Custom Search

         

Soft Tissue Pathology 

Pathology of Lipofibromatosis

Dr Sampurna Roy MD                 

 

                                                                                                                      

 

 

Lipofibromatosis is a rare pediatric lesion composed of abundant adipose tissue together with bundles of fibroblasts.

According to Fetsch et al - [ this tumour comprises part of the spectrum of what has been referred to in the literature as infantile/juvenile fibromatosis, its clinicopathologic features and, in particular, its distinctive tendency to contain fat as an integral component, warrant separate classification as a "lipofibromatosis."]

Age: 11 days to 12 years

Site:  The patients usually  presented with a soft tissue mass  involving upper and lower extremities, trunk , head or neck.

Microscopic features:

Two components:

1.Bundles of spindle shaped fibroblast-like cells with focal fascicular pattern

2. Disorganized mature adipose tissue.

The lesion mainly involved the septa of fat and skeletal muscle.

Mitotic activity is limited  - Less than 1/10HPF.

Cytological atypia is not prominent. 

The tumours entrapped vessels, nerves, skin adnexa and skeletal muscle.

Immunohistochemistry:  CD99 ; smooth muscle actin ; bcl2 - Focal Positivity  , S100 protein ; muscle actin ; EMA  - Less frequently positiveDesmin ; Keratins ; CD57 - Negative

Differential diagnosis:

-Fibromatosis;

-Fibrous hamartoma of infancy: In lipofibromatosis primitive nodular fibromyxoid component is absent. 

-Calcifying aponeurotic fibroma ;  Lipoblastoma

 

Further reading:

A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis.

Lipofibromatosis presenting as a pediatric neck mass.

Infantile lipofibromatosis of the upper limb.

 

 

 

 


 

 

Histopathology-India.net

 

Pathopedia-India.com

 

Surgical Pathology.com

 

Pathology-India.com

 

Dermpath-India

 

Infectious Disease Online

 

Pathology Quiz Online 

 

Paediatric Pathology Online

 

Pancreatic Pathology Online

 

Paraganglioma-Online

 

Endocrine Pathology Online

 

Eye Pathology Online

 

Ear Pathology Online

 

Cardiac Path Online

 

Pulmonary Pathology Online

 

Lung Tumour Online

 

Mesothelioma-Online

 

Nutritional Pathology Online

 

Environmental Pathology Online

 

Soft Tissue Tumour Online

 

GI Path Online-India

 

Gallbladder Pathology Online

 

E-book - History of Medicine  

 

Microscope - Seeing the Unseen

 

 roypath.in

 

 

Disclaimer

Privacy Policy  

Advertising Policy

Copyright 2017  histopathology-india.net