. Pseudotumorous Conditions. PORTNotes. In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created Feb 16, 2009 14:42. Last modified Nov 14, 2016 12:05 ver.28. Retrieved 2017-10-21, from http://www.orthopaedicsone.com/x/eQAjAQ.
- ± 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
- 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
- (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
- 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
- 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
- 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
- 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)
- Abdominal wall involvement (rectus abdominus) has been described, mimcking a soft tissue sarcoma
- Has been reported in the vastus lateralis
- Fluctuates with monthly cycles
- 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
- Nodular-cystic fat necrosis described related to trauma and subsequent rapid vascular insufficiency
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
Glial heterotopia (heterotopic neural rest)
- Brain tissue occurring outside the cranial vault has been reported on the chest wall, paraspinal region
- (From gossypion [Latin] = cotton), cotton balloma
- Retained surgical sponge with development of an aseptic granuloma
- Reported after up to 50 yrs!
Gout, tophaceous (tophi)
- Fanconi's anemia
- 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
- Extrusion of hematopoietic tissue through thinned trabeculae
- Spread through intercostal veins
- Spread from adjoining ribs
- Active stage
- Isointense in both T1 and T2
- Inactive stage
- Hypointense or hyperintense in both T1 and T2
- Active stage
- Hypervascular so think of bleeding complications
- Blood transfusion
- Target Hgb of at least 10 mg/dl
- 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
- Promotes fetal Hgb production
- Used when there is no urgency in management
- Blood transfusion
- Extramedullary hematopoiesis in Thalassemia Intermedia
- Sickle cell disease
- Has been reported to spontaneously regress
- ± 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
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
- 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
- Menkes syndrome
- Engelmann-Camurati disease
- Congenital syphilis
- Battered child syndrome
- Hypervitaminosis A
- 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
- Occurs in morbidly obese adults (mean pt weight 186 kg)
- Most occur in thigh but also in lower leg
- Misdiagnosis as liposarcoma or angiosarcoma
- 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
- (Focal myositis, focal interstitial polymyositis, interstitial nodular myositis, focal nodular myositis)
- 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
- 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)
- Particle-induced bone resorption
- Wear debris-challenged osteoblasts produce PGE2, IL-6: activators of osteoclasts
- They also produce IL-9 and MCP-1: chemotactic factors for leukocytes
- ± familial hx
Postsurgical ossified extradural cyst
- 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
- 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
- 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
- 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
- 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
- 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
- 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)
- 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
- 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
- Most often adjacent to silicone or metal prosthesis
- May mimic tenosynovial giant cell tumor (previously known as pigmented villonodular synovitis)
- 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
- Traumatic injury to periosteum, usually in the border digits resulting in an exostosis
- Familial hypercholesterolemia
- 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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