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.

Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center.

BACKGROUND/AIMS: The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center. MATERIALS AND METHODS: Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes. RESULTS: Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL. CONCLUSION: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA.

Neuroencorine Tumor Arising within a Tailgut Cyst in an Adolescent Boy.

INTRODUCTION: Neuroendocrine tumors (NET) within tailgut cysts (TC) are usually seen in middle-aged females. A 14 year-old boy who underwent surgical excision of NET within TC is presented to discuss the clinical features, diagnosis and treatment options. CASE REPORT: A 14-year-old boy with rectal bleeding and constipation had a 5 × 4 cm mass detected upon rectal examination. The cystic mass was totally excised. The histopathological evaluation revealed a low-grade NET within mucularis layer of the cyst showing a positive immunhistochemical staining for cytokeratin and synaptophysin. CONCLUSION: Although, the malignant transition of TC is commonly observed in middle age females, males and children can also be affected. This is first report of a pediatric TC with malignant transformation. Total surgical excision of cysts is mandatory for local control of the disease and chemotherapy should be reserved for the patients who have positive surgical margins.

Evaluation of surgically treated breast masses in children.

Dogan G, Soyer T, Ekinci S, Karnak I, Çiftçi AÖ, Tanyel FC. Evaluation of surgically treated breast masses in children. Turk J Pediatr 2017; 59: 177-183. We aimed to define the diagnostic and treatment characteristics of breast masses among female children who had undergone surgical treatment in order to figure out which children should be followed conservatively or treated surgically. We reviewed retrospectively 64 female patients operated for breast masses under the age of 18 years between 1977 and 2013. Patient demographics, symptoms, physical examination findings, size of the mass and its relation with menstrual cycle, follow-up period before and after surgery, diagnostic characteristics, laboratory analysis, diagnosis before and after surgery, indications for surgery, histopathological characteristics of the mass, postoperative complications, and recurrence rate were recorded. Histopathologic investigations among 41 girls with complete hospital records showed that 26 had fibroadenoma (FA; 63.4%). Eleven girls among the other 23 cases with incomplete hospital records but with histopathological results have also had FA (47%). The majority of breast masses in childhood are FA, the frequency and duration of follow up is important. If there is a mass that will not regress, that continues to grow or does not reduce in size during follow up, and for those with family history of breast cancer to definitely exclude the possibility of malignancy, surgical treatment may be appropriate.

Contralateral genitofemoral sympathetic nerve discharge increases following ipsilateral testicular torsion.

The decrease in blood flow due to the activation of sympathetic system has been suggested to play a role in contralateral testicular deterioration associated with unilateral testicular torsion. Sympathetic nerve discharges (SND) from the genitofemoral nerve were evaluated before and during unilateral testicular torsion. Under urethane anesthesia, arterial blood pressure and SND from splanchnic and right genitofemoral nerves were recorded in 12 male Sprague-Dawley rats, 8 of which were included in subsequent analyses. After control recordings of basal discharges for 2 min the left testis was twisted 720 degrees counterclockwise, and recording was resumed for an additional 30 min. Changes in nerve activity were calculated by measuring the area under the autospectrum curve, and alterations were compared. Following testicular torsion no significant changes were obtained for splanchnic SND, but the amplitude of SND from contralateral genitofemoral nerve showed an overall increase of 21.20+/-7.03% in six rats. This increase lasted about 10-15 min and activities returned to pretorsion levels. In two other rats no significant change was observed in either splanchnic or genitofemoral SND. Ipsilateral testicular torsion results in a transient increase in genitofemoral SND. A possible autonomic reflex mechanism may exist, and it may be activated by noxious stimuli from contralateral side. This reflex mechanism may initiate a series of events that lead to the injury of contralateral testis.