a collection of rare bone tumor and tumor like conditions for students of orthopaedic surgery.

1. Rare Bone Tumors
Vinod Naneria
Consultant Orthopaedic Surgeon
Choithram Hospital & Research Centre,
Indore, M.P., India

2. Contents
• Synovial Chondromatosis,
• Fibromatosis
• Heamengiomas
• Osteochondritis dissecans
• Multiple Myeloma
• Osteoid Osteoma
• Osteochondroma
• Lipoma

3. Contents….
• Fibroma
• Non-ossifying Fibroma
• Fibro-cortical defects
• Paget disease
• Osteogenesis Imperfecta
• Osteopetrosis
• Hyper-parathyroidism
• Neurofibromatosis
• Neurilemoma

4. Synovial chondromatosis
Monoarticular synovial proliferative disease in
which cartilaginous or osteocartilaginous
metaplasia occurs within the synovial
membrane of joints, bursae, or tendon
sheaths.
Three phases:
(1) early, with synovial chondrometaplasia but
no loose bodies,
(2) transitional, with active synovial disease
and loose bodies, and
(3) late, with loose bodies but no synovial
disease.

5. Routine investigations - X-ray, arthroscopy, CT scan,
& MRI.
common in the knee and hip, but almost any joint,
bursa, or tendon sheath may be affected.
Treatment is controversial;
synovectomy and removal of the loose bodies,
simple removal of the loose bodies, either by
arthroscopy or open operation.
Recurrence after surgery is not unusual, and there are
rare reports of malignant transformation to
chondrosarcoma.

6. Synovial Chondromatosis
cases…..
• A 60 yrs. male had some pain lt hip 1990
• Plain x-rays were negative.
• CT scan showed some loose bodies in
Fovea centralis.
• Since then managed conservatively.
• A 17 years follow up.

8. CT Scan 1990

10. MRI – 2006 to complete the investigation did not provide much information, a big
loose body is seen clearly. Joint appears normal.

11. Case 2

12. Case 3 Knee joint

14. Case 4 Large loose bodies

15. Case 5

16. Case 6 a 40 years female with chronic synovitis
Of the knee, underwent Arthroscopic debridement.
Multiple multi-color loose bodies removed.

17. Arthroscopic Removal of loose bodies

18. Case 7
3/2005
5/
2
0
0
6

19. Case 8

20. Case 9
Case 9

22. Clinical photos with pain free
Range of movements

23. Case 10 Two huge loose bodies in the sub scapular bursa
Accommodated in the infra-clevicular area. These bodies were
Removed 25 years ago.

24. Calcific Tendonitis of Ligamentum patellae

25. Skin painted with lead oxide (Geru)

26. Fibromatosis
Many fibrous proliferative lesions recur
after removal but do not metastasize. As a
group these are known as the
fibromatoses. Many have been defined
precisely and have been named, but
occasionally one is encountered that can
be recognized as a fibromatosis but
cannot be classified exactly.

27. • Desmoid tumors.
• Fibromatosis colli (congenital torticollis).
• Keloid.
• Irradiation fibromatosis.
• Palmar and plantar fascial fibromatoses.
• Peyronie disease.
• Juvenile aponeurotic fibroma.
• Elastofibroma dorsi.
• Progressive myositis fibrosa.
• Congenital generalized fibromatosis.
• Infantile dermal fibromatosis.
• Fibrous hamartoma of infancy.

28. Desmoid tumors are locally aggressive lesions of
connective tissue origin.
Infiltrate surrounding tissues and have a marked
propensity for persistence.
These lesions occur most frequently in the anterior
abdominal wall of women who have borne children.
In other locations are often known as extraabdominal
desmoids.

29. They are highly collagenized
and rather sparsely cellular and
infiltrate the muscle in which
they develop.
Grossly they are dense, hard,
rubbery, and grayish white.
Extraabdominal desmoids occur
most frequently in the shoulder
girdle, arm, thigh, neck, pelvis,
forearm, and popliteal fossa.

30. The natural history is usually a slow, relentless growth
with invasion of contiguous structures.
Spontaneous regression does occur, reported 29% of
patients treated by partial excision were without
evidence of disease at last follow-up.
Metastases have been reported but are extremely rare.
Most institutions currently recommend wide or marginal
excision followed by radiotherapy.
Regression following the use of indomethacin and
ascorbic acid, tamoxifen and testolactone, and
chlorothiazide has been reported.

33. Popliteal swelling with FFD

34. Swelling in popliteal space
Anterior knee- patient in prone position

36. Heamengiomas
• Cavernous hemangiomas consist of widely dilated
vessels or thicker vessels that may resemble veins;
Hemangiomas occur deep in skeletal muscle and other
soft tissues of the extremities.
• Recur unless completely removed.
• Occasionally a histologically benign hemangioma
stubbornly persists after surgery and becomes disabling.
Some intramuscular hemangiomas are infiltrative and
most difficult to excise except by radical surgery. Unlike
many soft tissue tumors, hemangiomas may be quite
painful.

37. Intrmascular Heamengioma
Intra-osseous,
Intra-mascular
Intra-neural
heamengioma

38. Intra mascular Heamengioma

39. OSTEOCHONDRITIS DISSECANS
OF KNEE
• The classic medial femoral condyle localization had a better
prognosis than an unusual one.
• Patients with no effusion, a lesion less than 20 mm, and no gross
detachment had significantly better results after conservative
treatment than those who had undergone operation. If signs of
detachment were present, the results were better after operative
than after conservative treatment.
• Arthroscopic drilling of osteochondritis dissecans lesions -no signs
of improvement after 6 months of conservative treatment.
• MRI is a highly sensitive method for detection of unstable
osteochondritis dissecans. The presence of an underlying high-
signal-intensity line, cystic area, or a focal articular defect indicates
instability and may help in preoperative planning. Whether the lesion
is drilled, excised, curetted, replaced and pinned, or bone grafted
depends on the size, stability, and weight-bearing nature of the
lesion, which can be determined only at surgery.

41. The loose body was found free & was removed arthroscopically

42. Multiple myeloma
• Multiple myeloma is the most common primary
malignancy of bone.
• 43% of primary malignancies of bone.
• Its peak incidence is in the fifth through seventh
decades with a 2:1 male predominance.
• Multiple myeloma and metastatic carcinoma
should be included in the differential diagnosis
for any patient over the age of 40 with a new
bone tumor.

43. Plasmacytoma

46. 2007
2002

48. Osteoid Osteoma
• Benign neoplasm most often seen in young males.
• Found in the first three decades of life, but an occasional lesion has
been reported in older patients.
• Almost any bone can be involved, although there is a predilection for
the lower extremity, with half the cases involving the femur or tibia.
• The tumor may be found in cortical or cancellous bone, producing a
distinct roentgenographic appearance of cortical sclerosis; 5% of
tumors are subperiosteal.
• Multicentric foci have been reported.
• No malignant change has ever been documented. The typical
patient has pain that is worse at night and relieved by aspirin.
• When the lesion is near a joint, swelling, stiffness, and contracture
may occur. When in a vertebra, scoliosis may occur.
• Occasionally, osteoid osteoma occurs with minimal pain. In children,
overgrowth and angular deformities may occur.

49. Osteoid Osteoma
• Routine roentgenograms often are diagnostic,
but bone scans or CT are often required to
localize the lesion accurately.
• CT may detect the nidus, whereas
roentgenograms show only sclerosis.
• A bone scan is helpful in detecting the quot;double-
density sign,quot; which is a focal area of increased
activity with a second smaller area of increased
uptake superimposed on it, is said to be
diagnostic of osteoid osteoma.

50. Osteoid Osteoma - Tx
• The entire nidus must be removed.
• Block resection of the nidus.
• An alternative method - shave the reactive bone
with a sharp osteotome until the nidus is
encountered, then curet the exposed nidus.
• Intraoperative localization of the nidus is
possible with preoperatively injected technetium-
labeled methylene diphosphonate and a sterile,
wrapped Geiger counter.
• If block excision is performed, intraoperative
roentgenograms of the specimen.

51. Osteoid Osteoma - Tx
• Excision of the osteoid osteoma nidus using CT–assisted
localization, a Kirschner wire inserted into the nidus, and a biopsy
punch inserted over the Kirschner wire into the bone.
• They recommend using a trephine 2 mm larger than the lesion for
complete removal.
• Recurrence after apparently complete excision has been reported
but is rare.
• Percutaneous radiofrequency ablation of osteoid osteomas.
• Spontaneous disappearance of osteoid osteomas after extended
observation and treatment of symptoms.

52. Giant osteoid osteoma

53. Tibia posterior &
lateral border

54. Lamina D11

55. Medial wall
Acetabulum

59. Osteochondroma
• The most common of the benign bone tumors.
• They are probably developmental malformations rather
than true neoplasms and are thought to originate within
the periosteum as small cartilaginous nodules.
• The lesions consist of a bony mass, often in the form of
a stalk, produced by progressive endochondral
ossification of a growing cartilaginous cap.
• Most lesions are found during the period of rapid skeletal
growth. About 90% of patients have only a single lesion.
Osteochondromas may occur on any bone preformed in
cartilage but usually are found on the metaphysis of a
long bone near the physis. They are seen most often on
the distal femur, the proximal tibia, and the proximal
humerus. Rarely do they develop in a joint.

68. Lipoma
• Lipomas most common benign tumors of connective
tissue.
• They may occur at any age and in either sex.
• These tumors usually develop subcutaneously but may
involve the deeper structures. They occasionally affect
the synovium (lipoma arborescens) and rarely the
periosteum.
• Clinically they are soft, circumscribed, movable masses
that are painless and grow slowly.
• On roentgenograms the larger ones appear as discrete
radiolucent areas within soft tissue.
• Grossly a lipoma is a well-encapsulated nodule of fat
that may contain fibrous tissue.
• Microscopically it is composed of mature fat cells.

70. Fibromas
• Most of these lesions are found in the upper
extremity and are 1 to 2 cm in diameter.
• They differ histologically from giant cell tumor of
tendon sheath by the absence of xanthoma cells
and giant cells.
• Proper treatment is marginal excision.
• In the hand, persistence as high as 24% has
been reported.
• Reexcision of the recurrence has been curative
in most patients.

71. Fibromas

72. NONOSSIFYING FIBROMA
• Nonossifying fibromas have the same histological
structure as fibrous cortical defects, but they are larger.
• Most occur in children and adolescents between the
ages of 10 and 20 years.
• The roentgenographic appearance usually is
characteristic.
• The osteolytic area is eccentrically located and oval.
Multilocular appearance or ridges in the bony wall,
sclerotic scalloped borders, and erosion of the cortex are
frequent findings.
• CT & biopsy be necessary for diagnosis.
• Regress spontaneously.

73. Non Ossifying Fibroma of right
Acetabulum

74. 1999 1999 2007 2007

75. Fibrous cortical defects
• Fibrous cortical defects, although classified as
bone tumors, are probably developmental
abnormalities and are believed to occur in as
many as 35% of children.
• Found incidentally.
• Metaphyseal region of long bones in ages of 2
and 20 years and occur predominantly in males.
Approximately 40% are found in the femur, 40%
in the proximal tibia, and 10% in the fibula,
although some occur in the upper extremity,
clavicle, ilium, ribs, vertebrae, skull, mandible, or
small bones of the hand and feet.

76. Fibro-cortical defects

78. Paget disease
Paget disease is an entity of unknown cause characterized by
osteoclastic resorption of bone followed by osteoblastic regeneration
of a primitive woven bone.
These processes may result in
(1) a roentgenographic appearance simulating a malignant tumor,
(2) deformity, or
(3) a pathological fracture of a long bone.
Biopsy may be required to establish the diagnosis. Intraosseous
arteriovenous shunts may develop and lead to impressive bleeding
during surgical procedures on involved bone. Bowing deformities of
long bones may make internal fixation of pathological fractures
technically difficult.
Nerves passing through foramina in quot;pagetoidquot; bone may be entrapped
and lead to troublesome symptoms. Occasionally decompressive
surgical procedures may be indicated. Sarcomatous change in
pagetoid bone is not rare (Fig. 23-15).

80. Osteogenesis Imperfecta
• Osteogenesis imperfecta is a disease
apparently of the mesodermal tissues with
abnormal or deficient collagen that has
been demonstrated in bone, skin, sclerae,
and dentine. The so-called diagnostic triad
of blue sclerae, dentinogenesis
imperfecta, and generalized osteoporosis
in a patient with multiple fractures or
bowing of the long bones usually is used
clinically.

83. Osteopetrosis
• osteopetrosis is failure of osteoclastic and
chondroclastic resorption;
- bones become exceedingly dense;
- marrow space is filled with unresorbed dense
bone;
• There is absence of marrow elements & these
patients are susceptible to infectious diseases.
• Most common is osteomyelitis of Mendible.
• Sub trochanteric fractures are common and
usually ends up in nonunion

84. A very old x-ray showing sclerosis at
Subtrochanteric level on both sides.
Developed fracture right femur at this site

85. Fractured healed after 3 surgeries

86. CT Scan
Showing
Bone
Marrow
obliteration
of femur

87. HYPERPARATHYROIDISM
Caused by overactivity of the parathyroid glands,
usually resulting from neoplasm or hyperplasia of the glands.
The symptoms may be renal, psychiatric, or skeletal.
The skeletal change usually is limited to diffuse demineralization.
Only rarely does the change become markedly focal
and produce a quot;brown tumor“.
Blood tests - serum calcium, phosphorus, alkaline phosphatase,
and hyperparathyroid hormone levels.
Sonography of neck for adenoma and abdomen for stones.

88. In hyperparathyroidism: Histopathology
(1) the giant cells are a little smaller, often occurring in a
somewhat nodular arrangement, especially around
areas of hemorrhage;
(2) the stromal cells are more spindle shaped and
delicate; and
(3) evidence of osseous metaplasia within the stroma is
prominent.
The bone surrounding the lesion should also be
examined;
in hyperparathyroidism it may show intense osteoclastic
and osteoblastic activity associated with peritrabecular
fibrosis.

92. quot;BROWN TUMORquot; OF HYPERPARATHYROIDISM

95. Neurofibromatosis
• Neurofibromas occur as a manifestation of von
Recklinghausen disease, in which many such
tumors may be found associated with café au lait
spots and various other lesions. Sometimes a
special type of neurofibroma occurs in which
entire peripheral nerves are involved, this is
referred to as a plexiform neurofibroma, and
excising it completely may be impossible. Other
manifestations of neurofibromatosis are
hypertrophy of soft tissue, including the skin,
hypertrophy of bone, scoliosis, bone cysts, and
other abnormalities.

96. Case history
• 4 yrs M, unable to stand due to painful
swelling in left gluteal region since infency.
• Clinically hyperalgesia in gluteal muscle
mass with defuse soft to firm and nodular
at places. Limb lengthening by 4cm.
• Skin – hyperpigmentation.
• Mother had same nodules and
pigmentation.

97. café au lait spots

98. X-ray MRI
Defuse soft-tissue tumor mass in the gluteal region and around sciatic
Nerve, extending into pelvis, upto spine S1 area. Also in brain.

100. Nerve sheath
Nerve sheath
Nerve sheath

101. Nerve sheath
Tumor mass
Normal Nerve

102. 14cm long tissue along the sciatic nerve
Tissue removed from the Gluteal region

106. Neurilemoma is typically a solitary
encapsulated lesion that may be cystic when
it is as large as 3 to 4 cm in diameter.
Usually it involves one of the larger
peripheral nerves.
There may be little if any neurological deficit.
Often the lesion simply spreads the nerve
fibers apart without anatomical or functional
interruption, so the tumor can be removed
by careful blunt dissection after a
longitudinal incision in the perineurium.

107. Thus there may be little if any increase in
the dysfunction of the nerve after surgery.
Occasionally the tumor may recur, but
usually the lesion or recurrent lesion can
be removed without sacrificing a
significant number of nerve fibers.
Excision that interrupts the continuity of
the nerve should be avoided. Often there
is cystic degeneration in the center of the
nerve.

108. Case 2
• A 40 M with chronic pain rt.leg 5 years
• Clinically a ill defined swelling in the
antero-lateral aspect of leg from head of
fibula to mid leg.
• A small swelling on left leg lower part on
anterior border of fibula.

112. DISCLAIMER
• Information contained and transmitted by this presentation is based
on personal experience and collection of cases at Choithram
Hospital & Research centre, Indore, India, during last 25 years.
• It is intended for use only by the students of orthopaedic surgery.
• Views and opinion expressed in this presentation are personal
opinon.
• Depending upon the x-rays and clinical presentations viewers can
make their own opinion.
• For any confusion please contact the sole author for clearification.
• Every body is allowed to copy or download and use the material
best suited to him. I am not responsible for any controversies arise
out of this presentation.
• For any correction or suggestion please contact
• naneria@yahoo.com