Lipoblastomatosis in a newborn: case report

A male newborn had a well-circumscribed, solid mass on the right anterior chest wall at birth. Computed tomography disclosed an infiltrating soft-tissue mass over the right 6th to 9th ribs near the costochondral junction. Surgical excision was done at the age of 5 days. Pathologic examination showed lipoblastomatosis.     

Ruptured giant intrathoracic lipoblastoma in a 4-month-old infant: CT and MR findings

Background. We describe a 4-month-old infant with a ruptured intrathoracic lipoblastoma arising from the parietal pleura and associated with a pleural effusion.¶Objective. The clinical presentation was rapidly evolving respiratory distress. The chest radiograph showed a large mass and a pleural effusion in the right thoracic cavity. CT demonstrated an inhomogeneous low-attenuation mass which was 7 cm in diameter and which showed areas of enhancement after intravenous contrast medium. MRI showed a fatty intrathoracic mass with intratumoral streaks and whorls, which were attributed to loose fibrovascular connective tissue on pathological examination.¶Results. Thoracotomy and pathological examination revealed a ruptured intrathoracic lipoblastoma arising from the parietal pleura.¶Conclusion. The pleural effusion might have suggested rupture of the tumour.     

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.     

Thoracic wall lipoblastoma: a case report and review of histopathology and cytogenetics.

A rare case of a successfully excised intra- and extrathoracic lipoblastoma of the anterior chest wall in a 13-month-old female infant is reported. Histopathology and cytogenetical analysis established the diagnosis of a lipoblastoma. The differential diagnosis, histology and cytogenetical evaluation of lipomatous neoplasms are discussed. Karyotypic analysis may be of use in diagnostically difficult cases owing to the characteristic alterations in 18q11-13. A complete resection of lipoblastomas is feasible and advantageous with no need for a mutilating radical excision.     

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.     

Lipoblastoma in a four-year-old African child

A four-year old Sudanese child presented with a growing mass in the medial aspect of the right thigh. The mass appeared during the neonatal period. On clinical examination a diagnosis of lipoblastoma was entertained on the basis of the patient's age and the clinical features of the mass. The tumor was completely excised surgically. The clinical diagnosis of lipoblastoma was confirmed pathologically. Follow-up of the patient for 6 months postoperatively showed no evidence of recurrence.     

Primary Cardiac Lipoblastoma

Lipoblastoma is a benign adipose tumor in children that has been described in various anatomic locations, most commonly the extremities. We describe the case of a 17-month-old boy diagnosed with cardiac lipoblastoma, a previously unreported primary cardiac tumor in children. Our patient presented with symptoms of coughing, wheezing, and hoarseness and was found to have a large mediastinal mass, which narrowed the left mainstem bronchus and compressed the right atrium and superior vena cava, causing superior vena cava syndrome. Surgical exploration revealed an intrapericardial soft tissue mass arising from the area of the posterior interatrial septum. Grossly, the resected mass was lobulated, pale yellow, and fatty with focal areas of gray myxoid tissue. Microscopically, the tumor consisted of both immature and mature adipocytes, with focal vascular myxoid areas containing lipoblasts, diagnostic of lipoblastoma. Two months after surgery, the patient was in good health without evidence of recurrence. Received April 13, 2000; accepted August 17, 2000.     

小儿漏斗胸Nuss手术对胸廓影响的研究

目的 探讨小儿漏斗胸Nuss手术对胸廓的影响.方法 对2004年至2008年采用Nuss手术治疗的33例漏斗胸患儿,主要利用超声及螺旋CT检查进行术后随访.项目包括:肋骨、肋软骨、胸壁第1至第6对肋骨与软骨关节(CCJ)、胸肋关节(CSJ)以及胸廓外观.结果 2例钢板移位;4例(12.1%)术后出现胸廓畸形;1例术后3年4个月发现右侧第四肋软骨胸骨端陈旧性骨折;1例术后1周发生右侧第五肋软骨横断骨折;8例(24.2%)术后近期随访发现不同程度的CSJ及CCJ的损伤;6例(18.2%)钢板平面以下的胸廓下陷;1例发现左侧第五肋软骨斑点状骨化.结论 Nuss手术虽然优点颇多,但也存在明显不足.利用钢板的外力强行将凹陷的胸骨顶起,对胸廓是一种创伤,该创伤累及胸廓的关节、肋骨、肋软骨,使CSJ和CCJ不同程度分离、移位,肋软骨骨化、肋软骨骨折以及胸廓畸形等.     

Benign mediastinal lipoblastoma in a 14-months-old infant

Lipoblastomas are rare benign tumors arised from the fetal-embryonal fat that occur almost exclusively in children. About 70% of them arise in the limbs but several other sites have been reported. To date there are only 4 cases of mediastinal lipoblastoma in children, described in the international literature. The radiologic examination, including the chest X-ray, sonography and CT scan, can be used to detect the mass and its relationship to surrounding structures, and to define the typical features of the fat-like tissue. A case of a mediastinal lipoblastoma in a 14-months-old infant is reported, with a respiratory stridor and occasional dyspnoea, appearing only in the supine position. The radiologic findings, treatment and follow-up are also described.     

An unusual case of grass inflorescence aspiration presenting as a chest wall tumour

A 9-year-old boy was referred to the Oncology Department because of a thoracic soft-tissue mass thought to be a chest wall tumour. He had a history of grass inflorescence (Hordeum murinum) aspiration 2 weeks prior to this admission. On physical examination a tender soft-tissue mass under the right scapula and diminished breath sounds from the right lower lobe were detected. Thoracic CT confirmed soft-tissue swelling of the right posterior chest wall. There was a hypodense area within the soft-tissue mass suggesting a foreign body and also focal consolidation of the right lower lobe adjacent to the soft-tissue swelling. We report here unique CT findings of grass inflorescence aspiration before and after its migration through the airways.     

Mesenchymal Hamartoma of the Chest Wall: A Cooperative Study with Review of the Literature

The clinicopathologic features of three examples of mesenchymal hamartoma of the chest wall are described. The entity has been recorded under a number of names including osteochondroma, osteochondrosarcoma, benign chondroblastoma, mesenchymoma, and chondromatous hamartoma. The condition is manifest at birth or shortly thereafter with deformity of the chest wall and respiratory distress. Radiographic examination reveals a well-defined, partly calcified mass involving one or more ribs. The tumor is composed predominantly of chondroid tissue with large endothelium-lined blood spaces and immature mesenchyme with osteoclastic giant cells and osteoid. We review the literature and suggest that the lesion should be distinguished from aneurysmal bone cyst, chondroma, and other mesenchymal neoplasms. In order to avoid local recurrence the recommended treatment is complete surgical resection.     

Mesenchymal hamartoma of the chest wall in infancy: 7-year follow-up

A case of mesenchymal hamartoma of the chest wall in infancy is reported. The presenting symptom was a bulging mass arising from the chest wall. Computed tomography showed the lesion to be multifocal. A large extrathoracic mass was removed and the defect in the chest wall was repaired with a Marlex mesh. Two smaller intrathoracic lesions were observed conservatively and have not enlarged during the past 7 years. Correspondence to: Y. Ishiguro     

Radiological–pathological correlation in lipoblastoma and lipoblastomatosis

Background Lipoblastoma and lipoblastomatosis are uncommon benign mesenchymal lesions that predominantly occur in infancy and early childhood.Objective To evaluate the imaging and histological features of lipoblastoma and lipoblastomatosis.Materials and methods Retrospective review of the radiological and pathological findings in children with lipoblastoma and lipoblastomatosis treated at a single centre between 1997 and 2004.Results Eight children (median age 18 months) had undergone imaging and surgery at our institution. An infiltrative growth pattern was identified at imaging in two children with lipoblastomatosis, and a well-defined mass in six children with lipoblastoma. In all patients, imaging showed a lesion composed mostly, but not entirely, of fat. There were no recurrences at follow-up of between 1 and 91 months.Conclusion In infancy and early childhood, the identification of a tumour composed mostly of fat should suggest the diagnosis of lipoblastoma or lipoblastomatosis.     

Rib fractures as a cause of immediate neonatal tachypnoea

Two macroscopic term neonates are described who presented with uncomplicated tachypnoea immediately following vaginal delivery. The tachypnoea was not associated with lung injury, metabolic, endocrine or cardio-respiratory disease but with multiple unilateral posterior rib fractures. The neonatologist should be aware that neonatal tachypnoea immediately after vaginal term delivery, especially in macrosomic infants, may be caused by the pain of fractured ribs. The diagnosis can be made only by careful inspection of the ribs on chest X-ray.     

The novel use of small intestinal submucosal matrix for chest wall reconstruction following Ewing’s tumour resection

Chest wall reconstruction following major tumour resection remains a challenging problem in the paediatric population due to the need for growth and development following the repair. Synthetic non-biodegradable grafts and materials have led to asymmetrical growth and significant functional and cosmetic compromise. The ideal graft material should be biological non-immunogenic which enables natural healing and facilitates further growth. Ewing’s sarcoma is an uncommon primitive neuroectodermal tumour which rarely occurs in the ribs. However, recent improvements in management have produced a significantly improved survival; increasing the importance of chest wall reconstruction. We prospectively assessed the utilisation of Surgisis (an acellular collagen matrix) as a replacement graft in two children undergoing primary chest wall reconstruction following tumour resection. Surgisis is derived from porcine small intestinal submucosa (SIS) that serves as a template for tissue reconstruction and remodeling. The graft was well tolerated; there was no herniation and it allowed normal symmetrical chest movement and growth to occur. The properties of SIS allows natural collagen growth and compares favourably with marlex mesh and other forms of chest wall reconstruction. We believe its unique properties allow it to act as a suitable framework for chest wall reconstruction allowing chest wall growth and development.     

Retroperitoneal lipoblastoma involving the right common iliac artery and vein.

A case of benign lipoblastoma of the retroperitoneum in a 12-month-old boy, presenting as a huge abdominal mass and right lower extremity swelling, was reported. Surgery revealed involvement of the right common iliac artery and vein by lipoblastoma, requiring the sacrifice of both vessels for complete removal of the tumour. Vascular continuity of both vessels was re-established by a saphenous vein graft interposition. Involvement of a great artery by benign lipoblastoma has not been reported before.     

Subdermal Fibrous Hamartoma of Infancy: Pathology of 40 Cases and Differential Diagnosis

Forty subdermal fibrous hamartomas of infancy occurred in 29 males and 11 females who were 7 months to 4 years of age at surgery. At least 4 were congenital. The lesions were situated in axilla, chest wall, and breast (17 cases); abdominal wall, inguinal region, and scrotum (8); buttock and lower limb (6); upper limb (4); neck and scalp (3); and low back (2). They were 0.7 to 10 cm, ill defined, and five underwent reexcision, which was curative in all. All had the characteristic mixture of fibrous and adipose tissue and nests of immature mesenchyme in different proportions, and nearly all showed lymphocytes and thick patent capillaries in the mesenchyme. However, the fibrous component varied considerably in amount, pattern, and cellularity, so that lesions that were typical in some areas, in others resembled collagenizing vascular granulation tissue, deep fibrous histiocytoma, or fibromatosis. Those in which adipose tissue predominated were distinguished from fibrolipoma by foci of immature mesenchyme and from lipoblastoma by their lack of a capsule and of a lobular pattern.     

Acute rib fracture diagnosis in an infant by US: a matter of child protection

Rib fractures in infants and children are highly specific for inflicted injury in the absence of a major accidental injury or underlying bone disorder. We present a 9-week-old infant diagnosed with an acute rib fracture by US at the site where physicians palpated chest wall crepitus when no rib fractures had been visualized on the skeletal survey, including oblique views of the ribs. Based on the US diagnosis of the acute rib fracture the infant was taken into protective custody. Follow-up skeletal survey 2 weeks later revealed healing fractures of the left 6th and 7th posterolateral ribs and right 7th, 8th and 9th anterolateral ribs. We were unable to locate previous reports describing the diagnosis of rib fractures in infants by US.     

Diagnostic imaging of primitive neuroectodermal tumour of the chest wall (Askin tumour)

Objectives. To describe the radiological features of primitive neuroectodermal tumour (PNET) of the chest wall (Askin tumour) at diagnosis and to analyse the radiological changes occurring as a consequence of treatment and during follow-up. Materials and methods. Nine children with histologically proven PNET were studied. At diagnosis, all patients underwent chest X-ray (CXR), chest CT and bone scintigraphy; three patients also had MR and three had US. During treatment and follow-up, CT was performed in all patients. Results. CT demonstrated a solid heterogeneous chest wall mass in all children at diagnosis and six had a rib lesion. Small nodular densities in the extra-pleural fat were identified in three patients at diagnosis. US, performed in three patients, excluded tumour infiltration of the lung or diaphragm, which had been suspected on CT. On MR, the lesions showed high signal intensity in T1-weighted/proton-density images and intermediate/high signal intensity in T2-weighted images compared with muscle. Minimal chest wall involvement was demonstrated in one case by MRI. Extensive necrosis of tumour mass with pseudo-cystic appearance was documented in the five patients who underwent chemotherapy. Macroscopically complete resection was performed in five patients but there was early local recurrence after surgery in two, identified by CT in one and by MR in the other. Conclusions. PNET of the chest wall should be considered in a child with a chest wall mass. CT is valuable for evaluating tumour extension at diagnosis, the effects of chemotherapy and assessing tumour recurrence after surgery. However, CT can overestimate pleural, lung or diaphragmatic infiltration, which are better evaluated by US. MR was superior to CT in the evaluation of tumour extension in one of three patients and may be considered complementary to CT, particularly in very large chest wall tumours. Received: 23 May 1997 Accepted: 23 February 1998     

Rapid-growing tumor of the cheek mimicking a malignant tumor: lipoblastoma of infancy

In this case report, we describe a 5-month-old girl with a rapid-growing mass of the lower lip extending to the buccal cheek. After surgical interference, the diagnosis lipoblastoma was made. Dealing with a fast-growing tumor in an infant, lipoblastic tumors belong in the differential diagnosis, however, a malignant process cannot be excluded. Rapid-growing lipoblastoma of infancy is a very rare benign tumor of embryonic white fatty cells. Magnetic resonance imaging might help with the diagnosis and often shows a lesion composed mostly, but not entirely, of fat. In this case report, we want to draw attention to the problems with diagnosis and therapeutic management of children with lipoblastoma.