We report the case of a 21-year-old female who presented with cervical lymphadenopathy and subsequently diagnosed with peritoneal mesothelioma. She had a medical history of Ph + chronic myelogenous leukemia, treated with total body radiation and bone marrow transplant 13 years earlier, then 10 years of imatinib for a relapse 3 years later. The mesothelioma responded well to combination therapy but recurred locally 3 years after diagnosis. This case report emphasizes that even without asbestos exposure, mesothelioma can present as a second malignancy in survivors of childhood cancer who were exposed to radiation therapy as part of their initial treatment.C hronic myelogenous leukemia (CML) is rarely seen in children. Secondary malignancies can present in patients who receive total body radiation (TBI) as a preparative regimen for bone marrow transplantation (BMT) for CML. We present a case of an unfortunate young female who suffered from two cancers-CML and mesothelioma-at a very young age.A 21-year-old female had been diagnosed with adult-type CML in 1997 at the age of 7. Her past medical history was unremarkable and her family history was positive only for breast cancer in both grandmothers at an advanced age. After confirmation of BCR-ABL translocation, she received an allogeneic matched unrelated BMT preceded by chemotherapy and TBI (1320 cGy in six fractions over 3 days). However, her disease control was temporary, and the patient had relapse of her CML in 2001. Fortunately, imatinib mesylate had just become available via a clinical trial for children, so she was started on this agent and had a complete cytogenetic response. In the years since, her quantitative PCR analysis for BCR-ABL kinase domain mutations has shown persistent major (highest level = 0.008%) or complete (undetectable level) molecular response.In the spring of 2010, the patient presented with left neck swelling. The left external jugular vein was distended and vague fullness was felt over her left lower neck. Computed tomography (CT) scan of the neck, chest, abdomen, and pelvis revealed a clot in the left internal jugular and subclavian veins and several enlarged left supraclavicular lymph nodes. Small bilateral pleural effusions were noted. A questionably abnormal cystic lesion was noted on the left adnexa. She was treated with enoxaparin and underwent left supraclavicular lymph node biopsy (Fig. 1).The biopsy tissue was felt to be a lymph node containing metastatic deposits of a carcinoma of unknown primary. Her immunohistochemical staining was positive for pan-keratin and cytokeratin 5/6 and negative for S-100, TTF-1, Villin, BRST-1, and WT-1. Since family history was positive for breast cancer in both grandmothers at an advanced age, she did not meet criteria for Li-Fraumeni or other genetic predisposition syndromes. Differential diagnosis included an adenocarcinoma (salivary gland, endometrial, pancreatic, or breast), mesothelioma, transitional cell carcinoma, squamous cell carcinoma, thymoma, or basal cell carcinoma. A subsequent posit...
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