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(BQ) Part 2 book Surgical pathology of the head and neck - Vol 2 has contents: Diseases of the bones and joints, hematopoietic and lymphoid disorders, the pathology of neck dissections, the occult primary and metastatic tumors to and from the head and neck.
17 Diseases of the Bones and joints LeonBarnes U/JiVel.Sity of fittSbt/rg/J School o f Medicine, and University oi Pittsburgh School o f Dental Medicine, Pittsburgh, Pennsylvania Robert S Verbin* and Billy N Appel* Unrversity of PittsOurgh School o i Dent,?/ Medic-ine, Pittshurgh, Pennsylva/~ia 11 Diseases of theJoints x o r Barws A Rheumatoid Arthrltis B Gout C CalciumPyrophosphateDihydrate Crystal Deposition Disease (Chondrocalcinosis, Pseudogout) D Synovlal Chondromatosis E.Piglnented Villonodular Synovitis F Ganglia-Synovial Cysts of the TemporomandibularJoint Nonneoplastic Diseases of Bone and Joints trrd Ro1wr-t S W h i t l , I 0.50 i050 I OS4 I OS7 I OS8 1061 1064 Lro,l Brrrrws Hi/!\ N AppcI 1066 1066 1077 1078 1079 1081 1084 1090 1005 1103 Osteomyelitls of the Jaws Rolx)r/ S K,r-hirr Osteoradionecrosis LCWIBt~r-ne.s Relapsing Polychondritis Lcorr Borne.s D Focal Osteoporotlc Bone Marrow Defect Ro1wr-r S V e r l h E CortlcalDefccts of the Mandible Robrr-t S V w h i r ~ F Paget's Disease o f Bone Roherl L feel G Fibrous Dysplas~a Lrorl Htrrl~es H Ccmento-Osseous Dysplasias Rohwr S k r - l i n Cherubism Rilly N A l p A B C Ill NeoplasticandNeoplastic-like Diseases of Bone A Solitary Hcmnngioma o f Bone Leorr Barws B Solitary Lyrnphanglorna of Bone Leorr Htrrrres C Angiomatosis of Bone Leon Bnr-ms D Tori and Multiple Exostoses Robert S krl>irr E Exostosis: Osteoma Lro,l Btrnws F Osteochondrolna Leor~Htrr-rres "Retired 1049 Leon Btrnzes ntld Rohc~r-t.S Wrhirz 1109 I I09 1112 1112 1114 1116 1121 1050 Barnes et al G H I J K L M N P Q R S T U V W Osteoid Osteoma Leorr Uorrre.s Osteoblastoma L c w H ~ trnles Ossifymg Fibroma Rober-?S Uvhiu Chondroid(Cartilaginous) Metaplasia of the Larynx Leotl Hrrme.s Chondroma and Chondrosarcoma of the Larynx I x o t l Ranzc s Chondroma and Chondrosarcoma of the Jaws and Craniofacial Bones Chondrosarcoma of the Skull Base Leon Rtrr-~re.~ Chondromyxotd Fibroma L c ~ r rRrrr-ties Chondroblastoma Leorr Brrr-rws Chordoma Lwrl Wrrrrw.s Giant Cell Tumor Leon Rcrr-~(,s Giant Cell Granuloma Roher-I S Uvhirr Aneurysmal Bone Cyst Leorl Btrrnc.,s Desmoplastic Fibroma Leon Rarnc~.s Fibrosarcornn L w r ~Bnrrres Osteosarcoma o f the Jaws, Skull and Larynx L c w Narrrc s Juxtacortical (Surface) Osteosarcoma Leon B~rr-rres Extraosseous Osteosarcoma Leon Uanrc~,s Angmxcoma of Bone LNNI B ~ I ~ I I P S Ewing’s Sarcoma Lcofr /hrr-~rc.s X Y Z References I DISEASES OF THE JOINTS I.A Rheumatoid Arthritis Introduction Rheumatoid arthritis (RA) is a chronic systemic disease characterized primarily by nonsuppurative symmetrical inflammation o f peripheral synovial joints Extra-articular manifestations however are frequent and include various hematological, neurological, cardiovascular, pulmonary, and cutaneous abnormalities Although the etiology is unknown, the disease is clearly mediated immunologically (1,2) It occurs in all age groups and affects about % of the adult population It is more common in women and has an average age of onset between 35 and 40 years (3) ClinicalFeatures There are many common anduncommon manifestations ofRA in the head and neck (4) Among these are the following: I k ~ r w r Involvement of the larynx byRAisnot unexpected because i t contains two paired diarthrodial, synovial-lined joints: cricoarytenoid and cricothyroid (5-16; Fig I and 2) Of the two, rheumatoid cricoarytenoid arthritis is the more common and also themost disabling because this joint is intimately involved in abducting and adducting the true vocal cords The frequency with which the larynx is affected in RA depends on the thoroughness of the examiner, the duration Leorr Hrrr7w.s 112s I l27 I130 1136 11.77 1142 I145 I l46 I14X I 151 I156 I IS9 I l64 l69 l171 117.3 1178 11x1 11x2 I185 1186 and severity of disease, and whether clinical or autopsy data are used Results from various studies suggest that 26-78% of patients will manifest laryngeal symptoms sometime during the course of their illness; 33-57% will show physical evidence of disease onindirectand 7.5% on direct laryngoscopy; 54-72% will exhibit abnormalities on computed tomography (CT): and 45-100%will have histological findings at autopsy (9-1 2,17) In general, the more severe the disease and the longer the duration, the more likely the patient will have laryngeal signs and symptoms In one series of 64 randomly selected patients withRAof years average duration (range months to 35 years) 17 (27%) hadlaryngeal symptonls (9) In another study of 45 patients with RA of 13.9 years average duration (range 0.5-35 years) seen in a university rheumatology clinic, 35 (78%) had one or more laryngeal complaints ( 12) Cricoarytenoid arthritis is usually bilateral and tends to be not only more common but also more severe in females (7) Symptoms in descending order of frequency include a sensation of a foreign body in the throat, hoarseness, fullness or tension in the throat, dyspnea referred otalgia odynophagia, stridor, dysphagia, and painful speech (7) Depending on whethcr the disease is in the acute or chronic phase, physical tindings at laryngoscopy may be entirely negative or may show a swollen, red mucosa; decrease range of motion, fixation, or ankylosis of the joints; joint deformities: or vocal cord immobility ( ) The correlation yroid 1051 Diseases of the Bones and Joints Cartilages of the Larynx Hyoid bone Epiglottic cartilage Arytenoid cartilage joint Cricoarytenoid Cricothyroid joint Cricoid cartilage between the extent of involvement of the cricoarytenoid joint and symptomatology isnot reliable, for some patients have shown severe joint disease and had no symptoms, whereas others have had no physical findings, but prominent symptoms (6,9) In general, patients who are symptomatic are more likely to have physical findings One of the most-feared complications of laryngeal RA is ankylosis of thecricoarytenoidjoints in astate of Figure Diagramshowing normalcartilagesand joints of the larynx adduction.Thisresults in significantnarrowing of the glottis, and any superimposed upper respiratory infection, no matter how trivial, may result in acute airway obstruction and the need for emergency tracheotomy TemporomandibularJoint Of the patients with RA, 50-75% will have involvement of thetemporomandibularjoint (TMJ) (18-21) When Figure Cricoid (C); thyrold (T) joint: Note the joint space (JS) and peripheral synovial lining (arrows) Barnes et al 1052 present, the joint involvement isusually bilateral, but rarely occurs early i n the course of the disease Signs and symptoms, which are rarely handicapping or as severe as in other joints, include morning stiffness, pain on movement of the jaws, difficulty in opening the mouth, referred otalgia, crepitus, and swelling of the jaw In more severe diseases, one may see mandibular micronathia, with malocclusion secondary to destruction of the condylar head, subluxation, and ankylosis Juvenile rheumatoid arthritis on the other hand, may be more serious and interfere with growth and development of the jaws In addition to the keratocol1.junctivitis sicca (dryeye) component of Sjogren’s syndrome, RAmaymanifest in the eye a s iritis iridocyclitis, and scleritis In mostinstances these are late manifestations of the disease Ecw Hearing impairment in patients withRAis a subject of controversy, and itsnature and extent hasnotbeen fully delineated (22-28) Since the incudomalleal and incudostapedial articulations are synovial joints with cartilaginous articular disks one would assume thattheywould be subjected to the same ravages of RA as seen in small joints elsewhere i n the body (37) Surprisingly, there have beenvery few histological studies ofthe temporal bone in patients with RA In those five instances in which the ossicles have been evaluated, the histological findings have been normal or equivocal at m o s t ( 3 ) Sensorineural hearing loss is one of the m o s t common auditory abnormalities i n RA I t occurs i n 2648% of patients andis presumably due to neuritis or vasculitis or both, of the auditory nerve or cochlea (27-30) There is no relation between hearing loss and duration or activity of RA; but, according t o Goodwill et a l patients with rheumatoid nodules are more likcly to experience sensorineural deafness (23) Although patients with RA frequently manifest an increased “stiffness” of the ossicles on specialized testing, clinically significant conductive hearing loss isunusual (28) Some drugs used in the treatment of RA, especially salicylates, are potentially ototoxic and may also contribute to hearing abnormalities S Nose In rare instances, patients with RA may experience spontaneous perforation ofthenasal septum (31,32) Although presumably caused by vasculitis or local vasospasm, biop- sies from the edges of these ulcers havenot evidence of vascular damage shown S u l i ~ ~ r rGlands: y Sjogrer? ‘ S Syclrotw Fifteen to 2.5% of patients with RA may manifest Sjogren’s syndrome, an idiopathic immunologically mediated, inflammatory-destructive disorder of thelacrimal and salivary glands, leading to dry eyes and mouth This syndrome is discussed i n greater detail i n Chapter 13 Lymph No&.s Lymphadenopathy, either regional or generalized, is not uncommon in patients with RA and is thought to be related to the immune response associated withthe disease In some instances the lymph nodes are actively involved in the production of rheumatoid factor Pathologists should be aware that patients with RA who are treated with a combination of methotrexate and prednisone may develop an Epstein-Barr virus lymphoid proliferation that may easily he mistaken for malignant lymphoma (33-35) Ifrecognized, reduction or cessation of immunotherapy will result in regression of the adenopathy X Rhcwrtrtrtoid N o c l ~ r l t ~ s Rheumatoid nodules develop in 25% of patients with RA and are associated with active disease high titersof rheumatoid factor, vasculitis, and a poor prognosis (3,36) They are frequently located over extensor surfaces, bony prominences, and other pressure areas, and occur within the dermis subcutaneous tissue, or periosteum They may also involve the synoviurn bone dura eye, and various internal organs, including the heart and lungs (37) Involvement of the larynx is distinctly unusual, but when it occurs thetrue vocal cords appear to bethemost frequent site ( ; Fig 3) They have also been described in the false vocal cords epiglottis and the mucosa and muscle adjacent to the cricoarytenoid joint (9,40) The nodules are soft and pale gray toyellow-white Rarely they may ulcerate Vtr.scx1iti.s Ten to fifteen percent of patients with RA will develop a vasculitis, either digital or systemic When systemic, it maybe impossible to separate from polyarteritis nodosa on histological examination alone Vasculitis is usually associated with rheumatoid nodules and high titersof rheumatoid factor I t is thought t o be the result of immune complexes and usually manifests as cutaneous-mucosal ulcers or a neuropathy (3,10,42.43) 1053 Diseases of the Bones and Joints Figure This 66-year-oldcoalminer, with a 12-year hlstory of rheumatold arthritis, died of respiratory failure secondarytoanthracosilicosis and pulmonary fibrosis An autopsyrevealedbilateral rheumatoid involvement of the cricoarytenoid joints and rheumatoid nodules In thesofttissue of theglottisandsubglottis The glottic nodules are illustrated Thyroid cartilage containingmarrow is present in the lower left hand comer (heX50) matoxylin and eosin [H&E] (Courtesy of G Perkins,Universityof Westem Ontario, London, Ontano and R Kessler, Montefiore Hospital, Pittsburgh, PA.) 10 Neuropathy 12 Amyloid Amyloid may develop in up to 20% of patients with RA The complication of neuropathy, whichmay be sensory or This subject is discussed in greater detail in Chapter 30 motor, occurs in about 10% of patients with RA In some instances, this is related to a small vessel angiitis involving Pathology The pathology is identicalwiththatobserved in peripheral joints Early in the course of the disthe nutrient blood vessels (vaso nervorum) of nerves, reease, the synoviumis congested, edematous, hyperplastic, sulting in demyelination or even frank necrosis A compressive (entrapment) neuropathy accounts for most of the and extensively infiltrated with plasma cells and lymphocytes As the abnormal synovium (pannus) enlarges, it exremaining cases, suchas in the carpel tunnel syndrome tends over the articular surfaceand destroys the cartilage, Involvement of the cervical spine is recognizedto be a common manifestation of RA and typically appears latein predisposing the joint to the ravages of secondary degenerative joint disease As the disease progresses, the pannus the course of the disease (44,45) Although about 25% of may become fibroticor even ossify, resultingin a “frozen” patients will exhibit radiographic abnormalities of the cerjoint vical vertebrae (usually varying degrees of subluxation), only a fewwill ever develop neurological symptoms (44) Rheumatoid nodules are composedof a central zoneof fibrinoidnecrosis and aperipheralborderconsisting 11 MyopathyandMyositis of palisading histiocytes, mononuclear inflammatory cells, and fibroblasts (Fig.3 and 4) Occasionally, multinucleated Muscle atrophy in RA may be due to disuse or ischemic giant cells may be observed and, in this instance, thenodneuritis-vasculitis (as noted earlier) The latter often reules may be confusedwith caseating granulomas sults in focal or diffusedemyelination with subsequent Treatment In general, medical treatment of the sysneurogenic atrophy temicdiseasewillalleviate or ameliorate many of the One may also see nodular collections of chronic inhead and neckmanifestations.Some of theproblems, flammatorycells,principallylymphocytes,lyingwithin however, may havetobeapproachedsurgically Some the muscle, which may also contribute to muscle weakpatients with severe cricoarytenoid arthritis may experiness In contrast with dermatomyositis, these inflammaence ankylosis of the joint(s) with fixation of the vocal toryfociare not associated with regenerativemuscle cords in a state of adduction and significant airway comchanges or necrosis promise In these instances, arytenoidectomy with vocal Barnes et al 1054 Figure Rheumatoidnodule composed of acentral zone of fibrinoid necrosis of histiocytes, andapenpheralborder fibroblasts and inflammatory cells (H&E, X 140) cord lateralization will ensure a safe airway and preserve by hyperuricemia and recurrent violent attacks of arthritis patient’s voicethe (13) intra-articular secondary to accumulation of monosodium urate crystals.As the disease progresses, the joints become I.B Gout become deposits urate and deformed, progressively grosslyvisibleastophi(Figure 5) Thediseaseisnot Introduction Gout, which may be primary or secuncommon, for about 5% of patients in major arthritis ondary, is a disorder of purine metabolism characterized clinics are estimated to have gout (1) ~~ d Figure Goutytophusonthehelix of theear(arrow).(Courtesy RE Lee, Unwersity of PittsburghMedicalCenter, Pittsburgh, PA.) 1055 Diseases of the Hones and Joints In humans, wholackthe enzyme uricase, the major end product of purine metabolism is uric acid In a normal healthy individual, about 700 mg of uric acid are turned over each day About two-thirds o f this is excreted i n the urine, and the remainder is eliminated through the gastrointestinal tract, where it is further degraded by colonic bacteria ( I ) Therefore hyperuricemia results when there is either an overproduction of purines or faulty excretion, or a combination thereof Prirrwry g o u t Primary gout accounts for 90-9S% of all cases and is the term usedto describe those individuals whosc hyperuricemia is due to an “inborn genetically determined metabolic defect leading to a pathologic de novo biosynthesis and/or retentionofuric acid” (2) Ofthis group about 90% are men:when i t occurs in women, mostarepostmenopausal The peak age of onset is the fifth decade Twenty-five percent o f the relatives of patients with gout have asymptomatic hyperuricemia ( I ) The severity and rate of progression of gouty arthritis are extremely variable The following four phases of the disease are recognized l A.s!‘rrll,torlltrtic’ Hyperuricewritr Overall about 5%-3S% of individuals with sustained hyperuricemia will develop gout The incidence, though varies withthe duration and degree of elevation ofthe serum uric acid level For example, if an individual maintains a serum level o f C) mg% or nlore for 14 years, he or she has an 83%: chance of developing gout ( I ,2) AcrIIcJ C ‘ o r r l ~Arthritis The initialattack is typically monoarticular and involves a peripheral joint usually the great toe (podagra) i n SO7 S % of the cases but occasionally the ankle, wrist, heel, or kncc The onset is sudden, reaches a maximum intensity i n a few hours, andwill resolve spontaneously i n a few days to weeks if left untreated Such attacks maybe precipitated by prolonged fasting, excessive alcohol consumption surgery joint trauma or ingestion of drugs that intcrfcre withrenal excretion of uricacid (especially thiazides) lr1tvrcriticwl (lrl/c~r\wl) Golrl Intercriticalgoutis the asymptomatic period between acute attacks I t mayvaryfrom weeks to years but most individuals will suffer another acute attack within months t o years after theinitial attack As the disease progresses, the attacks become more frequent and scverc, and multiple joints may become involved Chronic Topkcrceous Gout Gross deposits of monosodium urate crystals are referred to as tophi (from the Greek meaning “chalk stone”) Before the advent of effective drug therapy, 50-60% of patients developed tophi; nowadays, the incidence is 1335% ( l ) Their development is related to the length of the disease, degree of elevation ofthe serum uric acid, and the severity of renal involvement ( I ) In general, patients have had gouty arthritis for an average of years before tophi begin t o appear ( ) Thcy arc rarely observed at the time of the first attack Themost frequent sites are the synovium, subchondral bone, olecranon bursa, Achilles tendon subcutaneous tissue of the forearm near the elbow and thc helix of the ear (see Fig S) They are nonpainful, firm, and salmon pink when viewed through the skin Occasionally, the skin will ulcerate and drain white chalky material Large tophi may interfere with joint function, and some will erode bone and cartilage and produce secondary degenerative joint disease Patients with gout also have a higher incidence oidiabetes mellitus, obesity, atherosclerosis, hypertension, and cardiovascular disease In 2040% of cases, uric acid stones will develop and occasionally result in pyelonephritis ( 1.3) Kidney disease is the single most important cause of death in gout (3) Seco,1cltrry go111 Secondary gout accounts for the remaining S-lO% of cases and represents a complication of some otheracquircd disease in which there is accelerated nucleic acid turnover or impaired excretion of uric acid Hcmatological malignancies hypertensive cardiovascular disease and chronic renal disease are some of the more common causes I n the secondary form there is no hereditary tendency scrum uric acid levels are higher, the age of onset may be younger, and there is a higher incidence in women The pathogenesis ofthe acute attack of gout is complex As the urate crystals begin to nccumulate within the joints, they are immediately phagocytosed by neutrophils which, i n turn liberate lysosomal enzymes thathave ;I destructive effect on articular cartilage In addition the urate crystals activate Hagemm factor which stimulates cytokine production, resulting i n increased vascular permeability and a greater concentration of neutrophils The ensuing anaerobic metabolism lowers the pH which further promotes uric acid production Thus a vicious cycle is cstablished Hetrd trtd N d Mtrr1i~sttrtiorl.s Although gout may affect the head and necki n any stage of the disease, it generally occurs only in patients with long- Barnes et al 1056 crystals and changes of secondary degenerative joint disstanding disease.Tophi and cricoarytenoid arthritis are the prime manifestations(4-10) In this region, tophi are most eases Differential Diagnosis The differential diagnosis inoften observed on thehelix of the ear or in the vicinity of cludes Teflon granuloma and pseudogout.Patientswho the true and false vocal cords; however, they been have dehave unilateral vocal cord paralysis are often treated by scribed in thesoftpalate,uvula,glossoepiglotticfold, tongue, hyoid bone, thyroid cartilage, epiglottis, subglottis,injecting the paralyzed cord with Teflon, which expands the cord and, thereby, improves speech and lessens the tracheal cartilage, first part of the esophagus, sclera, condifficulty with secretions Teflon particles vary from round junctiva, and hypopharynx (5,6,8,10; seeFig ) toovoid and tendtohaveaclearcenter and athick Involvement of thecricoarytenoidjointresultsin border They also elicit a foreign body giant cell reaction hoarseness, dysphagia, dysphonia, pain,and possible joint and fibrosis and will polarize, which may result in confufixation, with aspiration pneumonia.As might be expected, sion with gout (see Chapter ; Figs 18 and 19) Sodium hoarseness is the chief symptom when tophi involve the urate crystals, however, are thinand elongated, not round true vocal cords Aural tophi can be confused clinically or ovoid In addition, the clinical history, normal serum with chondrodermatitis nodularis The presence of pain, uricacidlevel, and negativeDeGalanthastainforuric however,favorsthe latter Goutyinvolvement of the acid readily allow one to distinguish a Teflon granuloma temporomandibular joint is very rare from gout Pathology Tophi arecomposed of aggregates of Pseudogout or chondrocalcinosis may also be confused elongatedneedle-shaped,cleartolightbrowncrystals, with gout In this disease, calcium pyrophosphate dihyoften appearing as “bundles of wheat.” The crystals, in drate crystals are deposited in soft tissues or joints (14) turn, are immediately surrounded by foreign body giant The pyrophosphate crystals appear as short, blunt rods, cells,histiocytes, and densefibrousconnectivetissue rhomboids, or cuboids that are weakly birefringent under (3,ll-13; Fig ) Thecrystalsshownegativebirefrinpolarized light (Fig 7) gence When a primary color compensator is positioned Treatment and Prognosis Treatment consists of betweentwopolarizinglenses,thecrystals will change drugs, such as colchicine, allopurinol, probenecid, corticofrom yellow to blue on rotation of the polarizing lenses steroids, and nonsteroidal,anti-inflammatoryagentsdefrom parallel to perpendicular signed to intermpt the afore noted cycle of events or to When gout involves a joint, it may produce a gross deformity, with prominent intra-articular deposits of urate lower the serum urate level(15) If the gout is secondary, Figure Gouty tophus composed of needle-shaped urate crystals have evoked a forelgn body giant cell reactlon (H&E, X 350) that nes Diseases of the and Joints 1057 Figure Crystals of calciumpyrophosphatedihydrate(pseudogout)appear in tissue as rhomboids, cubes, or rectangles Note the multinucleated giant cells (H&E, X400) Compare with Fig then control of the primary disease is mandatory Tophi may dissolve with medication,butlargeronesthatare disfiguring or that interfere with joint function may have tobesurgicallyexcised.Jointsseverelydeformed by secondarydegenerativejointdiseasefrequentlyrequire arthroplastic procedures Although the purine content of daily food consumption does not substantially contribute to the serum urate concentration; nevertheless, moderation in dietary purine consumption is indicated (15) With the advent of effective antihyperuricemictherapy, the natural history of the disease has been altered Nowadays, only a few patients develop tophi, disabling joint problems, or life-threatening renal disease I.C Calcium Pyrophosphate Dihydrate Crystal Deposition Disease (Chondrocalcinosis, Pseudogout) Introduction Zitnan and Stag and McCartyetal wereamongthe first torecognize and characterizethe condition now known as calcium pyrophosphate dihydrate (CPPD) crystaldepositiondisease(chondrocalcinosis, pseudogout, CPPD arthropathy: 1-4) In this disorder, for reasonsunknown,crystals of CPPDaccumulateinthe joints,especiallytheknee,wrist,metacarpophalangeal, hip and spine, and occasionally, the para-articular tissues of older persons The disease is not uncommon It hasbeenobserved in the knee joint, for instance, either at autopsy or by radiographic examination, in 2.2-34% of patients older than 60 years of age ( ) It is more common in men by a ratio of 1.51 and occurs in patients averaging 72 years of age (5) Although most cases of CPPD disease are idiopathic and sporadic, a few are hereditary and are transmitted as an autosomal dominant trait, whereas others are precipitated by trauma or surgicalprocedures.About10% of cases are associated with a variety of metabolic disorders, especially hyperparathyroidism, hemochromatosis, hypothyroidism, gout, amyloidosis, Wilson's disease, and ochronosis (5,6) CPPD disease ranges from totally innocuous to incapacitating, and is often mistaken clinically for a variety of musculoskeletal disorders Ryan and McCarty recognize several variants of the disease, which they designate as type A through type F (5) In type A (pseudogout), the diseasepresents as an acute, self-limited, monoarticular arthritis that involves the knee in over 50% of the cases and is often confused with gout Type B (pseudorheumatoid arthritis) is characterized by multiple joint involvement, with subacute attacks lasting from weeks to several months.Thisvariant may bemistaken for rheumatoid arthritis About 10% of patients with this type will also have positive tests for rheumatoid factor, but only in low titers q p e s C and D (pseudo-osteoarthritis) involve the largejointsbilaterallyand,accordingly,arefrequently misdiagnosed as osteoarthritis Patients who give a history of superimposed acute joint symptoms are labeled astype C, whereasthosewhodo not have an inflammatory component are designated as type D Type E (asymptomatic),asthenameimplies,refers to thoseindividuals whohaveonlyradiologicevidence of disease,butno Barnes et al 1058 symptoms This variant may be the most frequent of all T \ p e F (pseudoncuropathic joint) istherarest and most severe form It resembles Charcot joint, but yet with few exceptions, these individuals not have an underlying neurological abnormality Patients with CPPD disease have normal levels of serum calcium, phosphorus and uric acid Theonly exceptions are those instances whenthe disorder may coexist with hyperparathyroidisti1 or gout As i n gout, the crystals may incite an inflammatory reaction that results in a cascade o f events that ultimately lead to joint destruction H c w l a r d Nock Mtrrli~~frsttr1iorl.s Rarely, CPPD disease involves theheadand neck, and when it does, the temporolnandibular joint (TMJ) is the most likely site (7-14) The disease in this area is typically unilateral occurs in patients between SO and 60 years of age, and manifests as a prealtricular swelling or discrete tnass that is often mistaken clinically for a tumor of thc parotid gland or external auditory canal.Some patients have complained of tenderness or pain, often referred to thcear and a “clicking” sensation on opening the jaws Deviation of the mmdible, difficulty on opening the mouth and decrease i n hearing, although described are unusual (9- 12) Radiography I n the knee, two of’ the earliest and most characteristic features of CPPD disease arc the prcsence o f linear punctuate calcitication o f the menisci and calcitic deposits in the midzone of the hyaline articular cartilage I n the TMJ thetypical appearance isthat o f either a slightly or heavilycalcified intra-articular mass with or without erosion of thc subjacent cartilage and bone ( I O 13) The calcification tends to be fluffy or granular Pathology TheCPPD crystals appear in tissue sections as slightly basophilic rhomboids, cubes or rectangles and, only rarely, as needles (IS-17; Fig 7) In general needles if seenat a l l tendto occur in acute disease whereas the other forms arc seen i n both acute and chronic conditions The crystals may lic free in the tissue without evidence of an inflammatory response or they may evoke acute and chronic inllammation with eventual multinucleated giant cell reaction and fibrosis Tophi similar to those seen in goutmay also occur (tophaceous pseudogout) However, in contrast with gout, the tophi of CPPD disease occur only i n the vicinity o f the diseased joint and are never widely dispersed Differential Diagnosis CPPD disease must be distinguished from gout This canltsually be nccomplished by obtaining a serum uric acid level and on viewing the crystals by both light and polarized microscopy (see Figs and 7) More sophisticated tests such as X-ray diffraction and infrared spectroscopy, can also be used Table I liststhe features that are useful in separating the two conditions Treatment and Prognosis Patients with limited, asymptomatic disease generally require no treatment Others however.may require ( a ) joint aspiration to remove the crystals (h) colchicine or nonsteroidal anti-intlammatory agents, (c) local injections of steroids, or even (d) joint immobilization Those with more severe joint diseasc may even require arthroplastic surgery Interestingly, when CPPD disease is associated with other metabolic diseases (hyperparathyroidism,hypothyroidism and such), corrcction of the underlying metabolic disorder has not resulted i n the disappearance of crystallinc deposits (S) I.D SynovialChondromatosis Introduction Synovial chondrotnatosis (SC) is a benign condition characterized by the occurrence of metaplastic cnrtilaginous or osteocartilaginous nodules within the synovial membrane of joints and, occasionally, of bursae and tendon sheaths As the disease progresses, the nodules may become detached to form intra-articular loose bodies Two forms o f the disease are recognized: primary and secondary ( I ) Pritnary synovial chondromatosis (PSC) is uncotntnon In this form of the disease, the metaplastic nodules appear de novo with no evidence of an underlying predisposing joint disorder The etiology is unknown but ;I benign neoplasm or a response to chronic, repetitive low-grade trauma have been proposed ( I,?) Compohition Shape Sodium 11rate Calcium pyro- 1-21 Cumulative Index Occupational exposures toxlns, effect of 449 Ocular adnexa diseases of, 1877-202 I fine-needle aspiration, 67-69, 69 lymphoid lesion of, 1341- I 342 Odontoallleloblastic odontogenic epithelium 1583-1 585, 1585, 1586 odontogenic carcinomas 1606-1 61X clear cell odontogenic carcinoma 161 1-1614 1612, 161.3, [Neuroendocrine tumor] clinical features, 197 differentla1 diagnosis 199-200 pathology 197, 198, 199 prognosis, 197-199 treatment, 197-199 Neurolibroma diffuse peripheral nerve sheath, 803 803-804 electron microscopy, 102 105 larynx 152-1 53 peripheral nerve sheath, 798-799, 799 Neurofibromatosis I , peripheral nerve sheath, 803-806 Neurofibromatosis peripheral nerve sheath 806-808 Neurofibrosarcoma 836-841, 838-839 Neuroglial tissue ectoplc 568-570 569, 570 Neuroma acoustic, peripheral nerve sheath, 808-809 mucosal peripheral nerve sheath, 809-8 I0 Neuropathy, rheumatoid arthritis, 1053 Neurothekeoma peripheral nerve sheath, 10-8 12 XI I Nevoid basal cell carcinoma syndrome, X 15-1 X 16 Nevus conjunctival, 1885-1 887 l886 iris 1937-1942 1938 1939t 1940 1941 IY42 larynx, 203 sebaceous 1839-1 840, 1840 spindlecell, 1827-1829, 1828, 18281 Nicotlna stomatitis, 246-248 248 249 Nodal metastases histopathology of, tissuc diagnosis 1410 1410-141 I , 1614 epithelial odontogenic ghost cell tumor, malignant 16 141615, 1615 1616, 1617 malignant ameloblastoma 1607- I6 10 ameloblasticcarcinoma, 16081, 1608-1610, 1609 peripheral ameloblastomas malignant behavior I610 odontogenic cyst malignant changes in, 1615-1618 1617, 1618 1619 primary intraosseous carcinoma, I6 I O - I6 1 Odontogeniccyst, 1439 1439-1472, 1440 1441, 1442, 1443 I472 malignant changes in l6lS-I6l8, 1617, 1618, 1619 Odontogenic ectomesenchyme included odontogenic epithelium 1597-1 606 benign cemcntoblastoma 1603-1606 1604, 1605 1606 odontogenictibroma 1597-1600 central odontogenic fibroma 1597, 1597-1 599, 1598 peripheral odontogenic fibroma 1599-1600 1600, 1601 odontogenicmyxoma, 1600-1603, 1602, 1603 Odontogenic epithelium with odontogenic ectomesenchyme, dental hard tissue 141I 1579-1 topography of 1409-1410 Nodular fasciitis, 924-927 926 Nodular goiter thyroid, 1692, I692 Nodular melanoma, skm I X2 I I822 Non-Hodgkm's lymphoma 55-60, 57, 58, 59, 60 1263-1 I2 B-cell I266 T-cell leukemia 1285-1 12 Nonallergic rhinosinusitis, with eosinophilia 473 Noncpithelial tumor 100-1 10 electron microscopy, 0 - I O Noninflammntory nonneoplastic enlargement, salivary gland 644-646 645 646 Nonkeratinizedpapilloma, 143-147 Nonproliferativediabetic retinopathy 1965-1967, 1966, 1967 1968 Nose rheumatoid arthrltrs 1052 squamous cell carcmonu 1808-1 809 Nuchal fibroma, 945-946 Oat cell carcinoma, trachea 620 Obstructive sleep apnea 257 clinical, 257 pathology 257 597 adenomatoid odontogenic tumor 1585-1589 1.587 1588 ameloblastic fibro-odontoma, 158 1-1 583, 1583 1584 ameloblastic tibrodentinoma, I 58 1-1583 158.3, 1584 ameloblastic fibroma 1579-1 581 15x1 calcifying odontogenic cyst, 1589-1595 peripheral 1592- I 593 solid, 1593-1 595 complex, compound odontomas 1595-1 597 1596 odontoameloblastic 1583-1585, 1585, 1586 without odontogenic ectomesenchymc 1558-1559 ameloblastoma, 1558 calcifying epithelial odontogenic tumor, 1577-1579 1578 1579 clear cell odontogenic tumor I579 desrnoplastlcalneloblastoma 1573 1.574 peripheral ameloblastoma, 1574-1575 solid ameloblastoma, 1558-1559, 1.560 1561 1563 1564, 1565 1566, 156x1 squamous odontogenic tumor 1575-1 577 1576 unicystic ameloblastoma 1569-1573 1570 1571.1572 Odontogenic tibroma, 1597-1 600 central, 1597 1597-1599, 1598 peripheral 1599-1600, 1600 1601 Volume 1, pp 1-786; Volume 2, pp 787-1436; Volume 3, pp 1437-2209 1-22 Cumulative Odontogenic keratocyst 1452-1 462 central combined para orthokeratotlc keratocyst 1462 centralorthokcratotic 1461, 1461 centralparakeratotic 1452-1461 1456 1457 13571 1458 peripheral 1462 Odontogenlc sarcomas I6 18-1 624 ameloblastic tibro-odontosarcolna 162 1-1 623 1622, 162.3 arnelohlastic fihrodentrnosarcoma 1621-1623, 1622, 1623 ameloblastic tibrosarcoma I6 I 8-1 62 1, 1620 odontogenlc carcinosarcoma, 1623-1 624 Odontogenic tumor 1557-1648, 1558t Olfactory neuroblastoma, 841-845, 8421 843, 844t electron n~icroscopy.97 100, 101 Oncocytes electron microscopy 97 102 Oncocyticcyst, 137, 138 Oncocytic lesion salivary gland 200-201 Oncocytic papillary cystadenoma salivary gland 677-678 Oncocytoma conjunctiva 1881 I882 salivary gland 678-683, 681, 682 68.3 Oncogenes I I S 16- I 17 Iq I3 amplitication I CCNDI I16 MYC, 117 RAS, 116-1 l7 Open-angle glaucoma primary I9 10- I9 I I secondary, I9 1 1912 Ophthalmia sympathetic 1922-1925 1925 Optic ncrvc tumor 1991 - I997 glioma, 1992-1995, lYY4 malignant gliomas of optic nerve 1995 Oral cavity, 362-365 anatomy of 375-377 376, 376t .?77, 3771 cancer of 369-438 cyst, cyst-like Icsion of 1437-1555 hcmangloma 900 leiomyosarcon~as, 979 lichen planus 364-365 lipoma 17-9 18 lymphoma, plasma cell lesion 1337-1339 neoplastic nonneoplastic lesion, benign 239-300 premolignant lesion 344-365 crythroplakia 360-362 361, 362, 3621 leukoplakia 344-360 lichen pl;~nus.364-365 sidcropcnic dysphagia 364 submucous fibrosis, 362-363 364 syphilis, 365 radiation injury 172-2 180 rhabdomyosarcolna, 962 sidcropenic dysphagta 364 syphilis, 365 Index Oral mucosa ulcerative lesion noninfcctious 301-332 vesiculoerostve lesion, noninfectious, 301-342 Oral nevi, 281-285 clinical features, 281 281 t, 2S2, 282t differential diagnosis, 2831 284 pathology 28 1-284 283 284 prognosis 284-285 treatment 284-285 Oral papilloma1osis florid 274 274 Oral pigmented lesion classification of, 283 Oral regions, metastatic tumor to, 1430-143 I Oral soft tissues metastases to 143I Orbit cyc, metastatic tumor to 143 lymphoid infiltrates, ocular adnexa eye 1979-1985 rhabdomyosarcoma 960-96 I 961, 9621 Orbltal space-occupying lesion 1985-1991 In adults, 1987-1991 cavernoushemangioma, 1990 l Y Y tibrous histiocytoma, 1990 Graves' disease 1987-1 989 hctnangiopertcytotna 1990 intlammatory pseudotumor, 1989, 1989-1 990 lacrimal gland tumor 1990-1991, 1991, IYY2 malignant lymphoma,I989 secondary neoplasm I99 I , IYY2 pediatnc 1985-1 987 capillary hemangioma, I985 dermoidcyst 1985-1986 IYX7 lymphangioma,I987 rhabdomyosarcoma 1986 19X6 19%' Organ of Chievltz 788 788 Oropharyngeal wall squamous cell carcinoma, 405-406, 406 Oropharynx anatomy of 375-377 376, 376t 377, 3771 cancer of clinical featurcs 240 240 neoplastic nonncoplastic Icsion, benign, 239-300 pathology, 240 240 prognosis 240-241 241 242 rhabdomyosarcoma, 962 treatment 240-241 241, 242 Orthokeratotic odontogenic kcratocyst central 1461, 1461 Ossifying tibroma, I 130-1 136 central 1130-1135 11.31, 11.32s 1133 11.34 peripheral l 135-1 136, 1136, 1137 Ossifying fibromyxoid tumor 954-957, 055, 955t, Y56 Osteoblastoma, I 127-1 130, I I28t 1/20, / , I 163-1 164, I164 aggressive, 129-1 I30 malignant I30 Osteochondroma I 1- 1 24 solitary, 1121-1 124 1122 1123 1/24 Volume 1, pp 1-786; Volume 2, pp 787-1436; Volume 3, pp 1437-2209 Cumulative Index 1-23 Osteochondromas, multiple I 124 Osteoid osteoma I25t I 125-1 127, I I26t Osteolipoma, 923 Osteoma, I 16- 12 I extraskeletal 952-953 Osteomyelitis jaw 1066-1077 sclerosing chronic 1070-107 I 107/ 1072 diffuse 1071-1074 10741 suppurative IIcUte 1066-1069 lU6X chronic, 1069-1070 Osteoporotlc bone marrow defect focal 1079-108 I , /OXO Osteoradionecrosis 1077-1078 Osteosarcoma extraosscous, I 18 1-1 I 82 high-grade, surface I I8 juxtacortlcnl I 178-1 I X I , I I791 larynx, 1173-1178, 11741 117.7 /176, 1/77 11781 parosteal 1179-1 180, / dedifferentiated, 180-1 I8 I periosteal, I I8 skull, 1173-1178 11741, 1/75 1/76, 1/77 11781 Otitis externa 2025-2027 bacterial 2025-2027 tilngal 2025-2027 malignant, 558-559, 2027 Otitis media, 559-561, 560 561 2027 acute 2027 chronic 2027 serous, 2027 Otosclerosis 572 572-573 Papillary endothelial hyperplasia intravascular 893 893-894 8Y4 Paget’s disease o f bone 1084-1090, IOX6 IOX7 IUXX /OXY, IOYO Palatal cyst median 1476 1476-1 477, 1477 Palatine papilla cyst, 1476 1476 Palatlnc tonsils lymphoid papilhry hyperplasla I260 Palpation thyroiditis 1683 Papillary adenocarcinoma low-gradc, S82-585 nasopharynx 532-534, 533 534 Papillary adenoma endolymphatic sac tumor Heffncr’s tumor 582-585 Papillary carcinoma columnar cell variant 1689-1690 diffuse sclerosis variant 1690 1690, / / encapsulated, 169 follicular 1688-1689 microcarclnonu, 1686-1687 16x7, 16XX solid varlant 1690- I69 I tall cell variant 1689 thyroid gland, 47 4X 1683-1692 typical 16X.I 1684-1 686 16X.5 variants of 1686-1692 Papillary hyperplasia inflalnmatory, 27 1-274 273 lymphoid palatine tonsils I260 thyroid gland 1683 Papillary keratosis 147 149 Papillary middle ear tumor, aggressive papillary adcnorna, 582-585 Papillary squamous carcinoma, 182-184 clinical features, 182-1 83 differential diagnosis 183-1 84 etiology I83 pathology, 183, 183, 1x4 prognosls, I83 treatment, 183 Papilloma, 143-147 269-27 I clinical features, 269 conjunctiva 1880-1881 /&S/ differential diagnosis, 270-27 etiology 269 fungiform, 4x7, 487t 487489 1x8, 4881 inverted 489497, 490, 4911 492, 49.3 4Y4 carcinoma and 491497 493 496 invertlng, 135 / keratinized, 147, 148 /4Y keratinlzed papilloma, 147 14X 14Y pathology 269-270 prognosis 270 respiratory papillomatosis, recurrent 143- 147 clinrcal features 143-144 ep~dcmiology,I43 pathology 144, 144-146 14.7 prognosis, 146- I47 terminology 143 treatment, 146-1 47 Schneidcrian, oncocytic, 497498, 4YX 498t 499 trachea 615-616 616 617 treatment, 270 Papillomatosis florid, oral 274, 274 Papillomavirus, human transmission of 124-2 128 21282/29 Papule, fibrous nng~ofibromn 1840-184 I , IX1l /