Presentation
It commonly presents as a painless subcutaneous soft tissue mass, but there are other unique clinical presentations that are important to recognize. The differential is broad and includes sarcoma, vascular tumor, myofibroma, and other fibromatoses. [ncbi.nlm.nih.gov]
Entire Body System
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Soft Tissue Mass
It commonly presents as a painless subcutaneous soft tissue mass, but there are other unique clinical presentations that are important to recognize. The differential is broad and includes sarcoma, vascular tumor, myofibroma, and other fibromatoses. [ncbi.nlm.nih.gov]
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Asymptomatic
Herein, we present a 2-month old asymptomatic boy diagnosed with an echogenic mass in the left ventricular outflow tract. The patient underwent surgical excision and histopathological evaluation revealed a lipoblastoma. [ncbi.nlm.nih.gov]
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Swelling
In our case, a child of age 1 year and 3 months presented with a swelling over the nape of the neck. The swelling had rapidly increased in size and was associated with difficulty in neck movements. [ncbi.nlm.nih.gov]
The third patient was an 18 month old boy with a swelling on the dorsum of his left forearm. The fourth patient had a swelling of his left thigh and two patients had swellings in their backs. [online.boneandjoint.org.uk]
Complete excision of one of his thigh swellings, as well as incision biopsy from other swellings, was performed without complications. [jmgims.co.in]
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Fishing
FISH revealed that five of seven cases in our series had a rearrangement of the PLAG1 gene. Occasionally, there can be difficulties in distinguishing a lipoblastoma from a conventional lipoma or a myxoid liposarcoma. [ncbi.nlm.nih.gov]
Additional FISH using an MDM2 gene probe and an BAC534N15 probe (containing sequences specific for the CDK4 gene) showed amplification of the CDK4 gene. [lirias.kuleuven.be]
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Developmental Delay
Ten patients had other medical conditions including macrocephaly, seizures, developmental delay, autism, congenital anomalies, Sturge-Weber syndrome, or a family history of multiple lipomas. [ncbi.nlm.nih.gov]
Her medical history was unremarkable, and her parents reported no trauma, unusual rashes or infections, and no physical or developmental delays. [mdmag.com]
There was no history of trauma, unusual rashes or infections, and no physical and developmental delay. There was no other similar swelling elsewhere. Family history was inconclusive. [journals.lww.com]
Associations (17%) ( 19738456 ) macrocephaly seizures developmental delay autism congenital anomalies Sturge-Weber syndrome family history of multiple lipomas hemangioma ( 9331284 ) lipoma ( 9331284 ) juvenile aponeurotic fibroma ( 9331284 ) The degree [humpath.com]
Respiratoric
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Aspiration
However, the fine-needle aspiration cytological features of a lipoblastoma are sufficiently characteristic to make a specific preoperative diagnosis. [ncbi.nlm.nih.gov]
Key Words: Lipoblastoma ; Fine needle aspiration cytology [jpatholtm.org]
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Respiratory Distress
We present a case of giant mediastinal lipoblastoma in a 16-month-old boy who presented with acute respiratory distress. The mass was completely excised through a left posterolateral thoracotomy. [ncbi.nlm.nih.gov]
In rare instances, they may present with respiratory distress. 2 Multiple cases of head and neck lipoblastomas have been published in the English-language literature. 3 Growing evidence supports that a chromosomal breakpoint abnormality at 8q11-q13 may [mdedge.com]
Other symptoms, depending on location with the head and neck, include respiratory distress and muscle weakness from nerve compression ( 1, 2 ). [frontiersin.org]
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Stridor
Head and neck region occurrences are rare; only 4 such cases involving patients who presented with stridor have been previously reported. [ncbi.nlm.nih.gov]
There was no history of trauma, infection, dysphagia, dyspnea, stridor, or fever. She had no other discomfort or any associated symptoms such as irritable crying, vomiting, fever, or body weight loss. [avicennajmed.com]
Table 1: Clinicopathological profile of cases of lipoblastoma/lipoblastomatosis Click here to view Case 1 A 12-months-old boy presented with dyspnea, stridor, and lethargy for 1 month. [cancerjournal.net]
Gastrointestinal
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Abdominal Mass
Asymptomatic abdominal mass, progressive abdominal distension and intraperitoneal radiolucent fat density mass on computed tomography are the main diagnostic criteria for omental lipoblastoma. [ncbi.nlm.nih.gov]
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Abdominal Distension
Asymptomatic abdominal mass, progressive abdominal distension and intraperitoneal radiolucent fat density mass on computed tomography are the main diagnostic criteria for omental lipoblastoma. [ncbi.nlm.nih.gov]
Tumors presented with a growing mass in 9 patients, abdominal distension in 2, and an incidental finding on chest radiography in one. Complete excisions were done in all patients. There was one recurrence in a patient with a scalp mass. [synapse.koreamed.org]
RESULTS: A soft tissue mass was the chief complaint in 7 patients, abdominal distension in 1 patient with a retroperitoneal mass, and defecation difficulty in 1 patient with a perirectal mass. In 4 patients, tumors occurred on the back. [thedoctorsdoctor.com]
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Rectal Bleeding
He presented with a history of reducible rectal prolapse, rectal bleeding, and episodic abdominal pain and was initially treated for constipation. [ncbi.nlm.nih.gov]
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Episodic Abdominal Pain
He presented with a history of reducible rectal prolapse, rectal bleeding, and episodic abdominal pain and was initially treated for constipation. [ncbi.nlm.nih.gov]
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Constipation
He presented with a history of reducible rectal prolapse, rectal bleeding, and episodic abdominal pain and was initially treated for constipation. [ncbi.nlm.nih.gov]
Skin
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Subcutaneous Mass
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. [ncbi.nlm.nih.gov]
To conclude although lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat in children, we should keep them as differential diagnosis of a soft subcutaneous mass. [jpss.eu]
Magnetic resonance imaging (MRI) visualized a well-circumscribed subcutaneous mass measuring 4.6 2.9 3.9 cm in size that was hypointense on T1-weighted images and hyperintense on T2-weighted images (Fig. 1 a,b), although it was not suppressed on fat-saturated [surgicalcasereports.springeropen.com]
(a, b) Coronal T1-weighted (500/14) (a) and axial proton-density fat-suppressed (2000/30) (b) MR images show a subcutaneous mass that is largely isointense relative to fat (arrows). [pubs.rsna.org]
Musculoskeletal
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Long Arm
Nowadays, cytogenetic analysis may contribute to the diagnosis by showing abnormalities of the long arm of chromosome 8, leading to rearrangement of the PLAG1 gene. Actual advances in cytogenetic molecular analysis may aid in accurate diagnosis. [ncbi.nlm.nih.gov]
Cytogenetic evaluation of tumour cells often reveals chromosomal anomalies, such as abnormalities of the long arm of chromosome 8 leading to rearrangement of the PLAG1 gene. [online.boneandjoint.org.uk]
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Macrocephaly
Ten patients had other medical conditions including macrocephaly, seizures, developmental delay, autism, congenital anomalies, Sturge-Weber syndrome, or a family history of multiple lipomas. [ncbi.nlm.nih.gov]
Associations (17%) ( 19738456 ) macrocephaly seizures developmental delay autism congenital anomalies Sturge-Weber syndrome family history of multiple lipomas hemangioma ( 9331284 ) lipoma ( 9331284 ) juvenile aponeurotic fibroma ( 9331284 ) The degree [humpath.com]
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Leg Pain
Leg pain and urinary incontinence developed in 1 patient but improved on conservative treatment. CONCLUSIONS: Lipoblastoma is a benign neoplasm but can frequently recur (25%) in spite of complete excision. [thedoctorsdoctor.com]
Eyes
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Strabismus
[J Pediatr Ophthalmol Strabismus. 2017;54:e67-e70.]. [ncbi.nlm.nih.gov]
Observation It was a two-year-old infant with a sibling of 2, from a non-consanguineous marriage, well vaccinated, followed by ophthalmology for convergent strabismus who had a left upper cervical mass (sub-angulomaxillary) left. gradually increasing [gavinpublishers.com]
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Prolapse
He presented with a history of reducible rectal prolapse, rectal bleeding, and episodic abdominal pain and was initially treated for constipation. [ncbi.nlm.nih.gov]
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Lacrimation
Once the frozen section showed fat and not lacrimal gland or malignancy, excision could proceed. The final sections alone established the correct diagnosis. [healio.com]
Psychiatrical
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Suggestibility
Lack of PLAG1 and HMGA2 expression in the majority of LLTV suggests that these lesions are distinct from "true" lipoblastoma. [ncbi.nlm.nih.gov]
Neurologic
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Delayed Milestone
A 1-year-old male child presented to us with delayed milestones, inability to move lower limbs since 6 months and difficulty in defecation and urination since 6 months. On examination, there was wasting of lower limb muscles. [pediatricneurosciences.com]
Urogenital
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Scrotal Mass
It represents one of several differential diagnoses of scrotal masses that include malignant tumors. Surgical extirpation is mandatory. We present a rare case. [ncbi.nlm.nih.gov]
masses such as malignant tumors. [pagepress.org]
We describe a lipoblastoma in a 12-month-old boy who presented with a rapidly enlarging scrotal mass. [thedoctorsdoctor.com]
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Urinary Incontinence
A 3-year-old boy had suffered from progressive urinary incontinence and uncontrolled defecation during past 3 months. [ncbi.nlm.nih.gov]
Leg pain and urinary incontinence developed in 1 patient but improved on conservative treatment. CONCLUSIONS: Lipoblastoma is a benign neoplasm but can frequently recur (25%) in spite of complete excision. [thedoctorsdoctor.com]
Workup
Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health for performing the PLAG1 immunohistochemistry workup. [surgicalcasereports.springeropen.com]
X-Ray
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Mediastinal Mass
Although extremely rare, mediastinal lipoblastoma can be life threatening; therefore, it should be included in the differential diagnosis of mediastinal mass in younger subjects. [ncbi.nlm.nih.gov]
Figure 1: (a) Preoperative computed tomography (CT) scan of chest showing a mediastinal mass. (b) The resected mediastinal mass measuring 6 5 3 cm. [cancerjournal.net]
Treatment
Treatment is surgical excision. An unusual case of omental lipoblastoma in a 2-year-old girl is presented in this report. [ncbi.nlm.nih.gov]
Complete surgical removal is the recommended treatment. Only 2 cases of lipoblastomas of the cheek have been reported in the English-language literature. [jamanetwork.com]
Prognosis
It has an excellent prognosis despite its potential to local invasion and rapid growth. [ncbi.nlm.nih.gov]
Etiology
MRI chest confirmed an 8 5 4cm right shoulder girdle, well encapsulated soft tissue mass probably arising from the subscapularis muscle and probably of neoplastic etiology. There was no intrathoracic or bony involvement. [indianpediatrics.net]
However, the etiological orientation and the extension assessment of the lesion remain limited. MRI is then often preferred [10] to the scanner to make a more precise assessment of the anatomical relationships of the tumor [10]. [gavinpublishers.com]
Epidemiology
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. [ncbi.nlm.nih.gov]
TERMINOLOGY Synonyms Fetal lipoma Infantile lipoma Embryonic lipoma Definitions Benign tumor resembling fetal adipose tissue Localized tumors designated lipoblastoma Diffuse tumors designated lipoblastomatosis CLINICAL ISSUES Epidemiology Incidence Rare [basicmedicalkey.com]
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. [afrjpaedsurg.org]
[…] tumor, either localized (lipoblastoma) or diffuse (lipoblastomatosis), resembling fetal adipose tissue Terminology Lipoblastoma: also called fetal/embryonal lipoblastoma; well circumscribed and subcutaneous Lipoblastomatosis: deep-seated and ill-defined Epidemiology [pathologyoutlines.com]
Epidemiology Primarily occurs in young children ( Clinics Presents in superficial tissues of arms and legs (deeper in lipoblastomatosis), though mediastinum, retroperitoneum, trunk, head and neck may be affected. [atlasgeneticsoncology.org]
Pathophysiology
Lipoblastoma: Pathophysiology and surgical management. J Pediatr Surg. 2001;36:229-31. Copyright 1999-2012 Indian Pediatrics [indianpediatrics.net]
Lipoblastoma: Pathophysiology and surgical management. J Pediatr Surg. 2001;36:229-231. Back To Top [appliedradiology.com]
Dilley AV, Patel DL, Hicks MJ, Brandt ML (2001) Lipoblastoma: pathophysiology and surgical management. J Pediatr Surg 36:229–231 CrossRef PubMed Google Scholar 15. [link.springer.com]
Lipoblastoma: Pathophysiology and surgical management. J Pediatr Surg 2001;36:229-31. 7. Bruyeer E, Lemmerling M, Poorten VV, Sciot R, Hermans R. Paediatric lipoblastoma in the head and neck: Three cases and review of literature. [avicennajmed.com]
Prevention
In such cases fluorescence in situ hybridization (FISH) analysis of PLAG1, a gene specifically rearranged in lipoblastoma, is necessary to prevent misdiagnosis. [ncbi.nlm.nih.gov]
Complete surgical resection is mandatory to prevent recurrence. [online.boneandjoint.org.uk]