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  Table of Contents  
Year : 2014  |  Volume : 51  |  Issue : 4  |  Page : 480

Patient with multiple metachronous primary cancers

1 Department of Radiation Oncology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
2 Department of Medical Genetics, Henry Ford Health System, Detroit, MI, USA
3 Department of Radiation Oncology, Henry Ford Health System, Detroit, MI, USA

Date of Web Publication1-Feb-2016

Correspondence Address:
Dr. J R Robbins
Department of Radiation Oncology, Medical College of Wisconsin, Milwaukee, Wisconsin
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.175346

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How to cite this article:
Robbins J R, Wang X, Tousignant R, Aref I, Siddiqui F. Patient with multiple metachronous primary cancers. Indian J Cancer 2014;51:480

How to cite this URL:
Robbins J R, Wang X, Tousignant R, Aref I, Siddiqui F. Patient with multiple metachronous primary cancers. Indian J Cancer [serial online] 2014 [cited 2021 Jul 24];51:480. Available from: https://www.indianjcancer.com/text.asp?2014/51/4/480/175346


A 61-year-old female presented to a radiation oncologist for consideration of stereotactic body radiation therapy for the right lung lesion. During the visit, an extensive cancer history was discovered. At age 19, she was diagnosed with osteosarcoma treated with an above-knee leg amputation. Thirty years later, at age 49, she developed right breast cancer that was treated with lumpectomy, chemotherapy, and radiation. Nine years later, at age 58, she was diagnosed with a synchronous adenocarcinoma of the left lung and mediastinum thymoma, which were treated simultaneously with left lower lobe lobectomy, mediastinal lymph node dissection, and postoperative radiation therapy. Three years later, a surveillance positron emission tomography scan detected the presenting lung lesion as well as a cecal lesion. Colonoscopy revealed a tubular adenoma of the rectum and an adenocarcinoma of the cecum, which was treated by a right hemi-colectomy. In addition, she reported a history of a resected lipoma and an excised benign nodule of her vocal cord. Her family history was unobtainable, because she was adopted and had no children secondary to an early hysterectomy for severe endometriosis. Given her history of multiple cancers, genetic counseling referral was made. Based on her constellation of primary cancers, a clinical diagnosis of Li–Faumeni syndrome (LFS) can be established by the stringent Chompret criteria: (1) Any individual with a LFS tumor before 46 years of age and one first or second degree relative with a LFS tumor before the age of 46 or with multiple cancers, or (2) any individual with multiple cancers, two of which are LFS-related cancers with the first diagnosed before the age of 46 years, or (3) any individual with an adrenocortical carcinoma or choroid plexus tumor.[1] LFS is an autosomal dominant hereditary cancer condition with predisposition to cancers as a result of TP53 gene mutation. Soft tissue sarcomas, osteosarcomas, premenopausal breast cancer, brain tumors, adrenocortical carcinomas, and leukemia are common LFS associated tumors. By age 30, gene carriers have a 50% likelihood of developing cancer. The chance approaches 90% by 60–70 years of age.[2] Approximately, 5–10% of cancers are due to hereditary cancer syndrome, therefore, a patient with early onset, multiple primary cancers, or a strong family history of cancers should receive genetic screening.[3] New genetic testing technologies and growing knowledge of cancer genomics may help provide an accurate diagnosis, identify high-risk individuals and facilitate the development of appropriate early detection and prevention strategies.[4]

  References Top

Bougeard G, Sesboüé R, Baert-Desurmont S, Vasseur S, Martin C, Tinat J, et al. Molecular basis of the Li-Fraumeni syndrome: An update from the French LFS families. J Med Genet 2008;45:535-8.  Back to cited text no. 1
Malkin D, Li FP, Strong LC, Fraumeni JF Jr, Nelson CE, Kim DH, et al. Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms. Science 1990;250:1233-8.  Back to cited text no. 2
Sifri R, Gangadharappa S, Acheson LS. Identifying and testing for hereditary susceptibility to common cancers. CA Cancer J Clin 2004;54:309-26.  Back to cited text no. 3
Stadler ZK, Schrader KA, Vijai J, Robson ME, Offit K. Cancer genomics and inherited risk. J Clin Oncol 2014;32:687-98.  Back to cited text no. 4


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