Carcinoma of Unknown Primary &amp;ndash; an Orphan Disease?

Krämer, Alwin; Hübner, G; Schneeweiß, Andreas; Folprecht, Gunnar; Neben, Kai

doi:10.1159/000136001

Cited by 12 publications

(20 citation statements)

References 65 publications

(51 reference statements)

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“…The proportions of patients with complete and partial responses to chemotherapy in the present study were similar to those reported in the literature [14,17,18,20,21].The response to chemotherapy showed a strong correlation with the PFS and OS, although the survival time did not differ significantly between the patients with a response and those with stable disease after chemotherapy. The choices of chemotherapeutic agents were based on the predicted primary site and were similar to those in the literature [9,22,23]. Platinum plus 5-fluorouracil and platinum plus taxane were the most commonly administered combinations.…”

Section: Discussionmentioning

confidence: 99%

“…The median PFS was 5 (1-26) months, and median OS was 7 (1-38) months. For the patients who had complete/partial responses after chemotherapy, the median PFS and OS were 14 (7-20) months and 18 (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)) months, respectively. For the patients with stable disease, the median PFS and OS were 10 (4-26) months and 16 (6-38) months, respectively.…”

Section: Survivalmentioning

confidence: 99%

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Unknown primary adenocarcinomas: a single-center experience

Uzunoğlu

Erdoğan

Kodaz

et al. 2016

Bosn J of Basic Med Sci

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This study aimed to elucidate the clinical and prognostic characteristics of a homogeneous group of patients with cancer of unknown primary (CUP). Between 1999 and 2014, CUP was diagnosed in 159 (1.3%) of 11,742 cancer patients at Trakya University Hospital (Edirne, Turkey). Ninety-seven (61%) of the 159 patients were retrospectively reviewed. Among these, 61 (62.8%) patients with adenocarcinoma were included in this study. The most frequently predicted primary tumor site was the lung (37.7%), and 59% of the patients were smokers. There was a significant relationship between smoking and the lung as a potential primary cancer site (p = 0.042). The most frequent site of metastasis was the liver (60.7%). The median number of metastases per patient was two, but patients with liver metastases had a median of five metastases. The overall median survival time was 7 months. Median survival was significantly longer in patients with a predicted primary site than in patients without the predicted site (7 vs. 6 months, respectively; p = 0.038). When the patients with predicted ovarian and peritoneal tumors were excluded from the comparison, the statistical p value was still close to significant (p = 0.07). Multivariate analysis revealed that smoking, liver metastasis, serum alkaline phosphatase ≥92 U/L, and progression in response to chemotherapy were independent predictors of a poor prognosis. The present study identified several independent prognostic factors in patients with unknown primary adenocarcinomas who received chemotherapy. Smoking, the presence of liver metastasis, and response to chemotherapy were independent risk factors for both progression-free and overall survival.

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Section: Discussionmentioning

confidence: 99%

Section: Survivalmentioning

confidence: 99%

Unknown primary adenocarcinomas: a single-center experience

Uzunoğlu

Erdoğan

Kodaz

et al. 2016

Bosn J of Basic Med Sci

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“…Furthermore, approaches to treatment must be informed by increasing evidence of significant heterogeneity among OPBC patients, with a recent study reporting outcome differences between OPBC patients with different immunohistochemical profiles. 7 If, as some would argue, 61 CUP is an orphan disease within medicine, OPBC is certainly an orphan disease within breast oncology. As with other rare diseases, evidence-based approaches to OPBC diagnosis and treatment would benefit from initiation of a prospectively accrued international patient registry.…”

Section: Discussionmentioning

confidence: 99%

Geographic and Temporal Trends in the Management of Occult Primary Breast Cancer: A Systematic Review and Meta-Analysis

et al. 2013

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Background Management of occult primary breast cancer (OPBC), including the role of magnetic resonance imaging (MRI), is controversial. We conducted a pooled analysis of OPBC patients and a meta-analysis of MRI accuracy in OPBC in order to elucidate current practices. Methods A literature search yielded 201 studies. Patient-level data for clinically/mammographically OPBC from studies published after 1993 and from our institution were pooled; logistic regression examined associations between patient/study data and outcomes, including treatments and recurrence. We report adjusted odds ratios (OR) and 95 % confidence intervals (95 % CI) significant at 2-tailed p<0.05. Meta-analysis included data for patients who received MRIs for workup of clinically/mammographically OPBC. We report pooled sensitivity and specificity with 95 % CIs. Results The pooled analysis included 92 patients (15 studies [n = 85] plus our institution [n = 7]). Patients from Asia were more likely to receive breast surgery (OR = 5.98, 95 % CI = 2.02–17.65) but not chemotherapy (OR = 0.32, 95 % CI = 0.13–0.82); patients from the United States were more likely to receive chemotherapy (OR = 13.08, 95 % CI = 2.64–64.78). Patients from studies published after 2003 were more likely to receive radiotherapy (OR = 3.86, 95 % CI = 1.41–10.55). Chemotherapy recipients were more likely to have distant recurrence (OR = 9.77, 95 % CI = 1.10–87.21). More patients with positive MRIs received chemotherapy than patients with negative MRIs (10 of 12 [83.3 %] vs 5 of 13 [38.5 %]; p = 0.0414). In the MRI-accuracy meta-analysis (10 studies, n = 262), pooled sensitivity and specificity were 96 % (95 % CI = 91–98 %) and 63 % (95 % CI = 42–81 %), respectively. Conclusions OPBC management varied geographically and over time. We recommend establishing an international OPBC patient registry to facilitate longitudinal study and develop global treatment standards.

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“…For CUP, identification of the primary site would be of utmost importance because therapy is typically devised based on clinicopathological considerations, of which the tumor origin is most important. The location of the affected lymph nodes may provide clues about the anatomic location of the primary tumor, guided by the lymphatic drainage system: head and neck lymph nodes are frequently affected by lung and head and neck cancers, axillary lymph nodes by breast and lung cancers and nodes below the diaphragm are affected by abdominal and (retro)peritoneal tumors 5‐8. Such empirical wisdom has been incorporated into the diagnostic guidelines of the European Society for Medical Oncology; patients who present with axillary nodal adenocarcinoma should be assessed by magnetic resonance imaging (MRI) of the breast and those who present with cervical nodal squamous cell carcinoma should undergo positron emission computed tomography (PET‐CT) of the head and neck region 9.…”

mentioning

confidence: 99%

Site‐specific cancer deaths in cancer of unknown primary diagnosed with lymph node metastasis may reveal hidden primaries

Hemminki

Bevier

Sundquist

et al. 2012

Intl Journal of Cancer

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Cancer of unknown primary site (CUP) is a fatal cancer ranking among the five most common cancer deaths. CUP is diagnosed through metastases, which are limited to lymph nodes in some patients. Cause-specific survival data could guide the search for hidden primary tumors and help with therapeutic choices. The CUP patients were identified from the Swedish Cancer Registry between 1987 and 2008; 1,444 patients had only lymph node metastasis of defined histology (adenocarcinoma, squamous cell or undifferentiated). Site-specific cancer deaths were analyzed by lymph node location and histology. KaplanMeier survival curves were compared with metastatic primary cancer at related sites. Among the patients with metastasis to head and neck lymph nodes, 117 (59.1% of the specific cancer deaths) died of lung tumors. Patients with axillary lymph node metastasis died of lung and breast tumors in equal proportions (40.2% each). Also, squamous cell CUP in head and neck lymph nodes was mainly associated with lung tumor deaths (53.1%). With a few exceptions, survival of CUP patients with lymph node metastasis was indistinguishable from survival of patients with metastatic primary cancer originating from the organs drained by those nodes. The association between lymph node CUP metastases with cancer deaths in the drained organ and the superimposable survival kinetics suggests that drained organs host hidden primaries. Importantly, half of all site-specific cancer deaths (266/530) were due to lung tumors. Thus, an intense search should be mounted to find lung cancer in CUP patients with lymph node metastases.

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Carcinoma of Unknown Primary – an Orphan Disease?

Cited by 12 publications

References 65 publications

Unknown primary adenocarcinomas: a single-center experience

Unknown primary adenocarcinomas: a single-center experience

Geographic and Temporal Trends in the Management of Occult Primary Breast Cancer: A Systematic Review and Meta-Analysis

Site‐specific cancer deaths in cancer of unknown primary diagnosed with lymph node metastasis may reveal hidden primaries

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