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1331
Musculoskeletal Margaret
Case
A. StuIl1
1: Parosteal
and
Case Mark
of the Day
Glass-Royal
Osteosarcoma
of the Humerus
About 6 months before presentation, this 62-year-old man noticed diffuse swelling and progressive loss of motion of the left shoulder and upper arm. About 4 months before presentation, he identified a nontender lump in the left shoulder. Physical examination revealed a large firm mass that extended into the root of the neck, medially to the chest wall, and inferiorly to the supracondylar region of the left elbow. Circulation, sensation, and function in the left hand were normal. Twenty years earlier, he had undergone biopsy for a left shoulder mass, which was diagnosed as an osteochondroma. The patient’s history did not reveal whether the mass had been completely surgically excised. Radiologic examination of the left humerus and shoulder (Fig. 1A) revealed a massive, amorphous, irregularly calcified, and ossified mass replacing and infiltrating the shoulder and arm as well as encircling the humerus. An anterior mTc methylene diphosphonate (MDP) scintigram (Fig. 1 B) showed markedly increased tracer accumulation in the mass in the left upper extremity. Intramedullary invasion, cortical destruction, and infiltration of adjacent structures by the juxtacortical humeral mass were shown by CT (Fig. 1 C). The extent of the tumor was delineated by MA examination. The Ti -weighted coronal MR images (Fig. i D) showed intermediate-signal tumor replacing the marrow of the left proximal humerus, enveloping the humerus, and infiltrating adjacent structures. The superior, medial, and inferior extent of the tumor was readily identified as bright signal infiltrating adjacent tissues on the T2-weighted MR coronal images (Fig. i E). Histologic examination revealed a parosteal osteosarcoma. Parosteal osteosarcoma is a rare, slow-growing, malignant, bone-forming neoplasm that arises from the surface of long bones. It has a better prognosis than conventional central osteosarcomas and other surface (juxtacortical) osteosarcomas [i -3]. Patients are generally affected in the second to fifth decades of life. They usually have gradual onset of pain, swelling, and a palpable mass [1 , 4]. The indolent behavior of the tumor delays diagnosis. As illustrated in this case, the tumor may attain enormous size before the patient seeks
1
Both authors:
Department
to M. A. Stull, coordinator AJR 154:1331-1336,
June
of Radiology,
Georgetown
University
evaluation [i , 4]. In addition, radiologic and histologic misdiagnosis as benign osteochondroma [2] may result in inadequate surgical excision of the tumor-a distinct possibility in this patient. The lesion most often arises from the metaphysis of a tubular bone, occasionally from the epiphysis, and rarely from the diaphysis [1 , 4]. The most common location is the posterior aspect of the distal femur, followed by the proximal humerus, the tibia, fibula, and ulna [i , 4]. This malignant neoplasm grows circumferentially along the cortex of the involved bone, eventually enveloping the bone shaft. Cortical destruction and neoplastic invasion of the medullary cavity are uncommon except in cases of prior treatment or a long history of growth, as illustrated in this case [3-5]. On radiographs, early parosteal osteosarcomas are radiodense, lobulated, or oval masses with a broad base of attachment to the external cortex. A thin, radiolucent cleavage plane between the tumor and underlying bone is characteristic but not a constant finding [i , 3-5]. Progressive growth of the tumor may obliterate this plane. This tumor ossifies from the base of the lesion to its periphery in contradistinction to myositis ossificans, which initially ossifies from the periphery [1 , 2, 4]. The differential diagnosis of a less advanced case of parosteal osteosarcoma includes osteochondroma, myositis ossificans, central osteosarcoma, extraosseous osteosarcoma, ossifying or calcifying hematoma, and exuberant callus [4, 5]. CT and MR studies assist in delineating the extent of the process and in preoperative planning [4, 5]. Margaret A. Stull Mark Glass-Royal clinical
Medical
Center,
REFERENCES 1 . Resnick
D, Kyriakos M, Greenway GD. Tumors and tumor-like lesions of bone: imaging and pathology of specific lesions. In: Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders, 2nd ed. Philadelphia:
Saunders, 1988:3648-3677 2. Hudson TM, Springfield DS, Benjamin M, tomography of parosteal osteosarcoma. 3. Schajowicz F, McGuire MH, Araujo ES, comas arising on the surfaces of long 1988;70:555-564
3800 Reservoir
of the Case of the Day series. 1990 0361-803X/90/1546-1331
C American Roentgen
Ray Society
Rd., N. W., Washington,
Bertoni F, Present DA. Computed AJR 1985;144:961-965
Muscolo DL. Gitelis S. Osteosarbones.
J Bone
Joint
Surg
[Am)
DC 20007. Address reprint requests
STULL
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i 332
AND
GLASS-ROYAL
AJR:154, June 1990
Fig. 1.-Case 1: Parosteal osteosarcoma of humerus. A, Radiograph of left humerus shows massive amorphous and irregular calcification surrounding and replacing shoulder structures and majority of humerus. Lobulated soft-tissue mass (white arrows) Is best seen along periphery of lesion. B, Anterior ““Tc-methylene diphosphonate scintigram shows inhomogeneous tracer uptake in a large, irregular, lobulated mass involving left shoulder structures and humerus. C, CT scan at level of left glenohumeral joint reveals extensive medullary invasion and cortical destruction by calcified juxtacortical humeral mass that Infiltrates adjacent structures also. D and E, TI-weighted, 600/25, (D) and T2-weighted, 2000/80, proximal left humerus (large straight arrow) and tumor extending also extends Into vicinity of left brachial plexus (curved arrow).
4. Smith J, Ahuja SC, Huvos AG, Bullough osteogenic sarcoma: a roentgenological Radiol 1978;29: 167-175
5. LindeIl MM, Shirkhoda
2: Osseous
PG. Parosteal
(juxtacortical)
of 30 patients.
J Can Assoc
A, Raymond AK, Murray JA, Harle TS. Parosteal
osteosarcoma: radiologic-pathologic 1987;148:323-328
Case
study
(E) coronal MR images of thorax and upper extremities show intramedullary invasion of medially, superiorly, and laterally into adjacent structures (small straight arrows). Tumor
Sarcoidosis
correlation
with emphasis
and Chronic
on CT. AiR
Polyarthntis
This 26-year-old black man, with a 5-year history of quiescent intrathoracic sarcoidosis, presented with intermittent swelling and tenderness of his fingers for the past 2 years. The patient’s symptoms partially improved by adjusting his steroid therapy. Physical examination revealed bilateral de-
formed, tender, and swollen fingers, hypertrophied nails, and skin lesions. In addition, multiple proximal and distal interphalangeal joints of both hands were tender and swollen. Other clinical findings related to active pulmonary sarcoidosis and left facial nerve involvement (i.e., Bell palsy) also had developed within the last 2 years and partially responded to steroid therapy. Radiographs of the hands (Figs. 2A and 2B) showed bilateral soft-tissue swelling, periarticular osteoporosis, deformity, and destructive osseous lesions of the digits. Localized “punched-out” Iytic lesions, trabecular alterations resulting in a latticework pattern, pathologic fractures, and fragmentation were identified in the phalanges. Periostitis was absent. The metacarpals and wrists were spared, except for
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AJR:154, June 1990
MUSCULOSKELETAL
CASE
OF THE
DAY
1333
Fig. 2.-Case 2: Osseous sarcoldosls and chronic polyarthritis. A and B, Radlographs of left (A) and right (B) hands show soft-tissue swelling, perlarticular osteoporosis, deformity, and destructive lesions of digits. “Punched-out” lesions (small arrows) are seen in multiple phalanges. Cortical tunneling and trabecular afterations produce a latticework appearance In affected phalanges (large arrows). Middle and distal phalanges of left middle finger, the distal phalanx of left ring and right middle fingers, and the base of distal phalanx of right thumb have pathologic fractures. Absence of perlosteal reaction Is strIking. An old healed lntraarticular fracture of head of right fifth proximal phalanx is noted. There is limited involvement of several distal metacarpals, bilaterally. wrists are spared. Joint spaces are relatively spared. C, Chest radiograph shows bilateral hilar and mediastinal adenopathy. Bilateral reticulonodular infiltrates are more prominent on right side.
minor alterations in several distal metacarpals. The joint spaces were relatively spared. All of these radiologic findings are characteristic of skeletal alterations in sarcoidosis. A concurrent chest radiograph (Fig. 2C) revealed mediastinal and hilar adenopathy and bilateral interstitial infiltrates consistent with stage 2 intrathoracic sarcoidosis. The cornbination of pulmonary and osseous abnormalities are characteristic of sarcoidosis. Sarcoidosis is a generalized granulomatous disorder of unknown cause characterized by noncaseating granulomas. The frequency of radiologically apparent osseous involvement in sarcoidosis ranges from i % to 1 3%, with an average of 5% [1 -4]. Any bone can be affected; however, the small bones of the hands and feet are most often involved, with the hands as the predominant site. Osseous alterations are usually asymmetric and involve the distal and middle phalanges more often than the proximal phalanges and metacarpals. The bone lesions are usually asymptomatic, but can cause severe symptoms, as in this patient [i , 2]. Soft-tissue swelling, tenderness, stiffness, and deformities can accompany osseous disease. Skeletal involvement is generally associated with cutaneous lesions, which were present in this patient. Radiologic evidence of pulmonary involvement is found in 80% to 90% of patients with osseous sarcoidosis [i , 2]. Skeletal lesions in sarcoidosis have several radiologic manifestations. Cortical and trabecular alterations resulting in a reticular pattern or latticework appearance are the most characteristic radiologic findings of osseous sarcoid [2, 5].
Punched-out lytic lesions of bone destruction also are commonly described. Calcification, nodular opacities, and residual trabeculae may be present in these lytic lesions. Occasionally, rapid bone destruction results in a permeative pattern of osteolysis with cortical disruption and sequestration. Pathologic fracture, fragmentation, and telescoping of the digit are associated with more aggressive osseous lesions. Periostitis is distinctly unusual. The joint spaces are relatively spared despite extensive osseous destruction. Acroosteosclerosis characterized by opaque nodules in the terminal phalanges and endosteal thickening is a radiologic finding in osseous sarcoidosis, but also is present in rheumatoid arthritis, systemic
lupus
erythematosus,
Hodgkin
disease,
and
sclero-
derma [i , 2]. Generalized osteosclerosis, usually involving the spine, pelvis, skull, and ribs, is a less typical osseous manifestation of sarcoidosis [11. Acute and chronic polyarthritis have been reported in i 0% to 35% of sarcoidosis patients [i , 2]. An acute symmetrical peripheral polyarthritis affects small and medium-sized joints early in the course of sarcoidosis. The condition usually resolves in 4 to 6 weeks. Recurrent episodes of acute exacerbation are typical of chronic polyarthritis, which affects the ankles, knees, wrists, shoulders, and smalljoints of the hands. Alterations related to joint disease found on radiographs consist of soft-tissue swelling, penarticular osteoporosis, mild joint-space narrowing, and eccentric erosions. Coexistent osseous lesions can extend to the subchondral bone and result in articular destruction and collapse [i , 2]. This patient
STULL
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i 334
AND
GLASS-ROYAL
AJA:154, June 1990
had a striking lack of interosseous alterations despite subjacent bone involvement and destruction. Other entities to be considered in the differential diagnosis
Case
of bone
consequent renal failure had required dialysis for the past 7 years. Her physical condition deteriorated progressively after a fall and injury to the extensor mechanism of both knees 5 years ago. The patient developed flexion contractures of both hips and knees related to these injuries. She complained of
lesions
in this
patient
include
tuberculosis
granulomatous infections, hemangiomatosis, skeletal metastases, and enchondromatosis. of intrathoracic
differential polyarthritis.
and
diagnosis
osseous
abnormalities
to sarcoidosis
with
and
other
xanthomatosis, The combination helps
narrow
associated
the
chronic
This
inability
Margaret A. Stull Mark Glass-Aoyal
REFERENCES 1 . Sartoris DJ, Resnick tions of sarcoidosis.
0, Resnik C, Yaghmai I. Musculoskeletal Semin Roentgenol 1985;20:376-386
2. Resnick D, Niwayama G. Sarcoidosis. Diagnosis
3: Primary
of bone
and joint
disorders,
manifesta-
In: Resnick D, Niwayama G, eds. 2nd ed. Philadelphia: Saunders,
1988:4012-4032 3. Yaghmai I. Radiographic, angiographic and radionuclide manifestations of osseous sarcoidosis. RadioGraphics 1983;3 :375-396 4. Subbarao K. Sarcoidosis of bone. In: Taveras JM, Ferrucci JT, eds. Radiology-diagnosis, imaging, intervention. Philadelphia: 1989;5: 1-5 5. Holt JF, Owens WI. Osseous lesions of sarcoidosis. Radiology 11-30
Lippincott, 1949;53:
Oxalosis
29-year-old
to walk
woman
and
with Renal with
Osteodystrophy
primary
subsequently
hyperoxaluria
underwent
and
bilateral
soft-
tissue contracture release procedures. Bone biopsy of the left iliac crest was performed at the request of the nephrology service. Histologic examination revealed numerous birefringent crystalline deposits consistent with calcium oxalate crystals within the bone tissue. Radiographs of the left shoulder and pelvis from a metabolic survey revealed evidence of osteitis fibrosa cystica and diffuse “Paget-like” osteosclerosis (Figs. 3A and 3B). Bilateral renal calcification
and
nephrolithiasis
also
were
identified.
A lateral
radiograph of the right foot (Fig. 3C) showed extensive vascular calcifications, which were denser than those commonly seen in diabetes or secondary hyperparathyroidism. Primary hyperoxaluria is a rare autosomal recessive metabolic disorder of glyoxylate metabolism, characterized by excessive production and urinary excretion of oxalic acid,
Fig. 3.-Case 3: Primary oxalosis with renal osteodystrophy. A, Radiograph of left shoulder shows subchondral resorption of distal clavicle and acromion, subligamentous resorption at sites of attachment of coracoclavicular ligament (white straight arrows), and subperlosteal resorption along medial aspect of humeral neck (black arrow). There is loss of normal trabecular pattern and diffuse patchy bone sclerosis. A focal soft-tissue calcification (curved arrow) is noted in region of tendinous portion of supraspinatus. B, Radiograph of pelvis reveals diffuse “Paget-like” osteosclerosis, especially about hips, symphysis pubis, lumbar vertebrae, and sacroiliac joints. Nephrolithiasis and diffuse nephrocalcinosis are present in visualized portion of kidneys (arrows), which are disproportionately small. C, Radiograph of right foot shows extensive vascular calcifications and diffuse osteosclerosis.
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AJA:154, June 1990
MUSCULOSKELETAL
calcium oxalate nephrolithiasis, and nephrocalcinosis with progressive renal failure that develops at an early age [1-4]. The metabolic derangement results from one of two enzymatic defects: the absence of a-ketoglutarate-glyoxylate carboligase activity resulting in glycolic aciduria (type i hyperoxaluria); or a deficiency of D-glycerate dehydrogenase causing 1 -glyceric aciduria (type 2 hyperoxaluria). The second type is exceedingly rare [i -4]. Extrarenal deposition of calcium oxalate crystals, termed oxalosis, accompanies primary hyperoxaluria and is accelerated on renal failure. Major extrarenal sites of accumulation include bone, small blood vessels, eyes, and soft tissues [i4]. There is also a predilection for sites of tissue injury. Crystal deposition is felt to be related to organ vascularity and not to active incorporation of oxalate crystals into growing tissue. Therefore, areas of high vascularity, such as the ends of bones, are preferentially affected [i]. The radiologic appearance of skeletal alterations in primary hyperoxaluria and oxalosis is produced by a combination of oxalate deposition and renal osteodystrophy, with the former predominating [i , 2]. Metaphyseal abnormalities in tubular bones include irregular transverse sclerotic bands and narrow translucent zones at the physis. Sclerosis in the subchondral areas of the humeri and femora can resemble osteonecrosis. Alternating lucent and sclerotic bands in the sternum and ilium have been described. Sclerotic zones along vertebral body end plates simulate the “rugger-jersey” spine of renal osteodystrophy. Delayed bone maturation and growth dysfunction resulting in drumstick-shaped metacarpals and epiphyseal invagination also have been noted. Eventually the trabecular pattern becomes blurred. Areas of cystic rarefaction followed by diffuse osteosclerosis are seen. End-stage renal failure leads to skeletal manifestations of renal osteodystrophy, including bone resorption, sclerosis, and osteomalacia [i , 2, 4]. In advanced disease, the skeletal alterations are produced not only by a direct response to oxalate deposition from hyperoxaluria, but also are affected by acquired or secondary oxalosis associated with chronic renal failure [4]. Secondary or acquired oxalosis is more common than the primary hereditary form. It is a known complication of renal failure, but also may result from excessive ingestion of substances that contain oxalate or are oxalate precursors, such as rhubarb, spinach, or ethylene glycol. In addition, pyridoxine deficiency and exposure to methoxyflurane anesthesia may result in secondary oxalosis. Patients with chronic gastrointestinal disorders, such as chronic inflammatory bowel disease, pancreatic and biliary tract diseases, bacterial overgrowth syndrome, and jejunoileal bypass surgery, which cause significant fat malabsorption, also may develop secondary oxalosis [3, 4]. The secondary causes of oxalosis usually do not produce skeletal and renal alterations as florid as those in the primary form. Margaret A. Stull Mark Glass-Royal
REFERENCES
1 . Day DL, Scheinman JI, Mahan J. Radiological aspects of primary hyperoxaluria. AIR 1986;146:395-401
CASE
OF
THE
i 335
DAY
2. Brancaccio D, Poggi A, Ciccarelli C, et al. Bone changes in end-stage oxalosis. AiR 1981;136:935-939 3. Williams HE, Smith LH Jr. Primary hyperoxaluria. In: Stansbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS, eds. The metabolic basis of inherited disease, 5th ed. New York: McGraw-Hill, 1983:204-228 4. Resnick D. Other crystal-induced diseases. In: Resnick 0, Niwayama G. eds. Diagnosis ofbone andjoint disorders, 2nd ed. Philadelphia: Saunders, 1988: 1804-1 814
Case
4: Gorham
Syndrome
of the Right
Clavicle
and
Scapula This 1 6-year-old boy suffered a fracture of the right clavicle on September i 0, i 987, while playing football. Radiograph of the right shoulder on the day of injury (Fig. 4A) showed a pathologic fracture of the clavicle with a moth-eaten pattern of osteolysis destroying the distal half of the bone. Because of this appearance, biopsy interpreted as compatible
was performed. Its results were with angioma or healing fracture,
without evidence of malignancy. Subsequent radiographs taken on June 20, 1989, revealed complete disappearance of the right clavicle, adjacent superior scapula, and coracoid process (Fig. 4B). A right axillary arteriogram (Fig. 4C) showed an abnormal hypervascular blush in the neck of the scapula, without pooling of contrast material or arteriovenous shunting. In light of the progression of radiologic findings, the original biopsy specimen was reevaluated and Gorham syndrome was diagnosed. Gorham syndrome (also known as Gorham disease, disappearing bone disease, phantom bone, massive osteolysis, hemangiomatosis with massive osteolysis, massive osteolysis, and angiomatosis) is a rare condition of unknown cause characterized by extensive localized osteolysis associated with proliferation of abnormal vascular channels [i , 2]. It is not hereditary, and no significant age or sex predilection is evident [3]. Approximately i 00 cases have been reported, the first in i 838 [4]. Clinical presentation is variable. Some patients complain of pain; others experience limitation of motion or obvious deformity, without significant pain [3]. Antecedent trauma is present in slightly more than half the patients [2]. Laboratory values are usually normal. Radiologically, the process is usually monocentric, but can spread to adjacent soft tissues and bones, despite an intervening joint space [3], as in our case. The process starts as small,
focal
areas
of
resorption
dissolution
of a portion
remaining
osseous
of bone
structure,
that
enlarge
results, resembling
until
with
tapering
a licked
complete
of the stick
of
candy [2]. Pathologic fracture is frequent. Any bone may be involved. Arteriography usually does not reveal profound vascular abnormality, although a faint blush in the affected area may be seen [3, 5]. On histologic examination, multiple thin-walled vascular channels associated with bony destruction are noted [i]. These channels may be of vascular or lymphatic origin [2]. Osteoclasts are scarce, and no evidence is present for malignancy. Therapeutic options that have been attempted include medical treatment, prosthetic devices, bone grafting, surgical extirpation, and radiotherapy; no clearly optimal approach has
STULL
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i 336
AND
GLASS-ROYAL
AJA:154, June 1990
Fig. 4.-Case A, Radiograph
4: Gorham syndrome of right clavicle and scapula. of right shoulder obtained on September 10, 1987, shows a pathologic fracture of distal third of clavicle (arrow). Note moth-eaten pattern of osteolysis involving distal half of this bone, which is tapered. B, Radiograph of right shoulderobtained on June 20, 1989, shows absence of right clavicle and coracoid process as well as permeative osteolysis of remaining upper scapula. C, Subtraction image from a right axillary arteriogram obtained on July 11, 1989, shows abnormal vascular blush In region of neck of scapula (arrow), in addition to absent clavicle. More inferior enhancement Is normal muscular blush, accentuated by subtraction technique.
been defined. The condition may be fatal if vital osseous
often arrests spontaneously, but structures are involved. Mark Glass-Royal Margaret A. Stull
REFERENCES 1. Gorham
LW, Wright
AW,
Shultz
HH, Maxon
FC. Disappearing
rare form of massive osteolysis. Am J Med 1954;17:674-682
bones:
a
2. Choma ND, Biscotti syndrome:
a case
Cv, Bauer TW, Mehta AC, Licata AA. Gorham’s report
83:1151-1156 3. Resnick D, Niwayama
and review
G. Osteolysis
of the literature.
and chondrolysis.
Am J Med
1987;
In: Resnick
D,
Niwayama G, eds. Diagnosis of bone and joint disorders, 2nd ed. Philadeiphia: Saunders, 1988:4141-4168 4. Jackson JBS. A boneless arm. Boston Med Surg J 1838; 18:368-369 5. Cannon SR. Massive osteolysis: a review of seven cases. J Bone Joint Surg [Br] 1986;68-B:24-28
1331
Musculoskeletal Margaret
Case
A. StuIl1
1: Parosteal
and
Case Mark
of the Day
Glass-Royal
Osteosarcoma
of the Humerus
About 6 months before presentation, this 62-year-old man noticed diffuse swelling and progressive loss of motion of the left shoulder and upper arm. About 4 months before presentation, he identified a nontender lump in the left shoulder. Physical examination revealed a large firm mass that extended into the root of the neck, medially to the chest wall, and inferiorly to the supracondylar region of the left elbow. Circulation, sensation, and function in the left hand were normal. Twenty years earlier, he had undergone biopsy for a left shoulder mass, which was diagnosed as an osteochondroma. The patient’s history did not reveal whether the mass had been completely surgically excised. Radiologic examination of the left humerus and shoulder (Fig. 1A) revealed a massive, amorphous, irregularly calcified, and ossified mass replacing and infiltrating the shoulder and arm as well as encircling the humerus. An anterior mTc methylene diphosphonate (MDP) scintigram (Fig. 1 B) showed markedly increased tracer accumulation in the mass in the left upper extremity. Intramedullary invasion, cortical destruction, and infiltration of adjacent structures by the juxtacortical humeral mass were shown by CT (Fig. 1 C). The extent of the tumor was delineated by MA examination. The Ti -weighted coronal MR images (Fig. i D) showed intermediate-signal tumor replacing the marrow of the left proximal humerus, enveloping the humerus, and infiltrating adjacent structures. The superior, medial, and inferior extent of the tumor was readily identified as bright signal infiltrating adjacent tissues on the T2-weighted MR coronal images (Fig. i E). Histologic examination revealed a parosteal osteosarcoma. Parosteal osteosarcoma is a rare, slow-growing, malignant, bone-forming neoplasm that arises from the surface of long bones. It has a better prognosis than conventional central osteosarcomas and other surface (juxtacortical) osteosarcomas [i -3]. Patients are generally affected in the second to fifth decades of life. They usually have gradual onset of pain, swelling, and a palpable mass [1 , 4]. The indolent behavior of the tumor delays diagnosis. As illustrated in this case, the tumor may attain enormous size before the patient seeks
1
Both authors:
Department
to M. A. Stull, coordinator AJR 154:1331-1336,
June
of Radiology,
Georgetown
University
evaluation [i , 4]. In addition, radiologic and histologic misdiagnosis as benign osteochondroma [2] may result in inadequate surgical excision of the tumor-a distinct possibility in this patient. The lesion most often arises from the metaphysis of a tubular bone, occasionally from the epiphysis, and rarely from the diaphysis [1 , 4]. The most common location is the posterior aspect of the distal femur, followed by the proximal humerus, the tibia, fibula, and ulna [i , 4]. This malignant neoplasm grows circumferentially along the cortex of the involved bone, eventually enveloping the bone shaft. Cortical destruction and neoplastic invasion of the medullary cavity are uncommon except in cases of prior treatment or a long history of growth, as illustrated in this case [3-5]. On radiographs, early parosteal osteosarcomas are radiodense, lobulated, or oval masses with a broad base of attachment to the external cortex. A thin, radiolucent cleavage plane between the tumor and underlying bone is characteristic but not a constant finding [i , 3-5]. Progressive growth of the tumor may obliterate this plane. This tumor ossifies from the base of the lesion to its periphery in contradistinction to myositis ossificans, which initially ossifies from the periphery [1 , 2, 4]. The differential diagnosis of a less advanced case of parosteal osteosarcoma includes osteochondroma, myositis ossificans, central osteosarcoma, extraosseous osteosarcoma, ossifying or calcifying hematoma, and exuberant callus [4, 5]. CT and MR studies assist in delineating the extent of the process and in preoperative planning [4, 5]. Margaret A. Stull Mark Glass-Royal clinical
Medical
Center,
REFERENCES 1 . Resnick
D, Kyriakos M, Greenway GD. Tumors and tumor-like lesions of bone: imaging and pathology of specific lesions. In: Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders, 2nd ed. Philadelphia:
Saunders, 1988:3648-3677 2. Hudson TM, Springfield DS, Benjamin M, tomography of parosteal osteosarcoma. 3. Schajowicz F, McGuire MH, Araujo ES, comas arising on the surfaces of long 1988;70:555-564
3800 Reservoir
of the Case of the Day series. 1990 0361-803X/90/1546-1331
C American Roentgen
Ray Society
Rd., N. W., Washington,
Bertoni F, Present DA. Computed AJR 1985;144:961-965
Muscolo DL. Gitelis S. Osteosarbones.
J Bone
Joint
Surg
[Am)
DC 20007. Address reprint requests
STULL
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i 332
AND
GLASS-ROYAL
AJR:154, June 1990
Fig. 1.-Case 1: Parosteal osteosarcoma of humerus. A, Radiograph of left humerus shows massive amorphous and irregular calcification surrounding and replacing shoulder structures and majority of humerus. Lobulated soft-tissue mass (white arrows) Is best seen along periphery of lesion. B, Anterior ““Tc-methylene diphosphonate scintigram shows inhomogeneous tracer uptake in a large, irregular, lobulated mass involving left shoulder structures and humerus. C, CT scan at level of left glenohumeral joint reveals extensive medullary invasion and cortical destruction by calcified juxtacortical humeral mass that Infiltrates adjacent structures also. D and E, TI-weighted, 600/25, (D) and T2-weighted, 2000/80, proximal left humerus (large straight arrow) and tumor extending also extends Into vicinity of left brachial plexus (curved arrow).
4. Smith J, Ahuja SC, Huvos AG, Bullough osteogenic sarcoma: a roentgenological Radiol 1978;29: 167-175
5. LindeIl MM, Shirkhoda
2: Osseous
PG. Parosteal
(juxtacortical)
of 30 patients.
J Can Assoc
A, Raymond AK, Murray JA, Harle TS. Parosteal
osteosarcoma: radiologic-pathologic 1987;148:323-328
Case
study
(E) coronal MR images of thorax and upper extremities show intramedullary invasion of medially, superiorly, and laterally into adjacent structures (small straight arrows). Tumor
Sarcoidosis
correlation
with emphasis
and Chronic
on CT. AiR
Polyarthntis
This 26-year-old black man, with a 5-year history of quiescent intrathoracic sarcoidosis, presented with intermittent swelling and tenderness of his fingers for the past 2 years. The patient’s symptoms partially improved by adjusting his steroid therapy. Physical examination revealed bilateral de-
formed, tender, and swollen fingers, hypertrophied nails, and skin lesions. In addition, multiple proximal and distal interphalangeal joints of both hands were tender and swollen. Other clinical findings related to active pulmonary sarcoidosis and left facial nerve involvement (i.e., Bell palsy) also had developed within the last 2 years and partially responded to steroid therapy. Radiographs of the hands (Figs. 2A and 2B) showed bilateral soft-tissue swelling, periarticular osteoporosis, deformity, and destructive osseous lesions of the digits. Localized “punched-out” Iytic lesions, trabecular alterations resulting in a latticework pattern, pathologic fractures, and fragmentation were identified in the phalanges. Periostitis was absent. The metacarpals and wrists were spared, except for
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AJR:154, June 1990
MUSCULOSKELETAL
CASE
OF THE
DAY
1333
Fig. 2.-Case 2: Osseous sarcoldosls and chronic polyarthritis. A and B, Radlographs of left (A) and right (B) hands show soft-tissue swelling, perlarticular osteoporosis, deformity, and destructive lesions of digits. “Punched-out” lesions (small arrows) are seen in multiple phalanges. Cortical tunneling and trabecular afterations produce a latticework appearance In affected phalanges (large arrows). Middle and distal phalanges of left middle finger, the distal phalanx of left ring and right middle fingers, and the base of distal phalanx of right thumb have pathologic fractures. Absence of perlosteal reaction Is strIking. An old healed lntraarticular fracture of head of right fifth proximal phalanx is noted. There is limited involvement of several distal metacarpals, bilaterally. wrists are spared. Joint spaces are relatively spared. C, Chest radiograph shows bilateral hilar and mediastinal adenopathy. Bilateral reticulonodular infiltrates are more prominent on right side.
minor alterations in several distal metacarpals. The joint spaces were relatively spared. All of these radiologic findings are characteristic of skeletal alterations in sarcoidosis. A concurrent chest radiograph (Fig. 2C) revealed mediastinal and hilar adenopathy and bilateral interstitial infiltrates consistent with stage 2 intrathoracic sarcoidosis. The cornbination of pulmonary and osseous abnormalities are characteristic of sarcoidosis. Sarcoidosis is a generalized granulomatous disorder of unknown cause characterized by noncaseating granulomas. The frequency of radiologically apparent osseous involvement in sarcoidosis ranges from i % to 1 3%, with an average of 5% [1 -4]. Any bone can be affected; however, the small bones of the hands and feet are most often involved, with the hands as the predominant site. Osseous alterations are usually asymmetric and involve the distal and middle phalanges more often than the proximal phalanges and metacarpals. The bone lesions are usually asymptomatic, but can cause severe symptoms, as in this patient [i , 2]. Soft-tissue swelling, tenderness, stiffness, and deformities can accompany osseous disease. Skeletal involvement is generally associated with cutaneous lesions, which were present in this patient. Radiologic evidence of pulmonary involvement is found in 80% to 90% of patients with osseous sarcoidosis [i , 2]. Skeletal lesions in sarcoidosis have several radiologic manifestations. Cortical and trabecular alterations resulting in a reticular pattern or latticework appearance are the most characteristic radiologic findings of osseous sarcoid [2, 5].
Punched-out lytic lesions of bone destruction also are commonly described. Calcification, nodular opacities, and residual trabeculae may be present in these lytic lesions. Occasionally, rapid bone destruction results in a permeative pattern of osteolysis with cortical disruption and sequestration. Pathologic fracture, fragmentation, and telescoping of the digit are associated with more aggressive osseous lesions. Periostitis is distinctly unusual. The joint spaces are relatively spared despite extensive osseous destruction. Acroosteosclerosis characterized by opaque nodules in the terminal phalanges and endosteal thickening is a radiologic finding in osseous sarcoidosis, but also is present in rheumatoid arthritis, systemic
lupus
erythematosus,
Hodgkin
disease,
and
sclero-
derma [i , 2]. Generalized osteosclerosis, usually involving the spine, pelvis, skull, and ribs, is a less typical osseous manifestation of sarcoidosis [11. Acute and chronic polyarthritis have been reported in i 0% to 35% of sarcoidosis patients [i , 2]. An acute symmetrical peripheral polyarthritis affects small and medium-sized joints early in the course of sarcoidosis. The condition usually resolves in 4 to 6 weeks. Recurrent episodes of acute exacerbation are typical of chronic polyarthritis, which affects the ankles, knees, wrists, shoulders, and smalljoints of the hands. Alterations related to joint disease found on radiographs consist of soft-tissue swelling, penarticular osteoporosis, mild joint-space narrowing, and eccentric erosions. Coexistent osseous lesions can extend to the subchondral bone and result in articular destruction and collapse [i , 2]. This patient
STULL
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i 334
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GLASS-ROYAL
AJA:154, June 1990
had a striking lack of interosseous alterations despite subjacent bone involvement and destruction. Other entities to be considered in the differential diagnosis
Case
of bone
consequent renal failure had required dialysis for the past 7 years. Her physical condition deteriorated progressively after a fall and injury to the extensor mechanism of both knees 5 years ago. The patient developed flexion contractures of both hips and knees related to these injuries. She complained of
lesions
in this
patient
include
tuberculosis
granulomatous infections, hemangiomatosis, skeletal metastases, and enchondromatosis. of intrathoracic
differential polyarthritis.
and
diagnosis
osseous
abnormalities
to sarcoidosis
with
and
other
xanthomatosis, The combination helps
narrow
associated
the
chronic
This
inability
Margaret A. Stull Mark Glass-Aoyal
REFERENCES 1 . Sartoris DJ, Resnick tions of sarcoidosis.
0, Resnik C, Yaghmai I. Musculoskeletal Semin Roentgenol 1985;20:376-386
2. Resnick D, Niwayama G. Sarcoidosis. Diagnosis
3: Primary
of bone
and joint
disorders,
manifesta-
In: Resnick D, Niwayama G, eds. 2nd ed. Philadelphia: Saunders,
1988:4012-4032 3. Yaghmai I. Radiographic, angiographic and radionuclide manifestations of osseous sarcoidosis. RadioGraphics 1983;3 :375-396 4. Subbarao K. Sarcoidosis of bone. In: Taveras JM, Ferrucci JT, eds. Radiology-diagnosis, imaging, intervention. Philadelphia: 1989;5: 1-5 5. Holt JF, Owens WI. Osseous lesions of sarcoidosis. Radiology 11-30
Lippincott, 1949;53:
Oxalosis
29-year-old
to walk
woman
and
with Renal with
Osteodystrophy
primary
subsequently
hyperoxaluria
underwent
and
bilateral
soft-
tissue contracture release procedures. Bone biopsy of the left iliac crest was performed at the request of the nephrology service. Histologic examination revealed numerous birefringent crystalline deposits consistent with calcium oxalate crystals within the bone tissue. Radiographs of the left shoulder and pelvis from a metabolic survey revealed evidence of osteitis fibrosa cystica and diffuse “Paget-like” osteosclerosis (Figs. 3A and 3B). Bilateral renal calcification
and
nephrolithiasis
also
were
identified.
A lateral
radiograph of the right foot (Fig. 3C) showed extensive vascular calcifications, which were denser than those commonly seen in diabetes or secondary hyperparathyroidism. Primary hyperoxaluria is a rare autosomal recessive metabolic disorder of glyoxylate metabolism, characterized by excessive production and urinary excretion of oxalic acid,
Fig. 3.-Case 3: Primary oxalosis with renal osteodystrophy. A, Radiograph of left shoulder shows subchondral resorption of distal clavicle and acromion, subligamentous resorption at sites of attachment of coracoclavicular ligament (white straight arrows), and subperlosteal resorption along medial aspect of humeral neck (black arrow). There is loss of normal trabecular pattern and diffuse patchy bone sclerosis. A focal soft-tissue calcification (curved arrow) is noted in region of tendinous portion of supraspinatus. B, Radiograph of pelvis reveals diffuse “Paget-like” osteosclerosis, especially about hips, symphysis pubis, lumbar vertebrae, and sacroiliac joints. Nephrolithiasis and diffuse nephrocalcinosis are present in visualized portion of kidneys (arrows), which are disproportionately small. C, Radiograph of right foot shows extensive vascular calcifications and diffuse osteosclerosis.
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AJA:154, June 1990
MUSCULOSKELETAL
calcium oxalate nephrolithiasis, and nephrocalcinosis with progressive renal failure that develops at an early age [1-4]. The metabolic derangement results from one of two enzymatic defects: the absence of a-ketoglutarate-glyoxylate carboligase activity resulting in glycolic aciduria (type i hyperoxaluria); or a deficiency of D-glycerate dehydrogenase causing 1 -glyceric aciduria (type 2 hyperoxaluria). The second type is exceedingly rare [i -4]. Extrarenal deposition of calcium oxalate crystals, termed oxalosis, accompanies primary hyperoxaluria and is accelerated on renal failure. Major extrarenal sites of accumulation include bone, small blood vessels, eyes, and soft tissues [i4]. There is also a predilection for sites of tissue injury. Crystal deposition is felt to be related to organ vascularity and not to active incorporation of oxalate crystals into growing tissue. Therefore, areas of high vascularity, such as the ends of bones, are preferentially affected [i]. The radiologic appearance of skeletal alterations in primary hyperoxaluria and oxalosis is produced by a combination of oxalate deposition and renal osteodystrophy, with the former predominating [i , 2]. Metaphyseal abnormalities in tubular bones include irregular transverse sclerotic bands and narrow translucent zones at the physis. Sclerosis in the subchondral areas of the humeri and femora can resemble osteonecrosis. Alternating lucent and sclerotic bands in the sternum and ilium have been described. Sclerotic zones along vertebral body end plates simulate the “rugger-jersey” spine of renal osteodystrophy. Delayed bone maturation and growth dysfunction resulting in drumstick-shaped metacarpals and epiphyseal invagination also have been noted. Eventually the trabecular pattern becomes blurred. Areas of cystic rarefaction followed by diffuse osteosclerosis are seen. End-stage renal failure leads to skeletal manifestations of renal osteodystrophy, including bone resorption, sclerosis, and osteomalacia [i , 2, 4]. In advanced disease, the skeletal alterations are produced not only by a direct response to oxalate deposition from hyperoxaluria, but also are affected by acquired or secondary oxalosis associated with chronic renal failure [4]. Secondary or acquired oxalosis is more common than the primary hereditary form. It is a known complication of renal failure, but also may result from excessive ingestion of substances that contain oxalate or are oxalate precursors, such as rhubarb, spinach, or ethylene glycol. In addition, pyridoxine deficiency and exposure to methoxyflurane anesthesia may result in secondary oxalosis. Patients with chronic gastrointestinal disorders, such as chronic inflammatory bowel disease, pancreatic and biliary tract diseases, bacterial overgrowth syndrome, and jejunoileal bypass surgery, which cause significant fat malabsorption, also may develop secondary oxalosis [3, 4]. The secondary causes of oxalosis usually do not produce skeletal and renal alterations as florid as those in the primary form. Margaret A. Stull Mark Glass-Royal
REFERENCES
1 . Day DL, Scheinman JI, Mahan J. Radiological aspects of primary hyperoxaluria. AIR 1986;146:395-401
CASE
OF
THE
i 335
DAY
2. Brancaccio D, Poggi A, Ciccarelli C, et al. Bone changes in end-stage oxalosis. AiR 1981;136:935-939 3. Williams HE, Smith LH Jr. Primary hyperoxaluria. In: Stansbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS, eds. The metabolic basis of inherited disease, 5th ed. New York: McGraw-Hill, 1983:204-228 4. Resnick D. Other crystal-induced diseases. In: Resnick 0, Niwayama G. eds. Diagnosis ofbone andjoint disorders, 2nd ed. Philadelphia: Saunders, 1988: 1804-1 814
Case
4: Gorham
Syndrome
of the Right
Clavicle
and
Scapula This 1 6-year-old boy suffered a fracture of the right clavicle on September i 0, i 987, while playing football. Radiograph of the right shoulder on the day of injury (Fig. 4A) showed a pathologic fracture of the clavicle with a moth-eaten pattern of osteolysis destroying the distal half of the bone. Because of this appearance, biopsy interpreted as compatible
was performed. Its results were with angioma or healing fracture,
without evidence of malignancy. Subsequent radiographs taken on June 20, 1989, revealed complete disappearance of the right clavicle, adjacent superior scapula, and coracoid process (Fig. 4B). A right axillary arteriogram (Fig. 4C) showed an abnormal hypervascular blush in the neck of the scapula, without pooling of contrast material or arteriovenous shunting. In light of the progression of radiologic findings, the original biopsy specimen was reevaluated and Gorham syndrome was diagnosed. Gorham syndrome (also known as Gorham disease, disappearing bone disease, phantom bone, massive osteolysis, hemangiomatosis with massive osteolysis, massive osteolysis, and angiomatosis) is a rare condition of unknown cause characterized by extensive localized osteolysis associated with proliferation of abnormal vascular channels [i , 2]. It is not hereditary, and no significant age or sex predilection is evident [3]. Approximately i 00 cases have been reported, the first in i 838 [4]. Clinical presentation is variable. Some patients complain of pain; others experience limitation of motion or obvious deformity, without significant pain [3]. Antecedent trauma is present in slightly more than half the patients [2]. Laboratory values are usually normal. Radiologically, the process is usually monocentric, but can spread to adjacent soft tissues and bones, despite an intervening joint space [3], as in our case. The process starts as small,
focal
areas
of
resorption
dissolution
of a portion
remaining
osseous
of bone
structure,
that
enlarge
results, resembling
until
with
tapering
a licked
complete
of the stick
of
candy [2]. Pathologic fracture is frequent. Any bone may be involved. Arteriography usually does not reveal profound vascular abnormality, although a faint blush in the affected area may be seen [3, 5]. On histologic examination, multiple thin-walled vascular channels associated with bony destruction are noted [i]. These channels may be of vascular or lymphatic origin [2]. Osteoclasts are scarce, and no evidence is present for malignancy. Therapeutic options that have been attempted include medical treatment, prosthetic devices, bone grafting, surgical extirpation, and radiotherapy; no clearly optimal approach has
STULL
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i 336
AND
GLASS-ROYAL
AJA:154, June 1990
Fig. 4.-Case A, Radiograph
4: Gorham syndrome of right clavicle and scapula. of right shoulder obtained on September 10, 1987, shows a pathologic fracture of distal third of clavicle (arrow). Note moth-eaten pattern of osteolysis involving distal half of this bone, which is tapered. B, Radiograph of right shoulderobtained on June 20, 1989, shows absence of right clavicle and coracoid process as well as permeative osteolysis of remaining upper scapula. C, Subtraction image from a right axillary arteriogram obtained on July 11, 1989, shows abnormal vascular blush In region of neck of scapula (arrow), in addition to absent clavicle. More inferior enhancement Is normal muscular blush, accentuated by subtraction technique.
been defined. The condition may be fatal if vital osseous
often arrests spontaneously, but structures are involved. Mark Glass-Royal Margaret A. Stull
REFERENCES 1. Gorham
LW, Wright
AW,
Shultz
HH, Maxon
FC. Disappearing
rare form of massive osteolysis. Am J Med 1954;17:674-682
bones:
a
2. Choma ND, Biscotti syndrome:
a case
Cv, Bauer TW, Mehta AC, Licata AA. Gorham’s report
83:1151-1156 3. Resnick D, Niwayama
and review
G. Osteolysis
of the literature.
and chondrolysis.
Am J Med
1987;
In: Resnick
D,
Niwayama G, eds. Diagnosis of bone and joint disorders, 2nd ed. Philadeiphia: Saunders, 1988:4141-4168 4. Jackson JBS. A boneless arm. Boston Med Surg J 1838; 18:368-369 5. Cannon SR. Massive osteolysis: a review of seven cases. J Bone Joint Surg [Br] 1986;68-B:24-28
