An unusual complication of tumor surgery: chylous leakage.

PURPOSE: This study was designed to evaluate the effectiveness of conservative treatment for chylous leak after tumor surgery and to propose a management algorithm. METHODS: The data of patients with postoperative chylous leak after tumor surgery in our institution between 2010 and 2019 were retrospectively reviewed. In this study, 469 laparotomies, 89 thoracotomies, and 57 cervical excisions were performed for tumor surgery in our institution. RESULTS: Twelve patients with a median age of 4 (IQR, 3-8) years had postoperative chylous leak. All patients received total parenteral nutrition for a median of 13 days. Five patients had intravenous somatostatin for a median of 14 days (IQR, 9-16) to decrease chyle production. Eventually, chylous leak ceased in all patients with conservative treatment and surgical drains were removed after no leak was observed with enteral feeding. CONCLUSIONS: The incidence of chylous leak in childhood tumor surgery is approximately 2%. Extended tumor resection and lymph node dissection lead to the injury of the delicate structures that drain chyle. Conservative treatment with total parenteral nutrition and somatostatin seems to be effective. In particular, somatostatin may be used in resistant cases. Conservative treatment can take up to 1 month. The algorithm consists of how to manage postoperative chylous leak in childhood.

Early postoperative complications in pediatric abdominal solid tumor surgery according to Clavian-Dindo classification.

PURPOSE: Postoperative period after abdominal solid tumor surgery is critical regarding complications. This study aimed to detect incidence and treatment of complications. METHODS: Single center retrospective study including years 2010-2019 for early postoperative complications were documented and graded according to Clavian-Dindo classification. RESULTS: The overall complication rate was 10% and they were chylous leak (n = 8, 2%), collection in surgical field (n = 8, 2%), acute renal failure (n = 7, 1.7%), hemorrhage (n = 5, 1.3%), intestinal obstruction (n = 4, 1%), surgical site infection (n = 3, 0.7%), thromboembolism (n = 3, 0.7%) and peripheric neuronal event (n = 2, 0.5%). Distribution of complications according to Clavian-Dindo classification was as follows: 2 grade I, 23 grade II, 9 grade IIIb, 5 grade IVa and 1 grade V. While age and percentage of benign or malignant disease were not different in groups with or without complications (p = 0.11, p = 0.24), males had more complications than female patients (p = 0.008). Having more than one surgery aiming tumor resection was associated with increased postoperative complications (p = 0.002). Incidence of complications were highest after extragonadal germ cell tumor (35%) and hepatic tumors (29%). CONCLUSIONS: Postoperative complications of abdominal tumor surgery are diverse in children. They are related with prolonged hospital stay and need for medical or surgical interventions. Number of surgeries, organ of origin and tumor type have an influence on risk of complications.

Biallelic ITGB4 variants in familial pyloric atresia without epidermolysis bullosa: Report of two families with five siblings.

Pyloric atresia (PA) is a rare gastrointestinal anomaly that occurs either as an isolated lesion or in association with other congenital or hereditary anomalies. Familial occurrence of PA with epidermolysis bullosa (EB) has been well documented and variants in ITGA6, ITGB4, and PLEC are known to cause EB with PA. However, no gene variants have been defined in familial isolated PA. Five siblings with familial isolated PA are presented that suggest biallelic ITGB4 variants may underlie the development of PA without EB. Five siblings from two unrelated families with isolated PA were studied with exome sequencing (ES) to identify the genetic etiology in isolated familial cases. Exome sequencing was performed in one affected patient from each family. Validation and segregation studies were done by Sanger sequencing. Parents were first cousins in one family but there was no consanguinity in the other family. Type-2 PA was detected in both families and none of the probands had associated anomalies. All patients underwent successful gastroduodenostomy and have been under follow-up uneventfully. All patients had biallelic ITGB4 variants, c.2032G > T p.(Asp678Tyr) being a novel one. Biallelic ITGB4 variants may underlie the development of PA without associated EB. Further detection of variants in this gene may establish any possible genotype-phenotype correlations.

Primary gastric lymphoma: A report of 16 pediatric cases treated at a single institute and review of the literature.

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.

Wilms Tumor Extension Into Duplex Ureter in a 10-Year-Old Girl.

Ureteral extension of Wilms tumor (WT) is a rare occasion. The association of duplex collecting system and WT is extremely rare. Ureteral extension of WT in a duplex collecting system is an unreported entity to date. A 10-year-old girl presenting with WT extending into upper pole ureter of duplex system is reported to emphasize the importance of preoperative diagnosis to plan step by step surgery in this rare coincidental situation.

Pulmonary Mucoepidermoid Carcinoma and Solid Pseudopapillary Neoplasm of the Pancreas: A Coincidence of 2 Rare Tumors in an 8-Year-Old Boy.

Primary pulmonary mucoepidermoid carcinoma (MEC) is extremely rare in children and is characterized by a mucus-secreting and squamous cell combination. Solid pseudopapillary neoplasm of the pancreas is a rare tumor of the pancreas, which is mostly seen in young women. We present the case of an 8-year-old boy previously diagnosed and treated by lung lobectomy for MEC. He was admitted to our hospital with an incidentally recognized mass at the pancreas during postoperative workout studies. Distal pancreatectomy was performed. Surgery was curative for both tumors. To the best of our knowledge, this is the first reported case of pseudopapillary neoplasm concomitant with MEC in the literature.

Sclerotherapy with bleomycin versus surgical excision for extracervical cystic lymphatic malformations in children.

PURPOSE: Sclerotherapy (ST) with bleomycin is an effective treatment for cervical cystic lymphatic malformations (LM) in children. However, its efficacy for treating extracervical cystic LM in children has not been investigated adequately. This retrospective study compares the efficacy of ST with surgery for treating extracervical cystic LM in children. METHODS: The subjects of this study were children treated for extracervical cystic LM at our hospital between 1970 and 2013. We evaluated retrospectively the hospital records of these children for age, gender, presenting symptoms, location of the lesion, radiological findings, treatments, complications, duration of hospitalization, and outcome. RESULTS: We analyzed the records of 70 children (M:F = 1:9) with a mean age of 52.57 ± 54.87 months (range 1-204 months). The number of children treated by surgery alone, ST alone, and surgery plus ST was 53 (77 %), 13 (18 %), and 4 (5 %), respectively. Surgery comprised total excision (n = 41), near-total excision (n = 9), partial excision (n = 6), and incisional biopsy (n = 1). The complication and recurrence rates were lower, the complete response rate was higher, and the length of hospitalization was shorter in the ST group than in the surgery group (5 vs. 15 % and 8 vs. 17 %, respectively, p < 0.05; 91 vs. 77 %, respectively, p = 0.05; and 2.42 ± 1.67 vs. 13.57 ± 16.24 days, respectively, p = 0.03). CONCLUSION: ST is as safe as surgery for extracervical macrocystic or mixed LMs in children, but is much more effective with higher success rates and lower recurrence rates. Thus, ST provides a cost-effective and appropriate mode of treatment for children with extracervical cystic LM.

Secretory breast carcinoma in a 6-year-old girl: mastectomy with sentinel lymph node dissection.

Secretory breast carcinoma (SBC) is a rare type of breast neoplasia that was originally described in children. SBC is an indolent breast tumor with good clinical outcome and rare systemic involvement. Since, majority of studies concerning pediatric SBC have been case reports, it has been difficult to clearly elucidate the characteristics and optimal treatment strategies for SBC in children. Although treatment recommendations vary, surgical excision is the primary mode of treatment. Also, necessity of axillary and/or sentinel lymph node dissection is another matter of discussion in children. We report a 6-year-old girl who was diagnosed as SBC was reported to discuss the use of mastectomy with sentinel lymph node dissection in the treatment of this rare tumor in children.

Pure endobronchial inflammatory myofibroblastic tumor in children.

Isolated endobronchial inflammatory myofibroblastic tumor is an unusual diagnosis among endobronchial masses in childhood. The presenting signs and symptoms may mimic asthma. Rigid bronchoscopy is effective for the diagnosis and treatment. Follow-up is mandatory to check for recurrent disease. Here in, the authors report on a 9-year-old girl with endobronchial inflammatory myofibroblastic tumor to emphasize the possibility of endobronchial lesion in children with longstanding obstructive symptoms.

Giant cervical polyp with mesonephric duct remnants: unusual cause of vaginal bleeding in an adolescent girl.

BACKGROUND: Cervical polyps (CP) are quite common in adults, they are extremely rare in children and adolescents. CP containing mesonephric duct remnants (MDR) are few millimeters in size and seldom grossly visible. CASE: A 14-year-old female admitted with massive vaginal bleeding with an intact wide hymeneal opening. Pelvic MRI revealed 47 mm × 25 mm × 35 mm polypoid mass originating from the endometrial cavity. During vaginoscopy, a polypoid mass was detected at 9 o'clock position of right lip of ectocervix and was totally excised. Light microscopy showed CP including small round or branched glands containing inspissated eosinophilic secretions and patchy chronic inflammatory infiltrate. The patient was diagnosed as gaint CP containing MDR. CONCLUSION: Giant CP is an uncommon variant of classical polyps and exhibit distinct clinical and pathologic features. However, MDR are benign lesions of cervix, detailed histopathological evaluation should be performed to all CP to differentiate malignant lesions.

Ganglioneuromas in Childhood: Hacettepe Experience With 70 Cases.

BACKGROUND: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. METHODS: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. RESULTS: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6-15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. CONCLUSIONS: Ganglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. LEVEL OF EVIDENCE: Case series, IV.

An unexpected diagnosis in children with male phenotype and bilateral nonpalpable gonad: congenital adrenal hyperplasia with female genotype.

PURPOSE: Female pseudohermaphroditism is the most frequent form of ambiguous genitalia in children with congenital adrenal hyperplasia (CAH). However, a small group of children with complete urethral development in contrast to 46XX karyotype can be encountered. We aimed to define the characteristics of patients with 46XX CAH but having fully developed male external genitalia. METHODS: The records of 11 children with CAH and 46XX karyotype but having male phenotype, encountered from 1990 to 2012 were reviewed retrospectively. The age, presenting signs and symptoms, diagnostic studies, surgical procedures, early results and outcome were noted. All patients were evaluated by gender assignment team and the decision of the family was also taken into consideration during gender assignment. RESULTS: Eleven children (mean age 3.64 ± 3 years) (range 5 days-10 years) were enrolled. The main presenting signs were nonpalpable gonads (n = 7), hyperpigmentation (n = 2), jaundice (n = 1) and electrolyte imbalance (n = 1). All patients had bilateral nonpalpable gonads and male phenotype with mean phallus length of 4.5 ± 1.7 cm. Urethral meatus is located at normal position (n = 6) or hypospadiac (n = 5). Labioscrotal fusion was complete in all cases and they were classified as 4th (n = 3) or 5th (n = 8) degree of virilization according to Prader's classification. All children had CAH and 46XX genotype. Pelvic ultrasound (n = 8) and genitocystogram (n = 9) were used, and genitocystoscopy was performed (n = 6). Male gender was assigned in most (n = 10) and female gender in one. Corrective surgery could be performed in 10 patients. The surgical procedures were hysterectomy + bilateral salphingo-oophorectomy + vaginectomy (n = 9), chordee release (n = 3) and then second-stage (n = 2) or one-stage urethra repair (n = 1), and pure one-stage urethra repair (n = 1). One case underwent surrenalectomy before the diagnosis of CAH. Mastectomy (n = 1) and fistula repair (n = 3) were additional operations. Only one child could be undergone feminizing genitoplasty and another was lost to follow-up. CONCLUSION: Unfortunately, most of the children underwent surgery in adverse to genotype because of constituted sexual identity. Children with male phenotype and bilateral nonpalpable gonads should undergo promptly biochemical analyses for CAH and early chromosomal analysis.

Invasive inflammatory myofibroblastic tumor of the spleen treated with partial splenectomy in a child.

Inflammatory myofibroblastic tumor (IMT) is a rare benign tumor that can be found in intra-abdominal organs such as the liver, intestine, extrahepatic bile ducts, and mesentery. The spleen is an extremely unusual location for an IMT. The authors report the case of a 14-year-old boy with invasive splenic IMT, present a review on the current literature about childhood splenic IMT, and emphasize the necessity of total excision of the tumor together with tumor-invaded surrounding tissues.

A unique case of magnet ingestion with respect to presentation and management.

Magnet ingestion may lead to serious complications with delay in diagnosis and treatment. The forceful attraction between magnets, with gastric and/or intestinal wall entrapped between them, can cause injury through pressure necrosis. The radiological appearance of more than one magnet on X-ray can be easily misinterpreted as belonging to only one rod-like radiopaque foreign body, even if the magnets are located in different parts of the gastrointestinal tract, thus delaying the management up to the onset of emergent surgical complications. A 17-month-old female with ingestion of a pair of magnets is presented, together with introduction of the clinical picture and therapeutic approach, which differed from the other previously reported cases. The ovoid shape of the magnets, their localization in the gastrointestinal tract (leading to entrapped gastric and intestinal wall between them), absence of any complication, and the therapeutic approach of endoscopic retrieval are the main distinguishing features of this case from those previously reported.

Why gastric perforation occurs in patients with isolated esophageal atresia: more vulnerable stomach?

Gastric perforation (GP) in patients with isolated esophageal atresia (EA) is seen more than expected. The etiology of the perforation may be vulnerability of these immature stomachs. Gastrostomy operation should be performed by gentle handling. The volume of gastrostomy feedings should be increased incrementally. The early diagnosis of perforation is only possible with a high index of suspicion. The stomach should be visualized by gastrography with soluble contrast material as soon as the perforation is suspected.

The prognostic factors in children undergoing pulmonary metastatectomy.

A retrospective analysis was performed to determine the prognostic factors affecting survival in children who underwent pulmonary metastatectomy. Seventeen patients who underwent pulmonary metastatectomy between 2000-2006 were evaluated retrospectively by means of age, sex, primary diagnosis, time of metastasis appearance, number of nodules found on imaging examinations, type of management, surgical data, and outcome. Video-assisted thoracoscopic surgery (VATS) was used in 11 patients, and all patients underwent thoracotomy consequently. The nodule was composed of tumor cells in 13 (76%) patients and had positive surgical margins in 5 (38%). The time of metastasis appearance, number of metastases and completeness of the nodule excision did not affect survival (p=0.31, p=0.87 and p=0.56, respectively). Nodule size >1 cm was associated with dismal survival (p=0.008). Time elapsed until the diagnosis of pulmonary metastasis, number of metastases and the completeness of metastatectomy do not have an impact on survival. The only significant prognostic factor is the size of the largest metastatic nodule. The presence of a metastatic nodule >1 cm is associated with a worse outcome in pediatric patients. VATS is an adjunct method to thoracotomy in the surgical management of pulmonary metastasis in children.

Postoperative intussusception in children: a keen diagnosis in postoperative ileus.

We investigated the patients who developed postoperative intussusception after a variety of intraabdominal procedures in order to identify the differentiating features and facilitate the prompt recognition and management of this entity. Fourteen patients with postoperative intussusception following an abdominal surgery between 1993 and 2010 were analyzed retrospectively. The primarily applied surgeries were: repair of diaphragmatic hernia (n=3), choledochal cyst excision (n=2), extraction of surrenal neuroblastoma (n=2), Duhamel operation (n=1), colostomy closure (n=1), Nissen fundoplication with (n=1) and without (n=1) gastrostomy, gastropexy (n=1), gastrostomy and jejunostomy (n=1), and manual reduction of ileocolic intussusception (n=1), with a median duration of 135 minutes (120-240). Patients were reoperated on the 3rd day (2-16); intussusception was ileoileal in 11, and was manually reduced in 12 of all patients. Postoperative intussusception differs from other cases of invagination with respect to the pathogenesis, clinical presentation and therapeutic approach. The original operations are mostly the major and lengthy ones, with vicinity to the diaphragm. Awareness of this entity by surgeons and differentiation from other causes of postoperative ileus are obligatory for prompt recognition and management.

Paediatric appendiceal neuroendocrine tumours: a review of 33 cases from a single center.

BACKGROUND: Appendiceal neurendocrine tumours (NETs) are rare neoplasms and diagnosis is commonly incidental following appendectomy. We aimed to review our experience with appendiceal NETs. METHODS: Records of children with appendiceal NETs were reviewed and data concerning demographic characteristics, clinical findings, surgical procedures, histopathological findings, management and outcomes were recorded. RESULTS: Between 1985 and 2021, 33 cases with appendiceal NETs (median age 11.8 years, range 7.8-16; male/female = 10/23) were identified. All but one patients presented with abdominal pain, six had vomiting, four had fever and they underwent appendectomies with presumed diagnosis of acute appendicitis. Abscess drainage and appendectomy was performed in a 16-year-old girl for suspected right ovarian mass, and tumour was positive in the omentum. Histopathological diagnosis was classical carcinoid tumour (NET) in all cases. Median tumour size was 0.9 cm (n = 26, range, 0.1-3.5 cm); tumours were ≤1 cm in 19 cases, 2 cm in one, 3.5 cm in another case. Tumours were located in the tip (n = 11), body (n = 6) and base of appendix (n = 1) (in others data unavailable). In 28 patients with data, tumour extended to submucosa in five, to tunica muscularis in seven, to subserosa in six, to serosa in six, to mesoappendix in three, to periappendiceal fat in one. Three cases were lost to follow-up, 31 cases were alive (median follow-up 53 months). CONCLUSIONS: Paediatric appendiceal NETs do not behave aggressively and appendectomy alone is sufficient for tumours ≤2 cm regardless of local invasion. The need for further extensive surgery in tumours >2 cm also remains controversial.

Giant cell tumor arising from the anterior arc of the rib: an extremely rare site in an adolescent girl.

BACKGROUND: Giant cell tumor is a rare and locally aggressive neoplasm of the long bones in children. Rib is the least frequently affected site, seen in less than 1% of all cases and most of them occur at the posterior arc. CASE: A 12-year-old girl presented with swelling and slight pain on the left inferior-anterior chest wall for two years. Physical examination revealed a giant, hard and fixed mass on the left chest wall. Hematological and biochemical test results were in normal limits but slight elevation of alkaline phosphatase level. Computed tomography of the chest showed a large expansive mass and lytic lesion with internal calcification arising from the anterior part of the 7th rib. En-bloc resection was performed including the 6th-8th ribs and a small part of the diaphragm. The pathological evaluation revealed giant cell tumor of bone. CONCLUSIONS: Herein, we aim to emphasize that giant cell tumor should be considered in the differential diagnosis of chest wall tumors in childhood whereby en-bloc resection and close follow up would be paramount.

Papillary thyroid carcinoma does not have standard course in children.

BACKGROUND AND PURPOSE: Papillary thyroid carcinoma has a favorable outcome in children. Recent experience with young children cases with early recurrences, after the total thyroidectomy and excision of palpable lymph nodes, in contrast to usual course of disease in adolescent cases, prompted us to review our experience on papillary carcinoma surgery. MATERIALS AND METHODS: Sixteen children who underwent surgery for papillary carcinoma between 1997 and 2010 were included. The charts and surgery notes were evaluated retrospectively and age, sex, complaints and physical findings at presentation, past medical history, results of laboratory tests, imaging findings, aspiration biopsy, extent of disease, details of surgery, complications and postoperative course were noted. RESULTS: Male to female ratio was 3:1. The mean age was 10.9 years (range, 5-16). The presenting symptom was palpable swelling in the neck (n = 12) or asymptomatic nodule was detected incidentally (n = 4). Physical examination findings were palpable nodule (n = 7), lymphadenopathy (n = 6) or both (n = 4). Thyroid ultrasound (US) revealed nodule (n = 9), heterogenisity of the parenchyma and enlargement of thyroid (n = 9), and microcalcifications (n = 5). Fine-needle aspiration biopsy was performed in 12 patients and was suggestive for malignancy in most cases (91%). Pulmonary metastasis was detected at presentation in two patients and appeared after thyroidectomy in one patient. Complementary thyroidectomy was performed in nine patients because the total thyroidectomy with or without cervical lymph node dissection was the treatment of choice (n = 15). Iodine scan was performed 4 weeks later following thyroidectomy. Radioactive iodine ablation (RIA) therapy was given to 11 patients who had residual or recurrent disease. Postoperative complications were permanent hypoparathyroidism (n = 4), recurrent laryngeal nerve injury (n = 3), lymphorea (n = 1) and Horner's syndrome (n = 1). CONCLUSION: Total thyroidectomy and excision of affected lymph nodes is the current mode of surgical management for thyroid papillary carcinoma in children. However, especially in young children (<10 years), modified lymph node dissection should be added to total thyroidectomy in order to avoid leaving the residual tumor foci which can impair the efficacy of the ablation therapy.