchronous or metachronous tumours Solid tumours tan to brown Well circumscribed ISUP 2 in most cases Morphologically distinct Negative staining with CK s(68%) Multifocal,young age, personal/family history of RCC or other tumours Risk of metastases High grade tumours subtle
Solid tumours tan to brown Well circumscribed ISUP 2 in most cases Morphologically distinct Negative staining with CK s(68%) Multifocal,young age, personal/family history of RCC or other tumours Risk of metastases High grade tumours subtle