Growing teratoma syndrome in children and adolescents: Prevalence and surgical outcome.

INTRODUCTION: Patients affected by metastatic germ cell tumors may occasionally experience enlargement of masses with concurrent normalization of tumor markers during or after chemotherapy. This phenomenon is described as growing teratoma syndrome (GTS). The aim of the pre sent study is to assess the prevalence of GTS in the pediatric population and its implications in terms of surgical outcome. PATIENTS AND METHODS: The clinical notes of patients diagnosed with stage III and IV malignant germ cell tumors from January 2010 until December 2020 at our Institution were retrospectively reviewed. The prevalence of GTS, treatment strategies, survival, and outcome were analyzed. RESULTS: Thirty-three patients with high-stage malignant germ cell tumors were diagnosed in our institution in the analyzed period. Nine patients (28%) had radiologic evidence of enlargement of persistent masses with normal markers after chemotherapy; these patients were classified as GTS patients. All nine patients underwent resection of metastatic lymph nodes, and six had surgery on visceral metastases. In six patients, radical excision of all metastatic sites was achieved; five patients are alive and in complete remission, while one died because of peri-operative complications. Out of the three patients who could not achieve radical excision of the metastases, two died of progressive disease, and one is alive with progressive disease. CONCLUSIONS: Patients affected by GTS have a risk of progression of chemotherapy-resistant disease and death. Radical surgical excision is essential to achieve disease control and long-term survival.

Femoral hernia in pediatric population: a diagnostic and surgical challenge.

PURPOSE: Femoral hernia (FH) is a rare and often misdiagnosed pathology in pediatric population. The aim of our study was to describe the experience of a Tertiary Center in children with FH, underlying diagnostic and surgical details that could improve its management. METHODS: A retrospective study of pediatric patients who underwent FH repair from January 2010 to June 2023 at our Institution was performed. RESULTS: In the analyzed period, 31 patients underwent surgical procedure for FH at our institution, of whom 16 (51.6%) were female. The mean age at time of surgery was 5.8 years (range 0.5-17.1 years). The rate of pre-operative misdiagnosis was 35.5% and open approach was adopted in all cases (16.1% with mesh application). Only two patients (6.4%) experienced surgical complications: one recurrence (repaired six months later) and one post-operative hematoma (treated successfully with conservative method). CONCLUSION: Due to the high rate of misdiagnosis, the variety of surgical approaches proposed, and the potential for intraoperative complications, FH poses a challenge for pediatric surgeons and urologists, as confirmed by the literature. It is essential to underline the importance of a proper clinical examination in order to correctly diagnose FH, make the best surgical plan for the patient and prevent post-operative complications.

Recurrent thoracic air leak syndrome in patients affected by pulmonary graft-versus-host disease: Surgical strategies and outcome.

BACKGROUND AND AIMS: Thoracic air leak syndrome (TALS) is a complication related to chronic pulmonary graft-versus-host disease (pGvHD) that affects approximately 0.83%-3.08% patients after allogenic hematopoietic stem cell transplant. Such complication is defined as the occurrence of any form of air leak in the thorax, including spontaneous pneumomediastinum or pneumopericardium, subcutaneous emphysema, interstitial emphysema and pneumothorax and has a negative impact on post-transplant survival. The aim of the present study is to describe a single-center experience in the surgical management of recurrent TALS in adolescents and young adults and its outcome. METHODS: We retrospectively reviewed the clinical notes of patients with previous allogenic hematopoietic stem cell transplant who underwent surgical procedures for recurrent TALS from January 2016 until March 2021. We analyzed clinical data, number of episodes of thoracic air leak, surgical procedures and relative outcome. RESULTS: In the examined period, four patients, aged 16-25 years, underwent surgical procedures for TALS, including thoracostomy tube placement, thoracoscopic pleurodesis and thoracotomy. All the patients had been diagnosed with pGvHD before the onset of TALS, with a mean time lapse of 276 days (range 42-513). These patients experienced on average 4.5 air leak episodes (range 3-6). All the patients experienced at least two episodes before surgery. One patient underwent emergency tube thoracostomy only, three patients underwent thoracoscopic pleurodesis and two patients underwent thoracotomy. After surgery, patients were free from air leak symptoms for a mean time of 176 days (range 25-477). Pulmonary function progressively deteriorated, and all the patients eventually died because of respiratory failure after a mean time of 483 days (range 127-1045) after the first episode of air leak. CONCLUSIONS: Surgery provides temporary relief to symptoms related to TALS but has limited effects on the underlying pathophysiologic process. The development of TALS in a sign of progressive pulmonary function worsening and is associated with high risk of respiratory failure and mortality.

Preoperative Spinal Angiography for Thoracic Neuroblastoma: Impact of Identification of the Adamkiewicz Artery on Gross Total Resection and Neurological Sequelae.

BACKGROUND: Patients with thoracic neuroblastoma (TNB) are at high risk of postoperative neurologic complications due to iatrogenic lesions of the artery of Adamkiewicz (AKA). The role of performing a preoperative spinal angiography (POSA) in these patients must be clarified. The present study sought to further understand the relationship between POSA and TNB, as well as the effects of identifying the AKA on surgical excision and neurological consequences. METHODS: Data from patients with TNB who underwent POSA between November 2015 and February 2022 at our tertiary pediatric center were retrospectively analyzed. RESULTS: Six patients were identified, five of whom (83%) were considered eligible for surgical excision. Gross total resection (GTR) was achieved in three patients (60%), which included two patients with an AKA contralateral to the tumor, and one with an homolateral AKAl. After a median follow-up of 4.1 years from diagnosis, no patients developed neurological complications; five (83%) were alive and well, and one died from refractory recurrence. CONCLUSIONS: Among patients with TNB, POSA was useful for identifying the AKA and defining the optimal surgical strategy. POSA should be considered in the preoperative evaluation of TNB to increase the likelihood of GTR and reduce the threats of iatrogenic neurologic sequelae.

Chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis: Active disease or scarring? A case report and literature review.

The second most common abdominal tumor in children is Wilms' tumor, and the lung is where it most often metastasizes. The typical metastases are multiple, peripherally located, round, and variable-sized nodules. Atypical patterns are also possible and may create diagnostic challenges, especially in patients treated with chemotherapy. Among these, cavitating metastases are an anecdotal type of atypical secondary lung lesions. Here, we report a case of a chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis discovered during the follow-up for an anaplastic nephroblastoma in a 6-year-old girl. Furthermore, we conducted a review of the existing literature on this exceedingly rare radiological pattern to establish its best management.

Uterine leiomyoma in pediatric population: A case report and review of the literature.

Uterine leiomyomas are rare in the pediatric population with less than 20 cases in adolescences reported in the literature. Furthermore, these masses represent a common presentation of gynecologic tumors with increasing age. We report a case of a 14-year-old female who presented with abdominal pain and increasing abdominal girth. Workup with ultrasound, CT and MRI demonstrated a large pelvic mass. Complete resection by median laparotomy was performed. The mass weighed 5,596 g and was 29.5 cm × 27 cm × 19 cm; the pathological examination confirmed the hypothesis of leiomyoma. The patient remained asymptomatic at 3 months follow up.

Case report: Bilateral pleural effusion secondary to late migration of a tunneled central venous catheter in a patient affected by high risk neuroblastoma.

The insertion of long-term central venous catheters is a standard of care for children affected by malignancies, although it can be associated with life-threatening complications. The present paper reports an unusual mechanical complication related to the use of a long term tunneled central venous catheter in a pediatric oncologic patient. An 18 months old child, diagnosed with stage M high-risk retroperitoneal neuroblastoma, underwent ultrasound-guided placement of a 6 Fr bilumen long-term tunneled central venous catheter in the right internal jugular vein prior to the beginning of induction chemotherapy. The correct position of the distal tip of the catheter was confirmed by fluoroscopy. After 4 months of regular use of the device, the patient experienced neck swelling during high-dose chemotherapy infusion. A chest x-ray showed a dislocated catheter and bilateral pleural effusion. CT scan demonstrated the tip of the catheter rupturing the medial wall of the right jugular vein and entering the mediastinum; furthermore, pneumomediastinum, subcutaneous neck emphysema and bilateral pleural effusion were noticed and a thrombus was evident in the right jugular vein at the insertion in the brachiocephalic vein. The patient was then transferred to the Intensive Care Unit and bilateral thoracostomy tubes were placed urgently (500 mL of clear fluid were evacuated from pleural spaces). The dislocated catheter was removed electively on the following day under fluoroscopy. Despite ultrasound-guided placement and long-term uneventful use of the catheter, life-threatening central venous catheter-related mechanical complications can occur; the current case report emphasizes the importance of careful monitoring of patients with central venous catheters in order to quickly diagnose and treat potentially lethal complications.

Vascular Access in Pediatric Oncology and Hematology: State of the Art.

Management and successful use of vascular access are critical issues in pediatric patients affected by malignancies. Prolonged course of disease, complex and various treatment protocols require long-lasting vascular access providing adequate tools to administrate those therapies and to collect routine blood sampling without painful and repeated venipuncture. For these reasons, central venous catheters are currently an important component in pediatric onco-hematological care, with a direct influence on outcome. Indeed, there are peculiar issues (techniques of insertion, management, complications etc.) which must be well-known in order to improve the outcome and the quality of life of children with cancer.

Case report: Primary ovarian Burkitt's lymphoma: A puzzling scenario in pediatric population.

Burkitt's lymphoma (BL) is defined as a highly invasive B-cell lymphoma, usually characterized by an excellent prognosis, more than 90% of children and adolescents being cured with highly dose-intensive multiagent chemotherapy. Primary ovarian localization without involvement of other organs is a rare manifestation of BL, especially in pediatric population. Symptoms at diagnosis are similar to other ovarian lesions and differential diagnosis may be challenging for clinicians. A 12-year-old girl was referred to our institution for abdominal pain and palpable mass observed by the pediatrician. Diagnostic work-up demonstrated a large mass arising from the right ovary, causing compression on abdominal aorta, inferior vena cava, ureters and bowel, with a second smaller lesion on the left ovary. At surgery, a 15 cm-large, ruptured mass arising from the right ovary was found, associated with a second lesion originating from the left ovary (8 cm) and multiple nodules of the greater omentum. Right salpingo-oophorectomy was performed, incisional biopsies were taken from the left ovary and omental nodules and peritoneal fluid samples were collected for cytology. Pathology revealed a Burkitt lymphoma and the patient underwent chemotherapy according to AIEOP LNH-97 Protocol, group R3 with Rituximab. Preoperative diagnosis of primary ovarian lymphoma is extremely difficult. Surgical exploration is often necessary in patients presenting with acute abdominal or pelvic pain; when the suspicion of primary ovarian lymphoma arises intraoperatively, every effort should be made to minimize invasive procedure in order to enhance post-operative recovery.

Hemorrhage During Induction Chemotherapy in Neuroblastoma: Additional Risk Factors in High-Risk Patients.

Background: Neuroblastoma is the most common solid extracranial tumor in children. Patients affected by neuroblastoma are stratified into low, intermediate, and high risk in terms of event-free and overall survival. Some high-risk patients have an additional risk of acute hemorrhagic complications during induction chemotherapy. Aim: To find easily and rapidly assessed parameters that help clinicians identify those patients affected by high-risk neuroblastoma who have an additional risk of hemorrhagic complications. Methods: The clinical notes of patients diagnosed with high-risk neuroblastoma from January 2013 until February 2021 were retrospectively reviewed. Clinical, demographic and laboratory data, biological characteristics of the tumor, and information about treatment and hospital stay were identified. Results: In the examined period, 44 patients were diagnosed with high-risk neuroblastoma. Four of these patients had hemorrhagic complications within 2-7 days after the initiation of induction chemotherapy; two patients had hemothorax, one patient had hemoperitoneum and one patient had hemothorax and hemoperitoneum. The patient with isolated hemoperitoneum was treated with blood components transfusions, clotting factors and colloids infusions; the three patients with hemothorax underwent thoracostomy tube placement and respiratory support. At initial presentation, patients who suffered from hemorrhagic complications had a higher degree of hypertension (stage 2, p = 0.0003), higher levels of LDH (median 3,745 U/L, p = 0.009) and lower levels of hemoglobin (mean 7.6 gr/dl, p = 0.0007) compared to other high-risk patients. Conclusions: A subgroup of "additional" high-risk patients can be identified within the high-risk neuroblastoma patients based on mean arterial pressure, LDH levels and hemoglobin levels at presentation. Further studies to define cut-off values and optimal management strategies for these patients are needed.

Burned-Out Testicular Tumors in Adolescents: Clinical Aspects and Outcome.

Purpose: Testicular germ cell tumors are the fourth most common neoplasm in adolescents, accounting for 8% of all tumors in the age group 15-19 years. On rare instances, the primary testicular lesion is not clinically or radiologically evident while nodal or visceral metastases represent the clinical manifestations of the disease. This phenomenon is described as "burned-out testicular tumor." In this paper, the authors report a single-institution experience with burned-out testicular tumors in adolescents and discuss their clinical implications. Patients and Methods: All the patients diagnosed with metastatic testicular germ cell tumors at Bambino Gesù Children Hospital between January 1, 2010, and June 30, 2020, were included in the study. Patients were categorized into two groups: "primary testicular" and "burned out." All the patients were staged and treated according to the AIEOP-TCGM 2004 protocol. Results: Eleven patients were classified as "primary testicular," and five patients were classified as "burned out." "Burned-out" tumors were associated with the presence of systemic symptoms compared to "primary testicular" tumors (80 vs. 0%; p = 0.0027) and higher aFP, hCG, and LDH levels (p < 0.00001). The "burned-out" population had a statistically significant higher incidence of relevant toxicity than the "primary testicular" population (80 vs. 18%; p = 0.0357) and a worse outcome in terms of both mean overall survival (15 vs. 43 months; p = 0.0299) and mean event-free survival (12 vs. 38 months; p = 0.0164). Conclusion: "Burned-out" testicular tumors seem to be a well-distinct clinical entity with a high treatment-related toxicity and poor prognosis. Further studies are needed to clarify the "burned-out phenomenon" and to identify more effective therapeutic strategies for these patients.

Renal pseudoaneurysm after blunt trauma in a 10-year-old girl: A case report.

INTRODUCTION: Renal trauma is a relevant cause of morbidity in children older than 1 year. Most patients are currently managed conservatively, even in case of high-grade traumas; nevertheless, harmful complications may occur even in hemodynamically stable patients. We present a case of grade IV blunt renal trauma complicated by post-traumatic pseudoaneurysm. CASE DESCRIPTION: A 10-year-old girl was referred to our institution for grade IV trauma of the right kidney. During observation she had persistent hematuria that caused anemia. A second contrast-enhanced computed tomography scan revealed a posttraumatic pseudoaneurysm that was successfully treated by angiographic embolization. CONCLUSIONS: Although extremely rare after blunt renal trauma, post-traumatic renal pseudoaneurysm may cause severe blood loss and anemia, and angioembolization is therefore indicated. This condition should be suspected and move physicians to investigate further.

Heterotopic pancreas in the gastrointestinal tract in children: a single-center experience and a review of the literature.

BACKGROUND: Heterotopic pancreas, that is the abnormal localization of a well-differentiated pancreatic tissue, is a rare occurrence in pediatric patients. Most lesions are found incidentally; in some circumstances, the presence of heterotopic pancreas may cause gastrointestinal symptoms, such as obstructive symptoms or bleeding. PATIENTS AND METHODS: The clinical notes of patients with histological diagnosis of heterotopic pancreas treated at Meyer Children's Hospital between 2009 and 2017 have been retrospectively examined. Four variables have been examined: clinical presentation, age at diagnosis, timing of surgery and localization of the heterotopic pancreas. Patients have been classified accordingly. RESULTS: Fourteen patients were diagnosed with heterotopic pancreas at a single institution. In half cases, heterotopic pancreas caused symptoms that warranted surgical exploration. Symptomatic patients were significantly older than patients in whom heterotopic pancreas was an incidental finding (mean age 9 years and 5 months vs 2 years and 9 months; p = 0.02). Heterotopic pancreas was more frequently found in patients who underwent urgent surgical procedure than in patients who underwent elective surgery (2.61% vs 0.22%; p < 0.0001). In all cases, foci of heterotopic pancreas were resected. CONCLUSION: Heterotopic pancreas is usually discovered in the submucosa of the stomach, duodenum and small bowel. Heterotopic tissue may cause symptoms related to mechanical complications, bleeding from the surrounding intestinal mucosa or, occasionally, to the development of malignancy. Heterotopic tissue is a rare but clinically relevant cause of gastrointestinal symptoms. The presence of heterotopic tissue should be considered in children with gastrointestinal symptoms of unclear origin and surgical resection is advisable.

Expanding intestinal segment using osmotic hydrogel: An in vivo study.

Intestinal circumferential expansion is essential for bowel lengthening in patients with Short Bowel Syndrome. We hypothesized use of an endoluminal osmotic hydrogel expander (EOHE) as a novel approach for intestinal expansion. An EOHE was introduced into an isolated intestinal segment of New Zealand rabbits, with a similar segment created as a control. After 4weeks, the segments were retrieved for analysis. Weight, inflammatory markers and fluoroscopy data was recorded weekly. EOHE allowed successful expansion of intestinal segments from 4.68 ± 0.35 to 9.79 ± 0.35 cm (p = 0.01). Increase in intestinal length was 167.8 ± 35.21% in segments with EOHE vs. 23.03 ± 4.2% in the control group (p < 0.01). A significant intestinal dilatation (214.4 ± 1.58 vs. 34.59 ± 1.23%, p < 0.01) was demonstrated. Hematoxylin and eosin stain revealed conservation of intestinal architecture with muscle hypertrophy and flattening of the epithelium possibly due to compression. No reduction of rabbit weight, inflammatory markers or liver damage was described. EOHE appears to produce safe intestinal expansion, achieving increased length and dilatation suitable for lengthening procedure. This approach may allow development of similar techniques to expand bowel in short bowel patients. © 2018 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater 107B: 1304-1309, 2019.

Successful Use of Negative-pressure Wound Therapy and Dermal Substitute in the Treatment of Gluteal Ecthyma Gangrenosum in a 2-year-old Girl.

Ecthyma Gangrenosum is a manifestation of Pseudomonas Aeruginosa infection, usually occurring in immunocompromised patients, which can be associated with Pseudomonas Aeruginosa bacteremia with potentially lethal outcome. The clinical appearance is of an inflammatory cutaneous lesion with a central necrotic spot; the lesion then rapidly progresses to a gangrenous ulcer with a gray-black eschar extending in the deep soft tissues. Treatment of Ecthyma Gangrenosum includes both aggressive systemic antibiotic therapy and surgical procedures. A 2-year-old girl affected by B-cell precursor acute lymphoblastic leukemia was admitted to our hospital for suspected sepsis; the diagnosis was later confirmed by blood cultures positive for Pseudomonas Aeruginosa. In the days following the diagnosis, the patient developed a necrotic lesion of the right gluteal area consistent with Ecthyma Gangrenosum. Aggressive surgical debridement was then performed, followed by negative-pressure wound therapy and reconstruction with dermal substitute and autologous skin graft, which were successful. Ecthyma Gangrenosum is a potentially lethal condition affecting especially immunocompromised patients; aggressive medical treatment with combination antibiotic therapy is warranted and multiple surgical procedures, including extensive surgical debridement and diverting colostomy, are needed. Various reconstructive techniques have been reported in the literature, although no gold-standard can be established to date. Since Ecthyma Gangrenosum lesions are characterized by the presence of both high inflammatory activity due Pseudomonas infection and extensive tissue loss, the association of negative-pressure therapy and dermal substitutes implant seem to have a rationale in the surgical treatment of Ecthyma Gangrenosum and should therefore be considered.

Surgical approach to giant ovarian masses in adolescents: technical considerations.

Ovarian neoplasms arising from the surface epithelium are rare in the pediatric population; their knowledge is therefore limited and the appropriate management is poorly defined. We describe our experience and suggest our surgical approach to adolescents affected by voluminous ovarian masses. Two 15-year-old adolescents were admitted to our institution in 2017 for multilobulated, fluid-filled masses measuring over 30 cm arising from the ovaries. The cystic component was drained intraoperatively with a spillage-free technique, consisting in the application of a sterile autoadhesive transparent drape on the cyst and the insertion of a 12 Ch pleural drain, secured with a purse-string suture. Unilateral salpingo-oophorectomy was then carried out. Histology revealed mucinous cystadenoma in both patients. Surgical treatment of ovarian masses should aim at both radically excising the tumor and preserving the fertility of the patients. Decompression with spillage-free techniques can be useful to achieve radical therapy with limited manipulation of tissues.

Role of thoracoscopy in traumatic diaphragmatic hernia.

Thoraco-abdominal trauma can in rare cases involve diaphragmatic rupture and subsequent herniation of intra-abdominal contents. We report a case of this complication in a 5-year-old boy who was injured in a car crash, and who manifested respiratory distress and hemodynamic instability after 48 h of being monitored in the pediatric intensive care unit. Multiple radiologic investigations were inconclusive and the definite diagnosis was established only on thoracoscopic exploration.