Background and aims Patients affected by metastatic germ cell tumors may occasionally experience enlargement of metastatic lesions with concurrent normalization of tumor markers after chemotherapy. This phenomenon is described as Growing Teratoma Syndrome (GTS). The aim of the present study is to assess the prevalence of GTS in the pediatric population and its implications in terms of surgical outcome. 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. Patients with residual disease after chemotherapy were studied; the occurrence 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. Eleven patients (34%) had radiologic evidence of enlargement of metastases after chemotherapy; nine of them (28%) had normal markers after chemotherapy and 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 for peri-operative complications. Out of the three patients who could not achieve radical excision of the metastases, two died for 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.

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% to 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. As well 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 to 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. When TALS develops, pulmonary function progressively worsens toward respiratory failure and death.

Thoracic air leak syndrome (TALS) is a complication related to chronic pulmonary graft-versus-host disease (pGvHD) and is defined as the occurrence of any form of air leak in the thorax. Four patients, aged 16 to 25 years, underwent surgical procedures for TALS between January 2016 and March 2021. All the patients experienced at least two episodes before surgery. After surgery, patients were temporarily free from air leak symptoms. All the patients eventually died because of respiratory failure. Surgery provides temporary relief to symptoms related to TALS. When TALS develops, pulmonary function progressively worsens toward respiratory failure and death.

Background: Thyroid gland malignancy is rare in pediatrics (0.7% of tumors); only 1.8% are observed in patients < 20 yrs with a higher prevalence recorded in females and adolescents. Risk factors include genetic syndromes - MEN disorders, autoimmune disease and ionizing radiation exposure. Radiotherapy is also linked with increased risk of secondary thyroid cancers. The present study describes the clinical features and surgical outcomes of primary and secondary thyroid tumors. Methods: Institutional data was collected on pediatric patients with thyroid cancer during 2000 - 2020 from 8 International Surgical Oncology centers. Statistical analysis was performed using GraphPad Prism. Results: Of 255 cases of thyroid cancer, only 13 (5.1%) were secondary tumors. Primary thyroid malignancies were more likely to be multifocal in origin (odds ratio [OR] 1.993, 95% confidence interval [CI] 0.7466-5.132, p 0.2323), had bilateral glandular location (OR 2.847, 95% CI 0.6835-12.68, p 0.2648) and proved metastatic at 1st diagnosis (OR 1.259, 95% CI 0.3267-5.696 p>0.999). Secondary tumors showed a higher incidence of disease relapse (OR 1.556, 95% CI 0.4579-5.57, p 0.4525) and surgical morbidity (OR 2.042, 95% CI 0.7917-5.221, p 0.1614) including hypoparathyroidism and recurrent laryngeal nerve injury. Overall survival (OS) was 99% at 1 year and 97% after 10 years. No EFS differences were evident with primary vs. secondary tumors (Chi square 0.7307, p 0.39026). Conclusions: This multicenter study demonstrates excellent survival for pediatric thyroid malignancy. Secondary tumors exhibit greater disease relapse (15.8% vs 10.5%) and a higher incidence of surgical related complications (36.8% vs 22.2%).

Background: Postoperative hypocalcemia is a frequent complication after thyroid surgery. Hypoparathyroidism may develop as transient (TtHP), with normalization within six months from surgery, or permanent (PtHP) if the patient requires replacement therapy. The present study analyzes rates and factors associated with the development of TtHP or PtHP following thyroid surgery in a pediatric population. Procedure: A retrospective multicenter study analyzing 363 patients was carried out. We recorded gender, age, tumor size, type of surgery, lymph node dissection, histology. Calcium levels were acquired daily for 72 hours after discharge. Subsequent sample collection was customized on the patient’s hypocalcemia severity. Results: We analyzed 363 patients aged ≤18 years (mean age 14.2 years) who underwent thyroid surgery clustered into age groups (≤15 or >15). Patients mean follow-up was 5.8 years (1-11yrs). At histology 310 (85%) were papillary carcinoma, 32 (9%) were follicular carcinoma, 6 (2%) presented diffuse sclerosing variant of papillary thyroid carcinoma whilst 15 (4%) had familial medullary carcinoma. TtHP developed in 36 (9,9%), PtHP in 20 (5.5%) cases. TtHP was more frequent in younger patients (p=0,009). Both PtHP and TtHP were increased in case of larger tumors (≥2 cm) (p=0,001). All TtHP and PtHP were in TT group. PtHP rate was increased if lymph node dissection was carried out (p<0.001). Conclusions: The risk of hypoparathyroidism is related to younger age, tumor size, TT and lymph node dissection therefore surgeons should tailor surgery as much as possible to avert such complication.

A 2-year-old boy with severe combined immunodeficiency developed gut graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation (HSCT), associated with massive intestinal pneumatosis (IP), pneumoretroperitoneum (PRP) and pneumomediastinum. His fair clinical conditions allowed conservative management, with progressive normalization of imaging findings. The patient did not require surgery and is alive and in good clinical conditions at follow-up. In children with GVHD-related IP but good clinical conditions and no signs of peritonitis, IP, is not a mandatory indication for surgery, despite its potentially striking imaging features. Conservative management, with intestinal rest, decompression and antibiotics, often allows regression of the clinical picture.