Oncocytic adrenocortical tumor with uncertain malignant potential in pediatric population: A case report and review of literature.

BACKGROUND: Oncocytic adrenocortical tumor (OACT) is rare, with few cases reported in the literature. No more than 20 cases in children have been reported. The clinical characteristics, diagnosis, treatment and prognosis of children with OACT are summarized based on a literature review, in order to improve the understanding of OACT in children. CASE SUMMARY: We report a case of a 17-mo-old patient who was admitted to our hospital due to symptoms of odynuria and fever, which are clinical features consistent with a functional adrenocortical tumor. The patient was diagnosed with OACT of uncertain malignant potential. Computed tomography indicated a soft tissue giant tumor in the right adrenal region, approximately 4.3 cm × 5.5 cm in size. Multiple nodular and speckled calcifications were observed in the lesion. The patient received robot-assisted laparoscopic right adrenal tumor resection. Postoperative pathological results were consistent with OACT, and immunohistochemical results showed cytokeratin+/-, chromogranin A+, synaptophysin-, neuron-specific enolase-, S100-, Ki67 about 10%, CD34- and D2-40-. After surgery, urinary tract ultrasonography was reviewed monthly, catecholamine hormone and sex hormone levels were examined every 2 mo and computed tomography was performed every 6 mo. To date, no tumor metastasis or recurrence has been identified in this patient. The levels of sex hormones and catecholamine hormones decreased to normal 1 mo after surgery. CONCLUSION: OACT is rare in the pediatric population, with few cases reported in the literature. Although most pediatric OACTs are benign, malignant cases have been reported. Surgical resection is the preferred option in most patients.

[Free grafting of internal preputial lamina for penile divided nevus in children].

OBJECTIVE: To evaluate the effect of free grafting of internal preputial lamina in the treatment of penile divided nevus in children. METHODS: This retrospective study included 5 children with penile divided nevus, 4 complicated by redundant prepuce and the other 1 by concealed penis, all treated by free grafting of internal preputial lamina from October 2015 to November 2017. The patients ranged in age from 6 years and 8 months to 10 years and 4 months, averaging 8 years and 6 months. The surgical procedure involved complete excision of the lesions underneath the foreskin with a safety margin of 2 mm, collection of healthy internal preputial lamina for free grafting onto the defected glans, and simultaneously circumcision or concealed penis correction. The removed tissue of the divided nevus was subjected to pathological examination. The patients were followed up for 4－29 (mean 18) months postoperatively. RESULTS: All incisions healed primarily and the free grafts of internal preputial lamina survived well, similar to the glans in color and with a desirable appearance. The patients lost no protopathic sensibility of the glans and experienced no recurrence. Pathological examination confirmed 1 case of intradermal nevus and 4 cases of compound nevus. CONCLUSIONS: Free grafting of internal preputial lamina is an ideal method for the treatment of penile divided nevus in children, with the advantages of easy graft collection, little color difference between the graft and surrounding skin, desirable external appearance, and definite clinical effect.

[Non-thoracoscopic Nuss procedure for correction operation of pectus excavatum ].

OBJECTIVE: To review the experience in correction operation of pectus excavatum with non-thoracoscopic Nuss procedure. METHODS: From September 2005 to August 2007, 108 patients with pectus excavatum were surgically corrected by non-thoracoscopic Nuss procedure. There were 91 male patients and 17 female patients. The age was from 2 years and 10 months old to 25 years old with an average of 7 years and 9 months old. The Haller indexes were from 3.6 to 10.1 before the operation. RESULTS: The operation in all patients had been performed successfully without any severe complications. The average time of operation was 40 minutes. The average bleeding volume during procedure was 10 ml. Uneventful recovery was achieved in all the cases. Excellent outcome was obtained in the follow-up of 2 months to 21 months in 92 patients. CONCLUSIONS: Non-thoracoscopic Nuss procedure for correction of pectus excavatum is safe and effective. It is unnecessary to perform the procedure into thoracic cavity so that there is less trauma and shorter time for the operation.

[Effect of hemin on the lung development and pulmonary arterial structural remodeling in congenital diaphragmatic hernia: experiment with rats].

OBJECTIVE: To assay the effects of prenatal hemin therapy on pulmonary hypoplasia (PH) and pulmonary arterial structural remodeling in congenital diaphragmatic hernia (CDH). METHODS: Six pregnant female SD rats were randomly divided into 3 equal groups: control group, undergoing gastric perfusion of olive oil once on day 9.5 and intraperitoneal injection of normal saline on days 11-14; CDH group, undergoing gastric perfusion of nitrofen 125 mg once on day 9.5 and intraperitoneal injection of normal saline on days 11-14; and hemin group, undergoing gastric perfusion of nitrofen 125 mg once on day 9.5 and intraperitoneal injection of hemin 15 mg x kg(-1) x d(-1) on days 11-14. On the gestational day 21 caesarean section was performed to take out the fetuses to undergo histological examination and image analysis. RESULTS: CDH were detected in 28 of the 44 (63.6%) fetuses from the 2 groups receiving nitrofen. The lungs of all CDH group fetuses were hypoplastic, and the fetuses of the hemin group showed improved lung development. The right lung/body weight ratio and pulmonary alveolar area ratio (PAA%) of the hemin group were (16.6 +/- 1.0) mg/g and (45 +/- 6)% respectively, both significantly higher than those of the CDH group [(14.6 +/- 1.7) mg/g and (28 +/- 6)% respectively, P = 0.03 and P < 0.01]. The alveolar septum area ratio (ASA%) of the hemin group was (44 +/- 6)%, significantly lower than that of the CDH group [(64 +/- 8)%, P < 0. 01]. The media thickness percentages (MT%) of pre-acinar artery (PAPA) and intra-acinar artery (IAPA) of the fetuses of the hemin group were (21.2 +/- 2.2)% and (18.2 +/- 2.1)% respectively, both significantly lower than those of the CDH group [(24.3 +/- 4.0)% and (21.9 +/- 3.9)% respectively, both P < 0.05], which were significantly higher than those of the control group [(20.0 +/- 2.4)% and (17.2 +/- 2.3)% respectively, both P < 0.01]. The component ratio of nonmuscularized artery (NMA) in the IAPA level of the hemin group was (78.2 +/- 3.0)%, significantly higher than that of the CDH group [(72.8 +/- 3.2)%, P = 0.001]. CONCLUSION: PH and pulmonary arterial structural remodeling are present in CDH. Although prenatal administration of hemin cannot prevent the genesis of CDH in rats, it may improve the pulmonary development, inhibit medial hypertrophy, and reduce the percentage of muscularized IAPA.