Gastric teratoma in children including the third reported female case

Gastric teratoma is a very rare benign tumor. Fifty-three cases, only two of which were females, have previously been reported. This report concerns two cases of teratoma arising from stomach. One of them is the third female case described in the literature.     

Hydatid disease presenting as an intrascrotal mass in a child.

A thirteen-year-old boy with the preliminary diagnosis of hydrocele of the cord who underwent surgery is presented. Ultimate diagnosis of hydatid disease was established by surgical exploration and histopathologic examination of the specimen. Although it is a rare occurrence, hydatid disease should be considered as a diagnostic possibility in endemic areas.     

The preventive effect of heparin on stricture formation after caustic esophageal burns

BACKGROUND/PURPOSE: Preventing thrombus formation after caustic esophageal ingestion has been proposed to have beneficial effects. Therefore, an experimental study was carried out to investigate the effects of heparin on the esophagus after caustic burns. METHODS: Caustic esophageal burns were produced in rats by irrigation with 50% NaOH as described by Liu. Rats were divided into four groups as follows: group A, animals with esophageal burns, received placebo and underwent autopsy 48 hours after caustic injury; group B, animals with esophageal burns, received subcutaneous heparin treatment and underwent autopsy 48 hours after caustic injury; group C, animals with esophageal burns, received placebo and underwent autopsy 28 days after caustic injury; group D, animals with esophageal burns, received subcutaneous heparin treatment for 7 days and underwent autopsy 28 days after caustic injury. Histopathologic evaluation was performed in all groups, and collagen content of esophageal sections was analyzed by determination of hydroxyproline levels. RESULTS: Submucosal vascular thrombosis was encountered in all group A animals but the submucosal venules and arterioles were patent in most of group B animals. Esophageal strictures did not develop in any of group D animals, although varying degree of esophageal stenoses were encountered in all animals of group C. The circumferences of the burned segment have been narrowed to 3+/-1 mm in group C rats. There was obvious collagen deposition in submucosa, and epithelial regeneration was not complete in group C rats. Submucosa and epithelial integrity seemed normal in group D animals. Hydroxyproline contents in group D were significantly lower compared with group C (P < .05). CONCLUSIONS: Heparin has ameliorating effects on stricture formation after caustic esophageal burn. Those effects may occur through possible anticoagulant, antithrombotic, and endothelial protective effects, and modifying effects of heparin on wound healing.     

Evaluation of thrombotic children with malignancy

The purpose of this study was to evaluate inherited and acquired prothrombotic risk factors among children with malignancies who have thrombosis and emphasize the importance of inherited prothrombotic risk factors. Thirty-seven consecutive children with thrombosis and malignancy were included in this study. The patients were evaluated separately for time of development of thrombosis, insertion of a central venous line (CVL), history of l-asparaginase usage, and recent infections. Prothrombotic risk factors such as factor V G1691A and prothrombin G20210A mutation, protein C, protein S, antithrombin III deficiencies, factor VIII and lipoprotein(a) elevation, and antiphospholipid antibodies were analyzed for all patients. Of 387 children with thrombosis, 37 (9.5%) had a malignancy. Thrombosis was detected in 9 patients at the time of diagnosis, during maintenance therapy in 25 patients, and after the discontinuation of treatment in 3 patients. One or two additional prothrombotic risk factors other than l-asparaginase therapy and insertion of central venous lines were present in 20 of these patients (54%). It was found that eight patients had the factor V G1691A mutation in the heterozygote state. One of them had the factor V G1691A mutation associated with a history of infection and one patient had the factor V G1691A mutation associated with factor VIII elevation. One had the the prothrombin G20210A mutation in the heterozygote state, four had lipoprotein(a) elevation, two had factor VIII elevation, one had a decreased protein S level, one had a decreased protein C level, one had antiphospholipid positivity, and two had histories of infection. Malignancy is an important risk factor for the development of childhood thrombosis. However, the risk of thrombosis increases when accompanied by additional prothrombotic risk factors. For this reason, especially children with malignancy and at high risk for the development of thrombosis, such as those who have received l-asparaginase or a replaced CVL during their therapy, might be screened for additional prothrombotic risk factors and appropriate measures might be taken to prevent the development of thrombosis.     

Giant lymphadenopathy infiltrated by gaucher cells mimicking lymphoma

Gaucher disease (GD) is a lysosomal storage disease characterized by deficiency of β‐glucocerebrosidase, which results in accumulation of glucocerebroside in reticuloendothelial system, bone marrow infiltration, progressive hepatosplenomegaly, and skeletal complications. Herein we report a 5‐year‐old female with GD receiving enzyme replacement therapy who had giant mesenteric lymphadenopathies. Pediatr Blood Cancer 2009;52:870–871. © 2009 Wiley‐Liss, Inc.     

A reevaluation of the structures accepted to represent the postnatal gubernaculum

INTRODUCTION: The structure called the residual gubernaculum (G) has been evaluated to define its origin. MATERIALS AND METHODS: Girls operated for inguinal hernia (n = 7) and boys operated for undescended testis (n = 12), inguinal hernia (n = 7), hydrocele of the cord (n = 1), and ectopic (n = 1) and entrapped (n = 1) testes were evaluated. Attachments of the structures coursing distally from the sacs were identified, and they were removed en block. Three samples, first from the distal part of the sac with the beginning of the fibrous structure, second from the midportion, and third from the most distal part, were prepared and stained with hematoxylin-eosin, trichrome and elastic van Gieson. Histologic structures within the samples were determined and compared with Mann-Whitney U test. RESULTS AND CONCLUSIONS: Structures called the residual G differed from the walls of accompanying sacs by only the absence of mesothelial lining. Therefore, the structure called the residual G in boys with an undescended testis and the round ligament in girls actually represent the obliterated processus vaginalis.     

Pheochromocytoma in children

BACKGROUND/PURPOSE: Etiopathogenesis, management, and outcome of pediatric pheochromocytoma (PHEO) still is obscure because of limited number of cases. Therefore, a retrospective clinical study was performed to present an updated picture of the entire spectrum of pediatric PHEO based on the authors' 30 years' experience consisting of one of the largest noncollected series treated in a single medical center. METHODS: Records of patients treated for PHEO in the authors' unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical and family history, clinical characteristics, diagnostic methods, treatment, pathologic findings, and outcome. RESULTS: Sixteen children with a mean age of 10.7 +/- 2.9 years consisting of 12 boys and 4 girls were treated for PHEO. Most of the tumors were right sided (n = 6) and bilateral (n = 6). Sporadic cases of PHEO accounted for 14 patients (88%), whereas 2 children had von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia type 2b (MEN2b). Hypertension was the most common symptom followed by headache and sweating. The diagnosis of PHEO was made by laboratory and radiologic studies. Preoperative medical therapy was done in all patients. Laparotomy confirmed that 11 patients had localized, 4 patients had regional, and 1 patient had metastatic disease. The localized tumors were excised totally by bilateral (n = 4) and unilateral (n = 6) adrenalectomy. Surgical procedures performed for regional disease were total excision (n = 2), incisional biopsy (n = 1) and partial excision (n = 1). Incisional biopsy could be taken only from a patient with metastatic disease at presentation. Two patients with localized disease and 2 patients with regional disease had benign recurrences in right (n = 2) and left (n = 2) adrenal glands within 3 to 7 years after operation. Total excision of the recurrent tumors was done in all patients. Pathologic examination found apparently malignant features in 3 patients who presented with regional (n = 2) or metastatic (n = 1) disease and underwent incisional biopsy (n = 2) or partial excision (n = 1). Pathologic features suggestive of malignancy were noted in 4 patients presenting with regional (n = 2) and localized disease (n = 2). Apparently benign pathologic features were noted in the remaining 9 patients. There was not any operative mortality in our series. Adjuvant chemotherapy was commenced postoperatively in all patients with malignant and suggestive of malignant pathologic features. During the long-term follow-up for 16 years, 3 patients died (19%). One patient with VHL disease died of astrositoma 5 years after her recurrent PHEO was excised. Of the 3 patients with malignant disease, 2 patients in whom only incisional biopsies were done had distant metastases and died of disease within 2 years. Another patient with malignancy who had MEN2b was lost to follow-up. CONCLUSIONS: Early diagnosis and total excision are the most important aspects of accurate treatment for childhood PHEO. Pre- intra- and postoperative medical management is as important as the surgical procedure. Our surgical treatment policy is mainly minimizing the risk of recurrence while preserving adequately functioning adrenal medullar tissue. Incomplete excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy. Because of the steadily increasing incidence of precancerous genetic syndromes related to adrenal glands and poor prognosis of advanced-stage PHEO, childhood cases of hypertensive disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults.