Assessment of prognostic factors in pediatric adrenocortical tumors: The modified pediatric S-GRAS score in an international multicenter cohort: A work from the ENSAT-PACT working group.

OBJECTIVE: Pediatric adrenocortical carcinoma (pACC) is rare, and prognostic stratification remains challenging. We aimed to confirm the prognostic value of the previously published pediatric scoring system (pS-GRAS) in an international multicenter cohort. DESIGN: Analysis of pS-GRAS items of pACC from six countries in collaboration of ENSAT-PACT, GPOH-MET and IC-PACT. METHODS: We received patient data of the pS-GRAS items including survival information from nine centers. PS-GRAS score was calculated as a sum of tumor stage (1 = 0; 2-3 = 1; 4 = 2 points), grade (Ki67 index: 0-9%=0; 10-19%=1; ≥ 20%=2 points), resection status (R0 = 0; RX/R1/R2 = 1 point), age (<4 years = 0; ≥ 4 years = 1 point), and hormone production (androgen production = 0; glucocorticoid-/mixed/-no hormone production = 1 point) generating eight scores and four groups (1: 0-2, 2: 3-4, 3: 5, 4: 6-7). Primary endpoint was overall survival (OS). RESULTS: We included 268 patients with median age of 4 years. The analysis of the pS-GRAS score showed a significantly favorable prognosis in patients with a lower scoring compared to higher scoring groups (5-year OS: group 1 98%; group 2 87%(HR of death 3.6, 95% CI of HR 1.6-8.2); group 3 43%(HR of death 2.8, 95% CI 1.9-4.4); group 4: OS 18%(HR of death 2.1, 95% CI 1.7-2.7)). In the multivariable analysis age (HR of death 3.5, 95% CI 1.8-7.0), resection status (HR of death 5.5, 95% CI 2.7-11.1), tumor stage (HR of death 1.9, 95%-CI of HR 1.2-3.0) and Ki67-index (HR of death 1.7, 95% CI 1.2-2.4) remained strong independent outcome predictors. Especially infants < 4 years showed more often low risk constellations with a better OS for all tumor stages. CONCLUSION: In an international multicenter study, we confirmed that the pS-GRAS score is strongly associated with overall survival among patients with pACC. Age, resection status, stage and Ki67-index are important parameters for risk stratification.

A Single-center Experience of Radiotherapy in Pediatric Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Chest Wall.

AIM: To evaluate the treatment results, prognostic parameters, and treatment-related toxicity in patients with Ewing sarcoma (ES)/primitive neuroectodermal tumor (PNET) of the chest wall who underwent surgery, chemotherapy, and radiotherapy (RT) in a tertiary referral center. METHODS: The data of 24 patients under 18 years of age with a histologic diagnosis of ES/PNET in the chest wall that received RT in our department between February 2003 and July 2020 were retrospectively evaluated. RT was applied to the primary site±whole involved chest wall and to the whole lung in patients with lung metastasis. RESULTS: The median age was 8.5 years (range: 1.5 to 17 y), 15 (63%) patients were female and 9 were male (37%). The tumor localization was extrathoracic in 18 (75%) and intrathoracic in 6 (25%) patients. Mediastinal lymph node and distant metastasis (DM) was present in 5 (21%) and 4 (16%) cases at diagnosis, respectively. The median follow-up after RT was 47 months (range: 11 to 162 mo). The 2-year and 5-year overall survival, event-free survival, local recurrence-free survival, and pleural recurrence-free survival were 83% and 48%, 48% and 42%, 74% and 48%, and 61% and 52%, respectively. The overall local control rate was 83% and the pleural control rate was 67%. RT was well tolerated, with 1 case of grade 3 acute dermatitis and 1 case of grade 3 subacute radiation pneumonitis. Late toxicity was observed in 3 (13%) cases. CONCLUSION: Long-term survival can be achieved with extended-field RT even in patients with ES/PNET of the chest wall with DM. The low toxicity rates allow us to draw the conclusion that RT with modern techniques is an effective and safe treatment modality for these patients.

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.

Esophageal Carcinoma in Children: Report of 2 Cases and a Review of the Literature.

Esophageal carcinoma in children and adolescents is extremely rare. Here, we report 2 cases of pediatric esophageal carcinoma presenting with progressive dysphagia. There was not any underlying specific risk factor in our cases. The histopathological subtypes were adenocarcinoma in one and squamous cell carcinoma in another case. Response to combined modality treatment was good in the case of adenocarcinoma, while the patient with squamous cell carcinoma was unresponsive to treatment and died of the progressive disease. We reviewed the pediatric cases of esophageal carcinoma reported in the literature. Progressive dysphagia was observed in 89% of these cases. One third of pediatric cases had underlying risk factors. Squamous cell carcinoma is a more common type of childhood esophageal carcinoma. In contrast to adults, pediatric esophageal squamous cell carcinoma may distribute throughout the esophagus. Esophageal adenocarcinoma was seen in the distal esophagus in pediatric cases. Metastatic disease was found in 48% of pediatric patients at presentation, and the prognosis is poor. Collaborative efforts are needed for success in the treatment of esophageal carcinoma.

Case Report: Severe McCune-Albright syndrome presenting with neonatal Cushing syndrome: navigating through clinical obstacles.

Background: Café-au-lait skin macules, Cushing syndrome (CS), hyperthyroidism, and liver and cardiac dysfunction are presenting features of neonatal McCune-Albright syndrome (MAS), CS being the rarest endocrine feature. Although spontaneous resolution of hypercortisolism has been reported, outcome is usually unfavorable. While a unified approach to diagnosis, treatment, and follow-up is lacking, herein successful treatment and long-term follow-up of a rare case is presented. Clinical case: An 11-day-old girl born small for gestational age presented with deterioration of well-being and weight loss. Large hyperpigmented macules on the trunk, hypertension, hyponatremia, hyperglycemia, and elevated liver enzymes were noted. ACTH-independent CS due to MAS was diagnosed. Although metyrapone (300 mg/m2/day) was started on the 25th day, complete remission could not be achieved despite increasing the dose up to 1,850 mg/m2/day. At 9 months, right total and left three-quarters adrenalectomy was performed. Cortisol decreased substantially, ACTH remained suppressed, rapid tapering of hydrocortisone to physiological dose was not tolerated, and supraphysiological doses were required for 2 months. GNAS analysis from the adrenal tissue showed a pathogenic heterozygous mutation. During 34 months of follow-up, in addition to CS due to MAS, fibrous dysplasia, hypophosphatemic rickets, and peripheral precocious puberty were detected. She is still regularly screened for other endocrinopathies. Conclusion: Neonatal CS due to MAS is extremely rare. Although there is no specific guideline for diagnosis, treatment, or follow-up, addressing side effects and identifying treatment outcomes will improve quality of life and survival.

Favourable outcomes of conservatively managed acute abdomen associated with multisystem inflammatory syndrome in children during the COVID-19 pandemic.

BACKGROUNDS: Abdominal pain is one of the most common symptoms of multisystem inflammatory syndrome in children (MIS-C). Abdominal pain can vary from mild to severe and may present as acute abdomen. Severe abdominal pain in patients with MIS-C should be differentiated from surgical causes of acute abdomen to prevent unnecessary surgery. METHODS: The diagnosis of MIS-C was based on WHO and CDC recommended criteria. Records of children treated for MIS-C between September 2020 and January 2021 were reviewed retrospectively. RESULTS: In a short time, we encountered seven patients who were diagnosed with MIS-C and showed acute abdomen findings. There were four male and three female patients. The median age was 9 years. Fever, abdominal pain and distension were present in all. The median duration of symptoms was 4 days. Five patients had general abdominal tenderness mimicking acute abdomen. Three patients had right lower quadrant tenderness mimicking acute appendicitis. After the initiation of immunomodulatory therapy and antibiotics, the physical examination findings were improved step by step in all. The median time to initiate oral feeding was 2 days. The median length of hospitalization time was 8 days. CONCLUSION: Serial abdominal examinations performed by the same surgeon enabled us to follow these patients conservatively and thus avoid unnecessary surgical intervention.

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.

Imaging findings of gastrointestinal tract tumors in children and adolescents.

Gastrointestinal (GI) tract tumors are rarely seen in children and adolescents, and can easily be misdiagnosed. Lymphoma is the most frequent GI tract tumor, and the common locations are ileum and ileocecal area. GI tract tumors may present as large heterogeneous mass lesions. For gastric and colonic tumors, increased wall thickening usually prompts the diagnosis of GI tract tumors. Computed tomography and magnetic resonance imaging might be used in clinically suspected cases for correct/appropriate diagnosis and management. Awareness as regards the most common tumors and their locations is paramount for radiologists. Likewise, the aim of this article was to define the imaging findings of primary benign and malignant GI tract tumors in children and adolescents.

Feminizing Adrenocortical Tumors as a Rare Etiology of Isosexual/Contrasexual Pseudopuberty

Objective: Estrogen-secreting adrenocortical tumors (ACTs) are quite rare with feminizing adrenocortical tumors (FATs) accounting for 0.37-2% of all ACTs. The aim was to evaluate clinical and hormonal characteristics of FATS as well as treatment options and follow-up in the pediatric age group. Methods: Medical records of children with ACTs presenting to a single center in the last two decades were reviewed. Literature review within Pubmed revealed 34 pediatric patients (22 boys) with FAT among 192 articles. Results: Among the 25 children presenting with ACTs in the last two decades, two new pediatric cases of FAT were identified, one benign and the other malignant, in two genders with different clinical presentations. Literature review showed that FATs are extremely rare tumors that are most commonly seen in men and boys presenting with gynecomastia. FATs are more common in children ≤8 years of age, with a median age at diagnosis of six years. While boys present with contrasexual pseudopuberty signs, girls present with isosexual pseudopuberty. A high estrogen level strongly supports diagnosis, while elevations in other adrenal hormones may be seen. FATs are usually malignant in adults and prognosis is generally very poor. However, in children approximately half are benign although assessment of malignant potential depends on clinical behavior of the tumor. FATs are very unpredictable so even after surgery long-term follow-up is required. FATs presenting in childhood may have a better prognosis than adult presentation tumors as most FATs in children are followed without recurrence of tumor. Conclusion: FATs are more common in children ≤8 years of age, with a median age at diagnosis of six years. FATs in childhood may have a better prognosis than in adult males.

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.

Reconstruction of congenital rectovestibular fistula in an adult. Case report.

Anorectal malformations are common congenital anomalies but diagnosis and treatment in adulthood are quite rare. Treatment during adulthood may be challenging due to anatomic and physiologic changes. Posterior sagittal anorectoplasty may provide good cosmetic and functional results even in adult patients. KEY WORDS: Congenital malformations, Rectovaginal fistula, Rectovestibular fistula.

Imaging findings of primary adrenal tumors in pediatric patients.

Apart from neuroblastomas, adrenal tumors are rarely seen in children. The most common adrenal tumors are adrenocortical carcinoma and pheochromocytoma. Adrenocortical carcinoma is usually a large heterogeneous, well-marginated mass with solid/cystic areas and calcifications, with poor prognosis. Most of the pheochromocytomas are benign tumors and usually show intense contrast enhancement, the pattern of which may be diffuse, mottled, or peripheral on computed tomography and magnetic resonance imaging. The purpose of this article is to evaluate primary nonneurogenic adrenal tumors.

Intestinal perforation in necrotizing enterocolitis: Does cardiac surgery make a difference?

BACKGROUND: The aim of this study is to evaluate the patients with intestinal perforation secondary to necrotizing enterocolitis (NEC) following cardiac surgery in the terms of risk factors and diagnosis/treatment process. METHODS: A series of cases operated for intestinal perforation secondary to NEC were retrospectively reviewed in two groups. Group I involved patients who had cardiac surgery for congenital heart disease previous to intestinal perforation secondary to NEC. Group II consisted patients who had intestinal perforation secondary to NEC without any previous cardiac surgery. Demographic characteristics, prenatal and postnatal features, and pre- and post-operative course of groups were statistically compared to define differences. RESULTS: Thirty-two patients underwent laparotomy secondary to intestinal perforation in this period. The gestational age and birth weight were smaller in Group II (p=0.001, p=0.001). Intrauterine growth retardation was more frequent in Group II (p=0.05). More Group I patients had hypotensive periods (p=0.018) before the diagnosis of NEC. Prostaglandin treatment and requirement of renal replacement therapy were more frequent in Group I (p=0.022, p=0.03). The mortality rate was higher in Group I (p=0.018). All patients in Group I were late stage NEC at the time of diagnosis. CONCLUSION: NEC developing after cardiac surgery is different from NEC seen in the neonatal period in the terms of etiology, facilitating factors, and clinical course. Mortality rate is higher in NEC after cardiac surgery. The diagnosis of intestinal perforation might be difficult in NEC after cardiac surgery due to insignificant physical examination findings and characteristic radiological signs of NEC. The history of prostaglandin usage and requirement of renal replacement were thought as alarming signs in terms of possible intestinal complications after cardiac surgery.

The effect of the COVID-19 pandemic on paediatric cancer care: lessons learnt from a major paediatric oncology department in Turkey.

The COVID-19 pandemic has disrupted cancer care. An audit at a major Paediatric Oncology Department in Turkey was performed to determine its impact on paediatric cancer care. A comparison was made among the number of daily paediatric cancer patients, diagnostic and treatment procedures. The data for the 'COVID-19 period' (10 March to 31 October 2020) were compared with the corresponding 'prior year control period' (10 March to 31 October 2019). Moreover, presentation delay (duration between first symptoms to healthcare visit) was calculated for new cases. The findings indicate that the mean 34.7 outpatients per day during 'COVID-19 period' was significantly lower than the 'prior year control period' (52.2). There were 17.7 inpatients per day during the 'COVID-19 period' which was significantly lower than 23.8 inpatients per day during the 'prior year control period'. Significant reduction in the daily mean number of patients undergoing chemotherapy, radiotherapy, surgery and imaging studies during the 'COVID-19 period' was also evident. A negative trend in the diagnosis of new paediatric cancers was evident with 128 new cancer cases during the 'COVID-19 period', whereas the corresponding number was 212 for the 'prior year control period'. The presentation delay (median 31 days) remain unchanged during the 'COVID-19 period'. The findings suggest significant damage to paediatric cancer care during the COVID-19 pandemic. Appropriate obligatory actions by oncology societies and policymakers can minimise longer term negative impacts.

Imaging findings of benign and malignant pediatric splenic lesions.

Splenic lesions in children have a wide histological spectrum. The majority of pediatric splenic lesions are benign and detected incidentally, and the most common benign lesions are cysts, followed by hemangiomas and lymphatic malformations. Most of the splenic malignancies in children are secondary to leukemia or lymphoma. The purpose of this article is to describe the ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) features of benign and malignant splenic lesions in the pediatric age group.

Extraovarian juvenile granulosa cell tumor in a prepubertal child: novel location of a rare tumor.

Juvenile granulosa cell tumor (JGCT) is the most common type of sex cord stromal tumor arising from gonadal structures of children and young adults. We present a 3.5-year-old girl with JGCT located in retroperitoneum without ovarian involvement. Extragonadal occurrences of other sex cord stromal tumors have been rarely reported, but this is the first case of JGCT in an extragonadal location. We speculate the possible underlying mechanism of sex cord stromal tumor formation in extragonadal locations. Furthermore, clinical presentation, differential diagnosis and management of this tumor in childhood are discussed.

Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center.

AIM: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center. MATERIAL AND METHODS: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively. RESULTS: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I131MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18. CONCLUSION: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors.

Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.

BACKGROUND: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management. METHODS: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively. RESULTS: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months. CONCLUSIONS: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.

Ultrasound-guided anatomical evaluation of right internal jugular vein in children aged 0-2 years: A prospective observational study.

BACKGROUND: The right internal jugular vein is commonly preferred for central venous cannulation, and ultrasonographic assessment and guidance is the recommended technique for this procedure. Despite the safety and reliability of this technique, it requires specific training and experience as well as thorough knowledge of anatomy. AIM: The position of the right internal jugular vein with respect to carotid artery and its transverse diameter free from overlap with the carotid artery were evaluated using ultrasound in patients aged 0-2 years. METHODS: This single-center, prospective, observational study included patients aged 0-2 years who underwent elective surgery between July 2018 and July 2019. The diameters and anatomical position were evaluated using ultrasound. All parameters were compared between groups classified according to postmenstrual ages. The correlations of these parameters with weight and postmenstrual ages were analyzed. RESULTS: A total of 156 patients were included. The diameters (P < .001) and depth (P = .012) were smaller in patients with postmenstrual age <45 weeks. The anteroposterior diameter was significantly correlated with weight in patients with postmenstrual age <45 weeks, but not in older patients. Right internal jugular vein was lateral and anterolateral with respect to carotid artery in 51.3% and 42.9% of patients, respectively, and >80% of the transverse diameter was free from overlap with the carotid artery. CONCLUSION: Our results support the current data for the high anatomical variability of the right internal jugular vein, highlighting the recommendation of the Rapid Central Vein Assessment (RaCeVA) protocol prior to and ultrasound guidance during catheterization of central veins.

The results and outcome of primary spontaneous pneumothorax in adolescents.

AIM: Primary spontaneous pneumothorax (PSP) is a rare pulmonary pathology that occurs in the absence of known lung disease. A retrospective study was performed to evaluate the results and outcome of PSP treatment in adolescents. METHODS: The cases with PSP from January 2004 to December 2017 were evaluated for age, sex, family and smoking history, clinical and radiological findings and results of treatment. RESULTS: Ten cases with PSP were included. The mean age of the patients was 15 years (10-17 years) and the male to female ratio was 9:1. Two of the patients (20%) had family history of PSP and four cases (40%) had smoking history. The initial complaints were chest pain (n=8), acute onset of cough (n=1) and breathing difficulty (n=1). Tube thoracostomy was performed in nine cases in which three of them were bilateral. Chest computed tomography (CT) demonstrated bullae (n=4; 40%) and subpleural blebs (n=2; 20%). Pleurodesis with talc was performed in four patients with pneumothorax for longer than a week in follow-up (n=4; 40%). Five cases had recurrent PSP within one year (n=5; 50%) and underwent pleurodesis with talc (n=4), autologous-blood (n=1) and bleomycin (n=1). Bleb excision was performed in two cases with persistent pneumothorax despite pleurodesis. CONCLUSION: Tube thoracostomy and oxygen supplementation, are considered as initial and adequate treatment of PSP in most of the adolescents. Prolonged air leaks require pleurosdesis as the first line treatment and surgical excision of blebs should be reversed for the patients who are unresponsive to other treatment options.