Gastrointestinal Stromal Tumour of the Appendix: A Very Rare Entity.

A 74-year-old man who presented with upper abdominal pain was found to have an incidental appendiceal mass on cross-sectional imaging. He underwent a laparoscopic appendicectomy with histopathological examination confirming a completely resected appendiceal gastrointestinal stromal tumour (GIST). Appendiceal GISTs are rare. Therefore, there is limited evidence to guide risk stratification and management with extrapolation of prognosis from data on GISTs at other sites. This paper highlights the rarity of these tumours and presents another case which correlates well with the existing but limited literature. There is a need to maintain a registry of this rare disease entity with the maintenance of longer-term follow-up data.

Efficacy of extended microthrombolysis in an elderly lady with purpura fulminans.

Purpura fulminans is a thrombotic emergency affecting small vessels of skin and of internal organs that may rapidly progress into necrotizing fasciitis, critical limb ischaemia and multiorgan failure; it often develops during an infection or as a postinfective 'autoimmune' disorder. Although supportive care and hydration are important, anticoagulation ought to be started to prevent further occlusions alongside blood products according to need. Herein, we describe the case of an elderly woman who received extended intravenous low-dose recombinant tissue plasminogen activator at the onset of purpura fulminans that salvaged her skin and prevented the development of multiorgan failure.

Primary intestinal follicular lymphoma presenting as multiple lymphomatous polyposis.

Multiple lymphomatous polyposis (MLP) is a rare condition, described in the literature as a presentation of extranodal mantle cell lymphoma. We report a rare case of follicular lymphoma presenting as MLP in a young woman with a short history of haematochezia who underwent colonoscopy. Immunohistochemistry on colonic biopsies confirmed follicular lymphoma. Microscopic examination found an extensive and dense lymphoid infiltrate, which demonstrated a follicular growth pattern. The neoplastic cells were positive with BCL2, BCL6, CD10 and CD20, and were negative with CD3, CD5, Cyclin D1 and SOX11. CT staging showed disseminated lymphadenopathy and the patient was commenced on chemotherapy. Endoscopic evaluation and histopathological analysis are vital for the accurate diagnosis of MLP. Our case demonstrates that follicular lymphoma should be considered as a differential, as not all cases of diffuse colonic MLP are related to mantle cell lymphoma. This distinction must be made to provide the best clinical management for the patient.

Segmentation defects of the human axial skeleton without dysostoses or skeletal dysplasias.

The incidence and associations of axial segmentation defects were examined in a series of perinatal autopsies. Variations in vertebral morphology (whether changes in overall numbers or homeotic transformations from one morphological type to another) and of number of well-formed ribs were common, and both were associated with extraskeletal malformations. Rudimentary cervical ribs were present in almost half of all cases and were not specifically associated with extraskeletal abnormalities. Radiographs of all fetuses and infants at autopsy are recommended as they may uncover axial skeletal abnormalities.