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Lipoma

DEFINITION AND PATHOGENESIS

  • Classification
    • Lipoma
      • Superficial (esp back, shoulders, neck, abdomen)
      • Deep (intramuscular, intermuscular, lumbosacral, perineural, lipoma of tendon sheath)
      • Intraarticular--not lipoma arborescens
    • Lipoma variants
      • Angiolipoma
      • Spindle cell lipoma
      • Pleomorphic lipoma
      • Benign lipoblastoma/lipoblastomatosis
      • Angiomyolipoma
      • Angiolipoma
      • Angiomylipoma
      • Chondroid lipoma
      • Lipomatosis
      • Myolipoma
      • Extra-adrenal myelolipoma
      • Extrarenal angiomyolipoma
      • Hibernoma
    • Lipomas intimately associated with other tissues
      • Intramuscular (infiltrating)
      • Intermuscular lipoma
      • Lipoma of tendon sheath (endovaginal lipoma)
      • Involving major nerves of the hand and wrist
        • Fibrolipomatous hamartoma
        • Neural fibrolipoma
        • Perineural lipoma
      • Lumbosacral lipoma
      • Periosteal (juxtacortical) lipoma
      • Involving the synovium (villous, lipomatous proliferation of the synovial membrane, lipoma arborescens)
      • Involving the bronchial tract
      • Intravascular lipoma
        • Intracardiac lipoma
      • Lipomas associated with the GI tract
    • Infiltrating proliferations of mature fat
      • Diffuse lipomatosis
      • Pelvic lipomatosis
      • Cervical symmetrical lipomatosis (Madelung's disease)
      • Adiposis dolorosa (Dercum's disease)
      • Steroid lipomatosis
    • Hibernoma (brown fat such as in hibernating animals)

IMPORTANCE

  • Most common tumor of soft tissue

CLINICAL FEATURES

  • Most common neoplasm of mesenchymal origin
  • Rare first two decades
  • M=F
  • Asymptomatic, slow-growing, round or discoid mass having a soft or doughy consistency (hardens after application of ice), rarely painful (except angiolipoma, most often forearm lesions)
  • Lipoma of tendon sheath occurs distally (50% bilateral)
  • Spindle cell and pleomorphic lipomas characteristically in male patients between 45-65 in the posterior neck and shoulder, painless and slowly growing
  • Lipomatous proliferation of the synovial membrane may be bilateral (?20%), of long-standing duration, and associated with DJD, DM, popliteal cysts (20%), joint trauma, psoriatic arthritis (knee most common, also wrist, shoulder, hip, subdeltoid bursa)
  • Average age 41 (9-90)
  • M:F = 1.3:1
  • Different than an intraarticular lipoma
  • Young to middle-aged BMs most often affected in pelvic lipomatosis
  • Intraneural fibrolipoma infiltrates large peripheral nerves (33% associated with macrodactyly/fatty overgrowth) with nerve compression sxs and gradual loss of function
  • Fatty growth involving neck ("horse collar") herald's Madelung's disease with EtOH abuse
  • Radicular sxs
    • Suprascapular nerve entrapment with large lipoma has been reported
  • Lipomas in the hand
    • May uncommonly be associated with compression of the median nerve and CTS
      • Complete return of function can be expected after resection
    • Can be an unusual cause of macrodactyly
    • Has been reported in an anomalous lumbrical muscle belly causing CTS and 'trigger wrist'
  • Lipomas of oral and maxillofacial region (in order of prevalence):
    • Parotid region
    • Buccal mucosa
    • Lip
    • Submandibular region
    • Tongue
    • Palate
    • Floor of mouth
    • Vestibule
    • Retropharyngeal
  • Unusual location

     

RADIOLOGIC FEATURES

  • Radiolucent round soft tissue mass ? calcification
  • CT and MRI reveal homogeneous fat density (both T1- and T2-weighted images)
  • MRI of hibernoma, because of hypervascularity may resemble liposarcoma, esp interscapular region, neck, mediastinum, retroperitoneum
  • PET
    • Mean SUVs lower than with liposarcoma
      • Cut-off value of 0.81 provides significant differentiation between benign lesions and wll-differentiated liposarcoma
  • Myelolipoma occurs in the adrenals, retroperitoneum and pelvic soft tissues

GROSS PATHOLOGY

  • Well delineated from surrounding tissues by a thin capsule
  • Orange yellow lesion in hibernoma
  • Villous fatty mass seen arthroscopically in lipoma arborescens
  • Fatty frond-like appearance

HISTOLOGIC FEATURES

  • Cell size varies slightly and slightly larger than mature fat cells (lipocytes have small nuclei at the periphery of the cell)
  • Rich vascularity not readily discernible (except in angiolipoma, which has diagnostic intraluminal fibrin thrombi as well as ± perivascular fibrosis)
  • Fibrous connective tissue may be part of tumor
  • Bone marrow elements and fat cells in myololipoma
  • Host tissue atrophied (muscle, nerve)
  • Hibernoma has lipocytes with vacuolated cytoplasm and dark-staining nucleus
  • Floret-type giant cells in pleomorphic lipoma have periperipherally placed nuclei
  • Uniform spindle cells + mature fat lacking pleomorphism ± mast cells ± vascular pattern ± myxoid change
  • Chondroid or osseous metaplasia is rare, calcification from infarction
  • ± necrosis, hemorrhage may be associated with inflammatory cells
  • Synovial lipoma with proliferation of fat within synovial villi
  • Replacement of subsynovial tissue by mature fat cells
  • Lumbosacral lipomas have neuroglia, ependymal elements, and smooth muscle
  • Liposfuscin pigment within cytoplasm of multivacuolated brown fat cells is golden-brown in hibernoma which typically grows within striated muscle
  • Special stains:
    • Trichrome highlights septae

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • With the advent of MRI, the diagnosis is only in question when areas of inhomogeneousy are present, giving a differential of the liposarcoma variants
  • Lipoma-like hibernoma
  • Histologically, leiomyoma or leiomyosarcoma are confused with myolipoma and angiomyolipoma which histologically contain smooth muscle elements
  • Occasionally angiosarcoma in angiolipoma
  • Rhabdomyoma, granular cell tumor, and round cell liposarcoma in hibernoma
  • Extramedullary hematopoiesis in myelolipoma

DISEASE COURSE AND TREATMENT

  • Recurrence with marginal excision is <5% (0-80% reported) (esp intramuscular lipoma which infiltrates skeletal muscle), and malignant changes are reportable
  • Marginal (not intralesional) resection recommended
  • Excision is never as easy as when first diagnosed
  • Multiple lesions in 5-6% of patients (small % with familial multiple lipomas), multiple angiolipomas are more common than solitary ones, are painful, and most commonly are in the forearm
  • Lipomatosis in Madelung's disease may be extensive to involvement of arms, axillae, mediastinum (and may compromise airway); halting with EtOH intake
  • Diffuse lipomatosis may cause bony hypertrophy
  • Pelvic lipomatosis associated with hydronephrosis and uremia or GI obstruction
  • Lumbosacral lipoma often (67%) associated with progressive radiculopathy (spina bifida and spinal cord lipoma)
  • Lipoma involving the synovium (lipoma arborescens), if not extensive, has been reported to have been txd with arthroscopic resection (bilateral knee tx also reported)
  • Tx is complete excision including synovium involved
  • Rarely reported in hip
  • Intraneural lipofibroma (lipofibromatous hamartoma) can be dissected free off fascicles with preservation of nerve function
    • Ulnar nerve involvement at the elbow reported
  • Endoscopic resection of GI lipomas reported
  • Laparoscopic resection of colonic lipomas reported 
  • Malignant epithelioid hemangioendothelioma arising in an intramuscular lipoma has been reported

SPECIAL CONSIDERATIONS

  • Multiple lipomas

    • (Superficial lesions)
    • Non-familial cases account for the majority of cases
  • Multiple Familial Lipomas

    • ? A
  • Lipoma Arborescens

    • Intra-articular lipoma

  • Lipomatoses

    • Nevus Lipomatosis Superficialis
      • Developmental nevoid form of lipoma characterized by the ectopic presence of mature adipocytes in the reticular dermis

  • Fibrolipoma 

    • Commonly occurs in the head/neck region (1-4% of lipomas)
    • Animal Model
      • Reported occuring in a retroparietal location in association with retroparietal osteochondroma in a calf
    • (Intra)Neural Fibrolipoma
      • Fibrolipomatous hamartoma of the median nerve
  • Bannayan-Zonana Syndrome

    • (Multiple lipomas, macrocephaly, hemangiomas)
  • Cowden Syndrome

    • (Multiple lipomas, hemangiomas associated with goiter, skin and mucosal lichenoid lesions)
  • Frohlich Syndrome

    • (Multiple lipomas, obesity, sexual infantilism)
  • Proteus Syndrome

    • Harmartomatous condition characterized by patchy or segmental overgrowthand hyperplasia with susceptibi8lity to development of tumors
      • Hemagiomas
      • Lipomas
      • Lymphangiomas
      • Varicosities
      • Verrucous epidermal nevi
      • Macrocephaly
      • Cranial exostosis
    • Hemihypertrophy and overgrowth reported presenting from 2 months to 10 years
    • (Multiple lipomatous lesions, feet/hand fibroplasia, skeletal hypertrophy, exostoses, scoliosis, pigmented skin lesions)(other neoplasms have been reported)
    • Acetabular dysplasia associated with intra-articular lipomatous lesions has been reported
    • Joseph Carey Merrick (the Elephant Man), described by Treves is thought to have had this syndrome (not NF1)
  • Spinal (Epidural) Lipoma, Spinal Angiomyolipoma, Spinal Angiolipoma

    • Usually related to steroid use
    • Neurological sxs vary depending on level involved
    • Mobilization off the compressed dura via laminectomies has been reported
    • May be due to venous thrombosis of the lesion
    • Extracanal extension into the posterior mediastinum has been reported
    • MRI diagnostic
  • Spinal (Epidural) Lipomatosis

    • Rarely found without steroid use or endocrinopathy
    • Obesity nearly always reported in idiopathic cases
    • M>F
    • MRI diagnostic
    • Thoracic level most commonly affected
    • Complete resolution of sxs with weight reduction
    • Laminectomy and fat debulking successful but not without risks

     

    • Spinal Cord Lipoma
      •  

        •  Dorsal lipoma

          •  Lipoma–cord interface is entirely on the dorsal surface of the lumbar spinal cord, always sparing the distal conus lipoma

    • Spinal (Epidural) Angiolipoma
      • Spindle shaped located in the posterior epidural space

      • Fat and vascular elements on MR imaging (either may predominate: type 1 predominantly lipomatous; type 2 predominantly vascular)

        • Vascular content:

          • Hypointense regions on T1-weighted imaging
          • Hyperintense regions on T2-weighted imagin
          • Marked enhancement

             

        • Fat content:

          •  Isointense with epidural fat

  • Intravascular Lipoma

 

    • Superior vena cava location reported
      • Nonenhancing 9 X 5 cm tumor occluding R subclavian vein, brachiocephalic vein, and SVC to the R atrium reported on CT
      • MRI reported as isointense lesion with fat
      • PET reported with no fludeoxyglucose F 18 uptake
      • Surgical resection through combined median sternotomy and R transclavicular cervicotomy
        • Control of SVC, subclavian, internal jugular and azygos vein with this approach
    • Intracardiac Lipoma

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