bb5_27.qxd 13.9.2006 13:53 Page 325
CHAPTER 19
Myogenic, Lipogenic, Neural,
and Epithelial Tumours
Smooth muscle tumours of bone, usually leiomyosarcoma, are
very rare. A metastasis from a distant site, especially the uterus,
has to be excluded before accepting the diagnosis of primary
leiomyosarcoma of bone.
Lipomas are not uncommon in bone and are incidental findings
on X-rays and frequently involve the calcaneus.
Roentgenograms show a well-circumscribed area of lucency
with a central area of calcification. CT and MRI help to confirm
the fatty nature of the tumour.
Neurilemmomas (schwannomas) occur rarely in bone. Along the
spine, especially in the sacrum, they may involve bone second-
arily. The most common location for an intraosseous neurilem-
moma is the mandible. The histological features are similar to
those of schwannomas elsewhere. Malignant peripheral nerve
sheath tumours (MPNST) do not occur in bone.
Adamantinoma has an epithelial phenotype and almost exclu-
sively involves the tibia. It is a low-grade malignancy with a
favourable clinical course. The roentgenographic, morphologic
and genetic features are often similar to those of osteofibrous
dysplasia.
Metastatic carcinoma is by far the commonest malignancy in the
skeleton, the most frequent primary tumours being carcinomas
of the lung, breast, prostate and kidney. Haematogeneous
metastasis of sarcomas to bone is a rare event.
bb5_27.qxd 13.9.2006 13:53 Page 326
E. McCarthy
Leiomyoma of bone
Definition tions. Uniform spindle cells are present a pattern identical to angioleiomyoma
A benign spindle cell tumour of bone in interlacing bundles. Mitotic figures are {2166}.
with smooth muscle differentiation. extremely rare. The cells are positive with
immunohistochemical stains for smooth Prognostic factors
Epidemiology muscle actin and desmin. Occasionally, Local excision results in a complete
Leiomyomas of bone are very rare. Most thick-walled blood vessels are present in cure.
patients are adults over age 30, although
a child age 3 years has been reported.
Males and females are equally affected
{2166}.
Sites of involvement
The facial bones are most commonly
affected by primary leiomyoma. The
most common site is the mandible. In the
extragnathic skeleton, the tibia is the
most common site {2166}.
Clinical features
Patients present with pain. Radiologi-
cally, lesions are radiolytic, often multi-
locular. A sclerotic rim may be present.
Occasionally there may be cortical
expansion.
Macroscopy
Primary leiomyomas of bone are firm
gray tan tumours. Most lesions are 3 cm
or smaller in maximum dimension.
A
Histopathology
Histologically, leiomyomas of bone are
identical to leiomyomas in other loca-
B
Fig. 19.01 Leiomyoma. CT scan showing a well Fig. 19.02 Leiomyoma. A Low power view showing bundles of uniform spindle cells. B Thick walled blood ves-
defined lytic lesion with a sclerotic rim in the ilium. sels admixed with spindle cells in a pattern of angiomyoleiomyoma.
326 Smooth muscle tumours
bb5_27.qxd 13.9.2006 13:53 Page 327
E. McCarthy
Leiomyosarcoma of bone
Definition Clinical features Histopathology
A very rare malignant spindle cell sarco- Pain, present from 2 weeks to 1 year prior Histologically, lesions are identical to
ma of bone which shows smooth muscle to diagnosis, is the most common symp- leiomyosarcomas in other locations.
differentiation with immunohistochemical tom. Approximately 15% of patients pres- Plump and pleomorphic spindle cells are
or electron microscopic studies. ent with pathological fracture. arranged in bundles or fascicles. Mitotic
Radiographically, it is an aggressive radi- figures are common. Often areas of
Epidemiology olytic lesion, with poorly defined margins, necrosis are present. Smooth muscle dif-
Although the reported age range is from a permeative growth pattern, and cortical ferentiation is demonstrable by positive
9 to 87 years, the mean age is 44 years destruction. MRI shows a hypointense immunohistochemical staining for
{165,1049,1932}. There is a slight male lesion on T1 and an iso-or hypointense smooth muscle actin and desmin.
predominance. lesion on T2 weighted studies {2056}. Electron microscopic studies demon-
strate fine filamentous actin fibrils in the
Sites of involvement Macroscopy cytoplasm.
Most lesions occur in the lower extremity Lesions are grey to tan, firm or creamy
around the knee, either in the distal masses, often with areas of necrosis or Prognostic factors
femoral metaphysis or proximal tibial cystic degeneration. Despite a broad Approximately 50% of patients develop
metaphysis. The craniofacial skeleton is range in size, lesions average 6 cm in metastases to the lungs within 5 years
the next most common area to be greatest dimension {68}. Cortical pene- {68}. Ultimately, 50% of patients die from
involved {68}. tration is common. leiomyosarcoma of bone {1099}.
A
B
Fig. 19.05 Leiomyosarcoma. A Low power photomi-
Fig. 19.03 Leiomyosarcoma. X-ray of a tumour in
Fig. 19.04 Leiomyosarcoma. Macroscopy of the
crograph showing bundles of spindle cells.
distal femur showing an aggressive, permeative
femoral lesion. Note both an intraosseous and an
B On high power magnification, note the cellular
lytic lesion with cortical destruction.
extraosseous component of the white fleshy tumour.
pleomorphism of the tumour cells.
Leiomyosarcoma of bone 327
bb5_27.qxd 13.9.2006 13:53 Page 328
A.E. Rosenberg
Lipoma of bone
J.A. Bridge
Definition Epidemiology cal surface that may show thickening or
Lipoma of bone is a benign neoplasm of Lipoma of bone is rare and accounts for a periosteal reaction. Similar to
adipocytes that arises within the less than 0.1% of primary bone tumours; intraosseous lipoma, the CT and MRI
medullary cavity, cortex, or on the sur- their incidence is not known. findings have the same features as sub-
face of bone. Intramedullary lipoma has a wide age cutaneous fat except if there is calcifica-
range (2nd-8th decades) but most tion, cartilage or ossification within the
Synonyms patients are approximately 40 years old lesion {1079,1752}.
Intramedullary lipoma, intracortical lipo- at the time of diagnosis {1458}. Males are
ma, ossifying lipoma, parosteal lipoma. affected more frequently than females at Macroscopy
a ratio of approximately 1.6:1 {1458}. Intramedullary lipoma is usually 3-5 cm
Parosteal lipoma usually develops dur- in size, is well defined, soft, and yellow.
ing adulthood and most patients are in The surrounding bone is often sclerotic.
their 5th-6th decade of life at the time of Parosteal lipoma is usually 4-10 cm in
diagnosis {1462}. There is a slight male greatest dimension, is well defined, soft
predominance (9:7) {1462}. and yellow. Some cases contain gritty
spicules of bone or firm nodules of carti-
Sites of Involvement lage in the base or scattered throughout
The vast majority of intraosseous lipomas the mass.
arise within the medullary cavity and
rarely develop in the cortex {2317}. They Histopathology
most commonly affect the metaphyseal Intramedullary lipoma is well defined and
regions of the long tubular bones, espe- consists of lobules of mature-appearing
cially the femur, tibia and fibula and the adipocytes that may replace the marrow
calcaneus. However, they have also and encase preexisting bony trabeculae.
been described in many bones including The adipocytes have a single large clear
the pelvis, vertebrae, sacrum, skull, cytoplasmic vacuole that displaces the
mandible, maxilla, and ribs. crescent shaped nucleus to the periph-
Parosteal lipomas generally develop on ery. Some tumours may demonstrate fat
the diaphyseal surface of long tubular necrosis with foamy macrophages and
bones, especially the femur, humerus, fibrosis. In ossifying lipomas delicate tra-
and tibia {1462}. beculae of woven and lamellar bone may
be present throughout the tumour {121,
Clinical features / Imaging 346}.
Intramedullary lipoma may be asympto-
matic or produce achy pain. Rarely, it
Fig. 19.06 Lipoma. Radiograph of intramedullary presents as a pathological fracture {822,
lipoma of humerus demonstrating an oval lytic
951, 1458}. Radiographically, intra-
lesion with a sclerotic rim.
medullary lipoma usually produces a
well defined lytic mass that is surround-
ed by a thin rim of sclerosis. The lesion
may also contain trabeculations or cen-
tral calcifications. Bony expansion may
occur in small caliber bones {822,951,
1458,1732}. CT shows that the fatty com-
ponent has a low attenuation value simi-
lar to that of subcutaneus fat and on MRI
the fat has high signal intensity on both
T1 and T2 weighted images {1732}.
Parosteal lipoma is frequently asympto-
matic and may present as a visible or
Fig. 19.07 Lipoma of calcaneous producing a well
palpable mass. Radiographs may reveal
defined lytic lesion with central mineralization. Axial Fig. 19.08 Lipoma. Axial CT showing that the lipoma
a radiolucent mass adjacent to the corti- has the tissue density of fat.
CT confirms the fatty nature of the lesion.
328 Lipogenic tumours
bb5_27.qxd 13.9.2006 13:53 Page 329
A B
Fig. 19.09 A Well defined intramedullary lipoma composed of sheets of white adipocytes. B Parosteal lipoma composed of lobules of white fat cells.
Parosteal lipoma is also well defined and Immunophenotype Prognostic factors
consists of lobules of mature appearing The neoplastic fat expresses vimentin Lipoma of bone has an excellent progno-
white adipocytes. The adipocytes have a and S100 protein. sis and rarely recurs.
single large clear cytoplasmic vacuole
that displaces the crescent shaped Genetics
nucleus to the periphery. Some cases The translocation t(3;12)(q28;q14) and
may have bone with or without a hyaline its associated fusion transcript HMGIC/
cartilage in the base of the lesion or scat- LPP characteristic of subcutaneous lipo-
tered throughout the mass in small ma has been detected in a case of
islands {1462}. parosteal lipoma {255,1698}.
Lipoma of bone 329
bb5_27.qxd 13.9.2006 13:53 Page 330
A.E. Rosenberg
Liposarcoma of bone
Definition that contains a finely arborizing vascular Ultrastructure
Liposarcoma of bone is a malignant neo- tree that has a plexiform pattern. Also The cytoplasm of the neoplastic cells
plasm whose phenotype recapitulates scattered throughout the tumour are contains membrane bound lipid droplets
fat. lipoblasts. Sheets of large pleomorphic of varying size. Dilated rough endoplas-
cells in which the cytoplasm is either mic reticulum and scattered mitochon-
Epidemiology eosinophilic or filled with round clear vac- dria are also present {1650}.
Liposarcoma of bone is an extraordinari- uoles characterize pleomorphic liposar-
ly rare neoplasm. Most cases are coma. Mitoses are usually numerous. Prognostic factors
described in the form of single case Prognostic information regarding liposar-
reports in older literature and the validity Immunophenotype coma of bone is scant. Generally, the
of the diagnosis in some cases has been There are no data regarding the immuno- behaviour of the tumour should correlate
questioned {457}. Liposarcoma of bone phenotype of liposarcoma of bone. with its histological grade.
occurs in all age groups although the
majority of patients are adults {15,457,
1090,2121}. Men are affected slightly
more frequently than women.
Sites of involvement
Liposarcoma of bone usually develops in
the long tubular bones especially the
tibia and femur and has been reported to
arise in the diaphysis, metaphysis, and
epiphysis {15,457,1090,2121}.
Clinical features / Imaging
Liposarcoma of bone presents as a
painful mass. Radiographically, the
tumour manifests as a well defined or
poorly defined mass {15,457,1090,
2121}.
Macroscopy
Most liposarcomas are large, lobulated,
soft to firm and are yellow to tan-white in
colour. Myxoid tumours may be glisten-
ing, slimy and mucinous.
Fig. 19.10 Myxoid liposarcoma consisting of scattered spindle and stellate cells and occasional lipoblasts
enmeshed in a frothy myxoid stroma that contains branching small caliber capillaries.
Histopathology
The histological variants of liposarcoma
reported in bone include well differentiat-
ed lipoma-like, myxoid and pleomorphic
types {15,457,1090,2121}. Well differenti-
ated lipoma-like liposarcoma consists of
sheets of mature appearing adipocytes
with scattered tumour cells having
enlarged hyperchromatic nuclei. Some
of these atypical cells are lipoblasts and
are distinguished by cytoplasmic vac-
uoles that are round, clear, and scallop
A B
the nucleus. Myxoid liposarcoma con-
Fig. 19.11 A Well differentiated liposarcoma, lipoma-like type, containing mature appearing white fat cells
sists of mildly atypical stellate and spin-
and scattered adipocytes that have enlarged hyperchromatic nuclei. B Sheets of pleomorphic cells includ-
dle cells enmeshed in a myxoid stroma
ing lipoblasts characterize pleomorphic liposarcoma.
Lipogenic / neural tumours
330
bb5_27.qxd 13.9.2006 13:53 Page 331
K.K. Unni
Schwannoma
Definition Clinical features / Imaging examples of malignant transformation of
Schwannoma is a benign neoplasm of Most neurilemmomas are asymptomatic, neurilemmomas in bone.
Schwann cell origin arising within bone. incidental findings on roentgenograms.
Occasionally, they produce pain and/or
ICD-O code 9560/0 swelling.
Synonyms Macroscopy
Neurilemmoma, neurinoma. Schwannomas of bone are extremely
well circumscribed and may show a
Epidemiology fibrous capsule. They are tan to white
Neurogenic tumours of bone are extre- and glistening. Foci of yellow discoloura-
mely uncommon. Although roentge- tion may be seen.
nographic abnormalities may be found
involving the skeleton in patients with Histopathology
A
Schwannoma is composed of spindle
cells with wavy appearing nuclei. The
nuclei frequently are arranged in a pal-
isading fashion. Areas of hypocellularity
may alternate with areas of hypercellular-
ity. Focally, the nuclei may be enlarged
and pleomorphic appearing. Mitotic
activity is rare. Schwannomas are always
diffusely and strongly positive with S100
protein.
B
Prognostic factors
Fig. 19.13 A Encapsulated mandibular Schwannoma
Schwannomas are benign lesions and
with tan and white areas. B Note the discrete ten-
complete, but conservative surgical
dency of spindle cell nuclei to palisade. The nuclei do
removal leads to cure. There are no not show cytological atypia.
Fig. 19.12 CT of a well-demarcated Schwannoma
of the maxilla.
neurofibromatosis, there are no well rec-
ognized examples of neurofibroma in
bone. All benign neurogenic tumours in
the skeleton are Schwannomas. They
compose less than 1% of all benign
tumours in the Mayo Clinic files (unpub-
lished statistics, Unni, K. K.).
Sites of involvement
The mandible and the sacrum are the
most common sites of involvement with
neurilemmoma. In the mandible, the
lesion almost always involves the mental
foramen. When neurilemmoma involves
the spine or the sacrum, it is frequently
difficult to know whether the tumour is
truly of bony origin. Fig. 19.14 Schwannoma. Note the hyalinization of vascular walls.
Schwannoma 331
bb5_27.qxd 13.9.2006 13:53 Page 332
P.C.W. Hogendoorn
Adamantinoma
H. Hashimoto
Definition ma, but very young patients with classic develop years after primary, intralesional
A low grade, malignant biphasic tumour adamantinoma (age 3) and older ones or marginal surgical treatment. On X-ray,
characterized by a variety of morpholog- with the osteofibrous dysplasia-like sub- typically a well circumscribed, cortical,
ical patterns, most commonly epithelial type (age 38) have been reported {918, (multi-)lobulated osteolytic lesion with
cells, surrounded by a relatively bland 1502,2069}. There is a slight predomi- intralesional opacities, septation and
spindle-cell osteo-fibrous component. nance in males. peripheral sclerosis is seen {217,987}.
Multifocality within the same bone is reg-
ICD-O code 9261/3 Sites of involvement ularly observed. The lesion commonly
The tibia, in particular the anterior seems to remain intracortical and
Synonyms (meta-) diaphysis, is involved in 85-90% extends longitudinally, but may also
Adamantinoma of long bones, extrag- of cases. In up to 10% this is combined destroy the cortex and invade the
nathic adamantinoma, differentiated with one or more lesions in the ipsilateral medullary cavity or surrounding perios-
adamantinoma, juvenile intracortical fibula as well. Rare other sites have been teum and soft tissue. This is usually
adamantinoma. reported, especially the ulna. accompanied by lamellar or solid
periosteal reaction. Aggressive tumours
Epidemiology Clinical features / Imaging occasionally present as single large lytic
Adamantinoma comprises about 0.4% of The main complaint is swelling with or lesions. MRI is useful to document multi-
all primary bone tumours {987,1503, without pain. Adamantinoma often dis- centricity, the extension of the lesion, and
1518}. Patients present with this tumour plays a protracted clinical behaviour. eventual soft tissue involvement.
from 3 up to 86 years, with a median age Clinical symptoms like swelling or radi-
of 25-35 years. The youngest age group ographic abnormality may last for more Macroscopy
predominantly includes patients with than 30 years prior to diagnosis, where- Classic adamantinoma usually presents
osteofibrous dysplasia-like adamantino- as local recurrences or metastases may as a cortical, well-demarcated, yellow-
ish-grey, lobulated tumour of firm to bony
consistency with peripheral sclerosis. It
may be a single lesion, but its multifocal
appearance with apparently normal cor-
tical bone lying in between is occasion-
ally striking. Small lesions remain intra-
cortical, and are usually white and gritty.
Larger tumours show intramedullary
extension and cortical breakthrough with
soft tissue invasion in a minority of cases.
Macroscopically detectable cystic
spaces are common, filled with straw-
coloured or blood-like fluid.
Histopathology
Classic adamantinomas are character-
ized by an epithelial and an osteofibrous
component, that may be intermingled
with each other in various proportions
and differentiation patterns. The four
main differentiation patterns of classic
adamantinoma are basaloid, tubular,
spindle cell, and squamous {2235}. The
first two patterns are encountered most
commonly, but all patterns may be pres-
ent in one lesion. The spindle cell com-
Fig. 19.15 Classic adamantinoma. The radiograph Fig. 19.16 Osteofibrous dysplasia-like adamantino-
ponent is more often observed in recur-
of the distal tibia shows an expansive, lobulated, ma. The lateral radiograph of the proximal aspect of
rences, lining cystic spaces, and in
lytic lesion with a defect of the outer surface of the the tibia shows a multilocular, lytic lesion with sur-
metastases. The osteofibrous compo-
cortex. rounding osteosclerosis of the anterior cortex.
Epithelial tumours
332
bb5_27.qxd 13.9.2006 13:53 Page 333
A B
Fig. 19.17 Adamantinoma. A Basaloid pattern. Easily distuingishable epithelial fields without clear pallisading. B Squamoid pattern.
A B
Fig. 19.18 Adamantinoma. A Spindle cell pattern. B Osteofibrous dysplasia like adamantinoma. Small epithelial clusters in a fibro-osseous stroma.
nent is composed of storiform oriented the fibrous tissue. Towards the periphery, epithelial cell keratins 5, 14, and 19. Also
spindle cells. Woven bone trabeculae the epithelial islands decrease to incon- keratins 1, 13 and 17 are variably pres-
are usually present in or next to the cen- spicuous elements and the osteofibrous ent. Keratins 8 and 18 are virtually
tre of the lesion, prominently rimmed by component gradually takes over with absent. In classic adamantinomas, the
osteoblasts, and with varying amounts of increasing amounts of woven bone tra- epithelial component is surrounded by a
transformation to lamellar bone at the beculae, transforming to lamellar bone. continuous basement membrane, where-
periphery of the tumour. Foam cells or In osteofibrous dysplasia-like adamantin- as less distinct epithelial islands show
myxoid change may be present, and oma, the centre is occupied by fibrous multiple interruptions or no surrounding
mast cells or multinucleated giant cells tissue with scanty and thin immature basement membrane at all {919}.
are occasionally detected. Mitotic activi- woven bone trabeculae with epithelial EGF/EGFR expression is restricted to the
ty is usually low. A fifth histological pat- elements. Small clusters of epithelial epithelial component. FGF2/FGFR1 is
tern, the so-called osteofibrous dyspla- cells are the only feature which differenti- present in both components {242}.
sia-like variant, is characterized by pre- ate osteofibrous dysplasia-like adaman-
dominance of osteofibrous tissue, in tinoma from osteofibrous dysplasia. Ultrastructure
which small groups of epithelial cells are Electron microscopic studies have con-
only encountered by careful search or Immunophenotype firmed the epithelial nature of adamantin-
immunohistochemistry. The majority of The fibrous tissue is vimentin-positive. oma, showing intracytoplasmic hemi-
classic and osteofibrous dysplasia-like The epithelial cells show co-expression desmosomes, tonofilaments, and micro-
adamantinomas display a "zonal" archi- for keratin, EMA and vimentin. Chain- filaments. Irrespective of histological
tecture. In classic adamantinoma, the specific keratin expression {917,1050} subtype, the epithelial cells are bound by
centre is usually dominated by the revealed a predominantly basal epithelial desmosomes and basement membranes
epithelial component, and only few, small cell differentiation, regardless of subtype, have been found to surround the epithe-
immature bone trabeculae are present in with widespread presence of basal lial nests.
Adamantinoma 333
bb5_27.qxd 13.9.2006 13:53 Page 334
Genetics Cytogenetic ana-lysis combined with behaviour {918,1084,1503}. Besides,
Adamantinomas, classic as well as FISH and RT-PCR of two cases formerly male sex {1050,1084}, females at
osteofibrous dysplasia-like, show recur- described as atypical or Ewing-like young age {1503}, pain at presentation
rent numerical chromosomal abnormal- adamantinoma revealed an (11;22) {1084}, short duration of symptoms
ities, mainly gain of chromosomes 7, 8, translocation, typical for Ewing sarco- {918,1084}, young age (<20 years)
12, and 19 {920,1058,1318,2004}. DNA ma {257}. Because of these findings {918}, and lack of squamous differenti-
flow cytometric and image cytometric these tumours were labelled "adaman- ation of the tumour {918, 1084} have
studies showed that in aneuploid tinoma-like Ewing sarcoma". The been associated with increased rates
tumours, the aneuploid population was t(11;22) translocation is not present in of recurrence or metastasis.
always restricted to the epithelial com- adamantinoma {908,1318}. Adamantinomas metastasise in 12-29%
ponent {916}. TP53 gene aberrations  of patients with comparable mortality
as detected immunohistochemically or Prognostic factors rates {918,1084,1503,1739}. Metastatic
by loss of heterozygosity analysis - are Risk factors for recurrence are intrale- tumours are all classic adamantinomas,
restricted to the epithelial component of sional or marginal surgery and extra- although rarely osteofibrous dysplasia-
adamantinoma. There have been some compartmental growth {918,1050,1084, like adamantinomas may metastasise
cases reported with histological fea- 1739}. Recurrence percentages after after recurrence and subsequent pro-
tures of adamantinoma as well as non-radical surgery may rise up to 90% gression to classic adamantinoma
Ewing sarcoma, sometimes called {918,1050,1084}. Recurrence is associ- {918}. The tumour spreads to regional
'atypical' or 'Ewing-like' adamantinoma ated with an increase in epithelium-to- lymph nodes and the lungs, and infre-
{741,1013, 1273,1400,1891,2178}. stroma ratio and more aggressive quently to skeleton, liver, and brain.
N.A. Jambhekar
Metastases involving bone
A. Borges
Definition identify the primary site in up to 85% of Skull base metastasis may cause Collet-
A tumour (usually malignant) involving cases {1812}. Sicard syndrome {1865}; hypercal-
bone, which has originated from another Although metastases are rare in children, caemia may accompany osteolysis
(distant) site. when they occur, they most often include {1520}.
neuroblastoma, rhabdomyosarcoma and Plain radiographs reveal lytic, blastic or
Synonyms clear cell sarcoma of kidney.
Metastatic carcinoma, skeletal deposits,
osseus metastasis, secondaries in bone, Sites of Involvement
bony implants. Metastatic carcinomas involve bones
with persistent red marrow such as verte-
Epidemiology bra, proximal femur ribs, sternum, pelvis,
The skeletal system is the third most skull and shoulder girdle. Out of 114 his-
common site to be involved by metastat- tologically evaluated lesions 44.3%
ic tumour after the lungs and liver {174}. involved axial skeleton, 28.8% the
Metastatic carcinomas are the most com- appendicular skeleton and 26.9%
mon malignant tumour affecting the involved multiple bones {504}. The lum-
skeleton {2154}. Over two-thirds of bar spine {757,1872} and proximal femur
patients with bone metastasis are {757} are favoured sites. Bones of the
between 40-60 years of age {504}. Most hands and feet are rarely involved {923,
metastases originate from common can- 1252,1433,1507,1925}.
cers namely breast, lung, prostate, kid-
ney and thyroid gland which account for Clinical features
93% of all deposits {504}. A complete Pain, swelling, fracture and neurological
Fig. 19.19 Permeative destruction of bone by a
radiographic and clinical search will symptoms (spine) are common {278}.
metastasis (primary tumour unknown).
334 Epithelial tumours
bb5_27.qxd 13.9.2006 13:53 Page 335
A B
Fig. 19.20 A Metastatic renal cell carcinoma showing an alveolar and nesting pattern. B Metastatic prostate carcinoma; note monotonous small cells and irregular
osteoid deposition.
mixed patterns {756}. Lung and breast making it valuable for identifying the tic metastases from the breast are grey-
deposits cause irregular lytic destruction, extent of the disease. CT scan is useful ish white firm, whereas renal cell carci-
but are occasionally osteoblastic {1460, for guiding needle biopsies. MRI has noma produces soft haemorrhagic
1514}. Thyroid and kidney deposits are also been used in some cases to detect deposits.
purely lytic; prostatic deposits are and delineate metastases.
osteoblastic. Solitary metastasis {2120}, Morphology
or an irregular periosteal reaction {1238, Aetiology Metastatic tumours attempt to recapitu-
1581} may simulate a primary bone sar- The location of the primary tumour and late the original tumour. Squamous car-
coma. the local pattern of blood flow determine cinomas from most sites look alike, how-
Plain radiographs are unreliable to detect involvement of skeletal sites. The verte- ever, many adenocarcinomas such as
vertebral deposits {707,1872} and bral venous plexus (Batson s plexus) is a renal cell, prostate and thyroid retain
despite gross evidence of spinal high volume, low pressure, valveless morphological similarities to the primary
deposits in 36% of 832 autopsied venous system independent of the pul- tumour. An accompanying fibroblastic,
patients dying of cancer, 26% had nega- monary, portal and caval systems; it com- vascular, osteoblastic and osteoclastic
tive plain X-rays {1872}. municates directly with veins of the response may be present. Sarcomatoid
Bone scintigraphy is a sensitive method pelvis, proximal half of lower extremity, (spindle cell) carcinomas originating in
for the detection of skeletal metastases, proximal half of upper extremity and head the kidney or the lung may simulate a
because it covers the whole skeleton, and neck {140}. Any increase in intrab- primary bone sarcoma.
dominal or intrathoracic pressure during
exhalation or straining causes a backflow Immunophenotype
into the vertebral plexus bypassing the Immunohistochemistry is useful when
heart and lungs. This explains the prefer- the diagnosis of metastatic carcinoma is
ential involvement of the vertebral and the straightforward but not distinctive
proximal appendicular bones, and the enough to identify the primary site, or,
occassional occurrence of extensive when the differential is broad and
skeletal deposits despite lack of visceral includes sarcoma, carcinoma and
involvement {1470}. melanoma {514}.
Macroscopy Prognostic factors
The macroscopic appearance of skele- Bone metastasis usually heralds incur-
tal metastasis varies depending upon ability and treatment is palliative. The
Fig. 19.21 Metastatic carcinoma. Scattered cytoker-
atin-positive tumour cells confirm the epithelial char- the amount of bone produced in outcome depends upon the primary site
acter of the lesion. response to the tumour. Thus, osteoblas- and the extent of disease.
Bone metastases 335


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