Case report: NTRK1-rearranged cervical sarcoma with fibrosarcoma like morphology presenting in a 13-year-old managed with a neo-adjuvant TRK-inhibitor and surgical excision.

This case report reviews the case of a 13-year-old patient who presented with a 9 cm NTRK1-rearranged cervical sarcoma with fibrosarcoma like morphology. At presentation the lesion filled her vagina and pelvis and any attempt at surgical removal would have been morbid and led to loss of fertility. These neoplasms are extremely rare with 18 cases of the uterine cervix reported in the literature, none of which have occurred in a paediatric patient, and none of whom have received neo-adjuvant therapy prior to excision. Based upon evidence that has shown good tolerability and responses of paediatric NTRK fusion-positive solid tumours to TRK inhibitors, both in the neo-adjuvant and upfront setting, this patient was managed with neo-adjuvant entrectinib. Following a dramatic reduction in tumour size confirmed by imaging, she underwent conservative fertility sparing surgery with final histopathology showing no residual disease.

Tumor cell distribution following laparoscopic colectomy in a porcine model.

UNLABELLED: A clinically relevant, laparoscopic colectomy model has been developed to quantify surgical practices that may affect the incidence of port wound tumor implantation. METHODS: Suspended 51Cr-labeled, fixed HeLa cells were injected intraperitoneally into pigs before laparoscopic colectomies were performed with or without insufflation. Tumor cell contamination of instruments, ports, stability threads, and excised port wound margins was determined by gamma counting. RESULTS: Tumor cells were distributed throughout the peritoneal cavity, and the number detected at wound sites was directly related to number injected. Ports used by the operating surgeon had more cells than those used by the camera operator or assistant surgeon. Postoperative withdrawal of contaminated ports through abdominal wound was associated with an increase in port site contamination. Although the port site distribution of tumor cells was affected, mechanical elevation of abdominal wall did not eliminate contamination at any site. CONCLUSION: These results demonstrate application of the porcine model to test current surgical practices and measures that might be used perioperatively to reduce the numbers of intraperitoneal tumor cells or their distribution to specific sites during laparoscopic or open surgery.

Operative factors affecting tumor cell distribution following laparoscopic colectomy in a porcine model.

BACKGROUND: An increased risk of laparoscopic port wound tumor implantation in the presence of overt or covert abdominal malignancy has been identified. PURPOSE: A porcine laparoscopic colectomy model has been used to quantify the influence surgical practices may have on tumor cell implantation. METHODS: 51Cr-labeled, fixed HeLa cells were injected intraperitoneally before surgery. Tumor cell contamination of instruments, ports, security threads, and excised wound margins was assessed by gamma counting. RESULTS: Greatest contamination occurred in ports used by the operating surgeon under pneumoperitoneum (64 percent of all port wound tumor cells) and mechanical elevation (76 percent). Gasless surgery in patients in the head-down position increased the rostral accumulation of tumor cells in the abdomen and right upper quadrant port wound by 330 and 176 percent, respectively. Under pneumoperitoneum, port movement was the major contributor to port leakage and wound contamination (21 percent of total recovered wound tumor cells per port). Tumor cells were not carried in aerosol form. Instrument passage and the withdrawal of security threads through the abdominal wall increased port wound contamination 430 and 263 percent, respectively, over pneumoperitoneum control ports. Preoperative lavage reduced by 61 percent, but did not eliminate, wound contamination. CONCLUSION: This porcine model may be used to evaluate surgical factors for the impact on port wound contamination.

Comparison of results of laparoscopic and open antegrade continence enema procedures.

Several modifications of the Malone antegrade continence enema (ACE) procedure have now been reported. In this study we have compared the results of our experience with the laparoscopic appendicostomy (LACE procedure) with the published results of previously described open ACE procedures. Children who had the LACE procedure at our institutions were reviewed. Intra- and postoperative problems were identified by review of hospital case notes and from a questionnaire completed by the children's caregivers. These results were compared with the reported results of the original Malone procedure, the Peña modification, and the open appendicostomy as described by Wilcox. Since 1994, 30 children have had LACE procedures performed at our institutions. Two have required conversion to an open procedure because of difficulty locating the appendix. The stoma is being used for regular antegrade colonic washouts in 29 of the 30, compared with 19 of 31 in Malone's series and 16 of 20 in Peña's. Improvement in soiling has been achieved in 27 (90%) of our patients, 15 of whom are completely clean, a rate similar to that of the other types of procedure (61%-78%). Stenosis of the stoma has occurred in 8 children (27%), compared with rates of 10%-33% in other series. Stomal leakage has been troublesome for 2 (6.7%), compared with leak rates between 5.6% and 15% in other series. The LACE procedure is technically the simplest. The laparoscopic approach to the ACE procedure appears to be a simpler and safe alternative to previously described methods. It causes minimal morbidity, has a high long-term viability rate, and resulted in improved control of soiling in 90% of the children with faecal incontinence in whom it has been employed.

Plastic migration from implanted central venous access devices.

BACKGROUND: This is the first reported study of histologically confirmed migration from intravenous access devices in children. METHODS: The capsules from around intravenous access devices were examined by light microscopy to determine the extent of the foreign body response; energy dispersive x ray analysis was performed to document the elemental content of the foreign material. RESULTS: A fibroconnective tissue capsule was found around all the samples. Elemental silicon was found in six of 13 tissue samples, and a foreign body giant cell reaction was seen in three of these. CONCLUSIONS: The pseudocapsule that surrounds an implanted vascular access device often has residual foreign material, including silicone.

Early experience with the laparoscopic ace procedure.

BACKGROUND: In 1990 Malone et al. introduced the reversed appendicocaecostomy as a technique to enable an antegrade continence enema (ACE) to be administered. The aim is to achieve colonic emptying and thus prevent soiling in children with faecal incontinence. The procedure has been modified by suturing the opened appendix directly to the skin as a stoma, and can now also be performed laparoscopically. METHODS: All children undergoing laparoscopic ACE procedures at Christchurch Hospital, Christchurch, New Zealand and Princess Margaret Hospital, Perth, Australia, were reviewed. Intra-operative and postoperative problems were identified retrospectively and questionnaires were completed by the children's caregivers detailing their experience with managing their children following the ACE procedure. RESULTS: Thirty children have had a laparoscopic ACE procedure performed since 1994. Two required conversion to an open procedure because of difficulty locating the appendix. Twenty-nine children are currently using the stoma for regular antegrade colonic washouts. Improvement in continence was seen in 27 children, 15 of whom have been completely continent. Stenosis of the stoma occurred in eight children, two of whom required operative revision of the stoma; the others have been managed by daily dilatation, either with the irrigation catheter or a small perspex dilator. More recently, spatulating the appendix tip has reduced the incidence of stenosis. Two children have had troublesome leaks. CONCLUSIONS: The laparoscopic approach to the ACE procedure results in an improved level of continence in this difficult group of children with faecal incontinence. It is a simpler alternative to previously described methods and causes minimal morbidity. Results to date suggest a satisfactory outcome for both the children and their families.

Tumour cell wound distribution after colectomy in a porcine model.

BACKGROUND: Concerns over tumour implants have impeded the adoption of laparoscopic surgery for cancer. Explanations assume an increased number of malignant cells present in trocar wound sites. The following are tested in the present paper: (i) that the magnitude of wound contamination following surgery is related to the location of the tumour cells; and (ii) the surgical approach. METHODS: We have used a porcine sigmoid colectomy model to compare the number of tumour cells on laparoscopic wounds after resections in the presence of intraluminal, intramural and intraperitoneal 51Cr-labelled, fixed HeLa tracer cells. Open colectomies were also performed in the presence of intraperitoneal tracer cells and their numbers on laparotomy wound surfaces were determined by gamma counting. RESULTS: With intraperitoneal cells, laparotomies had 1087 (+/- 106) tracer cells per mm (n = 4) while trocar wounds had 103 (+/- 54) cells per mm (n = 10) (P > 0.05). Resection of intramural tumours resulted in lower trocar wound contamination (0.9 +/- 0.6 cells/mm, n = 3). Resection of colon including intraluminal tracer cells resulted in 2.9 +/- 2.1 cells/mm on trocar wounds (n = 3). CONCLUSIONS: More tumour cells were deposited on open than laparoscopic trocar wound surfaces. Also, the risk of wound implantation is less with intraluminal or intramural tumours than with intraperitoneal cells (P > 0.05).

Case report: Colonic duplication: a rare cause of obstruction.

Complete duplication of the entire large bowel with partial ileal involvement is very rare and diagnosis can often be difficult as illustrated by this case report. We also review the other clinical associations of this rare condition and briefly discuss the embryology of duplications of the gastrointestinal tract.