Pulmonary lipoblastoma: a case report

We report a case of pulmonary lipoblastoma in a 2-year-old child. This pathology has not been previously reported in the English literature. Accepted: 3 July 2000     

A thoracic wall lipoblastoma in a 3-month-old infant: A case report and review of the literature

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.     

Mesenteric lipoblastoma: case report

We present a rare case of mesenteric lipoblastoma in a 16-month-old girl. The US, CT and MRI features of this unusual tumour are described and correlated with the pathology findings. MRI more clearly suggested the presence of fat components in the tumour. In addition, multiplanar MR images demonstrated the anatomical extent better, which was essential for successful complete tumour excision.     

儿童心脏脂肪母细胞瘤一例

患儿女,15月龄,以"孕6个月余排畸检查发现肺动脉狭窄,生后发现心脏杂音1年余"入院。平日除活动后偶有口唇发绀以外无特异临床表现。入院后行胸超声心动图检查提示肺动脉瓣上有一9.6 mm×9.2 mm近圆形异常囊性占位,内部呈无回声区,外壁呈中强回声,导致肺动脉瓣上形成狭窄(图1),狭窄区压差为61 mmHg(1 mmHg=0.133 kPa);房间隔中段回声断续脱失,累及范围约18.5 mm×15.0 mm×21.5 mm。术前诊断:①先天性心脏病,房间隔缺损(继发孔、筛孔型);②肺动脉瓣囊性占位(性质待定);③肺动脉瓣上狭窄(继发性);④三尖瓣反流(少量)。完善相关术前检查后行手术治疗。术中见心脏中度扩大,右心增大为主。肺动脉轻度扩张,压力不高,主动脉:肺动脉口径之比为=1∶1.2。     

Thoracic actinomycosis in children: case report and review of the literature

Thoracic actinomycosis is uncommon, indolent, and often not considered in children with chest wall masses, pneumonia, or empyema. We present a 27-month-old girl without risk factors for thoracic actinomycosis whose initial diagnosis was malignancy. She recovered completely after lobectomy, debridement, and antimicrobial therapy. Literature review since 1975 identified 54 additional cases; most were male, older, and had underlying risk factors.     

Thoracic myopericytoma in a child. A case report

Myopericytoma is a ubiquitous, benign tumor that is likely to be found in the muscular-cutaneous system. Symptoms are related to its location and to the mass effect it may cause on surrounding organs. Diagnosis is usually made by the pathologist and treatment is surgical. We report herein a rare thoracic location in a 4-year-old girl.     

Tropical pyomyositis: a case report and review.

Tropical pyomyositis is a disease of skeletal muscle characterized by single or multiple abscesses. The infective organism is most often penicillin-resistant Staphylococcus aureus. Only recently have case reports appeared in the literature from temperate zones; however, this disease is common in the tropics. This report reviews the literature and describes a child from rural North Carolina in whom tropical pyomyositis developed after incision and drainage of a furuncle.     

Chest wall lipoblastomatosis in a 2-year-old girl: a case report and literature review

Lipoblastoma is an uncommon tumor of adipose tissue that usually occurs in infancy and early childhood. They predominantly locate in the extremities, but are less frequently found in the head and neck region, trunk and various other locations. The most common sign is a rapidly growing mass. In spite of their potential for local invasion and rapid growth, they are benign tumors and have no distant metastasis. Although more than 180 cases of lipoblastoma distributed over various parts of the body have been reported, only nine cases were located in the thoracic wall. We present the case of a two-year-old girl with lipoblastomatosis of the chest wall that was identified by histologic examination. In this article, the English literature on this disorder is briefly reviewed.     

Thoracic ectopic kidney in a child: a case report

Congenital thoracic ectopic kidney is a very rare developmental anomaly and the rarest form of all ectopic kidneys. It is usually asymptomatic and discovered incidentally on a routine chest radiography. We report a thoracic ectopic kidney in a 19-month-old boy, which initially presented as a well demarcated mass at the base of the right lung on chest x-ray. Intravenous pyelography (IVP) and thoraco-abdominal computed tomography (CT) demonstrated a normal functioning transdiaphragmatic thoracic ectopic right kidney, but technetium-99m DTPA and DMSA scintigraphy demonstrated pelvic stasis. We hereby discuss the features of congenital thoracic ectopic kidney and review the literature. Although it is extremely rare, thoracic ectopic kidney should be considered in differential diagnosis of a mass with a well demarcated superior margin in the lower part of the thorax, and renal scintigraphy must be performed even if CT and IVP results are normal.     

Polyorchidism: a case report and review of the literature.

We report on a case of polyorchidism associated with left-sided inguinal hernia and hydrocele. Biopsies of both testes revealed normal histological pattern. After the hernia repair and hydrocelectomy both testes were left at their original places in the scrotum and fixed. Polyorchidism and its management are discussed briefly.     

Acute cerebellitis: case report and review.

We report a case of acute cerebellitis in a 7-year-old male presenting with headache, vomiting, ataxia and life-threatening hydrocephalus requiring emergent placement of an external ventricular drain. Unlike earlier reported cases which did not provide radiographic correlation of this disease, this report documents changes in the cerebellum with serial magnetic resonance and computer tomography scans. The signs, symptoms and differential diagnosis of this rare, self-limiting condition are briefly discussed.     

无脑回畸形儿1例报告并文献复习

目的探讨无脑回畸形的临床特征及其致病基因LIS1基因的检测特点。方法回顾性分析1例无脑回畸形患儿的临床、实验室检查及基因检测结果,同时复习相关文献。结果女性,5月龄,确诊癫痫20 d,3 d内再次抽搐发作30余次入院,抽搐发作表现为双眼凝视、上翻,口唇、面色发绀,口吐白沫,四肢强直,意识丧失,约2~3 min自行缓解。外周血白细胞计数13.67×109/L,血红蛋白108 g/L,红细胞计数3.90×1012/L,淋巴细胞10.26×109/L;心肌酶谱、肝肾功能均正常;血氨23μmol/L,乳酸2.11 mmol/L;长程视频脑电图显示高度失律,频繁部分性发作,有时继发全身阵挛发作。头部MRI提示无脑回畸形。口服左乙拉西坦片,约27 mg/(kg·d),托吡酯片约6.5 mg/(kg·d),目前暂无发作。患儿LIS1基因检测发现c.232del G杂合突变,导致蛋白移码突变(p.E78Nfs X25);患儿父母均未见突变。结论无脑回畸形患儿可合并癫痫,可能由LIS1基因突变所致,该基因c.232del G位点突变在国内外未见报道。     

半侧巨脑综合征1例附文献复习

半侧巨脑综合征(hemimegalencephaly,HME)是一种极为罕见的脑发育畸形,在难治性癫痼的手术治疗中有重要意义.现将我院诊治的1例报道如下.     

骨肥大血管扩张综合征1例报道及文献复习

骨肥大血管扩张综合征又称Klippel-Trenaunay-Weber syndrome (KTWS),是一种少见的神经皮肤综合征,多在生后或儿童期起病,临床以多发性皮肤血管痣(瘤)、肢体广泛静脉曲张、骨及软组织肥大三大症状和体征为特征,多为一侧躯体受累,异常血管可累及脊髓和脑。该文报道1例累及脑血管及脑,临床上罕见。其病因尚不清楚,认为与遗传有关,可能为血管壁间质组织先天性发育异常的遗传性疾病。本病目前尚无特殊有效的治疗方法,认为对于受压迫的异常静脉行外科松解手术治疗应越早越好,以可望不出现或减轻患肢增粗或增长的症状。近年来有报道利用超声波进行产前诊断,这对及时终止妊娠、优生优育有重大意义。     

Meconium periorchitis: case report and literature review.

Meconium periorchitis (MPO) is an uncommon entity associated with healed meconium peritonitis. The typical presentation is soft hydrocele at birth which becomes harder in weeks as the meconium calcifies. It mimics a scrotal mass, and, without knowledge of this rare disease, this may lead to unnecessary surgery. Both the masses and the calcifications have the tendency to resolve spontaneously without compromising the testicle. Sonographic features together with an abdominal plain film are diagnostic, and visualization of the normal testicle may be helpful in differentiating this tumor-like lesion from scrotal tumors. A case of a meconium periorchitis is reported on and the radiological and histological features are discussed with a literature review on the subject.     

Micrococcus luteus pneumonia: a case report and review of the literature.

The clinical course of a 69-year-old male with acute myelogenous leukemia is described who, while extremely leukopenic (less than 100 neutrophils/microliter) from chemotherapy, developed a cavitating pneumonia due to a gram-positive coccus, Micrococcus luteus. Aggressive antibiotic management and attainment of complete remission of his leukemia resulted in a successful outcome. A review of the literature regarding the pathogenicity of this organism and, in particular, its occurrence as a cause of pneumonia is presented.     

Congenital esophageal stenosis: a case report and review of the literature.

A child with congenital esophageal stenosis (CES) demonstrates the need for accuracy in the diagnosis and management of this rare problem. Other etiologies of dysphagia must be looked into, but when the expected results are not realized CES should be considered. A range of diagnoses and diagnostic studies may help to get children with dysphagia the treatment that they need to resolve their symptoms.