Zusammenfassung
Problemstellung
„Cancer of unknown primary“ (CUP) ist definiert durch das Vorhandensein einer metastasierten Erkrankung mit einem trotz leitliniengerechter Diagnostik nicht nachweisbaren Primärtumor zum Zeitpunkt der Erstdiagnose. Obwohl die Prognose der meisten CUP-Patienten schlecht ist, wurden bestimmte Untergruppen mit günstigerer Prognose identifiziert.
Diagnostik
Frauen mit isolierten axillären Lymphknotenmetastasen, die histologisch ein Adenokarzinom oder einen schlecht differenzierten Subtyp aufweisen, keine anderen Fernmetastasen und keine Hinweise auf einen Primärtumor inklusive eines primären Mammakarzinoms in der klinischen Untersuchung, Computertomographie (CT) des Thorax und Abdomens, Mammographie, dem Brustultraschall und der Magnetresonanztomographie (MRT) der Mamma aufweisen, stellen eine potenziell heilbare Untergruppe von Patienten mit CUP dar. Der Mamma-MRT kommt in der diagnostischen Aufarbeitung eines Breast-like-CUP-Syndroms für den Ausschluss eines Mammakarzinoms die größte Bedeutung zu.
Therapie
Breast-like-CUP-Patientinnen werden gemäß den Leitlinien für Patientinnen mit nodal-positivem Brustkrebs behandelt. Es sollte eine leitliniengerechte (neo)adjuvante Systemtherapie erfolgen. Operativ ist eine axilläre Lymphknotendissektion (ALND) indiziert. Bei fehlendem Nachweis eines Primarius in der ipsilateralen Brust wird diese nicht operiert. Eine adjuvante Bestrahlung der ipsilateralen Brust und der supra-/infraklavikulären Lymphknoten sollte diskutiert werden.
Abstract
Background
Cancer of unknown primary (CUP) is defined by the presence of metastatic disease with an undetectable primary tumor at the time of presentation despite standard-of-care imaging. Although the prognosis of most CUP patients is poor, certain subgroups with more favorable prognosis have been defined.
Diagnostics
Women with isolated axillary lymph node metastases and confirmed histologic adenocarcinoma or poorly differentiated subtype, no other distant metastases, and no evidence of a primary cancer including primary breast carcinoma evaluated by clinical examination, computed tomography of thorax and abdomen, mammography, breast ultrasound, and breast magnetic resonance imaging (MRI) represent a potentially curable subgroup of patients with CUP. Breast MRI is the most important radiological modality in the diagnostic workup of breast-like CUP to exclude a primary cancer in the breast.
Therapy
Breast-like CUP patients are treated according to guidelines for patients with node-positive breast cancer. Standard-of-care adjuvant systemic therapy should be given. Axillary lymph node dissection (ALND) is indicated. If no primary cancer in the breast is detected, surgery of the ipsilateral breast should not be performed. Radiotherapy of the ipsilateral breast and supra-/infraclavicular lymph nodes should be discussed.
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A. Schneeweiss hat Vortragshonorare erhalten von Celgene, Roche, Pfizer, AstraZeneca, Novartis, MSD, Tesaro, Lilly, Seagen, Gilead, GSK, Amgen, Pierre Fabre ohne Bezug zu diesem Artikel. L. Buschhorn gibt an, dass kein Interessenkonflikt besteht.
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Schneeweiss, A., Buschhorn, L. „Breast-like cancer of unknown primary“. Radiologie 63, 366–370 (2023). https://doi.org/10.1007/s00117-023-01136-5
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DOI: https://doi.org/10.1007/s00117-023-01136-5