Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infancy and early childhood. Lipoblastoma is present in 2 forms which are pathologically identical: circumscribed and diffuse. It is typically located in the extremities, and less frequently in head-neck region, trunk, and various organs. Lipoblastoma is a tumor with good prognosis with no reported metastases, despite its potential for local invasion and rapid growth. Our patient was a 3-month-old boy who was brought to our clinic for rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the lipomatous mass was removed by local resection. In addition to the patient's age, histologic and cytogenetic analyses assisted the diagnosis of diffuse lipoblastoma. In the postoperative period, the thorax wall was unaffected, and after 6 years of follow-up no recurrence was observed. In the English literature, 8 cases of thoracic wall lipoblastoma have been previously reported, and only 3 of diffuse form. Here, we report, at our knowledge, the fourth case of thoracic diffuse lipoblastoma, in which cytogenetic analysis showed a previously undescribed karyotype aberration involving chromosomes 8, 13, and 16.
Both incision and needle-core biopsying methods provided sub-optimal to optimal sampling adequacy rates in children affected by peripheral neuroblastic tumors. However, the former method was associated with a higher risk of both intraoperative and postoperative complications compared with the latter.
Background: Hypertension (HT) is rarely reported in patients affected by Neuroblastoma (NB), and management guidelines are lacking. Clinical features and perioperative medical treatment in such patients were reviewed to 1) ascertain whether a shared treatment strategy exists among centers and 2) if possible, propose some recommendations for the perioperative management of HT in NB patients. Methods: A retrospective multicenter survey was conducted on patients affected by NB who presented HT symptoms. Results: From 2006 to 2014, 1126 children were registered in the Italian Registry of Neuroblastoma (RINB). Of these, 21 with HT (1.8%) were included in our analysis. Pre-and intraoperative HT management was somewhat dissimilar among the participating centers, apart from a certain consistency in the intraoperative use of the alpha-1 blocker urapidil. Six of the 21 patients (28%) needed persistent antihypertensive treatment at a median follow-up of 36 months (range 4-96 months) despite tumor removal. Involvement of the renal pedicle was the only risk factor constantly associated to HT persistency following surgery. A correlation between the presence of HT and the secretion of specific catecholamines and/or compression of the renal vascular pedicle could not be demonstrated.
Ovarian neoplasms arising from the surface epithelium are rare in the pediatric population; their knowledge is therefore limited and the appropriate management is poorly defined. We describe our experience and suggest our surgical approach to adolescents affected by voluminous ovarian masses. Two 15-year-old adolescents were admitted to our institution in 2017 for multilobulated, fluid-filled masses measuring over 30 cm arising from the ovaries. The cystic component was drained intraoperatively with a spillage-free technique, consisting in the application of a sterile autoadhesive transparent drape on the cyst and the insertion of a 12 Ch pleural drain, secured with a purse-string suture. Unilateral salpingo-oophorectomy was then carried out. Histology revealed mucinous cystadenoma in both patients. Surgical treatment of ovarian masses should aim at both radically excising the tumor and preserving the fertility of the patients. Decompression with spillage-free techniques can be useful to achieve radical therapy with limited manipulation of tissues.
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