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We reported a perineal lipoblastoma in a 5-year-old girl located in the right labia mayor. The lesion gradually increased within 6 months. Ultrasound and magnetic resonance imaging (MRI) showed a limited solid heterogenous tumor with fatty component. After it had been surgically removed, the anatomopathological examination confirmed that it was a lipoblastoma. Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization; signs of compression of adjacent organs may be seen. This type of unusual soft tissue tumors occurred most often in under 3 years old. The localization of lipoblastomas is predominantly in the extremities but may be also found in other sites including the head and neck, trunk, mediastinum, kidney, mesentery, retropritoneum and perineum. The suspicion should be considered according to ultrasound and MRI findings., (Sociedad Argentina de Pediatría.)

Lipoblastoma-like tumor of the vulva (LBLTV) was first described as a benign mesenchymal neoplasia; it was not recognized as a separate diagnosis in the 2013 WHO classification of soft-tissue tumors. To date, only 19 cases have been reported. LBLTV differential diagnosis includes other tumors of the vulvoperineal region and tumors with adipocytic differentiation, most of which are benign and thus a misdiagnosis has few clinical consequences. However, LBLTV may also mimic some aggressive lipomatous neoplasms. We describe a case of LBLTV in a 28 year-old woman and review the literature., (Copyright © 2019 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: The hallmark of lipoblastoma is a PLAG1 fusion. PLAG1 protein overexpression has been reported in sporadic PLAG1-rearranged lipoblastomas., Methods: We evaluated the utility of PLAG1 immunohistochemical staining (IHC) in 34 pediatric lipomatous tumors, correlating the results with histology and conventional cytogenetics, FISH and/or next generation sequencing (NGS) results., Results: The study included 24 lipoblastomas, divided into 2 groups designated as "Lipoblastoma 1" with both lipoblastoma histology and PLAG1 rearrangement (n = 16) and "Lipoblastoma 2" with lipoblastoma histology but without PLAG1 cytogenetic rearrangement (n = 8), and 10 lipomas with neither lipoblastoma histology nor a PLAG1 rearrangement. Using the presence of a fusion as the "gold standard" for diagnosing lipoblastoma (Lipoblastoma 1), the sensitivity of PLAG1 IHC was 94%. Using histologic features alone (Lipoblastoma 1 + 2), the sensitivity was 96%. Specificity, as defined by the ability to distinguish lipoma from lipoblastoma, was 100%, as there were no false positives in the lipoma group., Conclusions: Cytogenetics/molecular testing is expensive and may not be ideal for detecting PLAG1 fusions because PLAG1 fusions are often cytogenetically cryptic and NGS panels may not include all partner genes. PLAG1 IHC is an inexpensive surrogate marker of PLAG1 fusions and may be useful in distinguishing lipoblastomas from lipomas.

Background: Lipoblastoma is a rare benign mesenchymal neoplasm of infancy that most commonly occurs on the extremities and trunk but can arise at variable sites of the body. Retroperitoneal lipoblastomas are particularly rare but can grow to enormous size, and preoperative diagnosis is difficult with diverse, mostly malignant differential diagnoses that would lead to aggressive therapy. Since lipoblastoma is a benign tumor that has an excellent prognosis after resection, correct diagnosis is crucial., Case Presentation: A case of a large retroperitoneal tumor of a 24-month old infant that was clinically suspicious of a malignant tumor is presented. Due to proximity to the right kidney, clinically most probably a nephroblastoma or clear cell sarcoma of the kidney was suspected. Radiological findings were ambiguous. Therefore, the mass was biopsied, and histology revealed an adipocytic lesion. Although mostly composed of mature adipocytes, in view of the age of the patient, the differential diagnosis of a (maturing) lipoblastoma was raised, which was supported by molecular analysis demonstrating a HAS2-PLAG1 fusion. The tumor was completely resected, and further histopathological workup led to the final diagnosis of a 13 cm large retroperitoneal maturing lipoblastoma. The child recovered promptly from surgery and showed no evidence of recurrence so far., Conclusion: Although rare, lipoblastoma should be included in the differential diagnoses of retroperitoneal tumors in infants and children, and molecular diagnostic approaches could be a helpful diagnostic adjunct in challenging cases.

Lipoblastomas are benign tumours of immature fat cells presenting in infancy. A lipoblastoma within the airway lumen causing airway compromise is an extremely rare occurrence. We present a 6 hours of life girl who had airway compromise due to a lobulated mass at the base of the tongue. The mass was excised after emergency endotracheal intubation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Lipoblastoma typically occurs in childhood and is associated with rearrangements of the PLAG1 gene. We present a patient with an isolated mass thought to be a lipoblastoma clinically, radiographically, and histologically. The lesion was diagnosed as a PIK3CA-adipose lesion after the tissue was negative for PLAG1 rearrangement and contained a somatic PIK3CA mutation (H1047R). Although PIK3CA variants are associated with PROS (PIK3CA-related overgrowth spectrum), this report illustrates a non-syndromic, lipoblastoma phenotype caused by a PIK3CA mutation., (© 2020 Wiley Periodicals LLC.)

Lipoblastomatosis is an extremely rare condition and is often not recognized prior to final pathologic analysis. In this case, a two-year-old female child presented with a non-tender, submental midline neck mass which had enlarged very slowly over the course of five months. The differential diagnosis included lipoma, lymphatic malformation, and thyroglossal duct cycst and a surgical excision was planned. The child was consented for a possible Sistrunk procedure if necessary. Intraoperatively, the mass was adherent to the hyoid bone and clear mucoid drainage was noted during resection leading the team to further weight the possibility of thyroglossal duct cyst and proceed with a Sistrunk procedure. Ultimately, final pathology revealed the diagnosis of lipoblastomatosis., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Ultrasonography (US) is usually the first imaging examination performed to evaluate palpable or visible superficial soft tissue lesions that are common in children. Although clinical assessments, such as age at presentation, clinical course, and overlying skin discoloration, are important for the differentiation of pediatric soft tissue lesions, US allows a specific diagnosis of some typical benign lesions and helps in guiding further investigation since it provides detailed information about the lesion location, characterization including solid versus cystic, vascularity, and compressibility. Therefore, sufficient knowledge of the normal anatomy, proper ultrasonographic techniques, and the imaging findings of common and uncommon soft tissue lesions in children are crucial for accurate assessment and management of patients. In this article, we review the techniques and imaging findings focusing on the ultrasonographic features of a variety of superficial soft tissue lesions detected in children., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2020 The Korean Society of Radiology.)

Lipoblastoma is a rare benign adipose tissue tumor that occurs mostly in infants and children. Histological diagnosis of lipoblastoma is sometimes difficult because it closely resembles other lipomatous tumors. The detection of PLAG1 gene rearrangement is useful for the diagnosis of lipoblastoma. Four PLAG1 fusion partner genes are known in lipoblastoma: HAS2 at 8q24.1, COL1A2 at 7q22, COL3A1 at 2q32, and RAB2A at 8q12. Herein, we describe a novel fusion gene in a case of lipoblastoma of left back origin. We identified a potential PLAG1 fusion partner using 5' rapid amplification of cDNA ends, and sequence analysis revealed the novel fusion gene, BOC-PLAG1. The BOC-PLAG1 fusion transcript consists of the first exon of the BOC gene fused to exon 2 or exon 3 of the PLAG1 gene. PLAG1 expression was found to be 35.7 ± 2.1 times higher in the tumor specimen than in human adipocytes by qRT-PCR. As a result of the translocation, the constitutively active promoter of BOC leads to PLAG1 overexpression. The identification of the BOC-PLAG1 fusion gene will lead to more accurate diagnosis of lipoblastoma., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Image-guided percutaneous core needle biopsy is a standard and safe procedure for the diagnosis of both solid and hematological malignancies in children. Despite recent improvements, nondiagnosis biopsies persist. Lipoblastoma is a benign adipocytic tumor composed of embryonal fat admixed with mature adipocytes and occurring before the age of 1 year in one-third of cases. Lipoblastoma is usually easily diagnosed, but in some cases, diagnosis may be difficult on percutaneous biopsies, when the lipoblastic component is not well represented or when the tumor contains a prominent myxoid component mimicking other myxoid tumors. We report here a case of lipoblastoma with a predominant myxoid component and discuss differential diagnosis of myxoid lesions of infancy. In such cases, pathologic examination enhanced by adjunct techniques, such as immunohistochemistry and cytogenetic or molecular genetic studies, is needed to achieve accurate diagnosis, particularly on fine-needle biopsies.

Lipoblastoma is a benign adipose tumor typically presenting in infancy in superficial soft tissues of extremities. Intestinal complications secondary to intraabdominal or retroperitoneal involvement are exceedingly rare. We describe a unique case of a primary intestinal lipoblastoma arising from the submucosa of the transverse colon in an otherwise healthy 18-month-old boy. He presented with a history of reducible rectal prolapse, rectal bleeding, and episodic abdominal pain and was initially treated for constipation. Imaging identified a short colo-colonic intussusception, confirmed at laparotomy, and a fatty mass thought to arise from the mesentery. Pathological examination of the resected transverse colon revealed a submucosal tumor composed of a mixture of mature adipose tissue, foci of myxoid mesenchymal tissue with desmin positive, HMGA2 negative spindle cells, and scattered lipoblasts, characteristic of lipoblastoma. Lipoblastoma should be considered as a potential albeit rare cause of intussusception in young children, where a pathologic lead point is infrequently identified.

Competing Interests: Competing interests: None declared.

Post-zygotic activating mutations in PIK3CA and other genes encoding members of PI3K-AKT-mTOR pathway have been found in various overgrowth syndromes that have been grouped together as PIK3CA-related overgrowth spectrum (PROS). We report a female patient with gait disturbance, leg pain, isolated macrodactyly of the foot, and mild intellectual disability. Imaging of the lower limb showed a lipoblastoma of the right thigh. A mosaic gain-of-function mutation in the catalytic domain of PIK3CA (c.3140 A > G; p.His1047Arg) was detected in the adipose tissue and in skin cultured fibroblasts from the macrodactyly but not in blood. The leg pain and the severe walking disturbance improved slightly over time and serial MRI of the lower limbs suggested that the size of the lipoblastoma relative to the lower limb muscles or to the whole lower limb was unchanged as consequence of limb growth. This case report illustrates that pain and gait disturbance can be features of PROS and highlights the need of better knowledge about the natural history of the disease., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Lipoblastoma is a rare tumor that is not commonly seen in the orbit. The authors present clinical features, histopathologic findings, changes in the radiologic findings over time, and the radiological-pathological correlation of orbital lipoblastoma in an infant. A 3-month-old male infant presented with a palpable mass on the left upper eyelid. The patient was observed for 1 year with magnetic resonance imaging. At the age of 15 months, the patient underwent excisional biopsy. Histopathologic examination showed features of hypocellular lobules with a mixture of adipocytes of various stages of maturity and myxoid stroma separated by prominent fibrous septa, confirming a diagnosis of orbital lipoblastoma. [J Pediatr Ophthalmol Strabismus. 2017;54:e67-e70.]., (Copyright 2017, SLACK Incorporated.)

Lipoblastoma is a relatively rare benign tumor. It derives from embryonic white fat cells. It almost exclusively affects children less than 3 years of age. We report a case of lipoblastoma of the left thigh detected in an older child (11 years) and a literature review. Diagnosis was based on histology while surgical treatment was based on total resection of the mass. The postoperative course was simple with a follow-up period of 9 months.

Background: Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization, and signs of compression of adjacent organs may be seen., Methods: A total of 12 children with pathologically proven lipoblastoma from 2000 to 2014 were reviewed retrospectively for their clinical features, treatment and follow up., Results: There were seven boys and five girls between 7.5 months and 7.5 years of age. The most common symptom was painless rapid-growing mass. The tumors were located in the neck, the abdomen, the groin, pelvis, axilla, glutea, labium majus, thigh and trunk. The largest tumor was in a retroperitoneal location and was 13 × 10 cm in size. Complete resection was performed in 10 patients. All of these patients were followed without any evidence of recurrence. Subtotal excision was performed in two patients. At a median follow up of 38 months (range, 1.8 months-10 years), all patients were disease free., Conclusions: Lipoblastoma should be considered in the differential diagnosis of rapidly growing mass in patients <3 years of age. Total excision is the treatment of choice with preservation of vital organs. The prognosis is excellent despite large tumor size and local invasion. Recurrence rate is high in incompletely resected tumors. Regular follow up is important for early detection of recurrence., (© 2017 Japan Pediatric Society.)

A 1-year-old boy with no underlying disorder presented with non-bilious vomiting since 4 days before admission. He was referred to our hospital and was diagnosed with a small bowel obstruction due to an intraabdominal tumor. Laparotomy revealed an intestinal volvulus with a soft and lobulated tumor arising from the mesentery. The resected tumor with a small part of the small bowel was diagnosed as lipoblastoma histologically. From a literature review, mesenteric lipoblastoma with an intestinal volvulus showed different characteristics such as greater frequency of vomiting and less frequency of abdominal mass as clinical symptoms, and the size of the tumor was smaller than that of the tumor without the intestinal volvulus., (Copyright © 2013. Published by Elsevier Taiwan.)

Introduction: Lipoblastoma is a benign neoplasia of the adipose tissue. It is a rare conditionand almost exclusively presents in children under 3 years old. It usually occurs in extremities as a painless volume increase of progressive growth, with the definitive diagnosis being established by pathological and cytogenetic analysis. The treatment of choice is complete resection, and follow-up period of up to five years is recommended due to a recurrence of up to 25%., Objective: To present an unusual location of this uncommon condition in an infant, and review the related literature., Case Report: A sixteen-month child with an increase in abdominal growth of six-months progression, associated with a decreased food intake, and with no other symptoms. The imaging study revealed a lipoid-like image compromising almost the entire abdominal cavity, very suggestive of lipoblastoma. A resection was performed on an 18cm diameter retroperitoneal tumour that rejected the adjacent organs. Histological analysis was enough to confirm diagnosis without the need for cytogenetic analysis. The follow-up showed no recurrence of the disease., Conclusion: Given the rarity of this disease and its unusual presentation, we communicate this clinical case, in order to be considered in the differential diagnosis of abdominal mass in chilhood., (Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)

Patients presenting with enlarging fibrofatty masses in the extremities pose an interesting dilemma to clinicians, as the differential diagnosis in such cases ranges from benign to malignant, and from lesions optimally managed operatively to those managed nonoperatively. The differential diagnosis includes benign lipoma, liposarcoma, lipoblastoma, and fibrolipomatous hamartoma (lipomatosis) of the nerves. The authors present the case of a 14-year-old girl with an enlarging fibrofatty mass of the forearm, initially thought, based on diagnostic imaging, to be a fibrolipomatous hamartoma of the median nerve, but found to be a lipoblastoma without direct nerve involvement based on histopathological examination of the operative specimen. This case serves to illustrate the diagnostic predicament that can exist with such masses. The authors advocate the need to establish a tissue diagnosis while having a contingency plan for each of the diagnostic possibilities because the management of each lesion is markedly different. In this report, the authors consider the differential diagnosis of fibrofatty masses of the extremities that the peripheral nerve surgeon may encounter, and they highlight the significant differences in management strategies for each possible diagnosis.

Objective: Lipoblastoma(toses) are benign neoplasms of embryonic white fat in a state of arrested maturation, and are seldom encountered in the head-neck region. We discuss the clinical details of lipoblastomatosis in the retropharyngeal space of a 9-year-old boy,with an emphasis on the head-neck area, the histopathological maturity of the tumor, and the practical roles of imaging, cytogenetics, and immunostaining in the diagnosis., Methods: Clinical record analysis; literature review., Results: A malnourished child presented with worsening respiratory distress and feeding difficulties. Imaging suggested a large, heterogeneous fibrofatty lesion in the retropharyngeal space. Surgery revealed an otherwise encapsulated mass that densely adhered to the pre-vertebral soft tissue. The findings were consistent with a diagnosis of maturing lipoblastomatosis on histopathology, which was supported by additional immunohistochemistry panel analysis., Conclusion: This is the first report of lipoblastomatosis with the retropharyngeal space as the epicenter of involvement/ origin. Although rare and seldom diagnosed before surgery, lipoblastoma(tosis) may be more common in the head-neck area than thought previously and should be an important differential diagnosis for pediatric fibrolipomatous neoplasms in this anatomic subsite.

Lipoblastoma is a rare fatty tumor that is diagnosed almost exclusively in children. Presentation often consists of respiratory symptoms; chest computed tomography shows a hypodense, low, attenuated mediastinal mass. Surgical approach and anesthetic management are dependent on the location of the tumor and the degree of airway compression; in most cases, a thoracotomy is performed, although a sternotomy is used in selected cases. Final diagnosis can be confirmed using molecular genetic analysis; a genetic hallmark of lipoblastoma is the rearrangement of chromosomal region 8q12 and the PLAG1 gene. Tumor recurrence is rare when a complete resection is performed., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Lipoblastoma is extremely rare and mainly occurs in children younger than 3 years old. It is predominantly found in the extremities and trunk. Head and neck region occurrences are rare; only 4 such cases involving patients who presented with stridor have been previously reported. We report the fifth case of lipoblastoma of the neck with stridor in a 9-year-old girl, which had gradually worsened over the previous year. Imaging showed a retrotracheal mass extending superiorly to the thyroid level and inferiorly to below the carina of the trachea. Total resection of the tumor was performed, and the histopathologic findings were consistent with lipoblastoma. Postoperatively, the patient was well with no complications.

Adipocytic tumors are the most common mesenchymal neoplasms, liposarcoma accounting for approximately 20% of soft tissue sarcomas. The differential diagnosis between benign and malignant tumors is often problematic and represents a significant proportion of consultation cases. The goal of this article is to review liposarcoma subtypes, the main benign adipocytic neoplasms: lipoblastoma, hibernoma, spindle/pleomorphic cell lipoma, chondroid lipoma, as well as non adipocytic neoplasms with a lipomatous component such as lipomatous solitary fibrous tumor, emphasizing on practical differential diagnosis issues, and immunohistochemical and molecular tools allowing their resolution., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Lipoblastoma is a rapidly growing, benign neoplasm in children. Surgical excision is usually curative, with a recurrence rate of about 20%. Because the histology of lipoblastoma is heterogeneous and overlaps with other lipomatous tumors, some lipoblastoma cases have been difficult to diagnose. The detection of PLAG1 gene rearrangement is useful for the diagnosis of lipoblastoma. Three fusion partner genes are known in relation to PLAG1 in lipoblastoma HAS2 at 8q24.1, COL1A2 at 7q22, and RAD51L1 at 14q24. Herein, we describe another two novel fusion genes in lipoblastoma tumor specimens. We checked six tumors for the presence of two known fusion genes, HAS2-PLAG1 and COL1A2-PLAG1. Only HAS2-PLAG1 was found in one of the cases. Next, we attempted to identify potential PLAG1 fusion partners using 5'RACE. Sequence analysis revealed two novel fusion genes, COL3A1-PLAG1 in three cases and RAB2A-PLAG1 in one case, respectively. As a result of the translocations, the constitutively active promoter of the partner gene drives the ectopic expression of PLAG1. We also evaluated whether a high level of PLAG1 expression can be used to help differentiate lipomatous tumors. PLAG1 expression was evaluated by real-time PCR in five lipoblastoma tumor specimens. The expressions were 70-150 times higher in lipoblastomas than in human adipocytes. However, PLAG1 expression was low in one case of lipoma. These results demonstrate that PLAG1 overexpression is a potential marker of lipoblastoma. Our findings, in agreement with previous studies, show that lipoblastoma is a group of lipomatous tumors with PLAG1 rearrangement and overexpression. © 2014 Wiley Periodicals, Inc., (Copyright © 2014 Wiley Periodicals, Inc.)

Lipoblastoma is a benign soft-tissue tumour of infancy and early childhood. The location of the tumour varies from that of the extremities, trunk, retroperitoneum, mediastinal, inguinal and scrotal regions. The most common symptoms are painless mass with or without increasing size. Complete surgical excision is the gold standard for managing this entity. Though rare, the recurrence occurs usually because of incomplete excision. We report a case of recurrent lipoblastoma of the upper extremity in a 9-year-old boy.

Aims: Lipoblastoma is a benign neoplasm of embryonic white fat tissue that results from the proliferation of primitive adipocytes, in which histological features can be ambiguous. In order to discriminate between lipoblastoma and other lipogenic and lipomatous tumours, we studied chromosomal alterations and protein expression in two cases of lipoblastoma in infants., Methods and Results: Standard cytogenetic analysis, fluorescence in-situ hybridization, array comparative genomic hybridization and Western blotting allowed us to demonstrate the presence of chromosome abnormalities involving the 8q11-13 region containing the pleomorphic adenoma gene 1 (PLAG1), which are classically reported in lipoblastoma, and aberrant expression of PLAG1., Conclusions: This report illustrates two different tumorigenic pathways implicating PLAG1 in lipoblastoma: amplification through multiple copies of a small marker chromosome derived from chromosome 8, and a paracentric inversion of the long arm of chromosome 8. Both these anomalies induced aberrant expression of PLAG1, emphasizing the role of PLAG1 in tumorigenesis. The aberrant expression of PLAG1 protein has been hypothesized, but this is the first report to demonstrate its occurrence in lipoblastoma., (© 2013 John Wiley & Sons Ltd.)

Background: Lipoblastoma is a rare benign mesenchymal tumour of embryonal fat that occurs almost exclusively in infants and children. This determined the epidemiological, clinical and therapeutic aspect of this disease., Materials and Methods: A total of 10 cases of pathologically proven lipoblastoma from 2003 to 2012 were reviewed., Results: There were six boys and four girls ranging in age from 7 months to 9 years. A soft-tissue mass was the main complain in nine patients. The various locations of the mass were mediastinal, thigh, buttock, inguino-scrotal, the greater omentum and the Latissimus dorsi. Lesions measured 5-15 cm. complete excision was done. The median time of follow-up was 42 months (ranges between 18 and 84 months). There were no recurrences., Conclusion: It is important to consider lipoblastoma in the diagnosis of a rapidly enlarging fatty mass in children. Complete resection is the only definitive treatment and should not be delayed when impingement on surrounding structures is imminent. There is a tendency for these lesions to recur despite presumed complete excision. Therefore, follow-up for a minimum of 5 years is recommended.

A rapidly growing soft tissue mass in the axilla of an infant raises the suspicion of a lipoblastoma or a liposarcoma. Excisional/incisional biopsy is vital in confirming the diagnosis and hence avoiding aggressive extirpation. This case report highlights the role of histopathology and immunohistochemistry as the gold standard in differentiating a lipoblastoma from a liposarcoma. In some cases where the histopathology is inconclusive, genetic rearrangement of the PLAG1 (pleomorphic adenoma gene 1) oncogene on chromosome 8q12 helps in confirming the diagnosis of lipoblastoma.

A 2-year-old boy was presented with symptoms of chest infection. The chest radiograph showed a large mediastinal mass, which led to further investigations including biopsy of the tumour. Histopathological analysis revealed a diagnosis of lipoblastoma. We highlight the imaging appearance of the lesion. Although histopathological analysis is required for the confirmation of the diagnosis, cross-sectional imaging is useful in evaluating the extent of the tumour for surgical planning.

The objective of the present work was to overview current concepts of epidemiology and clinical picture of a rare benign neoplasm, lipoblastoma, known to occur in the children, to report an original observation of one case of this condition, and to discuss specific features of its surgical treatment in the patient presenting with a rare localization of the tumour in the neck region extending onto certain vitally important anatomical features. Diagnosis of lipoblastoma of rare localization on the neck was verified in a 20-month old child. The necessity of differential diagnostics of lipoblastoma from other tumours (including malignant ones) in the neck region is substantiated along with the approaches to its surgical treatment bearing in mind the difficult of access localization of the tumour. It is concluded that the benign character of this neoplasm predetermines the favourable outcome of its treatment; however, there is a risk of incomplete dissection of the tumour and its relapse.

Background: Lipoblastoma is a rare, benign, and encapsulated tumor arising from embryonic white fat. Most of the cases occur in the extremities and the trunk; only a few cases in the head and the neck are reported. Thus, we present a case of lipoblastoma of the neck with a review of the literature., Patient and Method: A 1-year-old male infant presented to our hospital, with a history of painless swelling in the left side of the neck for 3 months that was rapidly enlarged. His birth history and medical history were unremarkable. A physical examination demonstrated a soft and compressible mass in the left side of the neck. The mass was nontender to palpation and mobile without cellulitic changes in the overlying skin. A computed tomographic scan showed that the mass is heterogenous, has low attenuation in nature, and is 3.8 × 2.8 × 9 cm in size., Result: Under general anesthesia, transverse cervical incision was made through the neck wrinkle, and there was no invasion of any of the neck structures. Complete surgical excision demonstrated yellowish-white, irregular lobules of immature fat cells separated by a loose and myxoid connective tissue. Grossly, the mass was a homogeneous tan-pink gelatinous mass. A microscopic examination demonstrated a small number of capillaries and mature fat cells, and differentiating immature lipoblastoma cells were detected in the myxoid stroma. A pathologic finding confirmed the diagnosis of lipoblastoma. The postoperative course was uneventful. The patient underwent follow-up for 1 year after the operation, and there was no evidence of recurrence., Conclusions: The most common presentation of lipoblastoma is a painless, rapidly enlarging neck mass. Published reports showed that most of them occur before the age of 3 years. Complete surgical excision is the treatment of choice. Although lipoblastoma is an extremely rare benign tumor, it should be considered in the diagnosis of neck mass in children younger than 3 years.

We report a case of giant omental lipoblastoma in a 13-month-old boy, which was treated successfully by total excision. Tumor cells were positive for S100, CD34 and CD56. This is the first report of lipoblastoma expressing CD56, a fact that could be used to differentiate lipoblastoma from liposarcoma.

Lipoblastoma is a rare benign neoplasm found exclusively in the pediatric population that can occur anywhere in the body, most commonly seen in the extremities but also found in the face. We report an 8-month-old female subject who presented with multifocal soft tissue masses of the face. The diagnosis of lipoblastoma was made in 2 separate locations after surgical resection. Subsequent surgery was performed at the cheek site in an attempt to remove further mass, resulting in discovery of 2 other discrete tumors found to be lipoblastomas. Although the literature reports recurrence rates ranging from 14% to 27%, to our knowledge, aside from a case of Proteus syndrome, there are no known reports of multiple lipoblastomas in the literature. Presentation of the case, review of pertinent literature, and consideration of congenital infiltrative lipomatosis of the face follow.

Lipoblastoma is a rare benign tumour arising from embryonic white fat. The tumours occur primarily in infancy and early childhood and usually arise from the limbs and the trunk, but neck involvement is rare. We report three cases of head and neck lipoblastoma. In all cases, imaging showed a well-delineated, fat-containing tumour. After surgical resection, the outcome of these patients was uneventful.

Retroperitoneal lipoblastomas are rare tumors found in young pediatric patients. Despite their large size at presentation, complete resection is usually achieved with minimal complications. Diagnosis is typically made after pathologic examination of the operative specimen, and cytogenetic analysis may be a key component in differentiating lipoblastoma from other lipomatous tumors. We present one such case of a large, retroperitoneal lipoblastoma and discuss the management of this uncommon entity., (Copyright © 2012 Elsevier Inc. All rights reserved.)

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.