Zusammenfassung
Cancer of Unkown Primary (CUP)-Syndrom beschreibt den histologischen Nachweis von
Metastasen ohne das gleichzeitige Auffinden eines Primärtumors. Der Begriff CUP ist
ein Oberbegriff für viele verschiedene Krebsarten. Somit sind Heilungschancen (Prognose)
und Behandlungsart für jeden Patienten anders, je nachdem welche Krebsart bei ihm
tatsächlich vorliegt. Spezielle histologische Untersuchungsmethoden wie z. B. immunhistochemische
Untersuchungen und moderne Röntgendiagnostik, wie z. B. Positron Emission Tomography
(PET) haben die Diagnostik bei Patienten mit CUP-Syndrom verbessert. Der Primärtumor
bleibt dennoch beim größten Teil der Patienten selbst nach Autopsie unbekannt. Eine
kleine Gruppe dieser Patienten weisen eine relativ gute Ansprechrate auf systemische
Chemotherapie in Kombination mit lokoregionäre Therapieverfahren auf. Beim größten
Teil der Patienten kann durch Therapiemaßnahmen keine Lebensverlängerung erreicht
werden. Aus diesem Grund muss sowohl bei der Diagnostik als auch der gewählten Therapie
die Lebensqualität und die zu erwartende Lebenserwartung des Patienten berücksichtigt
werden. Zusammenfassend bedarf die Diagnostik und Behandlung des CUP-Syndroms der
intensiven, interdisziplinären Zusammenarbeit der Inneren Medizin, Chirurgie, Pathologie,
Strahlentherapie, ggf. Urologie, Gynäkologie und Hals-, Nasen-, Ohrenheilkunde. Möglicherweise
kann in der Zukunft durch die Verwendung von molekularbiologischen und immunologischen
Methoden die Diagnostik und Therapie des CUP-Syndroms verbessert und dadurch das Überleben
dieser Patienten verlängert werden.
Abstract
Metastatic Cancer of Unknown Primary Site (CUP) accounts for approximately 3 % of
all malignant neoplasms. In those patients the site of origin for the metastatic disease
cannot be identified at the time of diagnosis. It is now accepted that CUP represents
a heterogeneous group of malignancies that share a unique clinical behaviour and,
presumably, unique biology. Specific histological examinations, i. e. immunohistochemistry
and modern imaging technology, i. e. high-resolution computed tomography, Positron
Emission Tomography (PET) scan, have resulted in some improvements in diagnosis; however,
the primary site remains unknown in most patients, even following autopsy. A small
group of patients with CUP syndrome have been identified, which are responsive to
systemic chemotherapy and/or locoregional treatment. Identification and treatment
of these patients is of paramount importance. For patients who do not fit into any
favourable sub-set treatment strategies should focus on life quality. In summary,
diagnosis and treatment of patients with CUP syndrome require an interdisciplinary
corporation involving disciplines, i. e. internal medicine, surgery, pathology, radiology
etc. In the future novel therapeutic approaches including immunotherapy and molecularbiology-based
methods might improve diagnosis of primary and improve survival in those patients.
Schlüsselwörter
Cancer of Unknown Primary - Diagnostik - Therapie - Metastasen
Key words
Cancer of Unknown Primary - Diagnosis - Therapy - Metastasis
Literatur
- 1
Volkmann R.
Das tiefe branchiogene Halscarcinom.
Zentralbl Chir.
1882;
9
49-51
- 2
Martin H E, Morfit H M, Ehrlich H.
The case for branchiogenic cancer (malignant branchioma).
Ann Surg.
2004;
132
867-887
- 3
Blaszyk H, Hartmann A, Björnsson J.
Cancer of unknown primary clinicopathological correlations.
APMIS.
2003;
111
1089-1094
- 4
Brigden M L, Murray N.
Improving survival in metastatic carcinoma of unknown origin.
Postgrad Med.
1999;
105
63-64
- 5
Daugaard G.
Unknown primary tumors.
Cancer Treat Rev.
1994;
20
119-147
- 6
Muir C.
Cancer of unknown primary site.
Cancer.
1995;
75
353-356
- 7
Silverberg E, Lubera J.
Cancer statistics, 1987.
CA Cancer J Clin.
1987;
37
2-19
- 8
Neumann G.
[Malignant neoplasms with secondary, unspecified or unknown site - significance for
cause of death statistics] Bosartige Neubildungen mit sekundarem, nicht naher oder
nicht bezeichnetem Sitz - ihre Bedeutung für die Todesursachenstatistik.
Öffentl Gesundheitswes.
1988;
50
13-19
- 9
Pavlidis N, Briasoulis E, Hainsworth J, Greco F A.
Diagnostic and therapeutic management of cancer of an unknown primary.
Eur J Cancer.
2003;
39
1990-2005
- 10
Ayoub J P, Hess K R, Abbruzzese M C, Lenzi R, Raber M N, Abbruzzese J L.
Unknown primary tumors metastatic to liver.
J Clin Oncol.
1998;
16
2105-2112
- 11
van der Gaast A, Verwij J, Planting A S, Stoter G, Henzen-Logmans S C.
The value of immunohistochemistry in patients with poorly differentiated adenocarcinomas
and undifferentiated carcinomas of unknown primary.
J Cancer Res Clin Oncol.
1996;
122
181-185
- 12
van der Gaast A, Verweij J, Henzen-Logmans S C, Rodenburg C J, Stoter G.
Carcinoma of unknown primary: identification of a treatable subset?.
Ann Oncol.
1990;
1
119-122
- 13
Gentile P S, Carloss H W, Huang T Y, Yam L T, Lam W K.
Disseminated prostatic carcinoma simulating primary lung cancer. Indications for immunodiagnostic
studies.
Cancer.
1988;
62
711-715
- 14
Wen P Y, Loeffler J S.
Management of brain metastases.
Oncology (Huntingt).
1999;
13
941-961
- 15
Le Chevalier T, Cvitkovic E, Caille P, Harvey J, Contesso G, Spielmann M, Rouesse J.
Early metastatic cancer of unknown primary origin at presentation. A clinical study
of 302 consecutive autopsied patients.
Arch Intern Med.
1988;
148
2035-2039
- 16
Karsell P R, Sheedy P F, O'Connell M J.
Computed tomography in search of cancer of unknown origin.
JAMA.
1982;
248
340-343
- 17
Baron P L, Moore M P, Kinne D W, Candela F C, Osborne M P, Petrek J A.
Occult breast cancer presenting with axillary metastases. Updated management.
Arch Surg.
1990;
125
210-214
- 18
Orel S G, Weinstein S P, Schnall M D, Reynolds C A, Schuchter L M, Fraker D L, Solin L J.
Breast MR imaging in patients with axillary node metastases and unknown primary malignancy.
Radiology.
1999;
212
543-549
- 19
Alberini J L, Belhocine T, Hustinx R, Daenen F, Rigo P.
Whole-body positron emission tomography using fluorodeoxyglucose in patients with
metastases of unknown primary tumours (CUP syndrome).
Nucl Med Commun.
2003;
24
1081-1086
- 20
Mantaka P, Baum R P, Hertel A, Adams S, Niessen A, Sengupta S, Hor G.
PET with 2-[F-18]-fluoro-2-deoxy-D-glucose (FDG) in patients with cancer of unknown
primary (CUP): influence on patients' diagnostic and therapeutic management.
Cancer Biother Radiopharm.
2003;
18
47-58
- 21
Rades D, Kuhnel G, Wildfang I, Borner A R, Schmoll H J, Knapp W.
Localised disease in cancer of unknown primary (CUP): the value of positron emission
tomography (PET) for individual therapeutic management.
Ann Oncol.
2001;
12
1605-1609
- 22
Jereczek-Fossa B A, Jassem J, Orecchia R.
Cervical lymph node metastases of squamous cell carcinoma from an unknown primary.
Cancer Treat Rev.
2004;
30
153-164
- 23
Milovic M, Popov I, Jelic S.
Tumor markers in metastatic disease from cancer of unknown primary origin.
Med Sci Monit.
2002;
8
MT25-MT30
- 24
Pasterz R, Savaraj N, Burgess M.
Prognostic factors in metastatic carcinoma of unknown primary.
J Clin Oncol.
1986;
4
1652-1657
- 25
Greco F A, Gray J, Burris H A, Erland J B, Morrissey L H, Hainsworth J D.
Taxane-based chemotherapy for patients with carcinoma of unknown primary site.
Cancer J.
2001;
7
203-212
- 26
Culine S, Ychou M, Fabbro M, Romieu G, Cupissol D.
5-fluorouracil and leucovorin as second-line chemotherapy in carcinomas of unknown
primary site.
Anticancer Res.
2001;
21
1455-1457
- 27
Culine S, Fabbro M, Ychou M, Romieu G, Cupissol D, Pujol H.
Chemotherapy in carcinomas of unknown primary site: a high-dose intensity policy.
Ann Oncol.
1999;
10
569-575
- 28
Ellerbroek N, Holmes F, Singletary E, Evans H, Oswald M, McNeese M.
Treatment of patients with isolated axillary nodal metastases from an occult primary
carcinoma consistent with breast origin.
Cancer.
1990;
66
1461-1467
- 29
Whillis D, Brown P W, Rodger A.
Adenocarcinoma from an unknown primary presenting in women with an axillary mass.
Clin Oncol (R Coll Radiol).
1990;
2
189-192
- 30
Nguyen C, Shenouda G, Black M J, Vuong T, Donath D, Yassa M.
Metastatic squamous cell carcinoma to cervical lymph nodes from unknown primary mucosal
sites.
Head Neck.
1994;
16
58-63
- 31
Hainsworth J D, Greco F A.
Treatment of patients with cancer of an unknown primary site.
N Engl J Med.
1993;
329
257-263
- 32
Motzer R J, Rodriguez E, Reuter V E, Bosl G J, Mazumdar M, Chaganti R S.
Molecular and cytogenetic studies in the diagnosis of patients with poorly differentiated
carcinomas of unknown primary site.
J Clin Oncol.
1995;
13
274-282
- 33
Pavlidis N, Kosmidis P, Skarlos D, Briassoulis E, Beer M, Theoharis D, Bafaloukos D,
Maraveyas A, Fountzilas G.
Subsets of tumors responsive to cisplatin or carboplatin combinations in patients
with carcinoma of unknown primary site. A Hellenic Cooperative Oncology Group Study.
Ann Oncol.
1992;
3
631-634
- 34
Kuttesch J F, Parham D M, Kaste S C, Rao B N, Douglass E C, Pratt C B.
Embryonal malignancies of unknown primary origin in children.
Cancer.
1995;
75
115-121
- 35
Sporn J R, Greenberg B R.
Empirical chemotherapy for adenocarcinoma of unknown primary tumor site.
Semin Oncol.
1993;
20
261-267
Prof. Dr. med. K. W. Jauch
Chirurgische Klinik und Poliklinik · Klinikum Großhadern · Ludwig-Maximilians Universität
Marchioninistr. 15
81377 München
Phone: 0 89-70 95-27 90
Fax: 0 89-70 95-56 74
Email: Karl-Walter.Jauch@med.uni-muenchen.de