Lipoblastoma

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Lipoblastoma
Lipoblastoma. Note the plexiform vascular pattern and small immature adipocytes.
SymptomsWell-defined large bump typically just under the skin of the arms, legs and trunk, and may infiltrate underlying muscle[1]
Usual onsetYoung children (<age 3-years)[2]
TypesLocal or diffuse[2]
CausesUnknown[2]
Differential diagnosisLipoma,[2] myxoid liposarcoma[1]
PrognosisGood after excision, can recur[2]
FrequencyMales>females, 75%-90% diagnosed before age 3-years[2]

Lipoblastoma is a type of non-cancerous white fat soft tissue tumor most often found in infants and young children.[1] It may be focal or diffuse (lipoblastomatosis).[3] It presents as a well-defined large bump typically just under the skin of the arms, legs and trunk, and may infiltrate underlying muscle.[1] If it occurs in the abdomen, mediastinum or neck, it may present with pressure symptoms.[2] The overlying color may be yellowish and the bump can reach around 5cm in diameter.[4] Some have associated developmental delay, seizures, congenital malformations, or a history of lipomas that run in their family.[2]

The cause is unknown.[2] Local recurrence can happen in up to 80% of incompletely resected tumours. Therefore, complete surgical resection is required to prevent recurrence. It arises from embyronic white fat that is rapidly enlarging.[5]

It is slightly more common in males and rare in adults.[2]

Types

Types include:[citation needed]

Pathology

Macroscopic

Grossly, it has a pale yellow, myxoid cut surface with small cystic foci.[5]

Microscopic

It has lobules consists of immature adipose tissue separated by fibrous septa and lipoblasts at different stages of maturation, without atypia or mitosis. Plexiform capillary network and mature adipose tissue are seen at the central part of the lobule.[5]

References

  1. 1.0 1.1 1.2 1.3 Creytens, David (2023). "5. Adipocytic tumors". In Folpe, Andrew L.; Nielsen, Gunnlaugur Petur (eds.). Bone and Soft Tissue Pathology: A Volume in the Foundations in Diagnostic Pathology Series (2nd ed.). Philadelphia: Elsevier. pp. 133–135. ISBN 978-0-323-75871-0. Archived from the original on 2023-02-24. Retrieved 2023-02-24.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 WHO Classification of Tumours Editorial Board, ed. (2020). "1. Soft tissue tumours: Lipoblastoma and lipoblastomatosis". Soft Tissue and Bone Tumours: WHO Classification of Tumours. Vol. 3 (5th ed.). Lyon (France): International Agency for Research on Cancer. pp. 20–23. ISBN 978-92-832-4503-2.
  3. Domanski, Henryk A. (2018). "5. Low grade superficial connective tumours". In Klijanienko, Jerzy; Marinšek, Živa Pohar; Domanski, Henryk A. (eds.). Small Volume Biopsy in Pediatric Tumors: An Atlas for Diagnostic Pathology. Springer. p. 217. ISBN 978-3-319-61026-9. Archived from the original on 2023-02-24. Retrieved 2023-02-24.
  4. Johnstone, Ronald B. (2017). "35. Tumors of fat". Weedon's Skin Pathology Essentials (2nd ed.). Elsevier. p. 642. ISBN 978-0-7020-6830-0. Archived from the original on 2023-02-26. Retrieved 2023-02-26.
  5. 5.0 5.1 5.2 Abdul-Ghafar J, Ahmad Z, Tariq MU, Kayani N, Uddin N (January 2018). "Lipoblastoma: a clinicopathologic review of 23 cases from a major tertiary care center plus detailed review of literature". BMC Research Notes. 11 (1): 42. doi:10.1186/s13104-018-3153-8. PMC 5773143. PMID 29343278.