scholarly journals Case Report: Long-Term Survival of a Pediatric Patient With an Intra-Abdominal Undifferentiated Carcinoma of Unknown Primary

2021 ◽  
Vol 11 ◽  
Author(s):  
Anja Stirnweiss ◽  
Hetal Dholaria ◽  
Joyce Oommen ◽  
Kathy Hardy ◽  
Gareth Jevon ◽  
...  

An 8-year and 10-month-old boy presented following 2 weeks of abdominal pain, vomiting, constipation, and rectal pain. A diffuse lower-abdominal mass was felt upon palpation, with radiological findings confirming the presence of a large, multilobulated intraperitoneal mass with mesenteric lymphadenopathy and hepatic metastatic disease. A biopsy of the mass revealed anatomical pathological findings consistent with a diagnosis of intra-abdominal undifferentiated carcinoma of unknown primary (CUP). The patient was treated with six cycles of carboplatin and gemcitabine prior to surgery. Following incomplete resection of the tumor, four further cycles were administered resulting in resolution of the pelvic mass, but progression in the right and left lobes of the liver. Therapy was accordingly adjusted, with administration of six cycles of ifosfamide and doxorubicin followed by 1 year of metronomic vinorelbine and cyclophosphamide maintenance therapy. The patient remains in remission 7 years from completion of therapy. Whole exome sequencing revealed missense mutations in the DNA-repair and chromatin-remodeling genes FANCM and SMARCD2, and a tumor-derived cell line revealed a complex karyotype suggesting chromosomal instability. CUP is an extremely rare diagnosis in the pediatric population, previously reported during adolescence. This report provides detailed characterization of CUP in a young child and in the absence of defined therapeutic guidelines for pediatric CUP, the successful treatment strategy described should be considered for similar cases.

2014 ◽  
Vol 53 (2) ◽  
pp. 159-162 ◽  
Author(s):  
Genta Ishikawa ◽  
Naoki Nishimura ◽  
Sayaka Mochizuki ◽  
Yasuhiko Yamano ◽  
Atsushi Kitamura ◽  
...  

2008 ◽  
Vol 26 (15_suppl) ◽  
pp. 15594-15594
Author(s):  
K. Sprenger ◽  
A. Kretzschmar ◽  
G. Folprecht ◽  
H. Link ◽  
V. Gruenwald ◽  
...  

2004 ◽  
Vol 26 (11) ◽  
pp. 780-782 ◽  
Author(s):  
Jerry C. Cheng ◽  
Samuel D. Esparza ◽  
Virginia M. Knez ◽  
Kathleen M. Sakamoto ◽  
Theodore B. Moore

2019 ◽  
Vol 2019 (8) ◽  
Author(s):  
Akihiro Shiroshita ◽  
Wataru Uegami ◽  
Ayumu Otsuki ◽  
Kei Nakashima ◽  
Keishin Sunagawa ◽  
...  

Abstract We report the case of a 61-year-old woman with Kartagener syndrome who presented with a 3-month history of chronic watery diarrhoea and severe hypoalbuminaemia. Histopathological examination of duodenum and large intestine biopsies showed amyloid A (AA) amyloid deposition. Scintigraphy and alpha-1 anti-trypsin clearance evaluations revealed protein-losing gastroenteropathy. Computed tomography with contrast and positron emission tomography showed a pelvic mass with multiple para-aortic lymph node enlargement. We suspected protein-losing gastroenteropathy secondary to AA amyloid produced related to malignant tumours. Following tumour resection, histopathological examination of the lesion revealed undifferentiated carcinoma of unknown origin. Postoperatively, the patient’s nutritional condition improved. There has been no recurrence of protein-losing gastroenteropathy 6 months postoperatively. This is the first report of protein-losing gastroenteropathy and AA amyloidosis secondary to undifferentiated carcinoma. Early recognition and intervention could increase the likelihood of amyloidosis remission.


2008 ◽  
Vol 6 (10) ◽  
pp. 1026
Author(s):  
_ _

Occult primary tumors, or cancers of unknown primary, account for 5% to 10% of all diagnosed cancers, and are manifested by a wide variety of clinical presentations, while conferring a poor prognosis for most patients. Even after postmortem examination, the primary tumor is not identified in 20% to 50% of patients. Multiple sites of involvement are observed in more than 50% of patients. Although certain patterns of metastases suggest possible primaries, occult primaries can metastasize to any site. In most patients, occult primary tumors are refractory to systemic treatments, and chemotherapy is only palliative and does not significantly improve long-term survival. However, special pathologic studies can identify subsets of patients with tumor types that are more responsive. Treatment options should be individualized for this selected group to achieve improved response and survival rates. Important updates for the NCCN guidelines include the additions of tables on tumor-specific markers and their staining pattern as well as analysis of undifferentiated carcinoma. For the most recent version of the guidelines, please visit NCCN.org


Lung Cancer ◽  
2007 ◽  
Vol 56 (1) ◽  
pp. 139-141 ◽  
Author(s):  
Chika Kusakari ◽  
Hiroshi Soda ◽  
Yoichi Nakamura ◽  
Hiroyuki Yamaguchi ◽  
Takeshi Kitazaki ◽  
...  

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