Friday 7 October 2005
Definition: Lipoblastoma is a rare benign tumor that arises from embryonic adipose tissue and usually occurs in young children. It is a lobulated, localized (lipoblastoma) or diffuse (lipoblastomatosis) tumor, resembling fetal adipose tissue.
82% of lipoblastomas had rearrangement of the 8q11 approximately q13 region. These rearrangements target the PLAG1 gene, which becomes upregulated through promoter swapping.
Both types of tumor are most commonly found in the first three years of life. They may occasionally be found at birth or in older children. There is male predilection.
The extremities are most common location but location in mediastinum, retroperitoneum and head and neck areas was described.
Most patients present with slow growing soft tissue nodule/mass, well circumscribed and confined to the subcutis in case of lipoblastoma , infiltrating deeper muscle in case of lipoblastomatosis.
Infants and young children ( < 5 years) almost exclusively1–5
Commonly proximal lower and upper extremities
sex ratio M/F = 1.7 (19738456)
Age at diagnosis ranges from 3 months to 16 years with 22% in the first year, 68% at 1 to 9 years, and 10% at 10 to 16 years. (19738456)
- trunk (64%)
- extremities (27%)
- head/neck (8%). Forty-six percent had
one or more recurrences (46%)
tumor diameter ranges from 1.2 to 15.5 cm
white to yellow cut surface
variable lobulation and myxoid change.
Benign, lobulated (lipoblastoma) or diffuse (lipoblastomatosis) adipose tumor of childhood, with a morphology that closely resembles the fetal adipose tissue.
soft and lobulated
relatively small lesions (2-5 cm), showing fatty looking tissue with gelatinous areas.
Lipoblastoma shows lobulated architecture with an admixture of mature and immature adipocytes, the latter corresponding to lipoblasts in various stages of development.
Depending of age of the patient, the lipoblast may be very scarse and lesion may be dominated by lipoma-like component (mostly in older patients).
Connective tissue septa separate lobules.
The lobulation is less prominent in lipoblastomatosis.
The matrix can be quite myxoid, with a plexiform vascular pattern, thus mimicking myxoid liposarcoma.
Occasionally lipoblastoma(tosis) may show extramedulary hematopoiesis or cells resembling brown fat. Cellular maturation has been described, leading to lipoma-like picture.
very focal lobulation
+/- atypical stromal nuclei
Closely resembles fetal fat
nodules of adipose and myxoid tissue demarcated by bands of fibrous tissue.
The cells displayed a range of differentiation from multivacuolated lipoblasts to mature adipocytes.
Mitoses are nonexistent to rare
Histologic variations :
- subtle zonal architecture of fat maturation
- abundant myxoid material
- primitive mesenchymal cells
- focal plexiform vascular pattern
- multinucleated cells.
- S100 (100%)
- CD34 (100%)
- Mib-1 reactivity : absent to low.
Associations (17%) (19738456)
family history of multiple lipomas
juvenile aponeurotic fibroma (9331284)
The degree of adipocytic differentiation does not predict biologic behavior.
- due to presence of: lipoblasts, plexiform vascular pattern, abundant myxoid stroma
- Ultrastructurally similar to myxoid liposarcoma
- lipoblastoma is distinguished from myxoid liposarcoma by: young age of patient, distinct lobulation, absence of giant cells or pleomorphic nuclei
pleomorphic liposarcoma of soft tissues
lipoblastomatosis (lipoblastoma with irregular margins)
In immunohistochemical studies, the neoplastic cells are positive for vimentin and stain negatively for other markers, including cytokeratins, CD34, desmin and NKIC3. These markers can be helpful in distinguishing lipoblastoma from the appendageal tumours and other mesenchymal tumours, such as spindle cell lipoma, angiomyofibroblastoma and lipofibromatosis.
82% lipoblastomas had rearrangement of the 8q11 approximately q13 region. These rearrangements target the PLAG1 gene, which becomes upregulated through promoter swapping.
As 8q11 approximately q13 rearrangements have been reported in only 3% of conventional lipomas and never in myxoid liposarcoma, cytogenetic analysis or FISH for the PLAG1 gene can provide useful differential diagnostic information.
8q22 rearrangements (PLAG1 rearrangements)
- t(3;8)(q12;q11.2) (1521225)
- t(8;15)(q12;q25) (8q12 and 15q25)
- t(7;8)(q31;q13) (7q31 and 8q13) (9530333)
- ins(8;6)(q11.2;q13q27) (9169035)
- masked 8q intrachromosomal rearrangement
PLAG1 rearrangements at 8q22 (69%) (11549588)
- PLAG1-HAS2 fusion by 8q rearrangement
- myxoid/round cell liposarcoma
- atypical lipomatous tumor tumor (well-differentiated lipoma-like liposarcoma) (12242528)
The histopathological findings of a lipoblastoma are characterized by lobular architecture and an admixture of mature adipocytes and lipoblasts in various stages of development. The matrix can be myxoid.
The histopathological differential diagnosis of a liposarcoma includes other tumours with adipocytic differentiation, such as atypical lipomatous tumours/well-differentiated liposarcomas, myxoid liposarcomas, and spindle cell lipomas.
Because of the presence of lipoblasts, a lipoblastoma may have a strong resemblance to well-differentiated liposarcomas. Cases with myxoid changes may be extremely difficult to distinguish from myxoid liposarcomas.
The recent innovation of the term lipofibromatosis also adds to the diagnostic difficulty. The degree of the fibroblastic component is generally greater in lipofibromatosis than in lipoblastoma.
Occasionally, there can be difficulties in distinguishing a lipoblastoma from a conventional lipoma or a myxoid liposarcoma. As 8q11 approximately q13 rearrangements have been reported in only 3% of conventional lipomas and never in myxoid liposarcoma. Cytogenetic analysis or FISH for the PLAG1 gene can provide useful differential diagnostic information.
Because lipoblastomas are usually well encapsulated and have little adjacent tissue, the definitive treatment is complete surgical excision.
However, focal recurrence can be a problem in up to 25% of cases, due to incomplete resection.
Despite their ability for local invasion and rapid growth, the prognosis is excellent.
Lipoblastoma is benign and malignant transformation or metastatsis does not occur. Recurrences are described in 9-22% of cases mainly in lipoblastomatosis.
- soft tissue tumors
- adipocytic tumors
- adipocytic tumors
Lipoblastoma (LPB): A Clinicopathologic and Immunohistochemical Analysis of 59 Cases. Coffin CM, Lowichik A, Putnam A. Am J Surg Pathol. 2009 Sep 4. PMID: 19738456
Bartuma H, Domanski HA, Von Steyern FV, Kullendorff CM, Mandahl N, Mertens F. Cytogenetic and molecular cytogenetic findings in lipoblastoma. Cancer Genet Cytogenet. 2008 May;183(1):60-3. PMID: 18474299
Collins MH, Chatten J. Lipoblastoma/lipoblastomatosis: a clinicopathologic study of 25 tumors. Am J Surg Pathol. 1997 Oct;21(10):1131-7. PMID: 9331284
Collins MH, Chatten J. Lipoblastoma/lipoblastomatosis: a clinicopathologic study of 25 tumors. Am J Surg Pathol. 1997;21:1131–1137.
Greco AMA, Garcia RL, Vuletin JC. Benign lipoblastomatosis. Ultrastructure and histogenesis. Cancer. 1980;45:511–515.
Mentzel T, Calonje E, Fletcher CD. Lipoblastoma and lipoblastomatosis. A clinicopathological study of 14 cases. Histopathology. 1993;23:527–533.
Rubin BP, Dal Cin P. The genetics of lipomatous tumors. Semin Diagn Pathol. 2001;18:286–293.
Vellios F, Baez MF, Schumacher HB. Lipoblastomatosis. A tumor of fetal fat different from hibernoma. Am J Pathol. 1958;34:1149–1155.
Coffin CM. Lipoblastoma. An embryonal tumor of soft tissue related to organogenesis. Semin Diagn Pathol. 1994;11:98–103.
Bolen JW, Thorning D. Benign lipoblastoma and myxoid liposarcoma. A comparative light- and electron-microscopic study. Am J Surg Pathol. 1980;4:163–174.
Chung EB, Enzinger FM. Benign lipoblastomatosis. An analysis of 35 cases. Cancer. 1973;32:482–492.
Fletcher CD, Akerman M, Dal Cin P, De Wever I, Mandahl N, Mertens F, et al. Correlation between clinicopathologic features and karyotype in lipomatous tumors. A report of 178 cases from the chromosomes and morphology (CHAMP) collaborative study group. Am J Pathol. 1996;148:623–630.
Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CDM, Mandahl M, et al. Combined morphologic and karyotypic study of 59 atypical lipomatous tumors: evaluation of their relationship and differential diagnosis with other adipose tissue tumors. Am J Surg Pathol. 1996;20:1182–1189.
Chung EB, Enzinger FM. Benign lipoblastomatosis. An analysis of 35 cases. Cancer 1973; 32: 482–492.
Hicks J, Dilley A, Patel D, Barrish J, Zhu SH, Brandt M. Lipoblastoma and lipoblastomatosis in infancy and childhood: histopathologic, ultrastructural, and cytogenetic features. Ultrastruct Pathol 2001; 25: 321–333.
Collins MH, Chatten J. Lipoblastoma/lipoblastomatosis: a clinicopathologic study of 25 tumors. Am J Surg Pathol 1997; 21: 1131–1137.
Lae ME, Pereira PF, Keeney GL, Nascimento AG. Lipoblastoma-like tumour of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation. Histopathology 2002; 40: 505–509.
de Saint Aubain Somerhausen N, Coindre JM, Debiec-Rychter M, Delplace J, Sciot R. Lipoblastoma in adolescents and young adults: report of six cases with FISH analysis. Histopathology 2008; 52: 294–298.
Miller GG, Yanchar NL, Magee JF, Blair GK. Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature. Can J Surg 1998; 41: 455–458.
Sciot R, De Wever I, Debiec-Rychter M. Lipoblastoma in a 23-year-old male: distinction from atypical lipomatous tumor using cytogenetic and fluorescence in-situ hybridization analysis. Virchows Arch 2003; 442: 468–471.
Do NY, Cho SI, Park JH, Choi JY. Lipoblastoma arising from the submandibular region. J Pediatr Surg 2008; 43: e13–e15.
Fletcher CDM, Unni KK, Mertens F, World Health Organization, International Agency for Research on Cancer. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press, 2002.
Ende L, Upton J, Richkind KE, Vargas SO. Lipoblastoma: appreciation of an expanded spectrum of disease through cytogenetic analysis. Arch Pathol Lab Med 2008; 132: 1442–1444.
Suster S, Fisher C. Immunoreactivity for the human hematopoietic progenitor cell antigen (CD34) in lipomatous tumors. Am J Surg Pathol 1997; 21: 195–200.
Silverman JS, Hamilton J, Tamsen A. Benign recurring lipoblastoma in an adult versus well differentiated subcutaneous myxoid liposarcoma: clinicopathologic, immunohistochemical and molecular analysis of a unique case. Pathol Res Pract 1999; 195: 787–792; discussion 93.
Fletcher JA, Kozakewich HP, Schoenberg ML, Morton CC. Cytogenetic findings in pediatric adipose tumors: consistent rearrangement of chromosome 8 in lipoblastoma. Genes Chromosomes Cancer 1993; 6: 24–29.