Mucinous adenocarcinoma arising from chronic perianal fistula

Perianal mucinous adenocarcinoma is a rare tumour which may be associated with long‐standing chronic perianal sepsis. Early diagnosis is challenging and is based on a high index of clinical suspicion and specific histological features. Definitive treatment is surgical, in the form of an abdomino‐perineal resection. We hereby describe a case of a perianal mucinous adenocarcinoma arising from long‐standing recurrent perianal fistula and complement this with a brief review of the literature pertaining in particular to the management of this condition.     

Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome: report of five cases

.We report five patients with Laurence-Moon-Bardet-Biedl syndrome (LMBBS) who had renal involvement. Intravenous pyelography showed bilateral or unilateral calyceal clubbing and blunting in all patients. In addition, one patient had a parapelvic cyst in the left kidney and another had bilateral lobulated renal outlines of the fetal type. One patient had a urinary concentrating defect and two patients showed increased fractional sodium excretion. Estimated tubular phosphate reabsorption values were in normal limits in all of five patients. No patient had a urine acidification defect, proteinuria, glycosuria, or hyperaminoaciduria. One patient died from end-stage renal failure. The remaining four patients had normal serum creatinine values and estimated creatinine clearances. ^99mTechnetium-diethylenetriamine pentaacetate renal scanning showed prolonged and delayed concentration and delayed excretion in three of the four patients who survived. A focal scar was determined on the left kidney of one of four patients by ^99mtechnetium-dimercaptosuccinic acid renal scanning. All LMBBS cases with or without renal symptoms should be routinely evaluated for renal abnormalities. Renal scanning is a valuable method, especially for determining the renal involvement in the early stage of disease. Received November 14, 1995; received in revised form and accepted May 23, 1996     

Reversed-J inferior sternotomy for awake coronary bypass.

Many approaches for minimally invasive coronary bypass surgery are available and to further decrease the invasiveness, coronary artery bypass grafting has been performed under high thoracic epidural anesthesia without endotracheal intubation in the last years. Less invasive approach to coronary artery bypass graft operations is possible through combination of the high thoracic epidural anesthesia and a reversed-J sternotomy, and coronary revascularization can be accomplished without any additional technical difficulties and with a good exposure of both the left anterior descending artery and the left internal thoracic artery. This technique is less traumatic for patients and provides practical better oxygenation and shorter hospital stay.     

Rotavirus diarrhea in Bangladeshi children: correlation of disease severity with serotypes.

To improve the understanding of the relative importance of serotypes of rotavirus in dehydrating diarrhea, we examined the correlation of clinical characteristics and disease severity with serotype in 2,441 diarrheal episodes among children younger than 2 years of age in rural Bangladesh. Of 764 rotavirus-associated episodes, a single G type (serotype 1, 2, 3, or 4) was determined by oligonucleotide probe in 485 (63%), while 233 episodes were nontypeable. Episodes with G types 2 and 3 were associated with more-severe dehydration than episodes associated with G type 1 or 4 or with nontypeable rotavirus. Episodes did not differ by G type in prevalence of vomiting, copious diarrhea, fever, abdominal pain, or length of treatment center stay. Rotavirus reinfections were detected in seven children, with homologous reinfection (G type 2) in one. Twelve children with diarrhea who died had rotavirus detected in stool specimens within 30 days of death. Children who died were more likely to be malnourished than were surviving children with rotavirus diarrhea. Of 40 specimens tested by polymerase chain reaction, 29 (72.5%) were P type 1, 9 (22.5%) were P type 2, 1 (2.5%) was P type 3, and 1 (2.5%) was nontypeable. One severely symptomatic diarrheal episode was associated with P type 3 rotavirus, a serotype usually found in asymptomatic nursery infections. Although G types 2 and 3 were associated with more-severe dehydration than other serotypes, the differences do not appear to be of major clinical importance. Effective vaccines should protect against all four major G types.     

Determination of allelic deletion of multiple endocrine neoplasm type 1 (MEN1) gene in acute myeloid leukemia (AML) by application of FISH-TSA technique

We have used the single and dual color fluorescence in situ hybridization (FISH) technique combined with a new detection system, tyramide signal amplification (TSA), by using the multiple endocrine neoplasm type 1 (MEN1) gene and chromosome 11 specific alpha satellite DNA probes for the study of the allelic deletion of the MEN1 gene. The MEN1 gene is a new tumor suppressor gene and has been recently cloned on chromosome 11q13. FISH combined with the TSA detection system was performed on bone marrow interphase nuclei of 22 patients with acute myeloid leukemia (AML). The FISH-TSA analysis revealed the mono allelic deletion of the MEN1 gene in 4 out of 22 patients (18.18%), 2 of 9 AML-M2 patients (22.2%), 1 of 6 AML-M4 patients (16.6%), and 1 of 4 AML-M5 patients (25.0%). Our study indicates that allelic deletion of the MEN1 gene is not a major cause or a primary event in tumorigenesis of AML, although the long arm (q13 region) of chromosome 11 involves a chromosomal rearrangement in AML.     

Telomere-specific fluorescence in situ hybridization analysis of couples with five or more recurrent miscarriages

Fluorescence in situ hybridization analysis using telomere specific probes has been used to detect cryptic translocations in the chromosomal telomeric regions. This study was performed in five clinically normal couples who have had five or more spontaneous abortions and whose karyotypes were found to be normal using conventional cytogenetic techniques. Using the telomere specific probes, in one couple we determined a cryptic translocation between chromosome 3 and 10, and, in another couple, the signal in chromosome 20 was detected in another chromosome, which was probably a D group chromosome. Additionally, in the latter and also in two other couples, we observed a polymorphism. The approach will be helpful for screening cryptic translocations using telomere specific multiple probe sets in couples with recurrent miscarriages. As prenatal diagnosis will be available for these couples for future pregnancies, it will be possible to help these families to have healthy fetuses.     

Leydig cell tumor of the testis: comparison of histopathological and immunohistochemical features of three azoospermic cases and one malignant case

Leydig cell tumors of the testis are rare, mostly presenting as a testicular mass or as endocrinological symptoms. Here, three patients who were admitted for investigation of primary infertility and one patient presenting with a testicular mass are reported. The histological features were reviewed and an immunohistochemical study was done using a panel of antibodies against cytokeratin, vimentin, inhibin A, S-100, Ki-67, follicle-stimulating hormone, luteinizing hormone, prolactin, p53, bcl-2, and c-erbB2. The latter case (lost during follow up of metastatic disease) demonstrated massive tumor necrosis, extension through the tunica albuginea, and a high mitotic activity and MIB-1 score. Only this malignant case was bcl-2 positive. Of the two oncogenic markers studied, none of the cases were positive for c-erb2, while p53 was positive in more than 50% of cells in the malignant case and in one case of infertility with a large tumor, hemorrhage, focal necrosis and atypical cytological features. We recommend the evaluation of infertile men for Leydig cell tumors, and we believe that a panel of antibodies, including Ki-67, p53 and bcl-2, used for immunohistochemical analysis could be of diagnostic value in the identification of malignant and borderline cases of Leydig cell tumor.     

3D-printed prostheses for large bone defect reconstruction following bone tumor surgery北大核心

BACKGROUND: Tumor segment resection is one of the standard methods for the treatment of bone tumors. However, the reconstruction of bone defects atumor resection faces many challenges. A growing number of researchers are focusing on 3D-printed prostheses for bone defect repair and reconstruction following bone tumor surgery. OBJECTIVE: To explore the feasibility of 3D-printed prostheses in the reconstruction of large bone defect following bone tumor surgery and to evaluate the postoperative outcomes. METHODS: Retrospective analysis of clinical data of 24 patients [19 males and 5 females, age 23.8 (6-61) years] who underwent bone tumor resection and 3D-printed prosthesis implantation in the Department of Bone Oncology, the First Affiliated Hospital of Xinjiang Medical University from December 2020 to September 2021 was conducted. There were 7 cases with distal femur tumor, 5 with pelvis tumor, 4 with proximal tibia tumor, 3 with middle femur tumor, 1 with distal tibia tumor, 1 with proximal humerus tumor, 1 with middle humerus tumor, 1 with scapula tumor, 1 with ulna tumor, and 22 cases with primary tumors (13 osteosarcoma, 4 Ewing sarcoma, 2 giant cell tumor of bone, 1 chondroblastoma, 1 chondrosarcoma, and 1 osteoblastoma), 2 metastatic carcinoma. Preoperative and postoperative imaging data were recorded and neoadjuvant chemotherapy was administered in 17 cases before surgery. The Musculoskeletal Tumour Society score was used to assess limb function before surgery and 6 months after surgery, and pain was assessed by the Visual Analog Scale, as well as the complications were recorded. RESULTS AND CONCLUSION: (1) All patients undergoing resection of the tumor segment and 3D-printed prosthesis implantation for the reconstruction of the bone defect were followed for 6-49 months, and the results showed that the length of osteotomy was (18.2 ± 7.3) cm and an average intraoperative bleeding volume was 740 (100-3 000) mL. (2) Two patients died of systemic metastasis, the remaining 22 had no pulmonary metastasis or recurrence during the follow-up period, and 1 patient developed aseptic loosening of the prosthesis at 25 months postoperatively. (3) The Musculoskeletal Tumour Society scores were significantly increased, while Visual Analog Scale scores were significantly decreased (P < 0.05) at 6 months postoperatively. (4) The Musculoskeletal Tumor Society score was rated excellent in all 22 patients at the final follow-up. (5) These results suggest that 3D-printed prosthesis is suitable for the reconstruction of large bone defects caused by bone tumor resection. Patients have good postoperative function and few complications. However, further investigations are needed to explore long-term follow-up results. © 2023, Publishing House of Chinese Journal of Tissue Engineering Research. All rights reserved.