Adenocarcinoma Arising from the Rectal Stump Eleven Years after Excision of an Ileal J-Pouch in a Patient with Ulcerative Colitis: Report of a Case

Adenocarcinomas in relation to the ileal J-pouch after restorative proctocolectomy for ulcerative colitis have been recently reported with increasing frequency. All previously reported cases have occurred in patients with their ileal pouch in situ. We report a case of adenocarcinoma in the anal canal 11 years after removal of a failed ileal J-pouch. Mucosectomy had been performed at the restorative proctocolectomy. The anus had been left in place at the pouch excision because of severe fibrosis in the pelvis. If it is decided to remove an ileal pouch permanently, a total abdominoperineal excision should be performed, particularly in patients with risk factors for cancer development. Reprints are not available.     

Adenocarcinoma at the Site of a Healed Gastric Ulcer after 10 Years of Endoscopic Observations

An adenocarcinoma developed in a 52-yr-old man during the course of a 10-yr gastroscopic and biopsy followup of a benign gastric ulcer that had remained healed for over 7 yr after a recurrence of the initially healed lesion. Histologically the lesion was an early gastric carcinoma of the intestinal type, with the rest of the mucosa showing an element of intestinal metaplasia and chronic gastritis. Development of a carcinoma in the healed scar of a benign gastric ulcer is rare; however, patients with healed gastric ulcers should be followed up endoscopically.     

Adenocarcinoma Developing at an Ileostomy: Report of a Case and Review of the Literature

Primary adenocarcinoma of a permanent ileostomy is a rare and unusual complication. We report a case of primary adenocarcinoma arising at an ileostomy site 46 years after total proctocolectomy for Crohn’s colitis. In addition, we performed a literature search and found 36 such cases reported. Based on the results of this case and literature review, we concur with the previously reported theory that the etiology of this phenomenon is likely the result of colonic metaplasia in the ileal mucosa, which eventually progresses to carcinoma. Common presenting symptoms include a bleeding, friable mass, difficulty fitting the stomal appliance, and bowel obstruction. Once confirmed by biopsy, appropriate surgical en bloc excision and stomal relocation is the mainstay of therapy. Lymph node metastasis occurs in 19 percent of patients and survival is at least 85 percent. Adjuvant therapy may be of additional benefit. Patient education is important for early detection as the lesion typically appears an average of 27 years after the original operation.     

Pseudomyxoma peritonei occurring after an uneventful 23 years interval from appendectomy

A 77-year-old male was admitted to our hospital for a bulky abdominal mass. He had a history of appendectomy under the diagnosis of appendiceal rupture 23 years previously. He also had received a radical lung resection for an early lung cancer 2 years earlier in another hospital. Tentative diagnosis of peritoneal metastases from the lung cancer was made. He then received 3 courses of chemotherapy, but failed to reach a remission. The final diagnosis of pseudomyxoma peritonei was made by means of abdominocentesis, and he underwent debulking surgery. However, he died on day 56 after the surgery. Pseudomyxoma peritonei requires careful observation, as it has the possibility to be detected after a long-term follow-up period of more than 20 years.     

Relation of an interleukin-23 receptor gene polymorphism to graft-versus-host disease after hematopoietic-cell transplantation

Polymorphisms in cytokine genes can influence immune responses and inflammation and thereby affecting the outcome of hematopoietic stem-cell transplantation. We analyzed a single-nucleotide polymorphism in the gene for the interleukin-23 receptor (IL-23R) (1142G>A) in a cohort of 221 transplant recipients and their human leukocyte antigen (HLA)-identical sibling donors and in a second cohort of 186 transplant recipients and their HLA-identical unrelated donors. Genotypes were tested for an association with graft-versus-host disease (GVHD) by multivariate analysis. The donor's IL-23R genotype was significantly associated with a reduced risk of acute GVHD in both cohorts for patients after transplant. Analysis of all 407 transplant recipients showed that IL-23R (1142G>A, Arg381Gln) genotype of the donor was associated with a decreased risk of grades 2-4 acute GVHD (31.6 compared to 51.0%, P=0.02) and grades 3-4 severe acute GVHD (3.9 compared to 23.4%, P=0.003). Death in remission was significantly lower in patients transplanted from donors with variant IL23-R (11.7 versus 27.7%, P=0.028), whereas overall survival or relapse rates were not influenced significantly by the IL-23R genotype. Among recipients of hematopoietic cells from HLA-identical donors, the IL-23R (Arg381Gln) gene variant on the donor side has a protective effect on the occurrence of acute GVHD in recipients after transplantation.     

Villous Adenocarcinoma Arising in the Bypassed Duodenum 18 Years after a Billroth II Subtotal Gastrectomy: Report of a Case and Review of the Literature

Eighteen years after a Billroth II procedure for a duodenal ulcer, a 65-year-old man presented with a large epigastric mass which was clinically interpreted as a pancreatic carcinoma. Fourteen months later, reevaluation of the enlarging mass led to a diagnosis of a villous tumor of the afferent limb of the gastroenterostomy. Laparotomy revealed an advanced villous adenocarcinoma. We could find only one other similar case in a 50-year literature review.     

Therapy-Related Acute Myeloid Leukemia 6 Years after Clonal Detection of inv(11)(q21q23) and MLL Gene Rearrangement

Results of recent studies with animal models suggest that expression of MLL fusion proteins promotes acute leukemogenesis. However, the most potent MLL fusion proteins are not sufficient for the development of acute myeloid leukemia (AML). The clinical data on the pathogenesis of this type of leukemia are limited. We analyzed the case of a patient with therapy-related AML with MLL rearrangement. The patient initially developed AML with t(8;21). Although the patient achieved complete remission with chemotherapy, an abnormal karyotype, inv(11)(q21q23), was detected. After 6-year persistence of a clone with the inversion 11 karyotype in the bone marrow, secondary AML developed. Results of fluorescence in situ hybridization analysis combined with magnet-activated cell sorting analysis showed that MLL rearrangement was detected in CD34+ and CD13+ fractions but not in a CD3+ fraction of the bone marrow. There were 2 important clinical findings. One was that MLL rearrangement was not sufficient for the development of leukemia. The other was that MLL rearrangement targets specific lineages.     

Developing an algorithm of informative markers for evaluation of chimerism after allogeneic bone marrow transplantation

Analysis of chimerism by polymerase chain reaction amplification of STR or VNTR has become a routine procedure for the evaluation of engraftment after allogeneic stem cell transplantation. Knowledge of the frequency of different STR or VNTR alleles in unrelated individuals in a population is useful for forensic work. In the context of HLA identical sibling bone marrow transplantation the informativeness of these markers needs to be evaluated. We evaluated five STRs (THO1, VWA, FES, ACTBP2, and F13A1) and 1 VNTR (APOB) for informativeness in stem cell transplants from HLA identical sibling donors. All four markers used individually allowed us to discriminate 20-56% of the patient donor pairs. Using a combination of all these markers along with a polymorphic marker in the beta-globin gene and the sex chromosome specific amelogenin marker, we were able to discriminate 99% of the patient donor pairs. We have established an algorithm for evaluating chimerism following HLA identical sibling donor transplants in the Indian population using molecular markers in 310 patients. Analysis of heterozygote frequencies in different populations is similar suggesting that this algorithm can be used universally for transplant centers to evaluate chimerism following allogeneic bone marrow transplantation.     

Multiple Malignant Lymphomas of the Bile Duct Developing after Spontaneous Regression of an Autoimmune Pancreatitis-like Mass

We herein report a 67-year-old woman with malignant lymphomas of the bile duct that developed after regression of a pancreatic head mass. Computed tomography suggested the mass was pancreatic head cancer. Endoscopic ultrasonography showed a low-echoic mass with hyperechoic strands resembling autoimmune pancreatitis. Her serum IgG4 concentration was elevated to 674 mg/dL. After the pancreatic head mass spontaneously diminished, three masses were detected in the common bile duct. A biopsy of the major papilla revealed high-grade B-cell lymphoma with MYC, BCL2 and/or BCL6 rearrangement. Systemic chemotherapy with rituximab plus etoposide, prednisolone, vincristine, cyclophosphamide and doxorubicin resulted in complete remission.     

11q23 Aberration is an additional chromosomal change in de novo acute leukemia after treatment with etoposide and mitoxantrone

We report on 2 patients with acute leukemia who had an 11q23 chromosomal aberration as an additional change after treatment with etoposide and mitoxantrone, agents that affect topoisomerase II (Topo II). One patient with Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (L2) received chemotherapy, including 1,000 mg of etoposide and 75 mg of mitoxantrone. She relapsed 10 months later. Analysis at time of relapse showed a chromosomal aberration of del(11)(q23) as an additional cytogenetic change. The other patient was diagnosed with acute monoblastic leukemia (M5a) and received two autologous peripheral blood stem-cell transplantations. Her cumulative doses of etoposide and mitoxantrone were 6,000 mg and 42 mg, respectively. She also relapsed, and analysis at that time revealed del(11)(q23) as an additional chromosomal aberration. The mixed lineage leukemia/(myeloid-lymphoid leukemia (MLL) gene was not rearranged in either case, making these cases distinct from previously described therapy-related leukemias caused by Topo II Inhibitors. Based on these two cases, it may be that Topo II inhibitors can cause clonal evolution affecting chromosome band 11q23. © 1996 Wiley-Liss, Inc.     

A Case in which an Intraductal Papillary Neoplasm of the Bile Duct Was Surgically Resected 12 Years after the Initial Diagnosis

A 66-year-old Japanese man was referred to our hospital with multiple giant liver cysts. The cysts had already been detected as multiple 3-cm cysts with small nodules at another hospital 12 years prior to this presentation. The cysts were diagnosed as an intraductal papillary neoplasms of the bile duct (IPNB) occupying the right lobe of the liver. Extended right lobectomy was performed. Based on the pathological findings, the tumor was diagnosed to be an oncocytic-type IPNB with minimal invasion. This experience suggests that the progression of IPNBs occur relatively slowly. The present case might provide important information for understanding the natural history of IPNBs.     

Symptomatic Pneumocephalus Occurring Years after Transphenoidal Surgery and Radiation Therapy for an Invasive Pituitary Tumor: A Case Report and Review of the Literature

We present the unusual case of a patient who experienced a CSF leak and subsequent pneumocephalus several years after transphenoidal surgery and radiation treatment for an ACTH secreting pituitary tumor. This patient underwent aggressive management including placement of a lumbar drain, and reoperation to stop her leak. Despite these efforts, her leak persisted resulting in significant tension pneumocephalus. Pneumocephalus is a rare but important potential complication of transphenoidal surgery. Here we discuss our management of this patient's condition, and review the existing literature.