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.     

A novel t(3;8)(p13;q21.1) translocation in a case of lipoblastoma

Lipoblastoma is a rare benign neoplasm of embryonic white fatty tissue primarily found in the extremities of children <3 years old (Batanian et al., Cancer Genet Cytogenet 125(1):10-13, 2001; McVay MR et al., J Pediatr Surg 41(6):1067-1071, 2006; Kamal et al., J Pediatr Surg 46(7):E9-E12, 2011). Translocations affecting the 8q11-13 region are commonly reported with lipoblastoma and proper diagnosis requires cytogenetic analysis to distinguish it from malignant myxoid liposarcoma (Miller et al., J Pediatr Surg 32(12):1771-1772, 1997; Morerio et al., Pediatr Blood Cancer 52(1):132-134, 2009). We describe an additional case of lipoblastoma containing a new translocation t(3;8)(p13;q21.1), which has not previously been reported in a healthy asymptomatic child.     

A rare case of inguinolabial lipoblastoma in a 13-month-old female

Lipoblastoma is a rare, rapidly growing, benign mesenchymal tumor composed of various stages of maturing adipocytes that most often occurs in children under the age of 3. The common locations are the extremities and the trunk. Presentation in the genitoinguinal area is rare. We report a case of a 13-month-old female infant with a 4-month history of a progressively enlarging left labial mass that encompassed her left labium majora and inguinal region. Pelvic MRI confirmed growth from previous ultrasound size of 3 × 2 × 1 cm to 7 × 2 × 2 cm. Composition was suggestive of adipose tissue. The mass was excised through a left inguinal incision. The final pathology results described a lipoblastoma. Six year follow-up has not revealed any signs or symptoms of recurrence.Circumscribed lipoblastomas should be distinguished from their infiltrative counterpart, diffuse lipoblastoma or lipoblastomatosis, which can be more difficult to excise and thus, more likely to recur. Lipoblastoma should also be distinguished from myxoid liposarcoma, which has malignant features, carries a high risk of recurrence, and requires a more aggressive management protocol. Although rare, lipoblastoma should be considered as part of the differential diagnosis of a rapidly growing vulvar mass in prepubertal children.     

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.     

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.     

Lipoblastoma: MRI appearances of a rare paediatric soft tissue tumour

Lipoblastoma is a rare, benign soft-tissue tumour derived from embryonic fat. Four patients with tumours located in the upper limb are reported, with special reference to imaging techniques and histology. Radical surgical excision is essential to prevent local recurrence and exact imaging techniques are thus crucial. MRI appears to be a reliable preoperative investigation and is the recommended radiological examination. In a child under 3 months of age, images showing a predominantly fatty but inhomogeneous soft-tissue mass are suggestive of lipoblastoma.     

Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature.

Lipoblastoma and lipoblastomatosis are rare benign neoplasms of foetal white fat tissue that occur almost exclusively in infants and children. Two cases are reported and a review of the literature is given. An almost one-year-old girl and a full-term male infant were brought to our hospital with a solid mass in the right thigh. Nine months after total excision of the lipoblastoma, the little girl developed tumour recurrence, with unexpected histopathological maturation. In the case of the little boy, lipoblastomatosis with infiltration of the surrounding muscles, together with involvement of the sciatic and posterior femoral cutaneous nerves was found. Histologically, the tumour showed an unusual inflammatory reaction.The diagnosis of this tumour was made by the pathologist, but the histopathological picture bears a striking similarity to myxoid liposarcoma, and may be indistinguishable. Recent studies describe rearrangements of chromosome 8 q11-q13 region as a new discriminative marker that distinguishes lipoblastoma and lipoblastomatosis from myxoid liposarcoma.     

Lipoblastoma/lipoblastomatosis: a clinicopathologic study of 16 cases in Taiwan

Lipoblastoma/lipoblastomatosis is an uncommon benign lipomatous tumor affecting mainly infants and children. It presents as a painless mass located frequently in upper and lower extremities. The present study was conducted to characterize the clinicopathologic features of lipoblastoma/lipoblastomatosis in a Chinese population. A retrospective survey of 16 cases of pathologically confirmed lipoblastoma/lipoblastomatosis from 1988 to 2002 was performed. The available clinical information and histological sections were reviewed. The study involved 10 males and 6 females ranging in age from 5 to 49 months (median, 11.5 months). The patients presented with mass involving neck (n=5), inguina (n=3), feet (n=2), arm (n=1), leg (n=1), thorax (n=1), mesentery (n=1), buttock (n=1), and presacral region(n=1). The lesions were removed surgically. Histologically, the majority (11/16) of primary tumors were of diffuse type. Follow-up ranging from 5 to 125 months was available in 13 patients. Four patients experienced a recurrence of tumors in the neck (n=2) and lower extremities (n=2) 11–84 months after operation. The most common site of involvement of lipoblastoma/lipoblastomatosis in our series was the neck. A follow-up period of more than 3 years is recommended for patients with this condition.     

Detection of a deep lipoblastoma by MRI and ultrasound

Lipoblastoma is a rare benign tumor of the soft tissue occurring predominantly during the first two years of life. We report here the application of MRI and ultrasound to the detection of a soft tissue lipoblastoma. By MRI and ultrasound, we precisely evaluated the extent of the tumor, the presence of atypical areas of cystic and mucoid degeneration within the tumor, and its lack of vascularity. Our patient's lipoblastoma did not demonstrate the signal characteristics of a fatty tumor on MRI.     

Rearrangement of HMGA2 in a case of infantile lipoblastoma without Plag1 alteration

Lipoblastoma is a rare benign adipocytic tumor that occurs usually in children. It can be difficult to distinguish a lipoblastoma from other lipogenic tumors. In such cases, the detection of a rearrangement of the PLAG1 gene by fluorescence in situ hybridization analysis is useful for characterizing a lipoblastoma. We present here a novel case of morphological infantile lipoblastoma showing a rearrangement of HMGA2 instead of the classical PLAG1 alteration. HMGA2 is the main target of clonal aberrations encountered in lipomas. This result supports the hypothesis that benign lipomatous tumors harboring PLAG1 or HMGA2 rearrangement could constitute a unique pathogenetic entity.     

Lipoblastoma and lipoblastomatosis in children.

Lipoblastoma and lipoblastomatosis are rare mesenchymal benign tumors of embryonal white fat. They are found primarily in children less than 5 years of age. This tumor presents in two forms: a localized well-circumscribed lesion (lipoblastoma), or a multicentric type (lipoblastomatosis). The authors presents three cases, two of them with a buttock lesion, the last with a paravertebral one. It is recommended a complete but conservative excision of the tumor because there is a natural tendency to involution, although in the first year of life a local reexcision for recurrence is sometimes described.     

Analysis of clinical features of lipoblastoma in children

Objective: A lipoblastoma is pathologically benign but often recurs. Due to its rarity, studies are scarce. The purpose of this study was to investigate the clinical characteristics of lipoblastoma occurring in children and to detect any correlations with the expression of Ki-67. Participants: From 1998 to 2010, 33 patients were diagnosed with lipoblastoma at Seoul National University Children's Hospital. Methods: Ki-67 immunohistochemistry staining of the tumor tissue was performed. Results: A total of 33 patients (64% males) were enrolled in the study, with a mean age of 28 month. Eleven and 22 lesions were deep and superficial, respectively. Complete excisions were performed for 30 patients, and three underwent incomplete excisions. Two patients who underwent incomplete excision subsequently underwent a second operation due to tumor regrowth, and one patient had a recurrence despite complete excision. There was no statistically significant correlation observed between the tumor size or recurrence and the expression of Ki-67. Conclusions: Lipoblastoma requires an accurate diagnosis and operative resection to alleviate the symptoms induced by its growth. Incompletely resected tumor may regrow; therefore, complete excision is the treatment of choice. Continuous follow-up is needed to monitor for recurrence of disease, even after a complete excision.     

Lipoblastoma: better termed infantile lipoma?

Lipoblastoma is a rare adipose tumor occurring exclusively in childhood. There have been no reports of metastases, making the designation “blastoma” confusing, since this term is usually reserved for malignant tumors. Two recent cases treated at our institution confirm its benign nature. In addition, a review of the literature supports the idea that the tumor may more accurately be described as an “infantile lipoma”. Infantile lipoma better reflects many of the tumor's characteristics such as, its early occurrence, it's ability to mature into a simple lipoma, it's cellular composition of mainly mature adipocytes, and its benign course. Although lipoblastoma is an uncommonly encountered tumor, making an effort to change its name to infantile lipoma will result in a more a accurate term that will facilitate treatment. Accepted: 18 March 1998     

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.     

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.     

Benign chest wall lipoblastoma of infancy producing underlying bone enlargement

We report a case of a chest wall lipoblastoma first presenting in a 6-month-old infant. Clinical presentation was with a posterior chest wall mass. Chest X-ray showed expansion of the posterior ribs on the left, a feature not previously described in this condition. CT scanning delineated a mass of predominantly fat attenuation, which was invading between the ribs to line the extra-pleural space, and was causing compression and atrophy of muscles locally. There were two recurrences requiring further surgery. Histology showed a well-differentiated lipoblastoma.     

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.     

Lipoblastoma of the neck

Lipoblastoma and lipoblastomatosis are benign tumours arising from embryonal fat cells. These rare tumours essentially occur in infancy and early childhood. The males are affected more than females often in the ratio of 3∶1. These tumours usually arise in extremities. The involvement of neck is rare with only 10 cases reported so far in English literature. We herein report lipoblastoma of the neck in a 3 year old girl.     

Diffuse fibromatosis on the scalp in infancy: A variant of juvenile hyaline fibromatosis

Various types of fibromatosis have been reported in infancy and early childhood. We describe an infant with diffuse fibromatosis on the scalp. A one year and five months-old girl showed a diffuse and hard mass 3 × 5 cm in diameter and no tenderness on the scalp. Two months later, the size of the mass had increased and several other tumors appeared on the lateral head. The magnetic resonance imaging (MRI) disclosed that a large and diffuse tumor had spread from the frontal to occipital head; a ‘helmet-like’ configuration of the tumor was exhibited on sagittal MR images. The tumor showed high signal intensity on T2-weighted images and was enhanced with Gd-DTPA. Histological examination showed a fibroblastic proliferation with intervening thick collagen bundles. The patient was diagnosed as having diffuse fibromatosis. The tumor at the resection site immediately recurred, whereas the tumor in the frontal head showed marked regression. Three months after the resection, new tumors appeared in the occipital head. The size and number of these tumors have remained unchanged for more than 18 months. The sites and appearance of the tumors were identical to that of juvenile hyaline fibromatosis (JHF) in this patient. However, JHF usually includes fibroblasts associated with large amounts of hyalinized collagen-like material, which were not present in our patient. The different histology of JHF comparing our case and other reported cases may depend on the different phase of the disease progression at resection. Long-term observation is necessary for the appropriate diagnosis and evaluation of prognosis in this patient.     

Giant pleural fibroma

Solitary fibrous tumors of the pleura are uncommon mesenchymal tumors that have been reported in adults. There are very few cases in children. These tumors are considered benign but their behavior is often unpredictable because they can invade nearby structures and transform into malignant variants after several years. Their clinical presentation is diverse but generally asymptomatic and they are detected incidentally in routine radiologic studies. Prognosis depends firstly on resectability of the tumour and secondly on its size, mitotic count, polymorphism, and necrosis within the tumour. Surgical resection is usually curative. Long-term follow-up is recommended because local recurrences can occur after surgery. We report the case of an asymptomatic 15-year-old girl with a giant thoracic mass revealed incidentally on a chest radiograph. Physical examination revealed thoracic asymmetry and no sounds of breathing were found in the right lung. Computerized tomography and magnetic resonance images showed the giant size of the mass. Preoperative fine needle biopsy was performed. Intraoperative biopsy confirmed the diagnosis. Although histologically benign, the tumors may enlarge rapidly. In the present case, because of the localization and size of the tumor, the patient was at increased risk. The tumour was resected and the postoperative course was uneventful. At present our patient remains under long term annual follow-up.