A Case of Precocious Pseudopuberty Associated With Ovarian Follicular Cyst

A case of precocious pseudopuberty associated with a follicular cyst in the ovary is described. A five-year-and-month-old Japanese girl had breast enlargement, areolar pigmentation and intermittent genital bleeding. On laparotomy, bilateral ovarian cysts were found. The left cyst (4.0 times 4.0 times 3.5 cm) was far larger than the right (1.5 times 1.3 times 1.3). The fluid in the left cyst contained a high concentration of estrogens. Therefore, the main cause of the precocious puberty was thought to be the left cyst. It was clearly detected by ultrasonography and computed tomography only when the signs of precocious puberty were remarkable and the serum estrogen level was increased; however, the right one could not be detected throughout the observation. We consider that in the case of intermittent puberty a functioning ovarian cyst can be identified more easily when the signs of precocious puberty are remarkable and the serum estrogen level is increased.     

Neonatal Ovarian Torsion: Report of Three Cases and Review of the Literature

Ovarian cysts are common incidental findings in term infants and, if unusually large, may result in dystocia, torsion, or rupture. Torsion and infarction of a normal ovary tend to occur in older childhood. During a 4-month period, 3 cases of neonatal ovarian torsion were observed after antenatal ultrasonography had detected fetal pelvico-abdominal cystic lesions. The three infants were explored between 4 and 16 days of age. Ovarian torsion was right-sided in all 3, and 1 ovary had been autoamputated. The resected specimens were nontense, thin-walled cysts, filled with hemorrhagic fluid, that measured between 4.5 and 8 cm in diameter. Microscopically, focal calcification and widespread necrosis precluded recognition of underlying histologic landmarks. Neonatal ovarian cysts or cystic ovaries greater than 4 cm in diameter should be excised, even if asymptomatic, because they are prone to, or have undergone, torsion.     

Ovarian tissue cryostorage and grafting: an option to preserve fertility in pediatric patients with malignancies

Fertility preservation in childhood cancer has become an important area of investigation due to increasing survival rates after cancer therapy. For these patients with an increased risk of infertility and premature ovarian failure, cryopreservation of ovarian tissue is a promising tool to preserve at least part of the reproductive potential. In recent years significant improvements have been achieved in this area, and 2 live births after autografting of frozen-thawed ovarian tissue have been reported. However, further research is needed to assess the clinical effectiveness of ovarian cryopreservation, to optimize the technique, and to limit the risk of reintroducing cancer cells in the patient with the graft.     

小儿急性出血坏死性胰腺炎的治疗

６例小儿急性出血坏死性胰腺炎，经手术治疗，治愈５例，死亡１例。对其降低死亡率、严格控制禁食时间、营养支持、腹腔引流及灌洗、“三造瘘”等问题加以讨论。     

CONGENITAL CYSTIC ADENOMATOID MALFORMATION OF THE LUNG: A Report of Two Cases

ABSTRACT. Michalsen, H. (Department of Paediatrics, Ullevål Hospital, Oslo, Narway). Congenital cystic adenomatdd malformation of the lung. Acta Paediatr Scand, 63: 793, 1974.—The clinical picture of the congenital cystic adenomatoid malformation of the lung is reviewed. Two patients suffering from this entity are presented, the first patient was a newborn girl with acute respiratory distress shortly after birth, and the seeond patient was 12 months old and had a history of repeated pulmonary infections. The importance of, and difficulty in, differentiation between diaphragmatic hernia and the congenital cysts of this type in newborn infants is emphasized.     

Prune Belly Syndrome: Report of Twelve Autopsy Cases and Possible Pathogenesis

Abstract Twelve autopsy cases (9 males, 3 females) of the prune belly syndrome are presented. Principal anomalies of this syndrome are a prune-like abdominal feature and a giant bladder. Urethral atresia was observed in most but two cases. Imperforate anus and rectovesical fistula were observed in 8 and 7 cases respectively. Associated anomalies were those which may or may not be embryologically related to the principal anomalies.
As regards the pathogenesis, the authors propose that a primary defect may occur during any developmental stage of the somitic mesoderm, genital tubercle and urethra. In conclusion, the prune belly syndrome may be of spectrum anomalies depending on the stage specificity mainly in the abdominal wall and genito-urinary organs.     

Dermoid Cysts of the Tongue: Report of Five Cases and Review of the Literature

Dermoid cysts of the tongue are uncommon. To date, there have been nine reported cases in the English language literature. In this article, we describe five cases accessioned at our institution over a 12-year period, two of which have previously been reported. The prevalence of dermoid cysts at our institution over this period was quite low. Of 324,042 surgical cases, 0.24% (765 cases) were dermoid cysts. Of these, five were from the tongue, representing only 0.7% of the dermoid cysts accessioned and 0.0015% of the total surgical specimens. The literature is reviewed and the possible origin of these lesions is discussed.     

脊髓纵裂的CT诊断价值（附17例报告）

１９７８～１９９２年共收治脊髓纵裂３９例，其中１７例术前行ＣＴ检查确诊。ＣＴ特征：椎管中央有纵行骨嵴将脊髓或马尾分隔，多数骨嵴两侧蛛网膜下间隙及脊髓不均称；该部椎管扩大；椎体及附件结构紊乱。ＣＴ不仅能准确诊断损害的平面，而且能显示椎管的自然状态和骨嵴的种类及轮廓。     

Mesenchymal Hamartoma of the Liver: Report of 30 Cases and Review of the literature

Thirty cases of mesenchymal hamartoma of the liver are presented and compared with 42 previously published cases. The patients, 69% male, range in age from newborn to 10 years (average age 15 months, median age 10 months). Except for occasional cases in which the lesion is an incidental finding at autopsy, most present with progressive abdominal enlargement over a period of days to months. Physical and radiologic examinations are nonspecific except for the demonstration of a mass within or attached to the liver. The masses are large, averaging 1311 g, and frequently contain cysts filled with clear fluid or gelatinous material. Histologic examination shows a mixture of loose mesenchymal tissue, bile ducts, connective tissue, and hepatocytes along with cysts formed either from degenerative areas of mesenchyme or from dilated bile ducts and lympathics. Treatment is partial or total excision of the lesion. Mortality (7-17%) is related to intraoperative or postoperative complications. Recurrence or malignant transformation has not been noted.     

Ovarian cancer in children and adolescents: A review of 15 cases

Fifteen patients, admitted between 1961 and 1975, from 8 to 19 years of age, with malignant ovarian tumors were reviewed. The histology was: dysgerminoma in 5, common “epithelial tumor” in 5, immature teratoma in 4, granulosa cell tumor in one. Of the 15 patients, 5 are alive and well, 4 of these had dysgerminoma, 1 had a papillary cystadenocarcinoma. Of 3 patients with dysgerminoma in Stage III, 2 are among the survivors. Dysgerminoma, in contrast with other types of germ cell tumors, may be curable even in advanced stage.     

Hereditary pancreatitis: Report of a family from Turkey

Chronic pancreatitis is a rare disease in children and is usually secondary to underlying diseases such as hereditary pancreatitis, cystic fibrosis, hyperlipidemia, prolonged malnutrition, gallstones or anomalies of the biliary-pancreatic duct system. Hereditary pancreatitis is a common cause of chronic pancreatitis in children but is often unrecognized until months or years later. We report here a family with hereditary pancreatitis in which four members are affected.     

325例小儿胚芽细胞瘤的诊断和治疗体会

１９６１～１９９４年共收治３２５例小儿胚芽细胞瘤。其中良好２８０例（８６．２％），恶性和潜在恶性４５例（１３．８％）。肿瘤术后复发、恶变和转移１３例，死亡２０例。笔者强调血清甲胎蛋白在诊断和治疗小儿胚芽细胞瘤的重要性，同时也强调早期诊断（包括产前Ｂ超诊断），早期手术，一旦肿瘤恶变、转移，预后极差。笔者推荐近来对恶性胚芽细胞瘤采用ＣＣＧ－８６１方案治疗，有明显疗效。     

Pleomorphic Xanthoastrocytoma: A Report of Two Cases

We report two cases of a recently isolated variety of astrocytoma, pleomorphic xanthoastrocytoma, affecting the cerebral cortex and meninges of children and young adults.

The first tumor was localized in the left frontal lobe of a 9-year-old girl. The second appeared in the right parietal lobe of a 14-year-old girl.

Both tumors displayed a pleomorphic cellular proliferation, with spindle-shaped and bizarre multinucleated giant cells, some positive for neutral lipids, with a prominent reticulin network and areas highly suggestive of malignant fibrohistio-cytomas (fibroxanthosarcomas).

The glial fibrillary acidic protein was demonstrated by the immunoperoxidase method in the cytoplasm of the giant and the spindle cells. Ultrastructural studies confirmed the glial nature of the tumor.

This type of tumor seems to carry a much better prognosis than other tumors from which it has to be separated, e.g., glioblastoma and meningeal sarcomas.     

幽门前瓣膜症：附4例报告

阐述幽门前瓣膜症的病因、分类及手术方式的选择。１９８８～１９９４年收治４例均为有孔瓣膜型，症状于出生１个月后出现。Ｂ超检查示幽门完全梗阻１例，不完全梗阻３例，均未见幽门肿块。ＧＩ检查常先行胃肠减压等治疗后继用少量造影剂检查，完全和不完全梗阻各１例，无征象２例。瓣膜位于胃窦部１例行单纯瓣膜切除术；幽门部２例行瓣膜切除加幽门成形术；合并胃溃疡１例行胃部分切除毕Ⅰ式吻合术。随访１～７年均无异常。本病罕见，症状出现的迟早取决于梗阻的严重程度。ＧＩ检查中注意胃肠减压等治疗对造影的影响。Ｂ超和内窥镜检查对确立诊断有重要价值。应根据瓣膜的解剖部位及合并症选择手术方式。     

SECONDARY LEIOMYOSARCOMAS: A Report of 4 Cases

Survivors of childhood cancer risk developing second malignant neoplasms (SMN). A small proportion of SMNs is represented by soft tissue sarcoma (STS), including leiomyosarcoma. The files of the STS Italian Cooperative Group (ICG) were reviewed and 4 patients with secondary leiomyosarcoma were identified, only two of them still alive. In 3 cases, the leiomyosarcoma occurred in the radiation field. The authors found no clear differences between primary and secondary leiomyosarcomas, but leiomyosarcoma proved to be the most frequent SMN registered by the ICG, confirming the need for further studies to identify any distinctive features or syndromes.     

Pseudo-Meconium Ileus Due to Cytomegalovirus Infection: A Report of Three Cases

We observed three cases of antenatal ileus associated with cytomegalovirus (CMV) infection of the fetus and placenta. Two were detected antenatally because of increased echogenicity of the lower abdomen. In the first fetus, the ileus was associated with abnormalities of amniotic fluid enzymes but it was transient and not present at autopsy and the CMV infection was mild, without inflammatory infiltration or necrosis. In the two others, the ileus persisted and CMV-associated lesions were severe. In all three cases the virus was demonstrable in ganglion cells or within myenteric and submucosal plexuses all along the small and large intestine; ileus was imputed to CMV, which caused a paralytic ileus, and in one fetus meconium ileus was also present. A transient episode of ileus does not indicate that the fetus is free of disease and a wide range of causes must be considered, including CMV infection as well as the more usual causes such as cystic fibrosis (CF) and Hirschsprung's disease.     

Zellweger Syndrome: A Histochemical Diagnosis of Two Cases

Until 17 years ago the diagnosis of the cerebrohepatorenal Zellweger syndrome (ZS) rested largely on clinical grounds confirmed by pathologic findings at autopsy. The observation that peroxisomes are not detectable morphologically or histochemically in liver or kidney of patients with this syndrome gave histopathologists the opportunity to make the diagnosis of this complex syndrome at biopsy. Catalase reaction of the peroxisomes can be used as a rapid and accurate method to differentiate between ZS and other clinical conditions in which the peroxisomes are present in normal or reduced number. We describe two patients in whom the diagnosis of ZS was made by the absence of histochemical staining for catalase in a liver biopsy. The findings were subsequently confirmed using standard biochemical tests.