In transit metastases in children, adolescents and young adults with localized rhabdomyosarcoma of the distal extremities: Analysis of the EpSSG RMS 2005 study.

In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS: Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS: One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION: Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.

Sacrococcygeal teratomas: midline reconstruction improves cosmesis without compromising outcomes.

AIM: Several studies have addressed the long-term functional, psychosexual and psychosocial outcomes following sacrococcygeal teratoma (SCT) excision. It is well reported that the classical chevron incision and reconstruction can leave a cosmetically unsatisfactory result; however, there is little in the literature focussed on improving this outcome. In our institution the preference is to perform a midline reconstruction, where possible, this is felt to improve appearance without compromising the oncological or functional outcome. The aim of this study was to evaluate patient-perceived cosmetic outcomes of the midline reconstruction. METHODS: All patients undergoing surgery for SCT between 2007 and 2020 were included in the study. Patient demographics, operation type, functional outcome and recurrence were all recorded. The primary outcome measure was patient/parent satisfaction with the cosmetic appearance. This was assessed using both qualitative and quantitative methodologies. Following ethical approval parents were asked questions from two existing validated patient outcome questionnaires: "Patient and Observer Scar Assessment Scale" (POSAS) v2.0 and the "Patient Scar Assessment Questionnaire". RESULTS: Thirty-two patients underwent surgery at our institution for SCT during the study period. Twenty-four had a posterior approach with midline reconstruction, two laparotomy and excision (excluded from this study) and six had a combined approach. Median follow-up was 35 months (8.5-96 months). There were no recurrences. 4/30 (13%) have persistent urological symptoms, and 1/30 (3%) has constipation requiring bowel management. Questionnaires were sent to 26/30 families with a 77% return rate. Median total score was 11 (7.4-17.5) on a 60-point scale (6, as normal skin, 60, worst imaginable scar). Twenty (95%) reported that the scar never affects the child's activities and 15 (71%) said they are "not at all" conscious of the scar. CONCLUSION: Scars can lead to an array of cosmetic, functional, and psychological consequences and as such consideration needs to be given to scarring following surgery for sacrococcygeal teratomas. This study demonstrates that a midline reconstruction produces a cosmetically favourable outcome. We, therefore, recommend where appropriate a midline reconstruction should be considered for SCT.

Ovarian tumors in children: how common are lesion recurrence and metachronous disease? A UK CCLG Surgeons Cancer Group nationwide study.

BACKGROUND: Ovarian tumors in children are rare, mature teratoma being the most common histological entity. Robust guidelines to aid patient follow-up after resection are distinctly lacking. Although mature teratoma has a very good prognosis following complete resection, small studies have reported the occurrence of metachronous disease and recurrence to a variable degree (2.5-23% of patients). Nevertheless, there are surgeons who recommend no follow-up is required for these children after primary tumor resection. We investigated the incidence of (i) recurrence and (ii) metachronous disease in pediatric patients following ovarian tumor resection. METHODS: Retrospective multicenter study amongst UK pediatric surgical oncology centers. Females <16 years with diagnosis of ovarian tumor from 2006 to 2016 were included. Functional/neonatal ovarian cysts were excluded. RESULTS: Three hundred ten patients with ovarian tumors treated at 12 surgical oncology centers were identified. Mean age at surgery was 11 years [IQR 8-14]. Most common diagnosis were mature teratoma (57%, 177 cases), immature teratoma (10.9%, 34 cases) and serous cystadenoma (7.7%, 24 cases). 8.1% (25 cases) of all females were identified with tumor recurrence/ metachronous disease. 5.1% (9 cases) of patients with mature teratoma had recurrent/ metachronous disease. Most of these patients were diagnosed at routine clinic follow-up. CONCLUSION: Our study clearly shows that ovarian tumor recurrence(s) and metachronous disease occur, even in "benign" ovarian tumors. We recommend female pediatric patients should have robust follow-up care plans after primary diagnosis and resection of ovarian tumor(s). LEVEL OF EVIDENCE STATEMENT: This is a level II evidence study. It is a retrospective multicentre collaborative study which summarizes data from a national cohort of children.

Congenital infantile fibrosarcoma of the colon: a case series and literature review.

Congenital infantile fibrosarcoma is rare and only three cases affecting the colon have previously been reported. We describe two further cases that presented in the neonatal period and were both successfully treated with surgical excision and have no evidence of recurrence or metastasis at 31 and 27 months follow-up, respectively.

Paediatric angiodysplasia of the jejunum: a case report and review of the literature.

Gastrointestinal angiodysplasia (GIAD) of the jejunum is a rare cause of acute upper and lower gastrointestinal bleeding in the paediatric population. This is the case of a previously well 10-year-old girl who presented with acute rectal haemorrhage, haematemesis and syncope. Despite an exploratory laparotomy, the cause of bleeding remained unknown. A computerized tomography angiogram was performed once she was haemodynamically stable. It indicated bleeding in the jejunum. Repeat laparotomy was performed including enterotomy, copious small bowel washout and visualization of the whole small bowel. The lesion was identified at approximately 100 cm from the duodenojejunal flexure and confirmed by isolating the lesion and testing for bleeding. A 30 cm length of jejunum was resected and primary anastomosis performed. She recovered well and was discharged three days postoperatively. She remained well at six-month follow-up. This case highlights the importance of considering upper GIAD in an acute paediatric gastrointestinal bleed and the way in which surgical management can prevent a potentially fatal outcome.

Bipartite anterior extraperitoneal teratoma: evidence for the embryological origins of teratomas?

Teratomas are thought to arise from totipotent primordial germ cells (PGCs) Dehner (1983) which may miss their target destination Moore and Persaud (1984). Teratomas can occur anywhere from the brain to the coccygeal area but are usually in the midline close to the embryological position of the gonadal ridges Bale (1984), Nguyen and Laberge (2000). We report a case of a bipartite anterior extraperitoneal teratoma. This is an unusual position for a teratoma, but one which may support the "missed target" theory of embryology.

Laparoscopic repair of late presenting congenital diaphragmatic hernia.

Congenital diaphragmatic hernia usually presents in the neonatal period, with delayed presentation being uncommon. Traditionally repair was performed by laparotomy or thoracotomy. We have performed laparoscopic repair of a previously undiagnosed congenital diaphragmatic hernia that presented acutely in a 10-year-old male. Laparoscopic repair of late-presenting congenital diaphragmatic hernia is a safe and effective approach even in an emergency. The laparoscopic approach has advantages including reduced hospital stay, excellent visualisation of the defect even for obese patients, and improved cosmesis.

Children's surgery performed by adult general surgeons in Scotland: the present and future.

OBJECTIVE: The aim of this study was to determine the extent and the future of paediatric surgery performed in Scotland outside of the designated surgical paediatric centres. MATERIALS AND METHODS: An anonymous questionnaire was sent to all 111 Scottish members of the Association of Surgeons of Great Britain and Ireland. There was a response rate of 69%. RESULTS: Overall, 45% of responders operated on children. This was independent of the surgeon's age but was related to the type of hospital that the surgeon worked in. Eighty-four per cent of responders had a lower age limit under which they would not operate and 94% stated that there were specific circumstances where they would not operate. A mean of 18.5 elective procedures (range 0-250, median two) and six emergency procedures (range 0-30, median five) were carried out by each surgeon operating on children under the age of five per annum. Only 13% of responders thought that their successor would operate on children. CONCLUSIONS: Non-specialist paediatric surgery in Scotland is currently provided by a significant number of surgeons whose successors will not continue to provide a comparative paediatric service. This has implications for local provision of care, emergency management and capacity of existing children's hospitals in the future.

Surgical implications of the Smith-Lemli-Opitz syndrome.

Smith-Lemli-Opitz syndrome (SLOS) is a syndrome of rare multiple congenital anomalies/mental retardation associated with low plasma cholesterol levels. Prior to receiving the diagnosis of SLOS, affected children may present as a neonatal surgical emergency with ambiguous genitalia, Hirschsprung's disease, and pyloric stenosis. We present two fatal cases of SLOS with near-total Hirschsprung's disease; the surgical, anaesthetic, and medical aspects of the cases are discussed, and a literature review is presented.