MULTHPLE PRIMARY LIPOSARCOMAS *

IAUm-V. ACx3MAN, MD.(From th Depwtmeu of Patkodogy, EW ish Stat Caxcff Hospita, Columia Mo.)

Adipoe tissue should be ndered as an organ subject to its owndiseases. Wells' stated it ver well: "Adipose tissue is not merely acommon connective tissue ldedwith stored simpe fats but, to alarge extent, s c a, deveopmentally and f nally an inde-pendent speial tissue more after the order of the true ductles gl."We see systemicdisease such as von Reckin 's neurofibro-matosis, lympsar , plasma cell myloma, and others fairly fre-quently. It seems reasonable, therefore, that fat tissue, behaving as asingle organ, could be involved by a lignant process.Trel sa are rare,'nlyjr2 and the occurrence of mileprim yliposarcomasis practically unknown in,th cntry. The

European literature, however, offers four art on this subject whichwM be reviewed later.L can occur in any area in whichfat is normally foumd, but arise most frequently in the popliteal spaces,the areas about the buttocks, and in the retrperitoneal areas. Theyoften appear enal , but it is probable that this e ain isfalse, as recurrences after su y areThe following case dem enstrates the appearlof multiple

maigant fatty tumos which were l d in the adie tissue, grewwith varymg speds, recurred m some areas, and finally caused deathafter io years. RamoiOF si

F. MK (E. F. S. C H. no. 328i), a white male, 54 years old, was amitted tothe sital on Sept r IS, zg4z. In I933, a min the right inferior guteafold had been noted, and in 938 it was removed at the Bell Moial Ho ital nKansas City. It sheEled out easiy. One and one-alf years before admissio to thishospital, the patient fell, striking his left hip, and 6 months later a hard, pinlswl above the left hip bone was felt. lhis grew slwiy. For I year to

there ad b pm on the outer surface of the left tih raAting downto the foot Four months previous to admission, he noticed a l inpi the rightpoiteal space. Weakness of the kgs had been marked for 2 week Phycal_exminati0n Levead a well wte male wo was quite we, p into lie in bed. On exio of the en, re ws a large, smooth, firmmass, felt in the right lower qant just th iIliac crest In the left lowerq_ ant, tr was a simil but sma mass. In the soft tissues of the ack,just above the Iliac crest an the left, there was a smooth, round, devated tumormassm about 2o m. in diameter and elevat about 6 cma above the sur-irond skin (Fig. I). It was nt ulcerated or attached to the oven skinThere wer also several small nevi and one cafJa--lat spot The extremities showedsigs of wasting and the tendon reflexes were absent These cages wer though

* Reeived for tipst pot,September 2 xg943.

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790 ~~ACKERMN

to be due to p on nerves by the large a inal masses. Just beneath theright piteal fossa in the soft tiWsse, there was a smooth, round, movable massmeasuring about 8 a in di . koentgenograms following a barim eashowed acement of the cecum, p asc colon and tminal ileum byan iC, hoefaction of the lower quadrant.On October 3, 194i, the tumor in the popliteal space was excised; on October

31st the mass on the back was rcted; on December 6th- the a inal mass wasincompletely resected. Tle nt was di a from the hospital aon Decem-ber H4th.He was dittedon February 4, 1942, becas of recurrece of thetumor in the itonel space. This was given intnie x-ray therpy760o r. over four lae fidds, but the tumor ed only slightly; He was dis-cgd fr the hospital onApil 7th. He ned on Je 6th, much worse. Inthe right adavicular fossa, there was a hard, movable mass about m. in diam-eter, wich was oved. After this, he returned to a county home, gadull oststrength, and died on-November 23, 1942.

AU of the surgically excised tumors had many similar caracteristics.TheY varied in siz frm 75 gin. (popliteal space) to 1200 g. (retro-peritoneal space). They were all in the soft tissue and led outeasily. Their ernal surfac were lobulated, and showed numerousconvolutions. In the grooves formed by these convolutions ran a fine*network of small blood vessels. On section, they cut with varyigdegrees of resistance depending on the amount of connective tissuepresent. The first one removed cut the easiest and looked very muchlike brain tissue. Itsa confonned to Shaiws descrIption oflposarcoma. All of the spemsshowed varyig degrees of hem-orhage and n ris lative to the cllularity and vascular suply.

AutopsyAnatomic diagnoses. Multiple primary liparcomas, arisng from

the subepicardial, ianal, mesenterc, peritoneal, retroperitoneal,subcutaneous, and bone marrow fat; metastatic liaroma of theliver.The body was e ed and showed numerous well healed surgicl

scars. There was a residual, soft tissue tumor of the right tih meas-uring about Io by 8 by 4 cm., and anoher in the soft tissue of theright popliteal space. At the initil icision, tumor was found in thescd and third costal tes s, apparently prolongations of atuor arisig from the metium In the p cavity, thesmall intestines were adherent to the lower half of the abdominal wall.The liver was rotated couter-dockwise about go degrees, so that thenormal supertor aspect was lying on the right side of the body. As awhole, the liver was lying in the left upper quadrant, disaced androtated by a large mass of neoplastic tissue occupying the normal liverspac. On the surface of the liver were several sharply dlin ,yelowish white nodules measuring from I to 6 am. in diameter. On

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MULTIPLE PRIMARY LIPOSARCOMS

section they were -soft, crcular and projected ilyabove the cutsurface. Tumor lety f the right iiac fossa and right lumbargutter and was covered by the iiopsoas muscle. In the left lowerquadrant, a similar mass of firm, grayish yellow astic tissue in-completly filled the left iliac fossa. Lymph nodes were apparentlyreplaed by tumor and measured p to 2 cm. in diamt er. The rightpltural cavity contained a large mass of soft, friable astic tissuewhich filled one-half to two-thirds of the space, compresed the lung,pushed its way through the intercostal spaces, and caused deviation ofthe mediasinum to the left. The left pleural cavity was free fromtumor. The heart weighed 300 gmi and its subepicardial fat wasmarkedly reduced in amount. At the apex, questionably arig fromthe subepiardial fat, there was an elevated tumor nodule mesuring3 cam in diameter whic had invaded the myocardium. The right lungshowed marked compression by tumor, but there was no definite tumorwithin the lung para chyma. The left lung was normal. The otherorgans shwed no gross evidence of tumor.

Microscopic Exaatio. The fat trom the pleura, pericardium,mesentery, peritoneum,'popliteal space and meiiain showed nu-merous tumor nodules. The cels varied i y m size and shape,had pale blue vesiaclar nudei, and fine ucudeoli. In many instanesthe nudei had been compressed to a crescentic outline by the largamounts of fat present. Other tumor cells showed oval, homogeneousnuclei with dear, pale cytoplasm and well defined ullar outlines.Sudanm stain showed large amunts of fat which was almost entirelycytoplasmic. Best's stain for glycog demonstrated largeamounts of cytoplasmic glycogen within practically all tumor cells.In the cells where globules of fat had compressed the cytoplasm, thiscompressed area was staied positively for gycogen. The tumor inthe liver was dearly demarcated, had an apparent connective tissuecasule, and was similar to tumor observed elsewhere. Te tumorthought to represent lymph node replacement was shown to be merelynodules of tumor of the same size as lymph nodes. Connective tissuestroma was extremely variable, and numerous areas of necrosis wereobserved. Thrombosis of the small vessels was widespread.

COMMENT

This patient had numerous fatty, soft-tissue tumors arising in areaswhere fat is normally present. Tumor was found in the three mostcomlmon sites: the popliteal spaces, buttocks and retroperitonealspaces, as well as other and rarer locations. There was recurrence oftumor growth after excision in both the popliteal space and the thigh.

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792 ACKEMAN

This type of liar grows r slowly. Tlere are numerousPles of soltary rted in the litte wih short

or no follow-up histories. It is ible that some of those patients mayevehtually develep otber tumrs.

lmere are four cases in the foreign literature which mustrat theoccurrn of mule primary fatty tus. In 1925, Fahr and Lu-ba3 described a case entitled "A Metas ng ipoma" in whichthere were multipe tumors, aarly of fatty origm, present aroundthe kidney, in the tlregion, in the mesentery, serosa,posterior meiastinm, the apex of the heart, the liver and the bonemarrow. T'ne authors thoght that the oinal tumor arose from thefat around the kidney and metastasized to the other siteS. Nihhuis,4too, rpted a case of multiple fatt tumors invo g mesentery,retreritonea tissues, pancreas, extradural tissues and thoracic verte-brae. lhese tm rs e of at least 2 years' duration and Nienhuisconclu at ody the bral and ral lesions were meta-

the otbers p imay malinant fatty turs. Hestated the se pted by Fahr and Lubarscpresented the samepicture, rather than a single p y h th metastases. In1934, Siegmud r ted a beautiful example of mutiple prmarymalinan fatty tumors ozurrig in a ma of 65 in whom the firsttumor was noted in the soft tissue of the thigh At autopsy, therewere meus indiviual fatty tu s arising withi the pleuralcavity, ometum, mesent, seroa of small and lare tei, alongthe aorta, suroding both ineys, in the soft tissues of the bac,igh and upper arms, the subepicardl fat, and in the bone marrow.He cuded that is was a ystem m ant disase of fatty tissueand should be d d as a 'i Sark e." He em-phasized that a alle might be n between this and othersystemic diseases such as urofib sisa lymphosarcoa. Healso i dtly m to the that Fahr and Lubarsch'scase represented multiple tuors. G matigh, in his ex-t ve reve of mdlignant fatty tuos 1936, mmented on Sieg-mund's case and reported an onal case of a 59-year-old womanwhose first tur was located in the left hal of the ab Radio-

was of no value. At auty, nrous individual mnaignntfatty tuos located in the soft tissue of the thigh, the base of theheart, in the gastroolic, gstr and s ligament of thelier, were foumd. Tnere were nmerous other tumors ariig fromthe fat of the serosa of the and lare ite , omentum andmesentery, making a total of 2 19 individual tumor masses. Tere was

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PRIMARY LIPOSAXCOMAS 793

no evidence of lymph node involvement The bns, unfortunately,were not em hnTese four wed ted cases and the case here red -

resent muIe primary manant fatty tmors and give furthlr sup-put to the foi at adi se shuld be c as asingle organ subject to its own diseases

REFERENCEi. WeIs, H. G. Adio , anled sect. J. A. M. A., I940, 114, 2177-

2183.2. Ackman, L. V., anmd Wheeler, P. omna S0th. M. 1., 1942, 3S, IS6-

I5s.3. Fahr, T., and 4h 0. In: F., and . andbuc der

spesmellen pathcloguschen Aatmie u lmdgic J. SriNr, Bedi, 1925,6,pt I, 603-6o6.

4. Nienhuis J. H] En p sisches Saxkm mit tase Zscr. f. Krebs-forsCh., 1925, 22, 435-445-

5. Siegmu, HE i astische Sakoratose. Virchows Arch. f. path. Axat.,.1934, 293, 458-463.

6. G m N., Vandednd, P., and de Puygeleyr, R. Lip1m et lipo-cytoses (hpoblastmmet p stses). Contriution i r'&de des lipor-

prOfosBds. Cucr B 1'udk,I93637, 1, 3-48.7. Shaw, B. F. A. Embryonal ce lipO . J. Path. & Bact., I936, 43, 277-

283.

[mus&ato foamo]

DES~RIPT1ON OF PLATS

PLATE 148Fun. I. Proie viw of soft-tissue of tbeck7M. 2. External UEmface iof nt-etuma tw, _ huatias.

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Ackerman Multiple Pimary Lipoacomas

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PLATT. 148

PLATE 149

Fi- 3. Cut section of r pritneal tumor.

Fi;. 4. Characteristic of one of the soft-tissue l s . X 303.

Fit S. Tumor liver parenchyma. X 97.

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S. 61 ilo II I 3 1'4 1 5 1:6 1I7

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PLX 149