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Pseudotumorous Conditions

Aneurysm

  • ± trauma hx
  • True aneurysm contains all 3 layers (intima, media, adventitia) of arterial wall
  • Etiologies: blunt trauma, connective tissue disorders, infection (mycotic), atherosclerotic, idiopathic

 

Arachnoid cysts (spinal meningeal cysts)

  • Radicular pain, progressive weakness, scoliosis, recurrent UTI
  • Types
    • Type I: extradural cyst without spinal nerve root fibers
    • Type II: extradural cysts with spinal nerve root fibers
    • Type III: intradural cysts
  • Tx: excision, fenestration, or shunting

Arteriovenous malformations

  • (Developmental with normal endothelial turnover)(hemangiomas are neoplastic with endothelial hyperplasia)
  • Mulliken and Glowacki classification
  • Predominant vessl found: venous, arterial, capillary, lymphatic, mixed
  • High versus low flow
  • Localized (involving a single tissue)
  • Diffuse (involving >1 tissue)
  • Recurrence after resection in up to 50%
  • Partial resection may give some relief
  • Bleeding has been reported to cause local neuropathy
  • PIN palsy reported

Bone infarct (osteonecrosis, avascular necrosis)

Bone marrow edema syndrome

  • Has been reported in large pelvic masses and increased intrapelvic pressure has been suggested as a cause

Brown tumor of hyperparathyroidism

Bursitis

  • Olecranon
  • Retrocalcaneal
  • MRI induced by stimulation of post-surgical metallic particles reported
  • Enlarged bursae may simulate tumor
    • Bicipital bursa has been reported to cause PIN compressive sxs

Calcific discitis

  • Ddx includes idiopathic, degenerative, spinal ankylosis, previous fusion, ankylosing spondylitis, DISH, juvenile-onset RA, Klippel-Feil syndrome, alkaptonuria, hemochromatosis, calcium pyrophosphate dihydrate crystal deposi-tion disease, acromegaly, hyperparathyroidism, amyloidosis, myositis ossificans progressiva

Calcific myonecrosis

  • Reported after compartment syndrome following trauma
  • Interval from injury reported to be 10-64 yrs
  • Long duration of enlarging mass
  • Peripheral plaque-like calcification ± periosteal reaction
  • Wound complications not unusual after biopsy or excision of the paste-like material

Charcot joint (neuropathic arthropathy)

  • Painless enlargement and joint crepitus and distention
  • Most common shoulder cause is cervical syrinx
  • Radiographic signs of joint destruction
  • Dislocation, fracture, fragmentation, heterotopic ossification, osseous debris
  • Nonoperative tx (bracing, observation) most often recommended to maintain a functional arc of motion
  • Operative tx of associated ulnar or radial compressive sxs reported

Congenital maldevelopment of an intervertebral disc

  • Has been reported to simulate an intraosseous spinal tumor

Dermatomyositis

  • May have dystropic soft-tissue calcifications

Diabetic amyotrophy (Bruns-Garland syndrome)

  • Pain attributed to ischemia of involved nerve

Diffuse idiopathic skeletal hyperostosis (DISH)(Forestier's disease)

  • Diagnostic criteria
    • Ossification anterolaterally along ³4 contiguous vertebrae
    • Disc height preservation and no significant DJD changes
    • No facet joint ankylosis; no joint erosions, sclerosis or intra-articular fusion

Dorsal defect of the patella (superolateral aspect of patella)

 

Endometriosis

  • Abdominal wall involvement (rectus abdominus) has been described, mimcking a soft tissue sarcoma
  • Has been reported in the vastus lateralis
  • Fluctuates with monthly cycles

Eosinophilic fasciitis

  • Edematous, indurated skin and soft tissues
  • ± associated joint contracture
  • Hypergammaglobulinemia, ? ESR, peripheral eosinophilia
  • Thickened, edematous fascia with infiltration of mononuclear cells
  • Judicious surgical management + prednisolone

Epidermoid cyst

Fat Necrosis

  • Nodular-cystic fat necrosis described related to trauma and subsequent rapid vascular insufficiency

Fluorosis

Focal fibrocartilaginous dysplasia

  • Tibia vara (9-28 months at presentation) that spontaneously corrects
  • Fibrocartilaginous tissue at a medial tibial metaphyseal cortical defect with surrounding sclerosis

Geode

  • Osteoarthritic

Glial heterotopia (heterotopic neural rest)

  • Brain tissue occurring outside the cranial vault has been reported on the chest wall, paraspinal region

Gossypiboma

  • (From gossypion [Latin] = cotton), cotton balloma
  • Retained surgical sponge with development of an aseptic granuloma
  • Reported after up to 50 yrs!

Gout, tophaceous (tophi)

Hematologic disorders

  • Fanconi's anemia
  • Hemoglobinopathies
  • Thalassemia (Cooley's anemia, Mediterranean anemia)
    • Extramedullary hematopoiesis in Thalassemia Intermedia
      • Liver, spleen, lymph node, paravertebral, intrathoracic, pelvic locations
      • Incidence in up to 20% of pts
        • Peak age incidence 21-30 yrs
      • Paraspinal location occurs in 11-15% of cases
      • 80% asymptomatic
      • Can present as tumor-like masses
      • M:F = 5:1
      • Pathophysiology
        • Extrusion of hematopoietic tissue through thinned trabeculae
        • Spread through intercostal veins
        • Spread from adjoining ribs
      • MRI
        • Active stage
          • Isointense in both T1 and T2
        • Inactive stage
          • Hypointense or hyperintense in both T1 and T2
      • Biopsy
        • Hypervascular so think of bleeding complications
      • Management
        • Blood transfusion
          • Target Hgb of at least 10 mg/dl
        • Radiotherapy
          • 10-30 Gy reported
          • Monotherapy with 50% excellent response within 5-7 days
          • High recurrence rate of 19-37%
          • Used in combination with surgery/hydroxyurea
        • Surgical decompression
          • Laminectomy when epidural involvement and neurological compromise
          • Bleeding and subsequent inadequate decompression a problem
        • Hydroxyurea
          • Promotes fetal Hgb production
          • Used when there is no urgency in management
  • Sickle cell disease

Hemophilic pseudotumor

  • Has been reported to spontaneously regress

Hematoma

  • ± hx of anticoagulation
  • ± compressive neuropathy
  • Hip pain reported from psoas hematoma
  • Tumors may masquerade as hematoma resulting in a significant diagnostic delay
    • Absence of subcutaneous ecchymosis in the majority of cases is an important clue of a malignant dx

Hernia

Lumbar hernia (Grynfeltt-Lesshaft hernia)

  • May simulate paraspinal mass

Hydatic (echinococcal) cyst

Hypertrophy/pseudohypertrophy of an extremity

  • Has been reported with chronic radiculopathy (neurogenic hypertrophy)

Idiopathic diabetic muscle infarction

  • May present with a mass and MRI findings suggestive of sarcoma
  • Histologic appearance includes hemorrhagic necrosis, lymphocytic infiltration

Incontinentia pigmenti

  • With onychodystrophic changes and painful "periungual tumors" ± associated lucent distal phalangeal changes (X-linked dominant)

Infantile cortical hyperostosis (Caffey's disease)

  • Self-limiting disease of infancy/early childhood
  • 3/1000 pts < 6 mos of age
  • Diffuse polyostotic periosteal reactions, esp mandible, tibia, clavicle, ulna
  • Has been reported in twins
  • Ddx
    • Menkes syndrome
    • Engelmann-Camurati disease
    • Osteomyelitis
    • Congenital syphilis
    • Battered child syndrome
    • Burns
    • Hypervitaminosis A
    • Scurvy
    • Hyperphosphatemia
    • Leukemia
    • Neuroblastoma
    • Iatrogenic from prostaglandin Rx

Intravascular papillary endothelial hyperplasia

  • 3 forms:
    • Found inside thrombosed vessels
    • Associated with vascular tumors or malformations
    • Arising within a hematoma (post-trauma or surgery)
  • Rare firm mass, commonly occurring in the hand, probably resulting from an alteration in thrombosis (exaggerated attempt at thrombus recanalization mediated by an autocrine loop of endothelial basic fibroblast growth factor)
  • ± overlying bluish/reddish discoloration of the skin
  • Important to distinguish from angiosarcoma (florid intravascular endothelial proliferation)
  • Has been misdescribed as a hemangioendothelioma

Massive lymphedema

Mercury granuloma

  • Radiopaque particles in subcutaneous tissue reported having entered through a cut after topical exposure
  • Surgical removal of particles is necessary
  • Serum mercury level
  • Penicillamine 10 mg/kg TID for systemic toxicity

Myositis

  • (Focal myositis, focal interstitial polymyositis, interstitial nodular myositis, focal nodular myositis)

Inflammatory myopathy

  • Unknown etiology
  • Enlarged mobile mass, typically unilateral and limited to a single muscle group
  • Duration of sxs: 2 wks - 3 yrs
  • Leg (38%), thigh (23%)
  • Rare locations: triceps, EDB, brachioradialis, rectus abdominus
  • Constitutional findings in up to 11%: fever, malaise, skin rash, generalized muscle dysfunction
  • ± ?? CK (24%), ESR (26%)
  • CT/MRI: irregular enlargement with no discrete mass, poorly defined fatty infiltration, inflammation, edema
  • Bx: inflammatory infiltrates (seen along endomysial sheaths mostly of small mature T-lymphocytes and macrophages) with variation in fiber size; randomly distributed necrotic cells with regenerative attempts
  • Tx: NSAIDs; surgical resection has been reported; may progress to polymyositis
  • ?? CK or ESR, multiple nodules, limb atrophy may indicate risk for recurrence or progression to multifocal inflammatory myopathy

Necrosis of muscle

  • Isolated brachialis muscle necrosis reported after exercise

Ochronosis

Os acromiale

  • Types: preacromion, mesoacromion, meta-acromion in ascending order of size may be confused with tumor

Ossification of the posterior longitudinal ligament of the spine

Osteochondritis dissecans (OCD)

Osteolysis

  • Particle-induced bone resorption
  • Metal-polyethylene
  • Wear debris-challenged osteoblasts produce PGE2, IL-6: activators of osteoclasts
  • They also produce IL-9 and MCP-1: chemotactic factors for leukocytes
  • Metal-metal

Osteomyelitis

Paget disease

  • ± familial hx

Postsurgical ossified extradural cyst

Proliferative myositis

  • Pseudosarcomatous lesion
  • Painful rapidly growing mass
  • ± hx of trauma
  • MRI shows an ill-defined mass
    • ? signal intensity on T1-weighted images
    • ? signal intensity on T2-weighted images
  • Giant ganglion-cell-like cells (+ for vimentin and smooth muscle actin) admixed with spindle cells
  • Mitoses common
  • ± focal calcification

    Pseudoaneurysm

  • Etiologies: penetrating trauma or fx, unusually blunt trauma
  • MRI with ? signal on T1- and T2-weighted images due to flow void

Pseudocysts of bone

  • Humeral pseudocyst
  • Lucency of the humeral head adjacent to the greater tuberosity, a normal variant

Pseudopathologic fx

  • External rotation of the femoral shaft results in an appearance similar to that of a pathologic fx of the femoral neck

Pulmonary hypertrophic osteoarthropathy

  • Diffuse periostitis of long bones
    • Primary
    • Familial
    • Secondary
    • Pulmonary tumor cause
      • Bronchogenic carcinoma (most common)
    • Metastatic osteosarcoma to the lung has been a reported cause
    • Non-pulmonary causes
      • Has been reported with an aortic, axillo-femoral, and axillo-axillary graft infection
      • Patent ductus arteriosus (PDA)
      • Venous stasis
      • Idiopathic periosteal hyperostosis with dysproteinemia (Goldbloom's syndrome)

Reaction to polylevolactic acid fracture fixation

Reactive angioendotheliomatosis

  • Cutaneous vascular proliferation presenting as multifocal erythematous macules, purpuric papules, ecchymoses, purpuric plaques ± ulceration
  • Intravascular proliferation of endothelial cells
  • Underlying coexistent systemic diseases: renal disease, valvular cardiac disease, alcoholic cirrhosis, glioblas-toma multiforme, rheumatoid arthritis, polymyalgia rheumatica

Rosai-Dorfman disease

  • Sinus histiocytosis with massive lymphadenopathy, with rare solitary or multiple bone lesions (8%) reported
  • Lucent lesion(s) ± surrounding sclerosis
  • Fever, leucocytosis, ? ESR, hypergammaglobulinemia, lymphadenopathy
  • Histiocytes, plasma cells, neutrophils, no eosinophils
  • DDx includes histiocytosis X and osteomyelitis

Schitzler's syndrome

  • Bone pain (70%), chronic nonpruritic urticaria, monoclonal immunoglobulin M gammopathy
  • ?? ESR (up to 101mm/hr reported)
  • ? CRP
  • ? WBC with neutrophilia
  • ? uptake on bone scan (40%)
  • ± periosteal reactions on plain radiographs
  • + anti-interleukin-1 autoantibodies
  • MRI: marrow infiltration, ? T1-weighted signal, ? T2-weighted signal
  • No observable bx abnormality of involved bones
  • DDx: Waldenstrom's macroglobulinemia, POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin lesions)
  • Tx: corticosteroids

Sclerosing extramedullary hematopoietic tumor

  • Myelofibrosis, anemia, and soft tissue masses
  • Encapsulated yellow-tan nodular growth with bizarre giant cells in prominent dense fibrous stroma
  • + factor VIII staining of the atypical giant cells
  • + CD43

Stress-induced osteolysis of the distal clavicle

  • (See also Chapter 11)

Stress fx

  • Transverse sclerotic line
  • Affects 0.96-64% of military recruits
    ? alk? (37.6 vs 26.2 units/L)
    ? osteocalcin (10.8 vs 8.8 ng/mL)
    ? 25-OH vitamin D (25.3 vs 29.8 ng/mL)
  • Bilateral stress fxs activity related
  • Femur: track, inline skating, competitive equestrian athletes
  • Insufficiency fx: due to normal stresses upon abnormal bone
  • Fatigue fx: due to abnormal forces applied to normal bone
  • Sites of involvement depend on diagnostic studies
  • Bone scan:
    • 49.1% tibia, 25.3% tarsals, 8.8% MTs, 72.% femur, 6.6% fibula, 1.6% pelvis, 0.9% sesamoids, 0.6% back
    • 16.6% bilateral
  • X-ray:
    • 35.2% MTs, 28% calcaneus, 24% tibia, 5.6% ribs, 3.2% femur, 3.2% fibula, 0.4% spine, 0.4% pubic ramus
  • Radiologic features
    • X-ray: normal to graying of cortex, fluffy or subtle periosteal reaction, fx, transverse or curvilinear sclerosis, multiple transverse lucencies, callus, cortical thickening
    • Bone scan: ? uptake, intensity depens of grade of stress injury
    • MRI: periosteal edema, bone marrow edema, fx line
  • Histologic features
    • First stage: within hours to days; hematoma
    • Second stage: within 48 hours; inflammation characterized by polymorphonuclear leukocytes, macro-
      phages, mast cells; osteoclasts begin removing c\necrotic bone
    • Third stage: 2-12 days; early granulation tissue formation with fibroblasts, initial collagen depostion; ? vascularity
    • Fourth stage: 1 wk to several months; soft callus; ? vascularity; proliferation of osteoblasts in cambium layer of periosteium (incipient periosteal new bone formation); external (surface) and internal (marrow) callus
    • Fifth stage: following 3-4 wks hard bony callus develops; bone forming by both membranous (periosteal) and endochondral ossification; matrix production is predominant with less evidence of inflamma-tion & vascularity
    • Sixth stage: several months; prolonged period of bone remodeling converting early stage of woven bone to more mature lamellar bone; "secondary" callus; intense osteoblast and osteoclast activity

Synovial metaplasia

  • Most often adjacent to silicone or metal prosthesis

Synovitis

  • Hemorrhagic synovitis

    • May mimic tenosynovial giant cell tumor (previously known as pigmented villonodular synovitis)

Tuberculosis

  • Subchondral osteolysis

Tuberous sclerosis (Bourneville-Pringle syndrome)

  • Triad: epilepsy (60-80%), mental retardation, skin lesions (hamartomas)(adenoma sebaceum (80-90%), shagreen patches, periungual fibromas, hypopigmented macules)
  • 1/6-10,000 incidence
  • Two genes involved: TSC1 (9q34) and TSC2 (16p13)
  • Giant cell astrocytomas
  • Renal angiomyolipomas (may present with abdominal mass)
  • Cardiac rhabdomyomas
  • Lung involvement in < 1%
  • Multiple sclerotic or radiolucent skeletal lesions (mimick metastases)
  • MRI has been used to identify tumors in brain and cardiac locations in early gestation

Turret exostosis

  • Traumatic injury to periosteum, usually in the border digits resulting in an exostosis

Vertebral pneumatocyst

Xanthoma

  • Familial hypercholesterolemia
  • AD
  • Affects LDL receptor gene
  • ? LDL, ? serum cholesterol
  • Premature coronary artery disease
  • Incidence reportedly 1/500
  • Complete excision may not be possible (because of intertwining with the involved tendons or NV structures)
  • 60% recurrence rate
  • Extensor tendons involved in 90%
  • DDx: GCTTS, lipoma, dermatofibroma, rheumatoid nodule, gouty tophi, keratogenous cysts, sarcomas with xanthomatous change
  • US: hypoechoic nodules or heterogeneous echo pattern
  • MRI: heterogeneous signal intensity on T1- and T2-weighted images, hyperintense to involved tendon
  • Cerebrotendinous xanthomatosis
    • Autosomal recessive lipid-storage disease
    • Bilateral Achilles tendon xanthoma
    • Bilateral cataract formation
    • Progressive neurological dysfunction
    • Pyramidal tract signs, cerebellar ataxia, cognitive impairment, peripheral neuropathy
    • Osteoporosis with ? risk of fx
    • Mutation in the sterol 27-hydroxylas (CYP27) gene
    • Oxidizes side chain of cholesterol for the formation of bile acids
    • Deficiency results in bile acid synthesis blockage and build up of bile acid intermediates
    • Leads to ? production of cholestanol
    • Tx with bile acid replacement therapy, ursodiol (ursodeoxycholic acid)
    • Tx with Chenix (chenodeoxycholic acid) to reduce cholestanol levels

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