PubMed · 14012205
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R ALMA, A LECCESE. [Appendicular agenesis].. https://pubmed.ncbi.nlm.nih.gov/14012205/
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Mucosa-associated lymphoid tissue (MALT) lymphomas usually arise at sites of acquired MALT and are uncommon in native MALT (eg, Peyer patches and tonsil). Malignancy in these low-grade lymphomas is often inferred by immunoglobulin light-chain restriction and expression of CD43; molecular genetic evidence is sought only if these are in doubt. We report 6 cases (4 tonsils, 2 appendixes) of marginal zone (MZ) hyperplasia in children aged 3 to 11 years that, despite histologic and immunophenotypic features indicative of lymphoma, were polyclonal by molecular analysis. No lymphoma-directed therapy was given and patients remain alive and well (5 cases, median follow-up 35.3 months). The involved tonsil and appendix showed florid MZ hyperplasia with prominent intraepithelial B cells (IEBCs). The MZ B cells and IEBCs showed a high-proliferation fraction and a CD20(+), CD21(+), CD27(-), immunoglobulin (Ig) superfamily receptor translocation-associated 1-positive (IRTA-1(+)), CD43(+), multiple myeloma oncogene 1 (MUM-1), IgM(+)D(+) phenotype. Polymerase chain reaction (PCR), cloning, and sequencing of rearranged IgH and Iglambda genes (whole tissue sections [6 cases]; microdissected cells [2 cases]) showed that the MZ B cells and IEBCs were polyclonal and the IgH genes nonmutated. In contrast, MZ (intraepithelial) B cells of 6 control tonsils had a similar immunophenotype, except for expression of CD27 and polytypic light chains, whereas molecular studies showed that they were polyclonal with mutated Ig genes.
The appendix has often been seen more as a nuisance rather than an important part of the human anatomy. Early misconceptions have led to the indiscriminate removal of the appendix from the body. Long thought to be an evolutionary remnant of little significance to normal physiology, the appendix has more recently been identified as an important component of mammalian mucosal immune function, particularly B-lymphocyte-mediated immune responses and extrathymically derived T-lymphocytes. This structure helps in the proper movement and removal of waste matter in the digestive system, contains lymphatic vessels that regulate pathogens, and lastly, might even produce early defences that prevent deadly diseases. The appendix is one of the guardians of the internal environment of the body from the hostile external environment.
A short ureteral length can preclude the use of a continent urinary diversion during urinary tract reconstruction after bladder removal. We present a surgical technique using the in situ appendix to provide length to a shortened left ureter, thus allowing formation of a continent ileocecal urinary diversion.