Case report 656

(1)

S k e l d Radio1 (1991) Ml: 137-140

Case report 656

AWn A. G d e r , M.D.',DeoisM. Regent,M.DmZ,Pierre

M.

Gûiet,M.D.l,PatriceG. Pwe,M.D.', Bernadette M. Aymard,

m.:

^{a d}Véronique Clement,

MD.'

Departmenta of Rhewtology, Rrtdiology, and Pathology, CBRU de Nancy, Hbpitd de Brabois, Vandoeuvre les N w : France

A 55-year-old man complained of progreasively worsening pain in the left ankle. The patient had a history of g e n e r a w psoriasis which had been treated 5 years before by exten- sive local eorticotherapy. He was a hoavy smokrr and iuffered from a severe armiopsthy. Alcohol abuse was also noted. without mncreatitis.

Physid examkation revealed ten- derness in the distal portion of the left leg. Laboratory findings and

. : blood studies were within normal

Mg. 1. A Anteroptcrior and B lateral v i m of the leR Iïbia show a metaphpeal, ilideiïneû, osteoly lie area with c o d a i erosion and srnaII, linear, perimteal reactioa. Axial CT scans of the distal and of the tibia (C goFt tissuc window seiiing and 1) bone window), 1-nmi section, demonstrate an ostoolytic lesion croding cortical h n e ; no soh tissue extension was deteded

Adrfress r e p h t r e w s l s tu: Pr Alain Gaucher, Clinique Rhumatologique, URACNRS 1288,

CHRU de Nancy, H&pital de Brabois, route de NeuCchâteau, F-54511 Vandomm ie Nancy, France

limits,

u i o g r a p h s of the left ankle, in- cluding tomographs, demonstrated a lytic, poorly marginated, polycyclic area in the distal tibial metaphysis (Fig. 1 A,

B).

Periosteal reaction and endosteal erosion with posterior cortical bone destruction were pres- ent. No soft tissue m s s was detected.

In other metapbyseal regions of both tibiae, there were sharply demarcated zones of inmeased densi ty suggesting multiple meduilary bone i n f m (BIS). Furthemore, frontal raentgen- ogramç ^ofthe right hip showed ne- crosis of the fernorat head, clinically latent.

A g9mTc-MDP bone scan demon- strated increased uptake in the distal end of the left tibia. The scan was otherwise normal. CT scana OP the left tibial metaphysis demonstrated a

ly tic tumor eroding the postenor cor- ticla, having its origin in a centro- osseous calcif~d a r w (Fig. 1 C).

(2)

A.A. Gaucher et al. : Case rcpart 456

Magnetic resonance imaging (MW) demonstrated in the four metaphyses of both tibiae and in the distal right femur serpiginous areas of low signal intensity on

Tl-

and T2-weighted im-

ages (Fig. 2) characteristic of _mature bone marïow infarction. Apart from these lesions, MRI sbowd in the dis-

ta1 third of the lefl tibia cortical ^ero- si on, suggesting malignant degenera- tion of one of the Bh (Fig. 3a). No

soft tissue mas was c i e t m .

An

open biopsy conformai corti- cd destruction by a soît tumor mass transgressing the perios teum. Micro-

s q i c a l examination reveald multi-

-

ple sarcomatons cells, growing in a

storiform pattern

(Fig.

4). Numerom focally necrotic zones, dystroghic calcifications, and nonviable larnellar bone trabeculae were present in _the tumor.

The patient underwent an _under- t h e k n a amputation. On gross ex- runination, the metaphyseal curtical bone was trangressd.

In

a sagittally cut section there was a yeilowish area

in the medulltiry ^cavitycorrespond- ing mcroscopically to infarcted bone. Around the a m O € the infarc- tion, a gray son tissue mass was pras- eut, destroying the posterior cortical bone and periosteum (Fig. 3B. C).

Microscopicdly, this sarcomatous tumor developed a triphasic pattern of ceIls: spindle *Ils with foamy cy-

toplasm, phmp celis with histiocytic appearance, and giant cells. The

spindle a l 1 wmponent was largely prcdominant and arianged in a stori-

form pattern. In some areas, numer- ous capillaries were present, suggest- ing an hemangiopericytorna-1ikepat- tern. Electran-microscopic evnlua- tion coniirmed the pre%ence of fibro- blastic, myofibrobiastic, and msilig-

nant histiocytelike cells. The lesion

m

was diagnoseci as a malignmt fibrous histiocytoma amund a bone infarct, withuut metastasis. No adjuvant treatment was prescritmi.

1

^Hg.^of^Lhe^2,^nght^Coronal^kna^MRIderoom1rathg low signal ^(TR:^$60^rns/TE:²⁶^ms) intensity areas in tibia1 and fcmoral metaphyses with a thin, w d l ~ n e d , low signal border

&tmt with two of the multiple mature borie marrow infarcts

FIg. 3. A Sadttal M M (TR: 480 msFE : 26 ms) o f the distal end of the left tibia shows a lypical image of a boae infarct surrounded by a fumoroubl Mon postaior ~ r i i a l bonc and t r a n m n g the pcriosteum (arrow).

8 Sagittally cut surface of the t h e i d pari of the left tibia demonstmtcs a c ~ n i d , ye1lowish Iibial infarct (star) surrounded by n tumor d i n g the cortat ( m w h h ) . C Contact mdiograph showing sndoiteal oateolysis and cortical dimption

Fig. 4. Phatomicrograph from tumor showing a moriform tirœ, witk niaügnant cells and Jigùf hyalinid stroma fianatoxy1in-eosin saffron,

K 801

Aith- malignant fibrous bistiocy- t o m i g well known amom the tumots of the somatic soft

tis-

(3)

A.A. Gaucher et al. : Case report 6%

s u s , it has a h recently k e n ^{m g -} nized as a primary tumor

in

bone [BI.

MFH of bone is rare (1 %-5% of psi- mary bone turnors) and sometimes develops in association witb pre-ex- isting bone abnormali ties (20%-25%

of MFHs), like bone infmt (BI) [14].

However, MFH arising within or as- socîated with skeletal infarction is ex- mptional, since only 32 cases (re- viewed in [4, 71) have been tabdated in the literature. Analysis of the re- ported cases reveals that such pa- tients had frequently large, multiple, metaphysodiaphyseal medullary in- fards OC the long bones. These BJs

were related to decompmssion sick- ness [IS], vasculitis 1131, alcohol abuse [7], sickle ce11 anemia

[a,

151, or steroid therapy [2,

1q. ^As

^{in the}

present case, these conditions have more o k n been associated with aseptic necrosis of the epiphyses, whch never degenerate. A Iong inter- vd between the occurrence of BI and MFH diaposis is frequently ob- served.

Our patient had abused atcohol and received seeroid therapy through extensive topical steroids for his gen- eralizcd psoriasis. Tn addition, our

casc documents the existence of a previous BI present for more than 5 years before the MHF was diag- nosed. An earlier arteriagram (1983) had clearly demonstrated jts pres- ence.

Typically, MFH associated with BI [l] occurs in a preponderence of men over 40 years of age. It involves almost exclusively the femur and the tibia. Pain and swelling are the main complaints.

RadiologicaUy, MFH is &arac- tcrized by a destnictive. purely osteo- lytic, illdefined, m~taphysodiaphy- seal area. The cortex is frequently destroyed. Periosteal reaction and

son tissue mass are often noticed.

Radiologîcal studies also demon- strate associated mature BIS charac- terized by large, serpiginous, medul- iary calcifications [5].

CT shows the ostmlytic tumor and its relationship with the centro-

osseous dcifications of bone mar- row infardon. Furthmore, endos- teal erosion ^andsoft tissue mass are a s i l y detectable. Operative planning requires knowledge of the local ex- tent of tbe tumor, and Cï is com-

monly required to 10-te it precisely.

9 9 T e M D P and citrate sctuis

demonstrate intcnse accumulation of the radiophamceuticais- in the tu- mor [i2], and the semainder of the scan can be used to search for possi- ble osseous metastases. T h e usefut- ness of angiography [9] has been re- ported in planning Ibe operative treatment.

MRI is a sensitive examination in the detection of avascular necrosis of the femorai head [3,13] and the knee [19]. T y p i d y , a low signal a m is present on T l - and T2-weighted im- ages. in some patients, the margins of the lesion become hypcrintense on long pulse squences, showing an in- flammatory tissue at the reactive in- letrace. These MR features are sjmi- lar for meduilary bone infarction [18]. Tbus, serpiginous rnetaphy so- diaphyseal zones of low signal inten- sity are detected in Ti-weighted im- ages, as well as similarly unenhanced signal in the TZweighted sequences.

A thin band of low signal delimits the mcirgins of the BI [2,1.6]. In some cases, the central portion is isointense with the surrounding normal bone marrow, indicating fatty areas.

In addition, our report suggests the ability of MRI to discriminate malignant sarcomatous transformation of medullary BIS by demonstrat- ing diaphyseal cortical erosion, Fur- thermore, MRJ delimits the tumor mass and de~ermines the presence or absence of soft tissue invasion. De- spite its classic lack of specificity, contrasting with its high sensitivity in detecting skeletal tumors, the asso-

ciation of medullary infarction with malignant transformation allows prediction of the prcscnce of an MFH.

Ris tologically

,

the following characteris tics typify MFH (141 : mononuclcar ovoid to spindly his tio-

cytic cella growing in a storiform pal- tern (characteristic but not pathog- nomonic of

MFH),

ana)lastic multi- nucleate giant cells, evidenœ or pha- gocytosis, heavy reticulin prodw- tion, and fat production.

MFH

of bones exhibits a

wide

range of histo- l o g i d patterns. With this in mind, Huvos et al. [IO] have subdivided MFH into three variants according to the histological appearimnce of growth: the prcdominantly Cibro-

blagtic pattern, the predominantly histiocytic or xanthomatous mor- phologic variant pattern, and the mdignant giant ditumor type. In our case the tumor had a predomi- nantly fibrobiastic pattern.

BI are chw&sed by dense bland fibrosis, dystrophie calcifica- tions, and foci of nonviable lamellar to woven bone. Around the BI, a chronic reparative process d d o p s at the revascularizing margins, and the proliferation of histiocytes is an essential component of this proœss, It has been suggested [15] that, iifter many years, in some instances, the histiocytic component may undergo sarcoma tous Iransformalion. Thus, the tumoral histiocyte a n differenti- ate along two major directions: to- wards phagocytic functions or fibro- genic functions. In the present case, the localization of the tumor around the BI reinforces this chronic repsra- tive theory for the malignant truns- formation of BIS [13].

In summary, a case is presented of a 55-year-old man with a MFH in a previous BI. The poor prognosis of such secondary MFH is related in part to the difficulty of its early diag- tiosis, its aggressive behavior, and its tendency to inetastasize (mainly to bone sites and lungs). Nevertheless, al though biopsy of asympioinalic bone marrow infurction is not indi- cated, MRI seemv consistent in de- tecting sarcomatous transformation of symptomatic BIS and plays an im- portant role in the patient's preoper- ative evatuation.

Acknowledgemcnts. WC wish tu h n k Mw.

J. ChmpigneulIe, M.D. (Laboratoire d'H- istologie) for hcr contribution in ultrastruc-

tuml study and Mr. P.M. Walker, Ph.D., Tor editing Ihe manuscript.

1. Abrahams TG, Hull M (1986) Cage re-

port 394. Skeletal Radiol 15: 578 2. Archer AG, Nelson MC, Abbondamo

SL, Bogumill OP (1989) C e report 554. Skektal R d i o l i 8 : 380

3. Betlran 6, Herrnan U, Burk JM, Z&

WA, Clark RN, Luuis JG, Weissl LD,

Yang A (1988) Femoral head i v a s ~ u l i r nerosis: M R imaging with clinicrii pathologie d radionuclide oomlrithn.

Radioiogy 146 : 21 5

4. Breen TF, Healy WL (1987} Mali&nant fibrous hiitiocytoma ariaing in medul- lary bone ~~: a case report. Orlho- pedics 10:1169

(4)

A.A. Gaucher et al. : Case report 656

5. Davies ^AM,Evans N, Grimer RJ (1989) C u of the month. Br J Kadiol62: 1.83 6. Fctdman F, _NormanD (1 972) Intra- and

exira- mafignant histioqioma.

Radiology 104 :497

7. Frieraion HF, Fechner EF, Stahg RD,

W~QJ GJ (1987) Malignant fibrous histioqtomn in b w t infarct: d a -

tion with s i d e & trait and alcohol abuse. 59 : 4%

8. Furey JG, Ferrer-Tm1 M, b g w JW (1960) Rbrosarcoma arising i t the site of bone infmt: a report of tw cases.

J Bone Joint Surg [Am] 42 : BO2 9. Hudson TM. Hawkins lF, Spanier ^SS.

h m k h g W F (1979) Angiography of malignant fibrous histioqtoma of bone.

Diagn Riidiol131:9

10. Hwm AG, Heilweil M, B M i SS

(1985) The pathology of malignant fi- brous histbqîoma of bone. A study of

130 patients. Am Surg h t h o l 9 : 8 5 3

i l . Lang P, Jergesea HE. Moseley ME,

Block JE, Chaîetz NI. Gsnmt HK (1988) Avasdar m s i s of the fernord h d : high fdd strtogth imaging wiîh histologie carrelation. Radiology 169:S17

12. Mackey JK, Abeva-Jackson B (1987) B w e and gallium scan findhgs in m l i g - nant fibrous histimytoma Case rtgort with radiographie and p~thologic corre- iatim. Ciin Nwl M d 12: 17

13. Michael RH, Dorfrnan HD (1 976) malignant fibrous histiocy tom aasociat- cd with bone infarcts. Clin Orthog i18:lSO

14. M i m JM (1 980) Bone tumors : diagno- sis and ireatment. Malignant fibrous histiocytoma in association witb bone idarc~s. Lippincott, Philadelpbia, PP 18%

15. Mirra JM, Oold RH, Marafiote R (1977) Malignant histimytoma arising in

d t i o n with a b n t infarct in slickle- ceii dissase: coincidence or cituse-and- effect? Canwr 39: 186

36. Munk PL, Hems CA, Holt

Ra

(1984) Irnmalurc b w e inïarcts: findings on plain radiographs and MR scrins. AJR

152:547

17. Ph8 P, Adolphe I, Deigoffc C, Froment N, Gaucher A (1 984) Ostkonécroses épi- physaircs, infarctus osseux multiples e l

histiocytomc fibreux. Rev Rhum Mal Osteoiirtic 5 1 : 427

18. PCrb P, Régent D, Vivard T, Gillet P, Gaucher A (1988) Imagerie par rése nance magnetique nucléaire dm infare

tus osseux. A propos de 2 obscrvatioius.

J Radio1 69: 597

19. Pollack MS, _DalinkaMK, K-1 HY, h t k c PA, Spritzer CE (1987) b g w l i c rcsonanoe irnaging in the evaluatiw of nuspied osteweaosis of the knee.

Skeleial W 0 1 1 6 : 121

Case report 656

Case report 656

M.

m.:

MD.'

B).

Tl-

An

-

(Fig.

In

m

1

tis-

in

[a,

1q. As

,

MFH),

MFH

wide

Ra

1q. ^As