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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.)

Background: Lipoblastoma is a rare and benign tumor arising from embryonal fat cells. It develops primarily in infancy and early childhood. We present a case study of a 10-month-old child treated for an extensive tumor of the infratemporal fossa and parapharyngeal space. The systematic review of 60 reports in 80 children with lipoblastoma of the head and neck is also reported., Material and Methods: Systematic review of all articles included lipoblastomas of the head and neck area in children published from 1964 to 2022 in the PubMed database was conducted. Clinical presentation of extensive lipoblastoma of the head and neck in a child., Results: On the basis of all inclusion criteria 83 articles were identified concerning pediatric lipoblastoma. There were 36 F (45%) and 39 M (48%), in 6 patients, (7%) gender was not specified. Ages ranged from 0mth (6h) to 15 yo (180mth). The tumor was located in the head in 22 (27%) cases, while the neck area accounted for 53 (65%) cases. General description without precise location was shown in 6 (7%) cases. All patients underwent complete surgical excision. Post surgical recurrence was noted in 6% clinical cases in the analyzed articles., Conclusions: Lipoblastoma is characterized by a rapidly growing predominantly adipose mass. The treatment of choice is complete surgical excision. In selected cases when the pathology is hard to reach, as a consequence of the extensive penetration of the mass, we recommend performing the procedure with an interdisciplinary team. Endoscopy assistance and microdebrider significantly facilitated the removal of pathology in the described advanced case of lipoblastoma. This is the only case reported in the pediatric literature of a head and neck lipoblastoma, where due to extensive involvement and location of the disease the cranial nerves V2 and inferior alveolar branch of V3 could not be spared. Long-term follow-up even to 10 years is required because there is a reported tendency for these tumors to recur., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Lipoblastomatosis of mesentery is an extremely rare benign fat-containing mesenchymal tumour in infants and young children. On imaging, it appears as a solid infiltrating mass interspersed with macroscopic fat. We describe the distinctive imaging features of a large mesenteric Lipoblastomatosis, corroborated with intraoperative and histopathological features. We hope the case report and short review about this rare entity will increase the diagnostic confidence of reading radiologists while formulating differentials for lesions of similar appearance in the paediatric age group., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Lipoblastoma-like tumours (LLT) are uncommon and their biologic nature is not well-understood. Complete resection of the primary lesion is usually curative. This is the first reported case of metastatic LLT., Case Presentation and Management: This patient originally had a large primary resected from the vulva. She subsequently developed tumours in multiple areas including her right pleura and lung. These were resected and pathology was consistent with LLT. On follow-up imaging, there was evidence of metastatic disease., Conclusion: The patient is currently undergoing detailed molecular analysis in the hope of detecting a molecular target. Given the infrequent occurrence of LLT, its behaviour is not well understood. Longer duration of follow up is required and prognostic markers need to be identified to better direct treatment., (© 2022 Royal Australasian College of Surgeons.)

Background: Lipoblastoma is a rare benign tumor of infancy and childhood, with similar radiographic imaging findings as malignant liposarcoma. Therefore, it is crucial to distinguish through other diagnostics and clinical features. We present our experience with the diagnosis and clinical management of this entity., Methods: A retrospective chart review was performed to identify children treated for lipoblastoma at our institution over a 30-year period. Charts were evaluated for diagnostic methods, treatment, and recurrence risk., Results: A total of 9 males and 3 females were identified. The age at diagnosis ranged from 8 months to 44 years. Imaging modalities employed included ultrasound (N = 5), MRI (N = 12), X-ray (N = 8), and CT (N = 3). The tumor location was reported in the lower extremity (N = 8) and upper extremity (N = 4). The tumor size ranged from 1.7 to 18 cm (mean=7.08 cm). All patients underwent complete resection. Available pathology reports noted masses featuring mature adipocytes (N = 1), fibrous septa (N = 2), focal myxoid changes (N = 1), and spindle cells (N = 2). Nine patients underwent chromosomal analysis, which demonstrated five chromosome 8 abnormalities. No recurrences were reported. Three patients had follow-up imaging, and 11 had follow-up visits between 2 weeks to 15 years., Conclusion: Lipoblastoma is an uncommon childhood neoplasm of embryonic white fat that can also be present in adolescence and adulthood. Imaging, especially MRI, is helpful in limiting the differential diagnosis, but definitive diagnosis requires tissue biopsy, which should be obtained with core biopsy rather than fine needle aspiration. Surgical resection is curative with few complications. We do not recommend routine monitoring as recurrence is unlikely., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Lipoblastoma is a rare benign tumor originating from adipose tissue, usually seen in infancy and early childhood. Here, we present a case of scalp lipoblastoma in a 4-month-old that we treated surgically and review the literature. Although lipoblastomas may be seen in various locations during infancy and early childhood, rarely, they can also develop in the scalp., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Lipoblastoma is a rare benign mesenchymal tumour derived from embryonic white fat tissue. Lipoblastoma generally occurs in children less than 3 years of age, and fewer than 200 cases have been reported in the English literature. There are hardly a dozen reports describing intrathoracic and thoracic involvement. We report a case of a 7-year-old boy who presented with a slowly growing right thoracic wall mass for a duration of 1 year. Radiological investigations (ultrasonography and contrast-enhanced CT of the chest showed the presence of a fat-containing, low density extrapulmonary soft tissue mass) could not confirm the diagnosis, and repeat biopsies also were not conclusive. The mass was excised and histopathology confirmed it as lipoblastoma. A review of literature pertaining to the clinical presentation, radiological features and histopathology of this rare condition has been discussed., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Lipoblastoma is a rare benign tumor of primitive white fat in children, which usually involves the trunk and extremities. It rarely involves retroperitoneum, head, neck, and mediastinum. Herein, the authors introduce a rare presentation of lipoblastoma as a large intrathoracic mass.

Lipoblastoma is a rare and benign tumour arising from embryonal fat cells, predominantly diagnosed in children younger than 3 years old. The most frequent locations are the extremities and trunk, while the head and neck areas are more rarely affected (10-15% of total cases). Clinically, the most common presentation is a fast-growing painless mass. Ultrasound is the first-line imaging examination, but Magnetic Resonance Imaging (MRI) allows for better definition of the relationships with the adjacent vascular and muscular structures. It can help to identify the lipomatous components, and it is useful for preoperative planning. However, the definitive diagnosis is provided by histopathological examination. Complete surgical excision is the first-line treatment, with a good prognosis in case of total eradication. We report the case of a 7-month-old male child with a rapidly growing mass that had typical radiological features of lipoblastoma., (© 2020. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

Lipoblastoma-like tumor is a very rare mesenchymal tumor believed to be restricted to female patients and only recently reported in the spermatic cord of a male patient. We describe herein an additional case of lipoblastoma-like tumor occurring in the spermatic cord, describing its histopathological, immunohistochemical, and molecular features.

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 and liposarcomas are rare causes of soft tissue masses in paediatric patients. In this retrospective clinical case series we identified 11 patients from our paediatric database (10 with a lipoblastoma and one with a liposarcoma) who had attended our hospital between 1998 and 2019. The median age of patients with lipoblastoma was 29 months. All lipoblastoma cases were managed with surgical excision and histological examination. The 18-year old patient with liposarcoma presented with a metastatic and unresectable tumour that was unresponsive to chemotherapy and radiation. Our experience demonstrates the importance of differentiating the type of soft tissue mass in children.

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.)

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.)

Lipoblastomas can occasionally require further molecular confirmation when occurring outside of the usual age groups or demonstrating unusual morphology. We reviewed 28 lipoblastomas with 16 controls. Lipoblastomas were subdivided into myxoid (n = 7), classic (n = 9), or lipoma-like (n = 12) subtypes. PLAG1 immunohistochemistry, PLAG1 fluorescence in situ hybridization (FISH), and targeted RNA sequencing were performed on formalin-fixed paraffin-embedded tissue. Karyotypes were available in a subset of lipoblastomas (n = 9). Gene rearrangements were identified in 17/25 (68%) lipoblastomas, including PLAG1 (15/25, 60%) and HMGA2 (2/25, 8%). Five novel fusion partners (DDX6, KLF10, and KANSL1L with PLAG1 and EP400 and FGD6 with HMGA2) were found. PLAG1 immunohistochemistry was positive (nuclear, moderate/strong) in myxoid and classic subtypes lipoblastomas with preferential expression in mesenchymal cells within myxoid stroma and fibrous septa and negative in all controls. When comparing PLAG1 immunohistochemistry with molecular testing (FISH and/or RNA sequencing and/or karyotype), concordant results were noted in 13/25 (52%) cases, increasing to 15/25 (60%) after slight adjustment of the PLAG1 FISH positive threshold. In myxoid and classic lipoblastomas, PLAG1 immunohistochemistry seems to be a better surrogate marker for PLAG1 rearrangement, as compared with lipoma-like subtypes. In lipoma-like subtypes, targeted RNA sequencing appears to detect PLAG1 fusions better than FISH and immunohistochemistry. The preferential expression of PLAG1 in the mesenchymal and fibroblast-like cells deserves further investigation as the putative cell of origin in lipoblastoma., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To describe two cases of mediastinal lipoblastoma, an infrequent and little-known patho logy, which is extremely rare in the mediastinum, with no cases reported in our country., Clinical Cases: Two case reports. Both patients were boys younger than three years, in which a mediastinal mass was found incidentally on a chest x-ray. The study was complemented with a CT scan and with a thoracoscopic biopsy in one of the cases. Complete resection of the tumor was achieved in both patients through thoracotomy. One of the patients presented Claude Bernard Horner syndrome as a complication from surgery, which resolved spontaneously after two years and the second case had no complications., Conclusion: Mediastinal lipoblastoma is a very rare pathology. Descriptions found in existing literature are similar to the cases presented in this article. We can conclude that mediastinal lipoblastomas in pediatrics present a very similar pattern and presentation, having a good prognosis if complete resection is achieved. Also, it is essential to distinguish it from its differential diagnoses in order to rule out malignancy.

Purpose: The objective of this study was to examine the long-term outcomes of pediatric patients who underwent surgical resection for lipoblastoma and lipoblastomatosis (LB/LBM)., Methods: A single-center retrospective study of pediatric patients with LB/LBMs seen between 1991 and 2015 was conducted. A systematic review, including studies published prior to late August 2018, was performed. Using a random effect meta-analysis, pooled weighted proportions and unadjusted odds ratios (OR) with 95% confidence intervals (CI) were calculated., Results: The retrospective study included 16 patients, while the systematic review included 19 published studies consisting of 381 patients. Among 329 (82%) patients with follow-up information, the pooled recurrence rate was 16.8% (95% CI 10.9-23.5%; I 2  = 59%). The reported time to recurrence ranged from < 1 to 8 years. Recurrence risk was greater for incomplete (n = 34) than complete resection (n = 150): OR 11.4 (95% CI 3.0-43.6; I 2  = 43%). LBMs (n = 35) had a greater recurrence risk than LBs (n = 116): OR 5.5 (95% CI 1.9-15.9; I 2  = 0%). Recurrences were higher for studies with approximately ≥ 3 years of follow-up versus studies with < 3 years of follow-up., Conclusion: Recurrences are more likely to occur with LBMs and/or incomplete resection. Follow-up beyond 3-5 years should be considered given that the recurrence risk appears to be greater in the long-term.

The article describes diagnostics and surgical treatment of a rare benign tumor (lipoblastoma) of four anatomical areas of the head and neck in an 8-months child. Diagnostic data, surgical procedure performed using microsurgical techniques and intraoperative neuromonitoring and the patient's condition in the long-term postoperative period are presented. The histological conclusion about the removal of the tumor with negative resection margins suggests favorable prognosis of the disease.

This case report describes the case of a 37-year-old man that noticed an intrascrotal right mass with 1 month of evolution. During physical exam presented with a large mass at the inferior portion of the right testicle, clearly separated from the testicle, with a tender consistency and mobile. An ultrasound was performed that showed a solid and subcutaneous nodular lesion, extra testicular, heterogeneous, measuring 7.2 cm. Pelvic magnetic resonance imageMRI showed a lesion compatible with a lipoma. The patient was subjected to surgical excision of the lesion by scrotal access, having histology revealed a lipoblastoma (LB) of the scrotum. Histological diagnosis was obtained by microscopic characteristics (well-circumscribed fatty neoplasm) and immunohistochemistry (stains for CD34, S100 protein and PLAG1 were positive; stains for MDM2 and CDK4 were negative). LB is extremely rare after adolescence in any location, being this first described case of intrascrotal LB described in adulthood., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

This case demonstrates successful resection of a rare, recurrent presacral-pelvic lipoblastoma in a 19-year-old female patient. Because of the anatomical location of the mass and its proximity to vital structures, the robotic approach allowed for both optimal visualization and effective debulking of the mass. Furthermore, with the use of an articulating laparoscopic camera, key visualization of the posterior lateral pelvis was possible. Using a wide breadth of technologies and resources is essential to broadening the surgical armamentarium and achieving resectability in otherwise challenging cases., (© 2018 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.)

Competing Interests: Competing interests: None declared.

Introduction: Diffuse or massive tumors involving the perineal and pelvic compartments may require aggressive surgical treatment in children. The authors, propose and present their results using a Posterior Sagittal Anorectal Mobilization (PSAM) METHODS: The oncologic patient is placed in a prone position with the pelvis elevated, without a previous colostomy. A 10 Ch bladder catheter is positioned. A midline sagittal incision is performed from the coccyx to the posterior margin of the anus. Fine electrocautery is used to obtain an accurate dissection all around the anal margin itself. The incisional so involves the midline dissection into the perineal body. Reconstruction is achieved by the classical pelvic-perineal anatomical structure approximation., Results: The authors describe for the first time a novel posterior anorectal mobilization technique for four children with pelvic-perineal tumors. No intraoperative or post-operative complications occurred. The oldest boy presented at follow-up a complete normal faecal and urinary continence (Krickenbeck criteria)., Conclusion: The presented technical approach, defined as PSAM, is completely suitable for surgical resection of perineal-pelvic tumors, without the need of more invasive operations including transpubic dissection and/or anorectal split. Moreover, nervous and muscular sphincter structures are easily identified and preserved., (© 2018 Wiley Periodicals, Inc.)

Competing Interests: Competing interests: None declared.

Lipoblastoma is a rare and benign tumor arising from embryonal fat cells. It is generally diagnosed in children younger than 3 years of age and can occur in the extremities or on the trunk. We present our series of 10 children with lipoblastoma treated at Schneider Children's Medical Center of Israel between 2011 and 2016. Six boys and four girls underwent tumor resection at a median age of 2 years and 3 months (range 5 months to 5.6 years). Locations were trunk (6), groin (2), perineum (1), and omentum (1). Follow up ranges from 1 to 5 years. Two patients had a local recurrence and required a second resection 2 years (perineal) and 6 years (trunk) after the first surgery without further recurrence at 1.9 and 2.9 years, respectively., Conclusion: Higher awareness of lipoblastoma enables optimal imaging strategies and resection. Long follow up is required due to local recurrences. The treatment of choice consists of complete, but non mutilating surgical resection. What is Known: • Lipoblastoma is a rare benign tumor of fatty tissue affecting children • Treatment consists of surgical resection What is New: • MRI is the modality of choice for follow up • Ten-year long-term follow up is required due to late recurrence.

Objective: Lipoblastoma is a rare neoplasm that occurs mostly in infants and children. Although benign, it has a tendency for local recurrence., Results: Clinical and pathological features of 23 cases of lipoblastoma described. Patients' age ranged from 8 months to 18 years with mean and median age 4.1 and 2.5 years, respectively. Male:female ratio was 2.8:1. Most common sites were lower extremities (9 cases), followed by abdominal cavity and retroperitoneum (4 cases), and scrotum/groin (3 cases). Grossly, 22 tumors were well circumscribed and multi nodular. All cases showed lobules composed of adipocytes and lipoblasts with intervening fibrous septa and fine vascular network. Myxoid change, capsule formation and septation were seen in all cases. Zonation was seen in 2 cases. Follow-up was available in 14 out of 23 patients. Of these, 13 were alive and free of disease with no evidence of any recurrent lesion. One patient with a mediastinal infiltrating lipoblastoma experienced 4 recurrences. Lipoblastoma is a benign adipocytic neoplasm of infants and young children. Correlation of clinical and histological features helps in reaching a correct diagnosis. Owing to a high recurrence rate following incomplete resection, a complete resection is essential. Prognosis is excellent after complete resection.

Background: Lipoblastomas are rare benign tumors that arise from embryonic white fat and almost always occur in babies and children. Here, we report a case of a giant popliteal lipoblastoma in a 23-month-old Japanese girl that was successfully treated via complete resection., Case Presentation: Our patient was a 23-month-old Japanese girl. At 6 months of age, she presented at a nearby hospital with a mass on the popliteal side of her lower right leg. She had no symptoms and was diagnosed as having a benign adipose tumor via magnetic resonance imaging. The mass gradually increased in size, and she was referred to our hospital at 1 year and 11 months of age. A physical examination and radiology revealed a localized mass 13 × 10 × 7 cm in size in the aforementioned area that restricted knee movement and caused proximal tibia deformity. Magnetic resonance imaging showed a giant circumscribed subcutaneous mass with multiple partitions that was hyperintense on T1-weighted and T2-weighted images but not fat-saturated on T2-weighted images. Based on these findings, she was diagnosed as having a lipoblastoma. Because the mass surrounded her popliteal artery and vein and part of the popliteal nerve, surgical resection was considered risky, and we opted to simply observe her. However, owing to rapid growth of the mass and the worsening of symptoms, she underwent complete resection at 2 years and 6 months of age. A histological examination confirmed the diagnosis of a lipoblastoma. She was discharged from our hospital 3 days after surgery with no symptoms. She could walk without pain at the 6-month follow-up, and no local recurrence was observed., Conclusions: We successfully treated a giant popliteal lipoblastoma without complications by performing a total resection. Our report provides evidence that lipoblastomas should be considered for surgical resection when they progress or symptoms appear.

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.)

Introduction: Lipoblastomas are benign tumors that arise from the proliferation of fetal adipose tissue. They are frequently found in the extremities, in the head, and in the neck. Dumbbell conformation (which refers to epidural tumors with extraspinal extension) has been described in only seven lipoblastoma cases so far., Case Report: We describe an 11-month-old patient with a large mediastinal dumbbell lipoblastoma. The mass was excised, but it recurred 1 year later when the MRI showed also multiple pleural lipoblastomatous lesions, thus establishing the diagnosis of lipoblastomatosis. The tumor has been removed with a second surgical procedure., Conclusion: Radical excision of lipoblastomas is the gold standard but is not always possible to achieve. Residual lesions can be managed conservatively, on the basis of the benign nature of the tumor and of its potential maturation to normal fat. Recurrence of lipoblastoma appears in 15 % of non-radical excisions but has not yet been described in the form of lipoblastomatosis. A second surgery is indicated if the tumor mass reaches large dimensions, if it increases in size, and if it threatens the functionality of vital organs such as the spinal cord.

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.)

Omental lipoblastoma is extremely rare among benign tumors. We herein report the case of a child who underwent laparoscopic extirpation of a large omental lipoblastoma. A 4-year-old girl was diagnosed with an intra-abdominal solid tumor. Abdominal imaging revealed a fat density mass that was well encapsulated and measured 18 × 15 × 7.5 cm in size. Considering the MRI findings and movability of the tumor, we strongly suspected that the lesion was an omental lipoblastoma. We initially decided to perform laparoscopic exploration and, if possible, extirpation of the solid tumor sequentially. A total of five trocars were used, and the tumor was found to originate from the omentum. We successfully performed complete resection of the tumor laparoscopically. A histological examination revealed lipoblastoma. For large abdominal tumors in children, the laparoscopic approach is recommended as the first procedure when the tumor is preoperatively considered to be benign and resectable., (© 2015 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and Wiley Publishing Asia Pty Ltd.)

Lipoblastoma-like tumor of the vulva (LLTV) is an exceptionally rare adipocytic mesenchymal tumor with only 4 cases reported previously. The aim of this study is to help characterize this tumor type. Eight cases of LLTV were identified in the consult files of one of the authors (C.D.M.F.). Clinical data and follow-up information were obtained from the referring pathologists. Detailed clinical information is available in all 8 cases. Patient age ranged from 17 to 46 years (median 27 y). Lesions presented as a vulvar mass with a variable growth rate, sometimes being painful. The most common preoperative clinical diagnosis was a Bartholin gland abnormality (4). The size of the mass ranged from 3.5 to 15 cm (median 5.6 cm). The lesions were described as grossly myxoid, mucoid, or gelatinous (4), well defined (4), and lobulated (3). None of the lesions exhibited necrosis. Histologically, LLTVs were lobulated and composed of variable proportions of mature adipocytes, bland univacuolated and bivacuolated lipoblasts, and spindle cells with short stubby nuclei in a diffusely myxoid background with prominent branching vessels. Nuclear atypia was minimal. No necrosis and only rare mitotic activity was identified. Only 1 of 7 tumors (in a 26-y-old patient) was positive for PLAG1 and retinoblastoma (Rb). Rb was lost, and PLAG1 was not expressed in all other tumors. HMGA2 was negative in 6 of 6 cases tested. Tumors were negative for S100 (5/5), MDM2, and CDK4 (5/6; 1 with just scattered cells positive for both antibodies). Two of 5 cases were positive for CD34. Fluorescence in situ hybridization for DDIT3 gene rearrangement was negative in 5 cases tested. The follow-up interval ranged from 4 months to 11 years (median 2.75 y). Three patients developed 1 local recurrence, 7 months, 2 years, and 8 years, respectively, after excision of the primary tumor. None of the patients has developed metastatic disease. LLTVs are indolent adipocytic mesenchymal tumors arising in adults. Lack of PLAG1 and HMGA2 expression in the majority of LLTV suggests that these lesions are distinct from "true" lipoblastoma. The loss of Rb in the majority of cases suggests a possible role of 13q chromosomal alterations and a possible relationship with the spindle cell lipoma tumor family.

A nine year-old boy presented with symptoms of dyspnoea. The chest radiograph and computed tomography scan revealed a large mediastinal tumour. A decision to operate on the patient was made. A huge 2.45 kg mediastinal lipoblastoma was successfully removed from the boy's chest. At three years no evidence of recurrence was found., (Copyright © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. 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.

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.