Magnified view of cancer cells.

Sarcomas — a rare form of cancer

Sar­co­mas are a very rare form of can­cer in the field of tumor dise­a­ses. They can appear any­whe­re in the body. In gyneco­lo­gy, sar­co­mas are even rarer, as they form a sub­fa­mi­ly of soft tis­sue sar­co­mas as part of the fema­le geni­tal tract. Sin­ce they initi­al­ly cau­se litt­le pain, sar­co­mas are often dis­missed as harm­less swel­lings. The rari­ty is due to the fact that the trea­ting doc­tor has signi­fi­cant­ly less lite­ra­tu­re and gui­de­lines to fall back on than is the case with com­mon types of can­cer such as breast or lung cancer.

A sar­co­ma is a mali­gnant tumor that deve­lo­ps in soft tis­sue (mus­cle, fat and con­nec­ti­ve tis­sue). The num­ber of new sar­co­ma cases in Ger­ma­ny is esti­ma­ted at around 5,000 per year. The­se most com­mon­ly occur on the extre­mi­ties (arms and legs). Howe­ver, sar­co­mas can also deve­lop in the fema­le geni­tal organs such as the womb (ute­rus), ova­ries (ova­ries), vagi­na (vagi­na) or vul­va. In this case, doc­tors usual­ly refer to this as gyneco­lo­gi­cal sar­co­mas. Com­pared to clas­sic gyneco­lo­gi­cal can­cers such as ova­ri­an or cer­vical can­cer, gyneco­lo­gi­cal sar­co­mas make up only a small pro­por­ti­on of gyneco­lo­gi­cal mali­gnant dise­a­ses. It is esti­ma­ted that only a maxi­mum of 2% of all gyneco­lo­gi­cal can­cers are gyneco­lo­gi­cal sarcomas.

Soft tis­sue sar­co­mas can occur at any age. Howe­ver, they occur more fre­quent­ly in child­hood and ado­le­s­cence as well as in adult­hood bet­ween the ages of 45 and 55. Gyneco­lo­gi­cal sar­co­mas such as B. a lei­o­myosar­co­ma of the ute­rus or a sar­co­ma of the ova­ry, almost never occur in child­hood, but are a dise­a­se of adult women.

Not every gynecologic sarcoma is the same

The­re are dif­fe­rent sub-forms, which dif­fer in their his­to­lo­gic com­po­si­ti­on. The most com­mon gyneco­lo­gi­cal sar­co­ma, at over 50%, is lei­o­myosar­co­ma of the ute­rus, the second most com­mon is endo­me­tri­al stromal sar­co­ma, which also occurs in the ute­rus. The­re are also many rare gyneco­lo­gi­cal forms of sar­co­ma, such as liposar­co­ma or angiosar­co­ma. Alt­hough it is more of a dise­a­se that occurs at an older age, women of any age can be affec­ted by this disease.

Diagnosis: How can a sarcoma be diagnosed?

Most sar­co­mas are dia­gno­sed very late, as they often only cau­se sym­ptoms when the tumor is in an advan­ced stage. For exam­p­le, a sar­co­ma can cau­se pul­mo­na­ry meta­sta­ses, which then rest­rict lung func­tion, i.e. cau­se short­ness of breath. A sym­ptom can also be that pain does not impro­ve over time, but mani­fests its­elf over many weeks and even wor­sens, such as: B. in a sar­co­ma of the ute­rus. In rare cases, aty­pi­cal blee­ding during or after mens­trua­ti­on can also be a first sym­ptom. If the sar­co­ma is loca­ted in the area of the ova­ry, the pati­ent usual­ly only feels sym­ptoms at a very advan­ced stage. It is often pain or an increase in abdo­mi­nal cir­cum­fe­rence that ulti­m­ate­ly prompts the pati­ent to see a doc­tor, who then dia­gno­ses a sar­co­ma. A real ear­ly detec­tion or pre­ven­ti­ve exami­na­ti­on is the­r­e­fo­re not pos­si­ble with sar­co­mas, not even with a smear in the area of ​​the uterus.

Therapy: How are sarcomas treated?

The tre­at­ment of gyneco­lo­gi­cal sar­co­mas is inter­di­sci­pli­na­ry (inclu­ding radio­lo­gy, patho­lo­gy, gynae­co­lo­gy, gene­ral sur­gery). It main­ly depends on the tumor stage, the tis­sue type and the health and phy­si­cal sta­tus of the pati­ent. In an ear­ly tumor stage, sur­gery alo­ne can be the only and suf­fi­ci­ent tre­at­ment. In the case of cer­tain risk fac­tors such as distant meta­sta­ses or tumor resi­dues at the sur­gi­cal mar­gins, che­mo­the­ra­py or radia­ti­on can be part of the so-cal­led mul­ti­mo­dal the­ra­py stra­tegy. In the case of advan­ced tumor stages with distant spread or in the event of recur­rence, medi­ca­ti­on – usual­ly che­mo­the­ra­py or a tar­ge­ted the­ra­py stra­tegy – is also used. The coll­ec­ti­ve term “tar­ge­ted the­ra­py stra­te­gies” sum­ma­ri­zes novel tre­at­ment approa­ches which, as the name sug­gests — in con­trast to the broad effect (“cell divi­si­on poi­son”) of clas­sic che­mo­the­ra­py — are used in a tar­ge­ted man­ner against a sin­gle, spe­ci­fic struc­tu­re. This can be an anti­bo­dy or so-cal­led “small mole­cu­les” that are so small that they can pene­tra­te the sar­co­ma cell and affect cell meta­bo­lism there.

What does the prognosis of my disease depend on?

Unfort­u­na­te­ly, a sar­co­ma is an aggres­si­ve form of tumor with a rather poor pro­gno­sis. Alt­hough many more women with gyneco­lo­gi­cal sar­co­mas are sur­vi­ving today than 30 years ago thanks to signi­fi­cant­ly impro­ved sur­gi­cal tech­ni­ques, litt­le pro­gress has been made in recent deca­des. The rari­ty of the dise­a­se poses a major pro­blem for the fur­ther deve­lo­p­ment of the­ra­py and thus the impro­ve­ment of the pro­gno­sis. If you look at the exam­p­le of breast can­cer, the most com­mon can­cer in women, you can see that the pro­gno­sis has impro­ved mas­si­ve­ly in recent deca­des. This made count­less stu­dies pos­si­ble, in which seve­ral hundred thousand pati­ents took part. As a result of this very inten­si­ve rese­arch acti­vi­ty, num­e­rous new insights were gai­ned. The field of sar­co­mas is only just beginning.

In prin­ci­ple, howe­ver, a cure is also pos­si­ble for mali­gnant tumors. The exact deter­mi­na­ti­on of the sar­co­ma sub­ty­pe is important for the tre­at­ment. This depends on the tumor bio­lo­gi­cal fac­tors, which are descri­bed in par­ti­cu­lar by the tumor stage. An ear­ly-stage tumor has a very high chan­ce of reco­very, while a later-stage tumor with distant meta­sta­ses is alre­a­dy pre­sent,
the chan­ces of reco­very are usual­ly only small. The chan­ces increase signi­fi­cant­ly if the sar­co­ma can be com­ple­te­ly remo­ved by sur­gery (with a bor­der of healt­hy sur­roun­ding tis­sue, sur­ge­ons then speak of a tumor com­part­ment). Howe­ver, such an ope­ra­ti­on is not always pos­si­ble. Even a suc­cessful ope­ra­ti­on does not neces­s­a­ri­ly gua­ran­tee a cure. In any case, sar­co­mas should be trea­ted in spe­cia­li­zed sar­co­ma centers.

The pro­gno­sis is influen­ced by many dif­fe­rent fac­tors. On the one hand, the­re are tumor-typi­cal fac­tors, such as B. the tumor size, the degree of dif­fe­ren­tia­ti­on of the cells and the tis­sue type, but on the other hand gene­ral fac­tors also play a role, such as the phy­si­cal con­di­ti­on of the pati­ent, her age and the func­tion­al sta­tus of her organs (blood count, kid­ney values, kid­ney func­tion, etc.).

Aftercare

After com­ple­ti­on of the the­ra­py, an indi­vi­du­al medi­cal after­ca­re plan should be dis­cus­sed in coope­ra­ti­on with the atten­ding phy­si­ci­an. This is valid for the next five years, in accordance with the gui­de­lines for gyneco­lo­gi­cal onco­lo­gy. Fol­low-up appoint­ments are initi­al­ly held about every three months. Later the exami­na­ti­on
inter­vals beco­me lon­ger. As a rule, the after­ca­re appoint­ments can be made on an out­pa­ti­ent basis with a spe­cia­list or in spe­cia­li­zed cli­nics such as the Cha­ri­té Women’s Cli­nic. The doc­tor in char­ge will con­stant­ly check whe­ther the con­se­quen­ces of the the­ra­py are lea­ding to impairm­ents and whe­ther new can­cer cells have for­med so that they can be trea­ted quick­ly if neces­sa­ry. Here, too, the fol­lo­wing appli­es: If a recur­rence dis­co­ver­ed ear­ly can be com­ple­te­ly remo­ved, the pro­gno­sis is good.

If you have any ques­ti­ons about sar­co­mas, you can also cont­act the can­cer infor­ma­ti­on ser­vice per­so­nal­ly: on the free tele­pho­ne num­ber 0800 — 420 30 40 or by e‑mail to krebsinformationsdienst@dkfz.de.

Sources:

Gesund.bund.de — Sar­ko­me
Ver­läss­li­che Infor­ma­tio­nen für Ihre Gesundheit

Deut­sche Sar­kom­stif­tung — sarkome.de
Infor­ma­ti­ons- und Aus­tausch-Platt­form für Pati­en­ten, Ange­hö­ri­ge, Medi­zi­ner, For­scher und ande­re Ver­tre­ter des Gesundheitssystems

Cha­ri­té — Uni­ver­si­täts­me­di­zin Ber­lin — https://frauenklinik.charite.de/
Sar­kom­er­fah­re­ne Medi­zi­ner und Sarkom-Zentrum

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