Silvia Strambi

Treatment of Ovarian Lesions in Children and Adolescents: A Retrospective Study of 130 Cases

In this study, we analyze the management of ovarian masses in a total of 130 children surgically treated for 137 ovarian lesions (7 bilateral). The most frequent symptoms were chronic (52.3%) and acute (25.4%) abdominal pain. Histological examination revealed 64 (46.7%) functional lesions, 59 (43.1%) benign neoplasms, 5 (3.7%) malignant ones, and 7 (6.6%) torsed normal ovaries. Ovarian torsion occurred in 36 cases (26.27%). A conservative treatment was performed in 81 (59.1%) girls: 61 (75.3%) treated in nonemergency and 20 (24.7%) in emergency surgery; laparoscopic approach in 35 cases (43.2%); and open surgery in 46 (56.8%). The remaining 56 (40.9%) ovarian masses underwent nonconservative surgery: 40 cases (71.4%) nonemergency and 16 (28.6%) emergency; laparoscopy in 20 patients (35.7%); and open surgery in 36 (64.3%). Fertility preservation should be a goal in the surgical treatment. The management of ovarian torsion should include adnexal detorsion and recovery of the ovarian tissue. In case of benign neoplasms, laparoscopic tumorectomy should be the gold standard; in early stage malignant tumors, fertility-sparing surgery with accurate staging is preferred.

Adnexal torsion in adolescents: update and review of the literature.

Purpose of review Adnexal torsion is a surgical emergency, which requires immediate recognition and prompt treatment. This article aims to systematically illustrate the most relevant approach to follow in cases of suspected adnexal torsion in adolescents. We based our evaluation of the role of clinical features, laboratory tests, and imaging on recent literature, till we consider the most appropriate surgical treatment. Recent findings The literature describes the conservative treatment for adnexal torsion, consisting of detorsion, as the best surgical approach to guarantee the future reproductive capacity of patients. Recent experimental studies have focused on the consequences of detorsion, which may cause ischemia-reperfusion damage and have speculated about the role of different drugs for its avoidance. Summary A systematic review of physiopathology, clinical picture, and imaging of adnexal torsion is certainly useful to identify as soon as possible this emergency condition. An early identification and an adequate treatment are indispensable in order to achieve a proper outcome for the patient. The choice of the appropriate surgical approach can be challenging and should be made on the basis of an accurate evaluation of the ovarian lesion. Therefore, it would be useful to perform an intraoperative frozen section analysis in selected cases.

Spigelian hernia in a 14-year-old girl: a case report and review of the literature.

Spigelian hernia (SH) is a surgical rarity in children, which occurs through slit-like defects in the anterior abdominal wall adjacent to the semilunar line, the convexity lateral line which joins the nine ribs to the pubic tubercle and signs the limit between the muscular and aponeurotic portion of transversus abdominis muscle. As there are no specific symptoms and signs, the diagnosis is difficult, especially in children. We report a case of SH that comes to our observation: a 14-year-old girl presented recurrent abdominal pain associated to intermittent palpable mass in the paraumbilical region. Starting from our case report, we review the literature of pediatric SH from 2000 to 2013 and we describe the anatomy, etiology, clinical presentation, instrumental diagnosis, and surgical technique of pediatric SH.

An Unusual Evolution of Krukenberg Tumour: A Case Report

Krukenberg tumours are rare metastatic tumours of the ovaries characterized by the presence of mucin-producing neoplastic Signet Ring Cell Carcinoma (SRCC). At first glance, this tumour may be confused with a primary ovarian tumour. Surgery and chemotherapy combination have led to improvement in prognosis, but it still remains severe. We report the case of a 60-year-old woman with a Krukenberg tumour rising from a low differentiated gastric adenocarcinoma. The patient was clinically stable for 26 months after surgery until she experienced a prompt decline and died of cerebral haemorrhage within two weeks. The aim of this article was to give an overview of the Krukenberg tumour starting from our case report and comparing it with clinicopathological characteristics of this pathology derived from a review of recent literature.

Update on the surgical management of ovarian neoplasms in children and adolescents: analysis on 32 cases

Abstract This study analyzes updated clinical, diagnostic, and surgical directions for the treatment of ovarian neoplasms in children and adolescents, comparing them with a retrospective analysis of 32 cases treated in two Pediatric Surgery University Institutions. From January 2005 to December 2015, 32 pediatric patients were surgically treated for 32 ovarian tumors: 28 (87.5%) benign and 4 (12.5%) malignant neoplastic lesions. Median age at surgery was 11.2 years (12.8 years in patients with benign neoplasms, 7.25 years in patients with malignant ones). All patients with malignant and 25% of patients with benign ovarian lesions had elevated serum level of tumors markers. The surgical approach was laparotomic in 62.5% and laparoscopic in 37.5%; 81.2% surgeries were performed in elective surgery and 18.8% in emergency. Intraoperative frozen section analysis was performed in 18.75% of patients. The most frequent surgery (96.8%) was unilateral oophorectomy. After a median follow-up of 76 months (range 6–132 months), 31/32 patients are alive and disease-free. In case of malignant tumors, fertility-sparing surgery with accurate staging must be performed. Laparoscopic multiport is the gold standard approach for benign pediatric ovarian neoplasms, but the use of laparoscopy in full respect of oncological principles also for early stage malignant tumors is currently increased.

Surgical Management of Medullary Thyroid Carcinoma in Pediatric Age

Medullary thyroid carcinoma (MTC) is a rare, accounting for 5% of thyroid malignancies. It is a neuroendocrine tumor wich origins from thyroid parafollicular cells. It may be sporadic, mostly in adult patients, or inherited as autosomal dominant pattern, mostly in pediatric patients. As familial cancer, MTC may presented isolated as familial medullary thyroid carcinoma (FMTC) - 10% of cases - or, most often, as part of multiple endocrine neoplasm type 2 (MEN 2A or MEN 2B) syndromes - 90% of cases. The therapy for sporadic or hereditary MTC is surgical resection and consists in total thyroidectomy associated with central compartment lymph nodal dissection; the radicality of this intervention is fundamental to obtain a definitive cure. Genetic screening in children of parents with MEN2 or FMTC has led to prophylactic total thyroidectomy. The type of prophylactic surgery required is focus of great attention, and it is reported that total thyroidectomy, central neck dissection associated with parathyroid autotransplantation is the proper management. In children, the recommended age of surgery based on genetic testing is 5 years for patients with MEN 2A and FMTC, and 1 year for children possessing the RET mutation responsible for MEN 2B. The surgical management changes depending on the type of codon mutated.

Habitual Joint Dislocations and Recurrent Multiple Hernias: An UnusualConnective Tissue Disease?

Purpose: Joint laxity and hernia defects are two well-defined conditions that are rarely described in association in literature. The purpose of this study is to describe a peculiar case of connective tissue disorder, not yet defined, whose features are habitual joint dislocations associated with recurrent and multiple hernias. Methods: This is an unusual case regarding a 22 years old male adult, who presented shoulder, patellar, mandibular habitual joint dislocations associated with groin, femoral, epigastric, umbilical, spigelian and lumbar hernias. His phenotype, consisting of tall stature and joint laxity, recalls a similar-marfanoid habitus. Results: Marfan Syndrome, Ehlers-Danlos syndrome, MEN-2B were excluded by specific assessments. The aspecific clinical features of the patient do not consent the identification of an exact diagnosis. Conclusion: This medical case, characterized by joint hypermobility and multiple recurrent hernias, probably consists in an unacknowledged peculiar case of connective tissue disorder. Further investigations and identification of subjects with similar features could be surely useful to diagnostic research and definitive characterization of patient’s disease.

Pediatric Differentiated Thyroid Cancer: When to Perform Conservative and Radical Surgery.

Thyroid tumors affect all age groups, including children and adolescents. Malignant cancer of the thyroid is a relatively uncommon disease in pediatric age. In the recent decades the incidence of paediatric differentiated thyroid carcinoma (DTC) has increased. DTC in paediatric age is rare and has an excellent prognosis. Compared to adult counterpart, DTC in childhood presents some different features as follows: larger volume at the diagnosis, more frequent multicentricity (both mono- and bilateral), earlier local involvement of soft tissue of the neck, earlier lymph node involvement, distant metastases 3-4 times more frequent (most often in the lungs and almost always functional) and more common post-operatory recurrence; nevertheless, the prognosis of DTC in childhood is better and the survival greater than in adult. Because of unusual association between aggressive presentation and good prognosis, the choice about the surgical treatment to perform in DTC is debatable, especially between conservative and radical approach in TNM stage I pediatric patients. To date, total thyroidectomy is the operation most often performed in children with DTC, although recently conservative surgery has been performed in solitary unifocal nodule without evidence distant metastases.

Primary retroperitoneal müllerian adenocarcinoma: a case report and literature review.

Primary retroperitoneal müllerian adenocarcinoma (PRMA) is an extremely rare clinical entity. We report the case of a 54-year-old woman who presented with a mass in the right lower retroperitoneum, identified during an ultrasound exam. Computed tomography confirmed a retroperitoneal mass measuring 11 cm. The patient underwent laparotomy and the mass was completely excised. The histopathological exam revealed PRMA.

Surgical management of follicular thyroid carcinoma in children and adolescents: A study of 30 cases

BACKGROUND/PURPOSE The purpose of the study is to describe the anatomoclinical, diagnostic, therapeutic and prognostic aspects of pediatric follicular thyroid carcinoma (FTC) in order to choose the best therapeutic strategy. METHODS Our study includes patients ≤18 years old surgically treated for FTC in four Italian Pediatric Surgery Centers from January 2000 to March 2017. The collected data were compared with those of 132 patients matched for age with a histological diagnosis of papillary thyroid carcinoma (PTC) surgically treated in the same institutions during the same period and with the data of patients diagnosed with FTC found in the literature; p-values <0.05 were considered significant. RESULTS 21 (70%) of the 30 patients with a histological diagnosis of FTC underwent hemithyroidectomy while 9 (30%) underwent total thyroidectomy. 11 (55%) out of 21 patients were subjected to a completion of thyroidectomy. All patients are alive (OS = 100%) without recurrence or relapse of the disease. Compared with PTC, FTC is significant for capsule infiltration (p < 0.0001), vascular invasion (p = 0.0014) and T-stage T3-T4 (p = 0.013). However, multifocality (p < 0.001), extrathyroid extension (p < 0.0001) and lymph node metastasis (p < 0.0001) are more evident in PTC. CONCLUSION The conservative approach seems to be a valid surgical treatment for pediatric patients diagnosed with MI-FTC. For patients with wide vascular invasion and/or a tumor >4 cm, especially with high after-surgery Tg rate, a completion of thyroidectomy is recommended. In patients with multifocal neoplasia, and/or tumor size ≥4 cm, and/or extrathyroid extension, and/or lymph node metastasis, and/or distant metastasis, total thyroidectomy followed by radioiodine therapy is generally indicated. LEVELS OF EVIDENCE II.