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Meconium ileus
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Robert J. Vandewalle, Frederick J. Rescorla
Neonates with complicated meconium ileus usually present with abdominal distension at the time of, or shortly after, delivery. In addition, bile-stained fluid is usually noted in the stomach. On physical examination, an abdominal mass may be noted. Neonates with meconium peritonitis occasionally have meconium in the scrotal sac or vagina as a result of passage of this material through a patent processus vaginalis or the fimbriated ends of the Fallopian tubes, respectively. In one unusual report, a meconium pseudocyst appeared as a buttock mass. The early management of these neonates includes IV hydration, antibiotics, and oral gastric tube decompression of the stomach.
Pediatric Oncology
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Stephen Lowis, Rachel Cox, John Moppett, Helen Rees
Most patients present with an abdominal mass. The right lobe is involved more frequently than the left, and both lobes are involved in 20–30% of cases. Multifocal HBL is recognized in around 15% of cases.121,122 Less common symptoms are anorexia, weight loss, and pain and an association with precocious puberty has been reported. Anemia and thrombocytosis are common, and a raised serum alpha fetoprotein (AFP) is found in >90% of cases. Lung metastases are seen in 10–20% at diagnosis.
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
Burkitt’s lymphoma may be divided into three clinical groups: endemic (African), sporadic (nonendemic), and AIDS-associated. Endemic Burkitt’s lymphoma was first described in equatorial Africa. Evidence of Epstein-Barr virus (EBV) infection is present in 95% of patients. The median age of patients with endemic Burkitt’s lymphoma is 7 years, with a boy-to-girl ratio of 3 to 1. The jaw, either the maxilla or mandible, is involved in 60% of patients. Patients also may present with large abdominal masses that involve retroperitoneal structures or gastrointestinal tract, or with involvement of the gonads.
Clinical and Pathological Features of Congenital Hepatic Hemangioma in Children: A Retrospective Analysis
Published in Fetal and Pediatric Pathology, 2023
Rong Wen, Zheng-Zhen Zhou, Wei-Jian Chen
In mesenchymal hamartomas of the liver (MHL), the pathogenesis is not yet clear, but abnormal development of the portal compartment is currently an accepted theory. Serum AFP may also be elevated accordingly [16]. It usually occurs in children before the age of two years. The typical clinical symptoms are abdominal distension or abdominal masses that are painless. Imaging features depend on the changes in interstitial components. Enhanced CT scans may reveal multiple small cystic masses. Under the microscope, various mesenchymal components such as dilated lymphatics, blood vessels, irregular bile ducts, mucosal matrix and collagen fibers can be seen as hyperplasia. Scattered or accumulated liver cells may also be seen. Invagination of blood vessels into the bile ducts, seen with NICH, is absent. MHL does not become malignant or recur after complete resection.
Metastatic malignant struma ovarii: a case report and review of the literature on the management of malignant struma ovarii
Published in Acta Clinica Belgica, 2022
Valerie Taelman, Mirra Boer, Paul Taelman
An 82-year-old woman presented with abdominal pain in 2006. She had a good performance status and a short medical history comprising a regular follow-up of a Barrett’s esophagus. An abdominal CT-scan revealed a large abdominal mass (22 × 14 × 10 cm). The mass consisted of solid tissue with multiple calcifications, adipose tissue and cystic components. Two calcified nodules cranial on the liver surface, a small amount of ascites and infiltration of the omentum majus, suggestive of metastasis, were also visualized. CA-125 was increased to 258 IU/mL (0–24 IU/mL), CEA was within the normal range. She was referred for an explorative laparotomy. A left salpingo-oophorectomy was performed. A biopsy was taken of the capsular liver lesion, and the omental lymph nodes were resected. Cryosection of the liver lesion confirmed metastasis. Considering the age of the patient and the presence of liver metastasis, a conservative policy was preferred; hence, no debulking procedure was performed.
Luteinized thecoma (thecomatosis) with sclerosing peritonitis: a systematic review of the literature of the last 25 years
Published in Expert Review of Anticancer Therapy, 2021
Leonardo Muratori, Elena Trevisi, Marco Donatello Delcuratolo, Paola Sperone, Massimo Di Maio
Thecomas are rare neoplasms that represent between 1 and 2% of all ovarian tumors, according to different statistics [1,2]. These neoplasms arise from granulosa and theca cells and they can produce estrogens or, less often, androgens. Although this neoplasm can present as a very large abdominal mass, in most cases the oncological behavior is benign (less than 5% are malignant). Luteinized thecomas are a histological subtype, characterized by the presence of hormone-secreting cells similar to lutein cells and without crystalloids of Reinke, about 50% of cases with estrogenic expression and about 10% with androgenic expression [3]. Luteinized thecoma (thecomatosis) with sclerosing peritonitis (LTSP) is a very uncommon entity, representing an extremely rare subset of ovarian thecomas, with very few cases described in literature. This condition is characterized by the presence of a luteinized thecoma associated with an abnormal proliferation of fibroblasts in the peritoneum that results in an apparently irreversible fibrotic process, which incarcerates and strangles the abdominal organs. The natural history of the disease leads inexorably to bowel obstruction with fatal consequences. Today, knowledge and the expertise about this condition are very limited, because of its extreme rarity and the consequent small amount of publications and studies. Therefore, we realized a systematic review of literature, with the aim of collecting the data contained in the few articles available and take stock of current knowledge about LTSP and its possible strategies of management.