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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Approximately 10% of adenocarcinomas are first seen without a detectable primary tumor. The clinical state that results when a metastatic deposit is more symptomatic than its primary I have termed the CUP (carcinoma unknown primary) syndrome. By definition CUP tumors are incurable by current treatment but their management often includes extensive and expensive efforts to detect the organ of origin. This article stresses the pitfalls in clinical diagnosis and management and outlines an empiric, palliative approach to therapy.
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PMID:Carcinoma with unknown primary tumor (CUP syndrome). 617 92

The patient with carcinoma of unknown primary site deserves prompt and efficient evaluation in an effort to locate a primary tumor in a treatable location. Early collaboration between clinician and pathologist is essential. The needs of patient and family must be considered, and the hospital stay should not be extended by unnecessary diagnostic tests that have no purpose other than delineation of extent of disease. Specific chemotherapy regimens should be instituted if evaluation reveals a potentially responsive tumor. All patients should receive palliative therapy directed at relief of symptoms and pain and improved quality of life. Refinement of immunologic and cytochemical techniques for primary tumor site localization, along with advances in the therapy of colonic, pancreatic, lung, and ovarian carcinomas, should make the outlook for the patient with carcinoma with an occult primary site considerably brighter.
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PMID:Carcinoma of unknown primary site. A prudent approach. 619 19

Surgery is considered the treatment of choice for solitary brain lesions, and radiation therapy is indicated for metastases only in vital or sensitive regions that cannot be excised without risk of disabling neurologic defects. In these cases, radiosurgery may be an alternative to conventionally fractionated radiation therapy. At the Heidelberg linear accelerator-based radiosurgery facility, 69 patients were treated for 102 inoperable brain metastases. The primary tumor sites included non-small cell lung carcinoma (n = 24), renal cell carcinoma (n = 14), melanoma (skin) (n = 14), colorectal carcinoma (n = 6), carcinoma of unknown primary (n = 4), and others (n = 7). Eleven patients were treated for relapse after surgery or after conventional whole-brain irradiation. The doses at the isocenter varied from 15-50 Gy (mean, 21.5 Gy). Ten patients with multiple metastases received a planned combination of whole-brain irradiation plus a single boost of 15 Gy. The median survival time for the entire group was 6 months, with a 1-year-survival of 28.3%. Factors associated with significant improvement of survival were brain metastases without other metastatic disease and good response to radiation therapy. Five of 22 patients (22.9%) with metastases located only in the brain survived longer than 2 years. An improvement in neurologic function was found in 81% within a period of 3 months. With imaging techniques, complete remission was found in 20%, partial remission in 35%, stable disease in 40%, and relapse in 5%. The authors concluded that radiosurgery is an effective and safe therapy for brain metastases. It can be applied as primary treatment, as boost in combination with whole-brain irradiation, or as treatment for patients with relapse in a previously irradiated field.
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PMID:Long-term follow-up for brain metastases treated by percutaneous stereotactic single high-dose irradiation. 843 11

Gastric metastasis from breast cancer is uncommon and typically occurs in patients with disseminated disease. The vast majority of patients with gastric lesions have a known preexisting diagnosis of breast cancer. In contrast, we describe a case in which a minimal breast cancer was found to be the primary tumor during the workup of a patient first diagnosed with carcinoma of unknown primary and subsequently presumed to have metastatic gastric cancer. Our case illustrates that a diagnosis of breast cancer metastatic to the stomach may require a high index of suspicion, as well as a meticulous breast workup. It also emphasizes that even tiny breast cancers have a small but real risk of metastatic spread. Determination of the correct primary source in these cases may not be only an academic exercise, since the treatment and prognosis of metastatic breast cancer (especially receptor positive) and metastatic gastric cancer are different.
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PMID:Metastatic breast cancer manifested as refractory anemia and gastric polyps. 1219 Feb 33

Carcinoma of unknown primary is defined as the histological diagnosis of metastasis without the detection of a primary tumor. In the literature, the incidence of CUP in all patients with a malignant disease is said to be between 3% and 15%. The most frequent histopathological results of CUP metastases are adenocarcinoma, followed by undifferentiated carcinoma and squamous cell carcinoma. In this retrospective investigation the clinical records of 167 patients were studied. All patients had been admitted and treated for cervical CUP at the Department of Otorhinolaryngology of the Grosshadern Clinic from 1979 to 1998. Cervical swelling was the first noted symptom in all cases, followed by pain and dysphagia. The study group comprised 134 men and 33 women with an average age of 55 years at admission. Squamous cell carcinoma (n=123) was the predominant histopathological finding of the cervical lymph nodes. During the 10-year follow-up, a primary tumor was detected in 36 (21.5%) of the 167 initially diagnosed CUP patients. In over 90% of these cases the tumor was localized in the head and neck region. The most frequent origin of the tumor was the tonsilla palatina (n=7). Neck dissection and additional postoperative radiotherapy was performed in 118 (70.7%) of the 167 CUP patients. Primary radiotherapy was the treatment of choice in 28 patients; eight patients received combined radio-chemotherapy as the primary treatment and seven patients were treated with chemotherapy alone. Six patients had no treatment. Comparison of different treatment protocols revealed a significant difference in patient survival: in comparison with primary radiotherapy alone or neck dissection and postoperative radiotherapy, the survival rate improved significantly in patients that received a bilateral tonsillectomy in addition to neck dissection and postoperative radiotherapy. The treatment of choice in patients with cervical CUP should be a surgical procedure including (radical) neck dissection and diagnostic bilateral tonsillectomy followed by postoperative radiation of the cervical lymph drainage. Bilateral tonsillectomy is especially important and is correlated with a significant improvement of the survival rate in CUP patients. Additional postoperative radiation of the entire pharyngeal and laryngeal mucosa should also be considered in order to treat a possible small primary tumor in this region.
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PMID:Diagnosis and management of carcinoma of unknown primary in the head and neck. 1268 29

Intratumoral genomic heterogeneity, which can be defined as both intersample and intrasample heterogeneity, is still a poorly understood phenomenon in head and neck squamous cell carcinoma (HNSCC) with presumed implications on tumor behavior and even prognosis. We analyzed 89 tumor specimen from 37 HNSCC patients by fluorescence in situ hybridization (dual-FISH) using specific DNA probes binding to centromeric sites of 6 chromosomes to investigate intratumoral heterogeneity. A derivation from disomy in at least 1/6 chromosomes was detected in 88/89 (99%) specimen. In 33% of these samples, a change in ploidy could be suspected. Intrasample heterogeneity was detected in 68/89 (76%). Intrasample heterogeneity was more pronounced in primary tumors than in metastatic tumors. Analysis of the intersample heterogeneity revealed notable differences between the 6 chromosomes with the highest discordance detected for chromosome 3 (46%) and the lowest for chromosome 11 (27%). Following our results, it seems important to us to underline that intratumoral heterogeneity exists as intra- and sample heterogeneity in HNSCC. Altogether, trisomic cells were significantly more frequent in primary tumors than in metastases (p=0.01) while, in turn, monosomic cells were significantly more frequent in metastases (p=0.029). In individual cases the extent of discordance between corresponding samples made a common clonal precursor unlikely. In these cases, the synchronous development of a primary tumor and a carcinoma of unknown primary ('CUP syndrome'), otherwise undetected, should be considered.
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PMID:Intratumoral genomic heterogeneity in primary head and neck cancer and corresponding metastases detected by dual-FISH. 1465 97

Cervical lymph node metastases of squamous cell carcinoma from occult primary constitute about 2-5% of all patients with carcinoma of unknown primary site (CUP). Metastases in the upper and middle neck are generally attributed to head and neck cancers, whereas the lower neck (supraclavicular area) involvement is often associated with primary malignancies below the clavicles. The diagnostic procedures include physical examination with thorough evaluation of the head and neck mucosa using fiber-optic endoscopy, biopsies from all suspicious sites or blindly from the sites of possible origin of the primary, computer tomography and/or magnetic resonance. A systematic tonsillectomy in the absence of suspicious lesions is often recommended since up to 25% of primary tumors can be detected in this site. The thoracic primary (tracheal, bronchial, lung, esophagus) has to be excluded, especially in the case of lower neck involvement. Positron emission tomography (PET) with fluoro-2-deoxy-D-glucose allows detection of primary tumor in about 25% of cases, but this procedure is still considered investigational. Therapeutic approaches include surgery (lymph node excision or neck dissection), with or without postoperative radiotherapy, radiotherapy alone and radiotherapy followed by surgery. In early stages (N1), neck dissection and radiotherapy seem to have similar efficacy, whereas more advanced cases (N2, N3) necessitate combined approaches. The extent of radiotherapy (irradiation of bilateral neck and mucosa versus ipsilateral neck radiotherapy) remains debatable. A potential benefit from extensive radiotherapy should be weighted against its acute and late morbidity and difficulties in re-irradiation in the case of subsequent primary emergence. The role of other methods, such as chemotherapy and hyperthermia, remains to be determined.
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PMID:Cervical lymph node metastases of squamous cell carcinoma from an unknown primary. 1502 33

The nodal status is one of the strongest prognostic factors in gynecologic malignancies. Metastatic involvement of regional and distant lymph nodes represents the selection basis for adjuvant therapy in a large number of solid neoplasms. The number of resected lymph nodes is one of the most important parameters in the quality control of the surgical procedure, in particular with respect to radicality. The present paper provides recommendations for gross dissection, laboratory procedures and reporting for lymph node biopsies, lymph node dissections and sentinel lymph node biopsies (SLN) for cancers of the vulva, vagina, uterine cervix, endometrium, Fallopian tubes and the ovaries, submitted for the evaluation of metastatic disease. The pathologic oncology report should include information about the number and size of resected lymph nodes, the number of involved lymph nodes with the maximum size of metastases and the presence of paranodal infiltration. In addition, the detection of isolated tumor cells should be reported, particularly with respect to the detection method (immunostains or molecular methods). In cases of metastatic disease and carcinoma of unknown primary (CUP-syndrome), information should be given regarding the primary tumor.
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PMID:[Recommendations for the handling and oncologic pathology report of lymph node specimens submitted for evaluation of metastatic disease in gynecologic malignancies]. 1591 29

Metastatic squamous cell carcinoma presenting in the neck from an unknown primary site represetns 2% to 6% of head and neck cancers. Optimal management of these cases remains controversial and continues to evolve with experience. We performed a retrospective analysis involving patients treated for unknown primary squamous cell carcinomas with metastases to cervical lymph nodes who presented to either the University of Kentucky or the Veterans Affairs Hospital of Lexington, Kentucky, from 1990 to 2000. Thirty-five out of 173 patients met inclusion criteria for carcinoma of unknown primary. The following data subsets were analyzed: age, gender, smoking and alcohol use, family history, diagnostic studies performed, radiation dose, surgical intervention, number and location of pathologic nodes, presence or absence of extracapsular extension, time between surgery and radiation, disease-specific and overall survival, response to treatment, emergence of a primary tumor, and duration of follow-up. Overall and disease-specific survivals were analyzed using, the Kaplan-Meier method and the log-rank test was used to assess differences in survival curves. The actuarial 5-year overall and disease-specific survival of all patients in this study was 54% and 63%, respectively. At 10 years, the overall survival declined to 37% with a disease-specific survival rate of 49%. The 5-year survival rates stratified by nodal stage were 80% for N1 patients, 64.7% for N2, 55.6% for N3, and 0% for any M disease. These rates declined to 60% for N1, 52.9% for N2, 11.1% for N3, and 0% for any M disease at 10 years (p<.0001). The presence of extracapsular spread, increased number of positive lymph nodes, and eventual discovery of a primary tumor did not significantly decrease survival in this series. The mean follow-up period for patients in this study was 54.8 months. We continue to refine our diagnostic and treatment strategies in this group of patients in an effort to improve long-term survival and reduce patient morbidity.
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PMID:Analysis of unknown primary carcinomas metastatic to the neck: diagnosis, treatment, and outcomes. 1659 71

FDG-PET can be successful in localizing the primary cancer when a metastasis is discovered but no primary tumor can be identified (cancer of unknown primary, or CUP) by physical examination, laboratory testing (for tumor markers, for example) or conventional imaging. The greatest number of PET studies in CUP concern secondary lesions in cervical lymph nodes, and PET is an established clinical use (highest ranking, 1A) according to the 3rd German Consensus Conference and an "option" in the French Standards, Options, and Recommendations. Success rates range from 30% to 50% in most studies using PET; a higher rate was reported recently with PET/CT. FDG-PET should be performed sufficiently early in cases of neurological paraneoplastic syndrome, because established lesions become irreversible. Identification of the antibody present helps to specify the organ and FDG-PET can then localize the lesion; together these techniques make it possible to perform curative surgery even when the primary tumor is not visible. The success rate is somewhat lower than in cases of metastasis, around 35%. The clinical utility of PET in other paraneoplastic syndromes has not yet been sufficiently studied, but these conditions are rare. It is precisely in cases with a kind of 'orphan' indication that FDG PET should be considered, as an effective "problem solver".
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PMID:[FDG-PET in localization of cancers of unknown primary origin]. 1696 33


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