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Lipoblastomatosis

Tumor of embryonic fat tissue

Summary

Tumor of embryonic fat tissue

Field	Value
name	Benign lipoblastomatosis
synonyms	Embryonic lipoma
field	Dermatology

Benign lipoblastomatosis is a tumor consisting of fetal-embryonal adipocytes, frequently confused with a liposarcoma, affecting exclusively infants and young children, with approximately 90% of cases occurring before 3 years of age. The term lipoblastomatosis was first used by Vellios et al. in 1958, at which point the tumor became generally accepted as a distinctive entity. Today Diffuse lipoblastoma is the preferred term for lipoblastomatosis. The tumor is rare, accounting for less than 1% of all childhood neoplasm, and it has been found to be more common in males than females. It often presents as an asymptomatic rapidly enlarging mass, occurring more often in the soft tissues of the extremities.

Signs and symptoms

Although they have also been reported in the head and neck, shoulder, groin, axilla, back, and abdominal cavity, these tumors typically manifest in the extremities. Although it is normally painless, it may cause symptoms such as vomiting, stomach pain, paralysis, or fecal or urinary incontinence, depending on where the mass is anatomically.

Causes

Lipoblastomatosis most likely results from new adipose tissue lobules and lipoblasts proliferating throughout the neonatal period.

Diagnosis

Although imaging modalities like magnetic resonance imaging (MRI) and computed tomography (CT) can be useful, histology confirmation is necessary for an accurate diagnosis.

Treatment

Prompt surgical resection is the advised course of treatment for lipoblastomatosis, particularly if the mass is close to any important organs.

References

Rapini, Ronald P.. (2007). "Dermatology: 2-Volume Set". Mosby.
(November 2015). "Rapidly-growing buccal mass in a 6-month-old infant". British Journal of Oral and Maxillofacial Surgery.
James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. {{ISBN. 0-7216-2921-0.
(November 1958). "Lipoblastomatosis: a tumor of fetal fat different from hibernoma; report of a case, with observations on the embryogenesis of human adipose tissue.". [[The American Journal of Pathology]].
(July 1983). "Benign Lipoblastoma in the Neck Causing Respiratory Insufficiency". [[The Laryngoscope]].
(2017). "Imaging of Soft Tissue Tumors".
(2020). "Revisiting the WHO classification system of soft tissue tumours: emphasis on advanced magnetic resonance imaging sequences. Part 1". Polish Journal of Radiology.
(2014-09-15). "Lipoblastoma and Lipoblastomatosis of the Lower Leg". Case Reports in Orthopedics.
(1998). "Congenital Lipoblastomatosis of the Lower Extremity in a Neonate". Pediatric Dermatology.
(2021). "Lipoblastomatosis: An unusual midline neck mass in a young child". Elsevier BV.
(2017). "Analysis of diagnosis and treatment of lipoblastomatosis". Science China Life Sciences.
(1997). "Lipoblastoma/Lipoblastomatosis: A Clinicopathologic Study of 25 Tumors". Ovid Technologies (Wolters Kluwer Health).

Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

dermal-and-subcutaneous-growths

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