Benign lipoblastomatosis | |
---|---|
Other names | Embryonic lipoma [1] |
Specialty | 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. [2] [3]: 626 The term lipoblastomatosis was first used by Vellios et al. in 1958, at which point the tumor became generally accepted as a distinctive entity. [4] [5] Today Diffuse lipoblastoma is the preferred term for lipoblastomatosis. [6] [7] The tumor is rare, accounting for less than 1% of all childhood neoplasm, [8] and it has been found to be more common in males than females. [9] [8] It often presents as an asymptomatic rapidly enlarging mass, occurring more often in the soft tissues of the extremities. [8] [9]
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. [10] 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. [11] [12]
Lipoblastomatosis most likely results from new adipose tissue lobules and lipoblasts proliferating throughout the neonatal period. [10]
Although imaging modalities like magnetic resonance imaging (MRI) and computed tomography (CT) can be useful, histology confirmation is necessary for an accurate diagnosis. [10]
Prompt surgical resection is the advised course of treatment for lipoblastomatosis, particularly if the mass is close to any important organs. [10]
Benign lipoblastomatosis | |
---|---|
Other names | Embryonic lipoma [1] |
Specialty | 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. [2] [3]: 626 The term lipoblastomatosis was first used by Vellios et al. in 1958, at which point the tumor became generally accepted as a distinctive entity. [4] [5] Today Diffuse lipoblastoma is the preferred term for lipoblastomatosis. [6] [7] The tumor is rare, accounting for less than 1% of all childhood neoplasm, [8] and it has been found to be more common in males than females. [9] [8] It often presents as an asymptomatic rapidly enlarging mass, occurring more often in the soft tissues of the extremities. [8] [9]
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. [10] 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. [11] [12]
Lipoblastomatosis most likely results from new adipose tissue lobules and lipoblasts proliferating throughout the neonatal period. [10]
Although imaging modalities like magnetic resonance imaging (MRI) and computed tomography (CT) can be useful, histology confirmation is necessary for an accurate diagnosis. [10]
Prompt surgical resection is the advised course of treatment for lipoblastomatosis, particularly if the mass is close to any important organs. [10]