Giant Multilocular Prostatic Cystadenoma in a 14-Year-Old Male: A Case Report of a Pediatric Pelvic Mass.

Giant multilocular prostatic cystadenoma (GMC) is an extremely rare, benign tumor seen in both adult and pediatric males. The neoplasm originates from prostatic tissue and is typically found within the rectovesical pouch, varying in both size and morphology. Microscopically, GMC contains both glandular and cystic prostatic tissue lined by cuboidal and columnar epithelium. Symptoms often arise once the pelvic mass begins to obstruct the surrounding structures and organs, although invasion into surrounding tissue is unlikely. Common symptoms include abdominal pain, urinary retention, and dysuria. The standard treatment for GMC is surgical removal of the mass with good outcomes and only 1 known case of recurrence. Here we present the case of a 14-year-old male with GMC-the youngest patient reported to date-who presented with abdominal pain, difficulty voiding, and hydroureteronephrosis.

Visual interpretation, not SUV ratios, is the ideal method to interpret 18F-DOPA PET scans to aid in the cure of patients with focal congenital hyperinsulinism.

INTRODUCTION: Congenital hyperinsulinism is characterized by abnormal regulation of insulin secretion from the pancreas causing profound hypoketotic hypoglycemia and is the leading cause of persistent hypoglycemia in infants and children. The main objective of this study is to highlight the different mechanisms to interpret the 18F-DOPA PET scans and how this can influence outcomes. MATERIALS AND METHODS: After 18F-Fluoro-L-DOPA was injected intravenously into 50 subjects' arm at a dose of 2.96-5.92 MBq/kg, three to four single-bed position PET scans were acquired at 20, 30, 40 and 50-minute post injection. The radiologist interpreted the scans for focal and diffuse hyperinsulinism using a visual interpretation method, as well as determining the Standard Uptake Value ratios with varying cut-offs. RESULTS: Visual interpretation had the combination of the best sensitivity and positive prediction values. CONCLUSIONS: In patients with focal disease, SUV ratios are not as accurate in identifying the focal lesion as visual inspection, and cases of focal disease may be missed by those relying on SUV ratios, thereby denying the patients a chance of cure. We recommend treating patients with diazoxide-resistant hyperinsulinism in centers with dedicated multidisciplinary team comprising of at least a pediatric endocrinologist with a special interest in hyperinsulinism, a radiologist experienced in interpretation of 18F-Fluoro-L-DOPA PET/CT scans, a histopathologist with experience in frozen section analysis of the pancreas and a pancreatic surgeon experienced in partial pancreatectomies in patients with hyperinsulinism.

Pancreatic uptake and radiation dosimetry of 6-[18F]fluoro-L-DOPA from PET imaging studies in infants with congenital hyperinsulinism.

METHODS: After injecting 25.6 ± 8.8 MBq (0.7 ± 0.2 mCi) of 18F-Fluoro-L-DOPA intravenously, three static PET scans were acquired at 20, 30, and 40 min post injection in 3-D mode on 10 patients (6 male, 4 female) with congenital hyperinsulinism. Regions of interest (ROIs) were drawn over several organs visible in the reconstructed PET/CT images and time activity curves (TACs) were generated. Residence times were calculated using the TAC data. The radiation absorbed dose for the whole body was calculated by entering the residence times in the OLINDA/EXM 1.0 software. RESULTS: The mean residence times for the 18F-Fluoro-L-DOPA in the liver, lungs, kidneys, muscles, and pancreas were 11.54 ± 2.84, 1.25 ± 0.38, 4.65 ± 0.97, 17.13 ± 2.62, and 0.89 ± 0.34 min, respectively. The mean effective dose equivalent for 18F-Fluoro-L-DOPA was 0.40 ± 0.04 mSv/MBq. The CT scan used for attenuation correction delivered an additional radiation dose of 5.7 mSv. The organs receiving the highest radiation absorbed dose from 18F-Fluoro-L-DOPA were the urinary bladder wall (2.76 ± 0.95 mGy/MBq), pancreas (0.87 ± 0.30 mGy/MBq), liver (0.34 ± 0.07 mGy/MBq), and kidneys (0.61 ± 0.11 mGy/MBq). The renal system was the primary route for the radioactivity clearance and excretion. CONCLUSIONS: The estimated radiation dose burden from 18F-Fluoro-L-DOPA is relatively modest to newborns.

Clinical management of infantile fibrosarcoma: a retrospective single-institution review.

BACKGROUND: Infantile fibrosarcoma (IFS) is an uncommon soft-tissue sarcoma. Here we review our experience treating this tumor. PATIENTS AND METHODS: We retrospectively reviewed records of patients with IFS treated at St. Jude Children's Research Hospital between 1980 and 2009. RESULTS: We identified 15 patients, 8 girls and 7 boys; 13 white and 2 black. Median age at diagnosis was 3 months. Primary sites included the leg (n = 3), chest wall (n = 2), foot (n = 2), and one each in the tongue, occipital region, axilla, parascapular region, arm, forearm, retroperitoneum, and thigh. All patients underwent resection; 11 upfront surgery, and 4 delayed. Complications included loss of the posterior tibial nerve and artery, axillary vein, biceps, pectoralis major, gallbladder, and transverse/sigmoid sinus. Eight received chemotherapy and three radiotherapy. Seven experienced local recurrence and three lung metastasis. Median follow-up was 65 months. At the time of the review, 12 patients were alive and 3 had died. All deaths were in patients older than 1 year at diagnosis with an axial primary site. CONCLUSIONS: Non-mutilating surgery should be the primary treatment for IFS. Neoadjuvant chemotherapy is indicated when upfront resection is unfeasible. Patients with positive surgical margins should receive adjuvant chemotherapy. Radiotherapy is indicated for axial primary sites where complete resection is impossible.

Thoracoscopic resection of computed tomography-localized lung nodules in children.

PURPOSE: Detection and treatment of small lung nodules are important in managing pediatric cancer. We studied the effectiveness of preoperative localization of pulmonary nodules by CT-guided needle hook wire placement followed by thoracoscopic resection in children with cancer. METHODS: We reviewed records of patients who underwent thoracoscopic resection of lung nodules localized preoperatively with CT-guided needle and hook wire placement at our hospital between March 1999 and April 2010 for nodule characteristics and outcomes of procedure. RESULTS: Thirty-seven patients (median age, 14years) with osteosarcoma or other cancers underwent thoracoscopic resection of needle-localized lung nodules. Lesion (median nodule size, 4mm) location was left lung (n=11), right lung (n=19), and bilateral (n=7). The procedure was successful in 36 (97.3%) patients. Five patients had a pneumothorax after localization but none required chest tube placement before thoracoscopy. All patients underwent thoracoscopy, but 4 required conversion to open thoracotomy. During thoracoscopic inspection, the hook wire slipped out of the lesion in 6 patients, of whom 1 needed thoracotomy to locate nodule. Lesions (malignant in 13 patients) were removed in all patients. Five patients with benign lesions had recurrent malignant lung nodules. CONCLUSIONS: Thoracoscopic resection of preoperatively localized small lung nodules is a safe and effective procedure in children.

Clinical management of Ewing sarcoma of the bones of the hands and feet: a retrospective single-institution review.

BACKGROUND/PURPOSE: Bones of the hands and feet are uncommon sites for Ewing sarcoma. In this study, we reviewed our experience in the management of these tumors. METHODS: We retrospectively reviewed clinical presentation, management, and outcome of patients with Ewing sarcoma of the bones of hands and feet treated at our institution (1981-2006). RESULTS: The cohort included 6 males and 3 females (8 white, 1 African American; median age at diagnosis, 15 years). Primary tumor site was the hand in 6 and the foot in 3 patients. Three patients had distant metastatic disease at diagnosis (lung [n = 2]; ipsilateral axillary lymph node[(n = 1]). All patients had painful swelling at the primary site, and 2 (22%) had pathological fracture. All patients received chemotherapy and local control measures (surgery [n = 6], radiation [n = 2], surgery and radiation [(n = 1]). Three patients received radiotherapy for distant metastases. Three patients had systemic recurrence (lungs [n = 2], lung and brain [n = 1]); none had local tumor recurrence. Median follow-up was 5 years. Five patients (55.6%) are alive at last follow-up. CONCLUSIONS: Chemotherapy and surgical excision of primary tumor are the mainstays of treatment. Radiotherapy is recommended for local control of lesions in the hand for patients declining excisional therapy.

Omental infarction: preoperative diagnosis and laparoscopic management in children.

BACKGROUND: Omental infarction (OI) is an unusual, poorly characterized cause of abdominal pain in children and is often mistaken for appendicitis preoperatively. We present our experience with this disease process over a 5-year period to identify preoperative factors to aid in timely diagnosis and treatment. METHODS: We retrospectively reviewed the medical records of all children that had OI and underwent laparoscopic omentectomy from November 2004 to June 2009. RESULTS: Ten patients with the diagnosis of OI were identified. OI occurred in 9 boys and 1 girl, with a median age at presentation of 8.5 years (range, 7-11). Median body mass index at presentation was 23.7 (range, 17-29), with 1 child categorized as healthy weight for age, 1 child as overweight for age, and 5 children as obese for age, based on Centers for Disease Control and Prevention criteria. All patients complained of right-sided abdominal pain; 4 patients complained of predominantly right-upper quadrant (RUQ) pain, 3 patients of right-lower quadrant (RLQ) pain, and 3 of combined RUQ/RLQ pain. On examination, 6 patients had RUQ tenderness and 4 patients had RLQ tenderness. The median duration of symptoms prior to seeking medical attention was 3 days (range, 2-7). All patients underwent computed tomography and the preoperative diagnosis of OI was established in 9 of 10 cases. Operative time was 48 ± 14 minutes. All patients underwent resection of the infarcted omentum; 2 patients underwent concurrent appendectomy. Median length of stay was 2 days (range, 2-4). CONCLUSIONS: OI occurs predominantly, but not exclusively, in obese preadolescent males. OI can be reliably distinguished from appendicitis on preoperative history, physical examination, laboratory analysis, and imaging. Laparoscopic omentectomy results in prompt resolution of symptoms and discharge.

On-pump versus off-pump coronary artery bypass surgery: a comparison of two consecutive series.

BACKGROUND DATA: On-pump and off-pump techniques are both widely used approaches to coronary artery bypass surgery. Yet, statistically valid comparisons of the results between the two groups have been limited, in part, by patient selection bias. METHODS: Two hundred sixty-nine consecutive patients undergoing off-pump coronary artery bypass and 379 consecutive patients undergoing on-pump bypass were compared in a retrospective chart review. The two groups were compared for preoperative characteristics as well as operative outcomes. To avoid selection bias, no on-pump coronary artery bypass surgery was performed during the off-pump coronary artery bypass series, and no patients were done off-pump during the coronary artery bypass series. RESULTS: There was no statistical difference in the groups pre-operatively except that there were slightly more patients with three-vessel disease in the on-pump group and more patients with single vessel disease in the off-pump group. Significant benefits were found in the off-pump group in that they required fewer re-operations for bleeding (0.8% vs. 5.7%, p-value < 0.002), and they left the hospital with higher hematocrits (32.1% vs. 30.8%, p-value < 0.001). Patients who had off-pump coronary artery bypass also had fewer sternal dehiscences (0% vs. 1.8%, p-value < 0.027). More patients receiving off-pump bypass demonstrated the need for prolonged mechanical ventilation (8.2% vs. 2.5%, p-value < 0.027), and they also had significantly fewer grafts (3 vs. 3.2, p-value < 0.005). There was no statistically significant difference among the other outcomes investigated. CONCLUSIONS: While there were no significant differences in some of the outcomes studied, others showed significant advantages in favor of off-pump surgery. Substantial advantages in off-pump coronary artery bypass were seen in bleeding reduction, improved sternal healing, and higher discharge hematocrits despite fewer transfusions. These advantages and others reported in specific high-risk patient groups, combined with documented cost reductions, warrant continued use of off-pump techniques. Off-pump coronary artery bypass is a safe, proven method with significant advantages over on-pump methods and, when appropriate, should be offered to patients undergoing coronary bypass surgery.