How Common Are Second Malignancies After a Primary Malignancy?

Patient Presentation
A 14-year-old female was referred from the emergency room with a one month history of constipation. She generally had regular soft stools but then began having bowel movements every 4+ days and later began having overflow encopresis. She said that she felt generally full in her abdomen and experienced pressure discomfort all the time but had no nausea or vomiting. She said that she had been eating less because she got full quickly. There was no blood or mucous in the stool.

The past medical history was positive for being a 5-year survivor of a primary neuroectodermal tumor of the brain that was successfully treated with chemotherapy and radiotherapy. The family history was non-contributory. The review of systems was negative for any weight loss, sweating, difficulty walking or urinating or seizure activity.

The pertinent physical exam showed a cooperative female in no distress with normal vital signs. She was at the 10% for height and 50% for weight. Her abdominal examination showed slight distention and was soft without hepatosplenomegaly. She had a mass in the left lower quadrant of the abdomen which was approximately 8 x 4 centimeters in size that was mobile. The rest of her examination was normal. The mass remained palpable after an enema produced a moderate amount of stool.

The radiologic evaluation of a computed tompgraphy showed a soft tissue mass arising from high up in the sigmoid colon.

A biopsy of the mass was most consistent with a the diagnosis of a dermoid ectodermal malignancy.
After a central line port was placed, she tolerated the first cycle of chemotherapy well, and was to be followed closely.

Figure 76 – Axial images from a computed tomography exam of the abdomen and pelvis perfomred with intravenous and oral contrast demonstates a large, ill-defined, heterogenous and necrotic mass arising from and filling the pelvis and extending up into the abdomen (lower image) that is associated with ascites and metastases to the liver and to the peritoneum anterior to the liver (upper image).

Discussion
About 12,400 children in the US are diagnosed with cancer each year, with 80% surviving. There are ~300,000 survivors of pediatric cancer in the US. Late complications of treatment may include problems with organ function, growth and development, neurocognitive function and academic achievement, infertility, and the potential for additional cancers, in addition to psychosocial consequences. It is estimated that 2/3rds of survivors will have at least one late-therapy complication and of those complications 1/4 will be life-threatening.

In 2002 the Children’s Oncology Group published their Long-term Follow-up Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancers at http://www.survivorshipguidelines.org/. In 2009, the American Academy of Pediatrics published a clinical report supporting their use to assist in tailoring care based upon specific treatment-related risk factors.

Learning Point
Second primary cancers (SPCs, not a recurrance of the initial primary cancer) unfortunately are not unknown nor rare. As improvements in treatment and supportive care occur, survivorship from cancer has increased. Therapy, especially radiotherapy, has emerged as one of the causes of SPCs in childhood cancer survivors. One study found that the leading cause of death after 15 years of survivorship from pediatric cancer was a SPC.

The Standardized Incidence Ratio (SIR) is a common statistical method used to describe incidence. The SIR is the ratio of the observed to the expected new cases of cancer. The expected number is based upon age-specific rates of cancer.

Overall the SIR for all pediatric cancers was 5.9. This SIR is highest in the first 1-9 years of survivorship and decreases to 4.1 after 20+ years of survival. The SPC with the highest SIR for all pediatric cancers were acute myeloid leukemia, bone, gastrointestinal tract, lung, and pancreas.

Acute lymphocytic leukemia (ALL) is the most common pediatric cancer accounting for ~25% of all malignancies. The SIR for ALL is 7.2 with the highest SIRs occurring in the 0-5 year survivorship group and decreasing to an SIR of 2.3 in the 11-15 years survivorship group. The SPC with highest SIR were a following ALL were acute non-lymphocytic leukemia and central nervous systems malignancies.

Questions for Further Discussion
1. What are the acute and long-term complications of chemotherapy?
2. What are the acute and long-term complications of radiotherapy?

Related Cases

Disease: Soft Tissue Sarcoma | Cancer | Brain Cancer | Cancer in Children

Symptom/Presentation: Constipation and Encopresis

Specialty: Emergency Medicine | Oncology | Preventive Medicine and Health Maintenance | Radiology / Nuclear Medicine / Radiation Oncology

Age: Teenager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Soft Tissue Sarcoma and Cancer in Children.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Lawless SC, Verma P, Green DM, et al. Mortality Experiences Among 15+ Year Survivors of Childhood and Adolescent Cancers. Pediatr Blood Cancer. 2007;48:333-338.

Krueger H, McLean D, Williams D. Prevention of Second Pediatric Cancers. Prog Exp Tumor Res. Basil, Switzerland. Karger. 2008:40:122-134.

American Academy of Pediatrics Section on Hematology/Oncology. Children’s Oncology Group. Long-term Follow-up Care for Pediatric Cancer Survivors. Pediatrics 2009 123: 906-915.

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