-
Experimental and Therapeutic Medicine Aug 2021Lipoblastoma (LB) and lipoblastomatosis (LBS) are uncommon benign mesenchymal tumors of embryonal fat, occurring almost exclusively in infancy and early childhood. These...
Lipoblastoma (LB) and lipoblastomatosis (LBS) are uncommon benign mesenchymal tumors of embryonal fat, occurring almost exclusively in infancy and early childhood. These fast-growing tumors have an excellent prognosis if properly treated. Eight consecutive children having pathologically demonstrated LB treated by the same surgical team were retrospectively reviewed. There were 5 boys and 3 girls between 7 to 36 months (median age 22 months). The localization of the tumors was on the thigh (1 case), abdomen (2 cases), axillary and pectoral region (1 case) paragluteal region (1 case), lumbar area (1 case), inguinal-scrotal (1 case), and in one case, presacral, gluteal and perirectal region (1 case). Five were focal and in 3 cases an infiltrative growth pattern was observed. One case exhibited a gross appearance resembling sacrococcygeal teratoma, with associated Dravet syndrome. No recurrence was noted in our series, after a mean follow-up of 28 months post operatory. Despite its rareness, LB must be kept in mind when diagnosing a rapidly growing fatty mass in children. Even when dealing with very large abdominal LB, complete surgical excision is possible, with an excellent prognosis. Due to the relatively high recurrence rate noted in the literature, particularly in LBS, follow-up is extremely important.
PubMed: 34257716
DOI: 10.3892/etm.2021.10335 -
International Journal of Pediatrics &... Mar 2022Lipoblastoma is a rare benign soft tissue tumor found in infants and young children. It presents as an enlarging mass commonly observed in extremities, trunk, head and...
Lipoblastoma is a rare benign soft tissue tumor found in infants and young children. It presents as an enlarging mass commonly observed in extremities, trunk, head and neck regions. Imaging features include a hyper-echoic mass on ultrasound, heterogonous fat density lesion on CT scan with enhancing septations and no calcification, and high T1 signal mass that drops the signal intensity on fat suppression MRI images. Total excision of such lesion is the treatment of choice, and follow-up is recommended to rule out recurrence.
PubMed: 35573075
DOI: 10.1016/j.ijpam.2021.05.004 -
Indian Journal of Pathology &... Feb 2024Lipoblastomas are benign tumors arising from embryonic white fatty cells that continue to proliferate and develop during the postnatal period. It commonly affects...
Lipoblastomas are benign tumors arising from embryonic white fatty cells that continue to proliferate and develop during the postnatal period. It commonly affects children 3 years of age or younger, with the neck being the most frequent site in the head and neck region. Only 10% of cases are seen at 10 years or above. The etiopathogenesis of lipoblastomas is linked to polysomy and rearrangement of chromosome 8q11-13 with or without the involvement of the pleomorphic adenoma gene 1 (PLAG1) gene. Here, we report one of the first cases of lipoblastoma of lip in a 10-year-old boy with atypical immunohistochemical features and the need for thorough clinical and histopathological evaluation of cases with atypical findings. The novel pathogenetic mechanism of lipoblastoma from the cluster of differentiation 34-positive (CD34+) stem cells has also been discussed.
PubMed: 38427759
DOI: 10.4103/ijpm.ijpm_841_23 -
Indian Pediatrics Jun 2013A 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...
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.
Topics: Axilla; Diagnosis, Differential; Female; Humans; Infant; Lipoblastoma; Liposarcoma, Myxoid
PubMed: 23942405
DOI: No ID Found -
Journal of the Belgian Society of... 2023Lipoblastoma should be considered in the differential diagnosis of painful rapidly growing fatty mass within the mediastinum in infants or young children under three...
Lipoblastoma should be considered in the differential diagnosis of painful rapidly growing fatty mass within the mediastinum in infants or young children under three years old.
PubMed: 37483991
DOI: 10.5334/jbsr.3166 -
Revista Chilena de Pediatria Apr 2020To 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...
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.
Topics: Child, Preschool; Humans; Infant; Lipoblastoma; Male; Mediastinal Neoplasms; Thoracotomy; Tomography, X-Ray Computed
PubMed: 32730544
DOI: 10.32641/rchped.v91i2.1223 -
Surgical Case Reports Dec 2016Intrascrotal lipoblastoma is a rare pediatric benign soft tissue neoplasm, and only 11 cases have been reported. The accurate preoperative diagnosis is difficult because...
Intrascrotal lipoblastoma is a rare pediatric benign soft tissue neoplasm, and only 11 cases have been reported. The accurate preoperative diagnosis is difficult because of its rarelity and the similarity with the other soft tissue tumors. Among them, accurate preoperative diagnosis had been made in only one case. Thus, almost all of the cases had required inguinal mass excision (and orchidectomy in one case). In this paper, we discuss the accurate preoperative diagnosis of intrascrotal lipoblastoma and subsequent simple tumorectomy via minimal invasive scrotal skin incision, in 1-year-old boy. On physical examination, intrascrotal extra-testicular lobulated mass was palpated on the right scrotum. An ultrasonography revealed the well-circumscribed, iso-echoic, scant blood-flow, and lobulated tumors with each lobules of 1 to 4 cm in diameter, and the tumor located outside of the tunica vaginalis testis. The serum values of alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (b-hCG) were within normal limit. The preoperative diagnosis of intrascrotal lipoblastoma was made, and the mass was excised via minimal scrotal incision. The right testicle and epididymis were normal. The lesion consisted of the distinct two lobulated tumors, and microscopic examination confirmed the diagnosis of intrascrotal lipoblastoma. The postoperative course was uneventful without evidence of recurrence. A rare intrascrotal lipoblastoma is seldom made accurate preoperative diagnosis; however, the accurate preoperative suspicion of this tumor leads to the minimal invasive tumorectomy via scrotal skin incision and favorable postoperative recovery without recurrence.
PubMed: 27059472
DOI: 10.1186/s40792-016-0160-7 -
Head and Neck Pathology Jul 2013Fat-containing tumors of the salivary glands are uncommon. Their wide histological spectrum varies from pure lipomatous neoplasms similar to their cutaneous and soft... (Review)
Review
Fat-containing tumors of the salivary glands are uncommon. Their wide histological spectrum varies from pure lipomatous neoplasms similar to their cutaneous and soft tissue counterparts to mixed lipoepithelial lesions specific to the salivary glands. With few exceptions, these uncommon lesions affect mainly the elderly, with a mean age at presentation of ≥ 50 years and show a predilection for males. A few cases occur in childhood; some of them represent congenital lesions. In decreasing order of frequency, ordinary (soft-tissue type) lipoma, oncocytic lipoadenoma, non-oncocytic sialolipoma, and pleomorphic adenoma/myoepithelioma with extensive lipometaplasia are the main variants of fat containing tumors encountered in the salivary glands. While pleomorphic adenoma/myoepithelioma with lipometaplasia behave in the same way as their non-fat-containing counterparts, other lipomatous salivary gland tumors listed above are cured with simple excision and do not carry a risk of recurrence. Other lipoma variants (spindle cell lipoma, osteolipoma, fibrolipoma, angiolipoma, pleomorphic lipoma, lipoblastoma and hibernoma) are exceptionally rare in the salivary gland. Atypical lipomatous tumors/liposarcoma have been only rarely reported in the salivary gland and they behave in a similar fashion to their soft-tissue counterparts. Diffuse lipomatosis and lobular fatty atrophy are the two tumor-like lesions that might closely mimic sialolipoma, particularly in limited biopsy material without knowledge of the gross findings. This review summarizes the clinicopathological features of the main types of salivary fat-containing lesions and discusses their differential diagnoses.
Topics: Aged; Female; Humans; Male; Middle Aged; Neoplasms, Adipose Tissue; Salivary Gland Neoplasms
PubMed: 23821211
DOI: 10.1007/s12105-013-0459-7 -
BMC Research Notes Jan 2018Lipoblastoma is a rare neoplasm that occurs mostly in infants and children. Although benign, it has a tendency for local recurrence. (Review)
Review
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.
Topics: Adolescent; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Lipoblastoma; Male; Neoplasm Recurrence, Local; Prognosis; Tertiary Care Centers
PubMed: 29343278
DOI: 10.1186/s13104-018-3153-8