Gastrointestinal stromal tumour of the duodenum in a 7-year-old boy

We report a 7-year-old boy presenting with an acute upper gastrointestinal (GI) haemorrhage subsequently diagnosed to have a very rare duodenal gastrointestinal stromal tumour (GIST). Endoscopy, pertechnetate and red cell scans were negative. Abdominal US detected a 17-mm mass lesion of the third part of the duodenum. This was confirmed on CT and shown to be hypervascular on selective angiography. At laparotomy, a 20-mm submucosal duodenal lesion was found associated with mucosal ulceration. Immunohistochemical analysis revealed it to be positive for CD117 (c-KIT protein) consistent with a GIST. We emphasize the importance of a thorough abdominal US examination in children with GI haemorrhage and the consideration of GIST in the diagnosis after the common causes have been excluded.     

Segmental omental infarction in childhood: a typical case diagnosed by CT allowing successful conservative treatment

Segmental omental infarction (SOI) is an uncommon cause of right lower quadrant pain in children that is often misdiagnosed as appendicitis. During the last decade, imaging findings of SOI have proved to be sufficiently typical to avoid unnecessary surgery in the majority of reported adult patients. The condition has a spontaneous favourable evolution under medical treatment. In children the surgical option remains controversial. We report a typical case of SOI in a 10-year-old boy. The diagnosis was suspected by sonography, unambiguously confirmed by multidetector CT and successfully treated conservatively. This report emphasizes the use of CT in selected acute abdominal situations, peculiarly in obese children, to avoid unnecessary surgery.     

Endodermal sinus tumor of the vagina

Malignant germ cell tumors of the vagina represent a critical site for local treatment. We report on our experience with two vaginal endodermal sinus tumors. Treatment with PEB regimen induced both tumor regression and alpha fetoprotein normalization. One patient had a residual lesion that was biopsied twice and no viable tumor cells were ever found. Both patients remained alive and disease-free, without having had surgical procedures except for biopsies. Platinum-based chemotherapy is able to achieve complete remissions and should be considered for vaginal endodermal sinus tumors.     

A case of Sertoli-Leydig cell tumour of the ovary with a multilocular cystic appearance on CT and MR imaging

We present a case of Sertoli-Leydig cell tumour of the ovary in a 14-year-old girl who presented with abdominal distension. Ultrasonography showed a multilocular cystic lesion filled with finely echogenic fluid. Contrast-enhanced CT demonstrated a huge multilocular cystic mass with thickened septa. At MR imaging, the capsule of the cyst was focally thickened, showing intermediate signal intensity on T2-W images. Although extensive cyst formation of Sertoli-Leydig cell tumour is rare, this tumour should be considered in the differential diagnosis of a multilocular cystic ovarian tumour in a young female.     

Colonic neuroendocrine carcinoma in a child

A 10-year-old boy with congenital immunodeficiency (X-linked agammaglobulinaemia) presented with loss of appetite and weight, right-sided abdominal pain, diarrhoea and low-grade fever. Radiological investigations with barium follow-through, CT, PET and octreotide scans revealed a primary caecal/ascending proximal colonic mass with liver and bony metastases. Urine screen for 5HIAA was positive. Percutaneous liver biopsy confirmed the diagnosis of neuroendocrine carcinoma. The radiological work-up and the usefulness of various imaging modalities in the diagnosis of this rare paediatric tumour are discussed. The PET scan demonstrated the primary tumour and the metastatic locations more vividly than the octreotide scan, which is currently considered to be the most specific imaging modality for neuroendocrine masses.     

Embryonal rhabdomyosarcoma of the omentum: two cases occurring in children

Rhabdomyosarcoma is a soft-tissue malignancy that represents approximately 4–8% of all solid tumours in children and commonly arises from the head and neck and genitourinary system. Intraperitoneal rhabdomyosarcoma, in particular with omental involvement, has been rarely reported in the literature. Furthermore, reports of omental rhabdomyosarcoma of embryonal origin do not exist, to our knowledge. We report two cases of omental embryonal rhabdomyosarcoma affecting children and illustrate the imaging characteristics of this rare tumour.     

Primary torsion of the omentum in children

Primary torsion of the omentum is an uncommon cause of abdominal pain in children. Patients usually present with a clinical picture of acute appendicitis. We report on three children with this condition who were managed by partial omentectomy with a satisfactory outcome in each case.     

Atypical inguinal malignant peripheral nerve sheath tumour with arteriovenous fistula of the left femoral nerve in a child

We report a 9-year-old girl who developed a malignant peripheral nerve sheath tumour (MPNST) with an arteriovenous fistula arising from the left femoral nerve and adjacent to the iliofemoral vessels in the ipsilateral groin, but without infiltrating them. We describe the MRI and MRA findings. Although MPNST is relatively well known and widely studied, the location of this mass is unique in a child. The mass was surgically removed.     

Burkitt's lymphoma with bilateral cavernous sinus and mediastinal involvement in a child

We report a 12-year-old boy who presented with incomplete right ophthalmoplegia, exophthalmos and headache. Initial CT and MRI revealed a mass in the right cavernous sinus. During tumour work-up, CT identified additional tumour within the mediastinum. Biopsy of the mediastinal lesion identified Burkitt's lymphoma. We report on this case because radiologists and clinicians should be alerted to identify sites of primary Burkitt's lymphoma outside of the central nervous system if clinical symptoms indicate, or imaging shows, CNS lesions. Primary CNS involvement in Burkitt's lymphoma is rare.     

Thoracic malignant peripheral nerve sheath tumour mimicking a pleural tumour: a rare pedunculated appearance

A malignant peripheral nerve sheath tumour (MPNST) generally occurs in adults and often in patients with neurofibromatosis-1 (NF-1). We present a rare case of a huge thoracic MPNST arising from the intercostal nerve in a 12-year-old girl without NF-1. In addition to the unusual occurrence in a child without NF-1, MRI demonstrated a unique pedunculated appearance mimicking a pleural tumour. In this report, we present the CT and MRI findings of our case, together with the histopathological findings, and review previous reports.     

MRI findings in a child with sigmoid sinus thrombosis following mastoiditis

We describe the MRI features of sigmoid sinus thrombosis following mastoiditis in a 3-year-old girl. The features consisted of increased signal from the sinus on T2-weighted images and absence of flow on MR venography. It is concluded that MRI enabled a timely diagnosis of this life-threatening disease. MRI, as a non-invasive technique that does not use ionizing radiation, should be considered the investigation of first choice, especially in young patients.     

Endodermal sinus tumors in the head and neck region

Extragonadal germ cell tumors of the head and neck region account for only 5% of all benign and malignant germ cell tumors. Endodermal sinus tumors (EST) of the head and neck region are rare. We report three patients with EST of head and neck region over a period of 10 years; the primary sites of tumor were orbit, maxillofacial region and retroauricular region. Histopathological examination revealed malignant teratoma with predominant endodermal sinus pattern in two, and pure EST in one patient. Serum alpha fetoprotein (AFP) was elevated in all three patients. Two patients had initial surgery but did not receive adjuvant chemotherapy, as the parents refused it. Partial remission was achieved in the other patient who received chemotherapy (cisplatin, bleomycin and vinblastin) and the patient died of infection after four courses of chemotherapy. Med. Pediatr. Oncol. 29:303–307, 1997. © 1997 Wiley-Liss, Inc.     

Primary cerebral leiomyosarcoma in a child

We present a 13-year-old boy with a low-grade primary intra-axial cerebral leiomyosarcoma. MRI showed a contrast-enhancing intra-axial mass with features of a cell-rich tumour. The diagnosis was established after neurosurgery and exclusion of an extracerebral primary tumour. The resection of local recurrence 6 months later confirmed the diagnosis, but a higher grade of malignancy was established. The patient died 15 months after the onset of symptoms from general intracranial and intraspinal spread.     

儿童内胚窦瘤临床治疗研究

目的 探讨儿童内胚窦瘤的治疗与转归。方法 回顾性分析2000 年4 月至2013 年7 月收治的12 例内胚窦瘤患儿的临床资料。其中男7 例,女5 例;11 例年龄在1~3.3 岁之间,1 例11 岁。Ⅰ期2 例,Ⅱ期4 例,Ⅲ期2 例,Ⅳ期4 例。1 例采用单纯手术,1 例采用手术联合VAC（长春新碱+ 放线菌素D+ 环磷酰胺）方案化疗,10 例采用手术联合以PEB（顺铂+依托泊苷+博来霉素）方案为主的化疗。结果 成功随访11 例,目前存活10 例,生存期为4.5~66 个月。10 例接受了PEB 方案化疗的患儿,8 例达到完全缓解,1 例达到部分缓解,1 例失访。PEB 方案治疗并发症主要包括骨髓抑制及胃肠道反应,未发现远期并发症。结论 手术联合PEB 化疗方案是有效、安全的治疗儿童内胚窦瘤的方法,但需积累更多病例进一步证实。     

小儿性腺外内胚窦瘤3例诊断与治疗分析

目的总结小儿原发性腺外内胚窦瘤的诊断与治疗经验。方法回顾性分析我院2003年10月-2011年9月收治的3例性腺外内胚窦瘤患儿的病历资料。结果术前均行PEB或JEB方案新辅助化疗,根治性手术切除,术后病理均为完全缓解,术后继续化疗。随访3个月～8年,2例无病存活,1例原位复发,经术前化疗、再次手术切除及术后化疗后,现随访25个月,无病存活。结论甲胎蛋白水平对小儿性腺外内胚窦瘤诊断及术后复发监测灵敏度较高,化疗与手术结合治疗效果良好。     

Segmental omental infarction in childhood: an unusual case of left-sided location with extension into the pelvis

Segmental omental infarction (OI) is a rare cause of abdominal pain in children. It generally occurs in the right lower and upper quadrants of the abdomen and only a few cases of other locations have been described in adults. We report a unique paediatric case of OI with an unusual left-sided location extending into the pelvis in a 6-year-old non-obese girl. The diagnosis was suspected on US and CT based on imaging patterns previously described in adults and children. Laparoscopic resection should be balanced with nonsurgical management.     

Imaging in unilateral Wilms tumour

Wilms tumour is one of the most common malignancies in children, with an excellent prognosis after therapy. There is a very diverse approach to treatment according to geographical location. This variation in therapeutic attitude toward Wilms tumour, particularly between the United States and Europe, has consequences for the choice of imaging modality at diagnosis. In Europe, the International Society of Paediatric Oncology (SIOP) treatment protocol is based on chemotherapy followed by surgery. Imaging (US, CT and MRI), clinical history and examination will help predict whether the findings are consistent with Wilms tumour. Furthermore, in the UK preoperative image-guided biopsy is advised to help identify the small group of patients who, despite typical imaging features of Wilms tumour, have other types of neoplasia that require alternative management. In the United States, the National Wilms Tumor Study (NWTS) advises surgery prior to chemo- and radiotherapy. Hence imaging must provide detailed anatomical information for surgical planning. This article discusses the role of imaging at diagnosis and the relative strengths and weaknesses of the available radiological techniques. We also focus on imaging the lung for metastatic disease and the consequences (to the patient’s ultimate outcome) of CT-diagnosed small pulmonary nodules and discuss the radiological diagnosis and consequences of tumour rupture present at diagnosis.     

女童先天性耻骨前窦道的诊断和治疗

目的 总结3例女童先天性耻骨前窦道的临床特点和治疗经验.方法 回顾性分析2011年11月至2014年3月,我院收治的3例女童先天性耻骨前窦道的临床表现、手术方法及疗效.患几年龄分别为0.45、1.5、2岁,均表现为下腹壁中线或靠近耻骨上方异常窦道.术前经窦道造影了解窦道位置、长度和走向,术中在美兰引导下进行窦道切除.结果 3例病例均Ⅰ期完整切除窦道,术前造影见窦道均延伸至耻骨后缘,其中2例穿过耻骨软骨、1例紧贴耻骨软骨表面,再经腹膜外延伸至膀胱前方.术中予窦道内注射美兰,证实窦道与泌尿生殖道及腹腔均无相连,窦道末端呈盲端.2例穿过耻骨软骨的患儿切除了与窦道关联的部分耻骨软骨.窦道长度分别为6.0、6.5、5.5cm,平均(6.0±0.5)cm,直径0.2～0.5 cm.术后病理见窦道内壁均被覆复层鳞状上皮细胞.术后分别随访0.25、2.5年,均未见窦道复发,伤口外观美观,患儿行走步态正常.结论 先天性耻骨前窦道是一种下腹壁中线部位罕见畸形,女婴尤为少见,完整手术切除窦道是治疗该病的有效方法.术前造影结合术中美兰引导有助于完整切除窦道.