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liveonearthThe "rare syndromes" that are associated with multiple lipomas, according to my minor surgery notes, are "Gardner's syndrome and two benign conditions--multiple symmetrical lipomatosis and Dercum's disease. In the latter, the lipomas are painful". I had to look it up because my sister's husband is covered in them, and because in the last 2 years I've begun to develop them all over my body including strange locations such as over the biceps femoris tendon in my posteriolateral popliteal area--very small so far--I suspect a hormonal etiology. More occur in women after menopause, and familial types onset at adolescence. With some types there is an increased incidence of breast, endometrial and thyroid cancer. Sounds like it may be related with estrogen dominance. This would be supported by the increased incidence with obesity. There is also an association with multiple endocrine neoplasia type I (lipomas and pancreatic, parathyroid, pitutitary tumors). The list of hormonal associations increases the deeper I dig. There are also fat deposits associated with spina bifida. Looks like nobody has studied hormones in lipomatosis patients, but based on my short survey I have just now formed the opinion that is expressed in the title of this entry.
MULTIPLE LIPOMAS
fat cells in the subcu grow into masses that can be moved underneath the skin
the most common benign tumor
lumps range from pea sized to egg or MUCH bigger
familial
dt mitochondrial DNA mutation???
vast majority are painless
mc locations: arms, forearms, legs, trunk
also occurs: neck, face, buttocks
may reach hundreds
most appear during 40's and 50's
lipomas themselves seldom become malignant but lipoma pts have more cancer
INCIDENCE
about 1/3 of cases with large numbers are familial
43% of b9 mesenchymal tumors in Scandinavians were lipomas in one study
solitary more in women
multiple more in men
rare in children and infants
TYPES OF LIPOMAS
(including solitary lipomas, composite from many sources, many overlap)
--adiposis dolorosa (Dercum Disease): multiple painful subcutaneous lipomas, may not be circumscribed, usu on legs, pelvis or trunk, freq assoc with obesity or alcoholism
--angiolipoleiomyoma: solitary, acquired, asx, acral, incl sm muscle, BVs, CT and fat
--angiolipoma: painful, tender, soft, subcutaneous nodules, frequently multiple, occasionally familial, often present in adolescence, mb multilobulated and firm, vaguely painful w/ pressure or spontaneously
--Bannayan-Riley-Ruvalcaba / Bannayan-Zonana: multiple congenital lipomas, macrocephaly, hemangiomas, mental retardation
--benign symmetric lipomatosis (Madelung disease), men 4x more, head, neck, shoulders, and proximal upper extremities, pt hx often incl excessive alcohol or DM, see Madelung
--chondroid: deep, firm, yellow, on legs of women
--corpus callosum lipoma: rare, congenital, 1-3cm-->larger and sounds like a very bad location
--Cowden syndrome: multiple lipomas, GI hamartomatous polyps, trichilemmomas (sebaceous cysts), incr breast, endometrial and thyroid carcinoma
--Dercum's disease (adiposis dolorosa): rare, lipomas are multiple, large and painful, mc on extremities of obese postmenopausal women, mb assoc w/ alcoholism, emotional instability, depression
--diffuse congenital lipomatosis: usu on trunk or large area of extremity, usu occurs before age 2, overgrowth of mature fat, involves muscle and subcutaneous tissue but not nerves, recurrent
--encephalocraniocutaneous lipomatosis (Haberland syndrome): lipomatous hamartomas of the head and face, usu unilateral, congenital, cranial asymmetry and mental retardation
--familial multiple lipomatosis: few to many small encapsulated lipomas usu on extremities, usu appears during or after adolescence, neck and shoulders usu spared (opp: b9 symmetric), autosomal dominant inheritance
--fibrolipomatous hamartoma of nerve/neural fibrolipoma: mature adipose tissue with a fibrous component, infiltrating/surrounding a major nerve mc: median n, loc: epineural and perineural, metaplastic bone has been reported-->macrodactyly, usu under age 30, s/sx assoc with nerve impingment
--hibernoma: brown fat lipoma, usu asx, solitary well-circumscribed nodules, loc: interscapular, axillae, neck, or mediastinum, histol: embryonic brown lipoblasts termed mulberry cells
--intradermal spindle cell lipoma: in women, distrib around body
--lumbosacral lipoma/lipomatosis: diffuse incr in mature fat over lumboscral spine, always occurring with spina bifida or laminar defect, possible meningocele or myelocele, infants and children, may have associated skin tags, hemangioma or hair overgrowth
--Madelung’s Disease (Launois-Bensaude syndrome): multiple subcutaneous lumps AND others that impinge on larynx, esophagus, intestine, lungs, heart, usu not painful, associated with malig tumors of upper airways, hyperuricemia, obesity, renal tubular acidosis, peripheral neuropathy, and liver disease, see b9 symmetric, usu neck, shoulders, upper arms, described as pseudoathletic or horse collar, frequently associated with diabetes and alcohol abuse, mc in men of Mediterranean origin
--MERRF / Ekbom syndrome: myoclonus, epilelpsy and ragged-red fibersm lipomas, skeletal deformities, cerebellar ataxia, photomyoclonus, mtDNA tRNA mutation, multiple endocrine neoplasia type 1: lipomas and pancreatic, parathyroid, pitutitary tumors
--neural fibrolipoma: fibro-fatty tissue along nerve trunk-->nerve compression
--pelvic lipomatosis: usu in black males age 9-80, perivesical and perirectal overgrowth, rarely involves retroperitoneum and mesentery, frequent cystitis glandularis, may develop adenocarcinoma (see proteus synd)
--pleomorphic lipomas: mostly on backs/necks of elderly men, floret giant cells with overlapping nuclei
--Proteus syndrome: congenital pelvic lipomatosis, macrodactyly, exostoses, hemangiomas, linear sebaceous nevi
--solitary lipomas: mc, superficial, small, develops with weight gain but doesn't shrink with weigh loss
--spindle-cell lipoma: asx, slow growing, same loc as pleomorphic
--superficial subcutaneous lipoma: mc type, usu on trunk, thighs, forearms
TREATMENT
excision or liposuction
most will grow back and must be removed repeatedly
tumors may infiltrate muscle and be harder to remove
multilobular harder to remove
PATHOPHYSIOLOGY of SOLITARY
increased levels of lipoprotein lipase
presence of a larger number of precursor cells
~60% of solitary cutaneous lipomas display clonal alterations
mc: breakpoint on bands 12q13-15
also: karyotype aberrations on arms 6p and 13q
SOURCES SURFED
http://surgpathcriteria.stanford.edu/softfat/lipoma/multiple_lipomas.html
Robert V Rouse MD rouse@stanford.edu
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
http://www.medonc.com/lipomas.htm
http://www.ahealthyme.com/topic/sublipomas
http://en.wikipedia.org/wiki/Lipoma
STILL NEED TO HARVEST INFO FROM
http://www.lipomadoc.org/3501.html
HOPI WOMAN WITH DERCUM'S
http://azdailysun.com/news/local/article_e54fc832-3e9b-11e0-aed9-001cc4c002e0.html
MULTIPLE LIPOMAS
fat cells in the subcu grow into masses that can be moved underneath the skin
the most common benign tumor
lumps range from pea sized to egg or MUCH bigger
familial
dt mitochondrial DNA mutation???
vast majority are painless
mc locations: arms, forearms, legs, trunk
also occurs: neck, face, buttocks
may reach hundreds
most appear during 40's and 50's
lipomas themselves seldom become malignant but lipoma pts have more cancer
INCIDENCE
about 1/3 of cases with large numbers are familial
43% of b9 mesenchymal tumors in Scandinavians were lipomas in one study
solitary more in women
multiple more in men
rare in children and infants
TYPES OF LIPOMAS
(including solitary lipomas, composite from many sources, many overlap)
--adiposis dolorosa (Dercum Disease): multiple painful subcutaneous lipomas, may not be circumscribed, usu on legs, pelvis or trunk, freq assoc with obesity or alcoholism
--angiolipoleiomyoma: solitary, acquired, asx, acral, incl sm muscle, BVs, CT and fat
--angiolipoma: painful, tender, soft, subcutaneous nodules, frequently multiple, occasionally familial, often present in adolescence, mb multilobulated and firm, vaguely painful w/ pressure or spontaneously
--Bannayan-Riley-Ruvalcaba / Bannayan-Zonana: multiple congenital lipomas, macrocephaly, hemangiomas, mental retardation
--benign symmetric lipomatosis (Madelung disease), men 4x more, head, neck, shoulders, and proximal upper extremities, pt hx often incl excessive alcohol or DM, see Madelung
--chondroid: deep, firm, yellow, on legs of women
--corpus callosum lipoma: rare, congenital, 1-3cm-->larger and sounds like a very bad location
--Cowden syndrome: multiple lipomas, GI hamartomatous polyps, trichilemmomas (sebaceous cysts), incr breast, endometrial and thyroid carcinoma
--Dercum's disease (adiposis dolorosa): rare, lipomas are multiple, large and painful, mc on extremities of obese postmenopausal women, mb assoc w/ alcoholism, emotional instability, depression
--diffuse congenital lipomatosis: usu on trunk or large area of extremity, usu occurs before age 2, overgrowth of mature fat, involves muscle and subcutaneous tissue but not nerves, recurrent
--encephalocraniocutaneous lipomatosis (Haberland syndrome): lipomatous hamartomas of the head and face, usu unilateral, congenital, cranial asymmetry and mental retardation
--familial multiple lipomatosis: few to many small encapsulated lipomas usu on extremities, usu appears during or after adolescence, neck and shoulders usu spared (opp: b9 symmetric), autosomal dominant inheritance
--fibrolipomatous hamartoma of nerve/neural fibrolipoma: mature adipose tissue with a fibrous component, infiltrating/surrounding a major nerve mc: median n, loc: epineural and perineural, metaplastic bone has been reported-->macrodactyly, usu under age 30, s/sx assoc with nerve impingment
--hibernoma: brown fat lipoma, usu asx, solitary well-circumscribed nodules, loc: interscapular, axillae, neck, or mediastinum, histol: embryonic brown lipoblasts termed mulberry cells
--intradermal spindle cell lipoma: in women, distrib around body
--lumbosacral lipoma/lipomatosis: diffuse incr in mature fat over lumboscral spine, always occurring with spina bifida or laminar defect, possible meningocele or myelocele, infants and children, may have associated skin tags, hemangioma or hair overgrowth
--Madelung’s Disease (Launois-Bensaude syndrome): multiple subcutaneous lumps AND others that impinge on larynx, esophagus, intestine, lungs, heart, usu not painful, associated with malig tumors of upper airways, hyperuricemia, obesity, renal tubular acidosis, peripheral neuropathy, and liver disease, see b9 symmetric, usu neck, shoulders, upper arms, described as pseudoathletic or horse collar, frequently associated with diabetes and alcohol abuse, mc in men of Mediterranean origin
--MERRF / Ekbom syndrome: myoclonus, epilelpsy and ragged-red fibersm lipomas, skeletal deformities, cerebellar ataxia, photomyoclonus, mtDNA tRNA mutation, multiple endocrine neoplasia type 1: lipomas and pancreatic, parathyroid, pitutitary tumors
--neural fibrolipoma: fibro-fatty tissue along nerve trunk-->nerve compression
--pelvic lipomatosis: usu in black males age 9-80, perivesical and perirectal overgrowth, rarely involves retroperitoneum and mesentery, frequent cystitis glandularis, may develop adenocarcinoma (see proteus synd)
--pleomorphic lipomas: mostly on backs/necks of elderly men, floret giant cells with overlapping nuclei
--Proteus syndrome: congenital pelvic lipomatosis, macrodactyly, exostoses, hemangiomas, linear sebaceous nevi
--solitary lipomas: mc, superficial, small, develops with weight gain but doesn't shrink with weigh loss
--spindle-cell lipoma: asx, slow growing, same loc as pleomorphic
--superficial subcutaneous lipoma: mc type, usu on trunk, thighs, forearms
TREATMENT
excision or liposuction
most will grow back and must be removed repeatedly
tumors may infiltrate muscle and be harder to remove
multilobular harder to remove
PATHOPHYSIOLOGY of SOLITARY
increased levels of lipoprotein lipase
presence of a larger number of precursor cells
~60% of solitary cutaneous lipomas display clonal alterations
mc: breakpoint on bands 12q13-15
also: karyotype aberrations on arms 6p and 13q
SOURCES SURFED
http://surgpathcriteria.stanford.edu/softfat/lipoma/multiple_lipomas.html
Robert V Rouse MD rouse@stanford.edu
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
http://www.medonc.com/lipomas.htm
http://www.ahealthyme.com/topic/sublipomas
http://en.wikipedia.org/wiki/Lipoma
STILL NEED TO HARVEST INFO FROM
http://www.lipomadoc.org/3501.html
HOPI WOMAN WITH DERCUM'S
http://azdailysun.com/news/local/article_e54fc832-3e9b-11e0-aed9-001cc4c002e0.html
