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Peritonealkarzinose beim CUP‑Syndrom

Diagnostik und Indikationsstellung für die multimodale Therapie

Peritoneal carcinomatosis secondary to CUP syndrome

Diagnosis and indications for multimodal treatment

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Zusammenfassung

Klinisches Problem

Die Peritonealkarzinose als Folge eines CUP-Syndroms („cancer of unknown primary syndrome“) ist eine seltene Entität, für die es keine einheitlichen Therapieempfehlungen oder Leitlinien gibt. Die mediane Überlebenszeit liegt bei 3 Monaten.

Diagnostik

Die Computertomographie (CT), die Magnetresonanztomographie (MRT) und die 18F‑FDG-Positionen-Emissions-Tomographie (PET)/CT sind valide Untersuchungsmodalitäten für den Nachweis einer Peritonealkarzinose. Die Sensitivität aller Verfahren ist am höchsten für große, makronoduläre Manifestationen. Eine Limitation aller bildgebenden Verfahren besteht bei limitierter und kleinknotiger Peritonealkarzinose. Auch die Darstellung von flächig ausgebreiteter peritonealer Metastasierung im Bereich des Dünndarmmesenteriums oder der Zwerchfellkuppeln lässt sich nur mit niedriger Sensitivität darstellen. Deswegen sollte in diesen Fällen eine explorative Laparoskopie als nächster diagnostischer Schritt erwogen werden. So kann bei etwa der Hälfte der PatientInnen eine unnötige Laparotomie vermieden werden, falls bei der Laparoskopie ein diffuser, kleinknotiger Befall der Dünndarmwand und somit ein irresektabler Befund festgestellt wurde.

Therapie

Bei ausgewählten PatientInnen stellt die Durchführung einer kompletten Zytoreduktion mit anschließender hyperthermer intraabdomineller Chemotherapie (HIPEC) eine gute Therapieoption dar. Für das Festlegen der immer komplexer werdenden onkologischen Therapiestrategien ist die möglichst genaue Identifikation des Ausmaßes einer peritonealen Tumormanifestation daher von enormer Wichtigkeit.

Abstract

Clinical problem

Peritoneal carcinomatosis secondary to cancer of unknown primary (CUP) syndrome is a rare entity for which there are no uniform treatment recommendations or guidelines. The median survival time is 3 months.

Diagnosis

Computed tomography (CT), magnetic resonance imaging (MRI), and 18F‑FDG positron emission tomography (PET)/CT are valid imaging modalities for the detection of peritoneal carcinomatosis. The sensitivity of all techniques is highest for large, macronodular peritoneal carcinomatosis manifestations. A limitation of all imaging techniques is limited and small-nodular peritoneal carcinomatosis. Also, peritoneal metastasis in the small bowel mesentery or diaphragmatic domes can only be visualized with low sensitivity. Therefore, exploratory laparoscopy should be considered as the next diagnostic step. In half of these cases an unnecessary laparotomy can be avoided, because the laparoscopy revealed diffuse, small-nodule involvement of the small bowel wall and thus an irresectable situation.

Treatment

In selected patients, performing complete cytoreduction followed by hyperthermic intra-abdominal chemotherapy (HIPEC) is a good therapeutic option. Therefore, the identification of the extent of peritoneal tumor manifestation as accurately as possible is important for the definition of the increasingly complex oncological therapy strategies.

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Abbreviations

CT:

Computertomographie

CUP:

Cancer of unknown primary

CRS:

Zytoreduktive Chirurgie

DWI:

Diffusion-Weighted Imaging

18F‑FDG:

18F‑Fluorodeoxyglukose

HIPEC:

Hypertherme intraperitoneale Chemotherapie

MRT:

Magnetresonanztomographie

PCI:

Peritoneal Cancer Index

PM:

Peritoneale Metastasierung

PET:

Positronen-Emissions-Tomographie

SPPC:

Seröses papilläres Peritonealkarzinom

Literatur

  1. Acs M, Dadras A, Blaj S, Leebmann H, Piso P (2022) Selection criteria for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy with special emphasis on laparoscopy as an efficient tool. In Vivo 36(3):1367–1374

    Article  PubMed  PubMed Central  Google Scholar 

  2. Archer AG, Sugarbaker PH, Jelinek JS (1996) Radiology of peritoneal carcinomatosis. Cancer Treat Res 82:263–288

    Article  CAS  PubMed  Google Scholar 

  3. Ben-Baruch G, Sivan E, Moran O, Rizel S, Menczer J, Seidman DS (1996) Primary peritoneal serous papillary carcinoma: a study of 25 cases and comparison with stage III–IV ovarian papillary serous carcinoma. Gynecol Oncol 60(3):393–396

    Article  CAS  PubMed  Google Scholar 

  4. Bochtler T, Löffler H, Krämer A et al (2018) Diagnosis and management of metastatic neoplasms with unknown primary. Semin Diagn Pathol 35(3):199–206

    Article  PubMed  Google Scholar 

  5. Briasoulis E, Kalofonos H, Bafaloukos D et al (2000) Carboplatin plus paclitaxel in unknown primary carcinoma: a phase II Hellenic Cooperative Oncology Group Study. J Clin Oncol 18(17):3101–3107

    Article  CAS  PubMed  Google Scholar 

  6. Carboni F, Federici O, Zazza S, Corona F, Massimi F, Sperduti I, Valle M (2021) Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy for peritoneal metastasis of nonprimary origin. Langenbecks Arch Surg 406(8):2817–2825

    Article  PubMed  Google Scholar 

  7. Ceelen WP, Bracke ME (2009) Peritoneal minimal residual disease in colorectal cancer: mechanisms, prevention, and treatment. Lancet Oncol 10(1):72–79

    Article  PubMed  Google Scholar 

  8. Coakley FV, Choi PH, Gougoutas CA et al (2002) Peritoneal metastases: detection with spiral CT in patients with ovarian cancer. Radiology 223:495–499

    Article  PubMed  Google Scholar 

  9. De Bree E, Koops W, Kröger R, van Ruth S, Witkamp AJ, Zoetmulder FA (2004) Peritoneal carcinomatosis from colorectal or appendiceal origin: correlation of preoperative CT with intraoperative findings and evaluation of interobserver agreement. J Surg Oncol 86(2):64–73

    Article  PubMed  Google Scholar 

  10. De Vuysere S, Vandecaveye V, De Bruecker Y, Carton S, Vermeiren K, Tollens T, De Keyzer F, Dresen RC (2021) Accuracy of whole-body diffusion-weighted MRI (WB-DWI/MRI) in diagnosis, staging and follow-up of gastric cancer, in comparison to CT: a pilot study. BMC Med Imaging 21(1):18–15

    Article  PubMed  PubMed Central  Google Scholar 

  11. Fizazi K, Greco FA, Pavlidis N et al (2015) Cancers of unknown primary site: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 26(Suppl 5):v133–v138

    Article  PubMed  Google Scholar 

  12. Forstner R, Hricak H, Powell CB et al (1995) Ovarian cancer recurrence: value of MR imaging. Radiology 196:715–720

    Article  CAS  PubMed  Google Scholar 

  13. González-Moreno S, González-Bayón L, Ortega-Pérez G, González-Hernando C (2009) Imaging of peritoneal carcinomatosis. Cancer J 15(3):184–189

    Article  PubMed  Google Scholar 

  14. Heinemann V, Boeck C, Hinke A, Labianca R, Louvet C et al (2008) Meta-analysis of randomised trials: evaluation of benefit of gemcitabine-based combination chemotherapy applied in advanced pancreatic cancer. BMC Cancer 8:82–93

    Article  PubMed  PubMed Central  Google Scholar 

  15. Hull NC, Granberg CF, Gargollo PC et al (2022) Imaging of pre- and post-cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pediatric intraperitoneal malignancy. Pediatr Radiol 52:2254–2266

    Article  PubMed  Google Scholar 

  16. Jacquet P, Sugarbaker PH (1996) Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res 82:359–374

    Article  CAS  PubMed  Google Scholar 

  17. Karavasilis V, Malamou-mitsi V, Briasoulis E, Tsanou E, Kitsou E, Kalofonos H, Fountzilas G, Fotsis T, Pavlidis N (2005) Angiogenesis in cancer of unknown primary: clinicopathological study of CD34, VEGF and TSP‑1. BMC Cancer 5:25

    Article  PubMed  PubMed Central  Google Scholar 

  18. Killackey MA, Davis AR (1993) Papillary serous carcinoma of the peritoneal surface: matched-case comparison with papillary serous ovarian carcinoma. Gynecol Oncol 51:171–174

    Article  CAS  PubMed  Google Scholar 

  19. Klumpp BD, Aschoff P, Schwenzer N, Fenchel M, Koenigsrainer I, Falch C, Bruecher B, Claussen CD, Koenigsrainer A, Pfannenberg C, Kramer U, Miller S (2012) Peritoneal carcinomatosis: comparison of dynamic contrast-enhanced magnetic resonance imaging with surgical and histopathologic findings. Abdom Imaging 37:834–842

    Article  PubMed  Google Scholar 

  20. Königsrainer I, Struller F, Horvath P, Klumpp B, Königsrainer A, Beckert S (2013) Peritonealkarzinose: Präoperative Diagnostik und Patientenselektion. Viszeralmedizin 29:208–211

    Google Scholar 

  21. Kyriazi S, Kaye SB, de Souza NM (2010) Imaging ovarian cancer and peritoneal metastases—Current and emerging techniques. Nat Rev Clin Oncol 7:381–393

    Article  PubMed  Google Scholar 

  22. Leebmann H, Piso P (2022) Aktuelle Behandlungsempfehlungen beim Pseudomyxoma peritonei. Chirurgie 93:1152–1157

    Article  CAS  PubMed  Google Scholar 

  23. Levy AD, Shaw JC, Sobin LH (2009) Secondary tumors and tumorlike lesions of the peritoneal cavity: imaging features with pathologic correlation. Radiographics 29(2):347–373

    Article  PubMed  Google Scholar 

  24. Low RN, Semelka RC, Worawattanakul S et al (2000) Extrahepatic abdominal imaging in patients with malignancy: comparison of MR imaging and helical CT in 164 patients. J Magn Reson Imaging 12:269–277

    Article  CAS  PubMed  Google Scholar 

  25. Marin D, Catalano C, Baski M et al (2010) 64-section multi-detector row CT in the preoperative diagnosis of peritoneal carcinomatosis: correlation with histopathological findings. Abdom Imaging 35:694–700

    Article  PubMed  Google Scholar 

  26. Mugerwa S, Lekharaju V, Kiire CF (2009) Management of peritoneal carcinomatosis secondary to metastatic cancer of unknown primary in men. Eur J Cancer Care 18:22–27

    Article  CAS  Google Scholar 

  27. Ono K, Ochiai R, Yoshida T et al (2009) Comparison of diffusion-weighted MRI and 2‑[fluorine-18]-fluo-ro-2-deoxy-D-glucose positron emission tomography (FDG-PET) for detecting primary colorec-tal cancer and regional lymph node metastases. J Magn Reson Imaging 29:336–340

    Article  PubMed  Google Scholar 

  28. Pavlidis N, Briasoulis E et al (2003) Diagnostic and therapeutic management of cancer of an unknown primary. Eur J Cancer 39:1990–2005

    Article  CAS  PubMed  Google Scholar 

  29. Pavlidis N, Fiazai K et al (2009) Carcinoma of unknown primary (CUP). Crit Rev Oncol Hematol 69(3):271–278

    Article  PubMed  Google Scholar 

  30. Pavlidis N, Pentheroudakis G et al (2012) Cancer of unknown primary site. Lancet 379:1428–1435

    Article  PubMed  Google Scholar 

  31. Pentheroudakis G, Pavlidis N (2010) Serous papillary peritoneal carcinoma: unknown primary tumour, ovarian cancer counterpart or a distinct entity? A systematic review. Crit Rev Oncol Hematol 75:27–42

    Article  PubMed  Google Scholar 

  32. Pentheroudakis G, Briasoulis E, Karavassilis V, Fountzilas G (2005) Chemotherapy for patients with two favourable subsets of unknown primary carcinoma: Active, but how effective? Acta Oncol 44(2):155–160

    Article  PubMed  Google Scholar 

  33. Pentheroudakis G, Briasoulis E, Pavlidis N (2007) Cancer of unknown primary site: missing primary or missing biology? Oncologist 12:418–425

    Article  CAS  PubMed  Google Scholar 

  34. Pentheroudakis G, Lazaridis G, Pavlidis N (2010) Axillary nodal metastases from carcinoma of unknown primary (CUPAx): a systematic review of published evidence. Breast Cancer Res Treat 119:1–11

    Article  PubMed  Google Scholar 

  35. Pfannenberg C, Koenigsrainer I, Aschoff P et al (2009) F‑FDG-PET/CT to select patients with peritoneal carcinomatosis for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol 16:1295–1303

    Article  PubMed  Google Scholar 

  36. Pfannenberg C, Schwenzer NF, Bruecher BL (2012) State-of-the art imaging of peritoneal carcinomatosis. Fortschr Röntgenstr 184:205–213

    Article  CAS  Google Scholar 

  37. Piso P, Arnold D (2011) Multimodal treatment approaches for peritoneal carcinosis in colorectal cancer. Dtsch Arztebl Int 108(47):802–808

    PubMed  PubMed Central  Google Scholar 

  38. Piso P, Glockzin G, von Breitenbuch P et al (2009) Patient selection for a curative approach to carcinomatosis. Cancer J 15(3):236–242

    Article  PubMed  Google Scholar 

  39. Piso P, Leebmann H, März L, Mayr M (2015) Cytoreductive surgery for malignant peritoneal tumors. Chirurg 86(1):38–46

    Article  CAS  PubMed  Google Scholar 

  40. Piso P, Stierstorfer K, Gerken M, Klinkhammer-Schalke M (2018) Benefit of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy in patients with isolated peritoneal metastases from colorectal cancer. Int J Colorectal Dis 33(11):1559–1567

    Article  PubMed  Google Scholar 

  41. Quénet F, Elias D, Roca L et al (2021) Cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy versus cytoreductive surgery alone for colorectal peritoneal metastases (PRODIGE 7): a multicentre, randomised, open-label, phase 3 trial. Lancet Oncol 22(2):256–266

    Article  PubMed  Google Scholar 

  42. Rajha A, Piso P, Halmy L et al (2022) Rare histologies and infrequent indications for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Anticancer Res 42(7):3681–3692

    Article  CAS  PubMed  Google Scholar 

  43. Rau B, Brandl A, Piso P et al (2020) Peritoneal metastasis in gastric cancer: results from the German database. Gastric Cancer 23(1):11–22

    Article  PubMed  Google Scholar 

  44. Sadeghi B, Arvieux C, Glehen O, Beaujard AC, Rivoire M, Baulieux J, Fontaumard E, Brachet A, Caillot JL, Faure JL, Porcheron J, Peix JL, Francois Y, Vignal J, Gilly FN (2000) Peritoneal carcinomatosis from non-gynecologic malignancies: results of the EVOCAPE 1 multicentric prospective study. Cancer 88:358–363

    Article  CAS  PubMed  Google Scholar 

  45. Satoh Y, Ichikawa T, Motosugi U, Kimura K, Sou H, Sano K, Araki T (2011) Diagnosis of peritoneal dissemination: comparison of 18F-FDG PET/CT, diffusion-weighted MRI, and contrast-enhanced MDCT. AJR Am J Roentgenol 196(2):447–453

    Article  PubMed  Google Scholar 

  46. Schorge JO, Muto MG, Lee SJ et al (2000) BRCA1-related papillary serous carcinoma of the peritoneum has a unique molecular pathogenesis. Cancer Res 60:1361–1364

    CAS  PubMed  Google Scholar 

  47. Solass W, Struller F, Horvath P, Königsrainer A, Sipos B, Weinreich FJ (2016) Morphology of the peritoneal cavity and pathophysiological consequences. Pleura Peritoneum 1:193–201

    Article  PubMed  Google Scholar 

  48. Sugarbaker PH (1994) Pseudomyxoma peritonei. A cancer whose biology is characterized by a redistribution phenomenon. Ann Surg 219(2):109–111

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Sugarbaker PH (1995) Peritonectomy procedures. Ann Surg 221:29–42

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Sugarbaker PH (2007) Peritoneum as the first-line of defense in carcinomatosis. J Surg Oncol 95:93–96

    Article  PubMed  Google Scholar 

  51. Van der Speeten K, Govaerts K, Stuart OA et al (2012) Pharmakokinetiks of the perioperative use of cancer chemotherapy in peritoneal surface malignancy patients. Gastroenterol Res Pract 2012:378064

    Article  PubMed  PubMed Central  Google Scholar 

  52. Van’t Veer LJ, Weigelt B (2003) Road map to metastasis. Nat Med 9:999–1000

    Article  PubMed  Google Scholar 

  53. Yan TD, Haveric N, Carmignani CP, Chang D, Sugarbaker PH (2005) Abdominal computed tomography scans in the selection of patients with malignant peritoneal mesothelioma for comprehensive treatment with cytoreductive surgery and perioperative intraperitoneal chemotherapy. Cancer 103(4):839–849

    Article  PubMed  Google Scholar 

  54. Zissin R, Hertz M, Shapiro-Feinberg M et al (2001) Primary serous papillary carcinoma of the peritoneum: CT findings. Clin Radiol 56:740–745

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Allgemein- und Viszeralchirurgie, Department für Chirurgie, Klinik für Gastrointestinale und Kolorektale Chirurgie, Asklepios Klinik Barmbek, Rübenkamp 220, 22307, Hamburg, Deutschland

    M. Jusufi

  2. Allgemein- und Viszeralchirurgie, Krankenhaus Barmherzige Brüder Regensburg, Regensburg, Deutschland

    P. Piso

  3. Radiologie, Neuroradiologie und Nuklearmedizin, Krankenhaus Barmherzige Brüder Regensburg, Regensburg, Deutschland

    N. Zorger

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Correspondence to M. Jusufi.

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Interessenkonflikt

M. Jusufi, P. Piso und N. Zorger geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Jusufi, M., Piso, P. & Zorger, N. Peritonealkarzinose beim CUP‑Syndrom. Radiologie 63, 371–380 (2023). https://doi.org/10.1007/s00117-023-01140-9

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