Case Report
Familial Multiple Lipomatosis : A rare case report
Abstract: familial multiple lipomatosis is abenign
hereditary syndrome
INTRODUCTION
Lipomas are the most common soft tissue
tumor, lipoma is a benign tumor of adipose
tissue, it is called as universal tumor of adipose
tissue (ubiquitous tumor) as it can occur
anywhere in the body (except brain), with an
incidence of 2.1 per 1000 people. Multiple
lipomas localised in different areas of the body
are rare and can occur in specific hereditary
syndrome. Although most lipomas are
sporadic, 2 rare distinct familial types of
lipomatosis have been identified: familial
multiple lipomatosis(FML) and multiple
symmetric lipomatosis (MSL) (also known as
Madelung disease) Familial multiple
lipomatosis is a benign hereditay syndrome
occurring in a proposed Autosomal Dominant
inheritance have also been reported. FML
occur with incidence of 0.002% has been
reported. Men are affected twice as commonly
as women.FML usually appear mostly in third
decade of life.
The treatment of lipoma can include simple
excision, endoscopic removal, or liposuction.
The object of presenting the caseis to highlight
this rare case .
History:A 45 year old man presented with
multiple small swellings of 25 year duration.
Since the patient do not report the disease in
his family First swelling appear in left upper
half of chest. He developed few similar
swelling on left arm ,right thigh and buttock
swelings are painless. his general physical and
systemic examination were within normal
limits. the largest lipoma is about 3.4;2cm in
size spherical to oval in shape, smooth soft in
consistency ,temperature is normal , overlying
skinis normal slipsignis positive, not fluctuant
nor tranilluminating and is non reducible with
negative cough impulse .
DISCUSSION
Familial multiple lipomatosis is a very rare
benign condition. It is usually transmitted by
the autosomal dominant route of inheritance,
although cases with recessive inheritance have
alsobeen reported. FML mustbe distinguished
from MSL, which is a condition of diffuse and
symmetric fatty infiltration that occurs among
middle-aged men and those with a history of
alcoholism. A distinction between the two
conditions is the relative sparing of the neck
and shoulders in FML and the presence of
discrete lipomas rather than the diffuse and
symmetric lipomatous infiltration in MSL.
Clinical features of FML include multiple, well
encapsulated, oval-to-round, subcutaneous,
rubbery lipomas that range from a few
millimeters to 25 centimeters in size.
Spontaneous regression and malignant
degeneration are rare. The disease is not
associated with any abnormalities in lipid
metabolism. In the present study male/famale
ratio was found 6:1. Lipomas in FML begin to
appear during the third decade of life and may
continue to develop through the fifth decade
of life. Lipomas are generally restricted to the
arms, lower trunk, and thighs, and are
asymptomatic. Patient concerns with FML
usually are cosmetic. Lipomas are generally
painless and do not affect the daily activities of
afflicted individuals. Treatment can include
simple excision, endoscopic removal, or
liposuction. Surgical techniques have been
developed toremove dozens of tumorsat once
and with a minimal number of incisions and
scars. Ersek et al stated that FML is not
transmitted through simple dominant or
recessive genes, and the authors entertained
the possibility of it being a sexlinked disease,
affected by numerous ancillary variables. FML
is a rare autosomal-dominant inherited
disease. Although much is known of the
genetic abnormalities of sporadic lipomas, the
specific germ line genetic abnormality
responsible for FML is unknown. It also is not
known if the same genetic abnormality is
present in all families affected by FML or if
different genetic abnormalities produce the
samephenotype in different families.Basedon
the family tree prepared we concluded that
the disease is autosomal dominant inherited,
and also x-linked dominant inheritance is
possible. Further research is needed in this
respect.

Case report

  • 1.
    Case Report Familial MultipleLipomatosis : A rare case report Abstract: familial multiple lipomatosis is abenign hereditary syndrome INTRODUCTION Lipomas are the most common soft tissue tumor, lipoma is a benign tumor of adipose tissue, it is called as universal tumor of adipose tissue (ubiquitous tumor) as it can occur anywhere in the body (except brain), with an incidence of 2.1 per 1000 people. Multiple lipomas localised in different areas of the body are rare and can occur in specific hereditary syndrome. Although most lipomas are sporadic, 2 rare distinct familial types of lipomatosis have been identified: familial multiple lipomatosis(FML) and multiple symmetric lipomatosis (MSL) (also known as Madelung disease) Familial multiple lipomatosis is a benign hereditay syndrome occurring in a proposed Autosomal Dominant inheritance have also been reported. FML occur with incidence of 0.002% has been reported. Men are affected twice as commonly as women.FML usually appear mostly in third decade of life. The treatment of lipoma can include simple excision, endoscopic removal, or liposuction.
  • 2.
    The object ofpresenting the caseis to highlight this rare case . History:A 45 year old man presented with multiple small swellings of 25 year duration. Since the patient do not report the disease in his family First swelling appear in left upper half of chest. He developed few similar swelling on left arm ,right thigh and buttock swelings are painless. his general physical and systemic examination were within normal limits. the largest lipoma is about 3.4;2cm in size spherical to oval in shape, smooth soft in consistency ,temperature is normal , overlying skinis normal slipsignis positive, not fluctuant nor tranilluminating and is non reducible with negative cough impulse . DISCUSSION Familial multiple lipomatosis is a very rare benign condition. It is usually transmitted by the autosomal dominant route of inheritance, although cases with recessive inheritance have alsobeen reported. FML mustbe distinguished from MSL, which is a condition of diffuse and symmetric fatty infiltration that occurs among middle-aged men and those with a history of alcoholism. A distinction between the two conditions is the relative sparing of the neck and shoulders in FML and the presence of discrete lipomas rather than the diffuse and symmetric lipomatous infiltration in MSL. Clinical features of FML include multiple, well encapsulated, oval-to-round, subcutaneous, rubbery lipomas that range from a few millimeters to 25 centimeters in size. Spontaneous regression and malignant degeneration are rare. The disease is not associated with any abnormalities in lipid metabolism. In the present study male/famale ratio was found 6:1. Lipomas in FML begin to appear during the third decade of life and may continue to develop through the fifth decade of life. Lipomas are generally restricted to the arms, lower trunk, and thighs, and are asymptomatic. Patient concerns with FML usually are cosmetic. Lipomas are generally painless and do not affect the daily activities of afflicted individuals. Treatment can include simple excision, endoscopic removal, or liposuction. Surgical techniques have been
  • 3.
    developed toremove dozensof tumorsat once and with a minimal number of incisions and scars. Ersek et al stated that FML is not transmitted through simple dominant or recessive genes, and the authors entertained the possibility of it being a sexlinked disease, affected by numerous ancillary variables. FML is a rare autosomal-dominant inherited disease. Although much is known of the genetic abnormalities of sporadic lipomas, the specific germ line genetic abnormality responsible for FML is unknown. It also is not known if the same genetic abnormality is present in all families affected by FML or if different genetic abnormalities produce the samephenotype in different families.Basedon the family tree prepared we concluded that the disease is autosomal dominant inherited, and also x-linked dominant inheritance is possible. Further research is needed in this respect.