Metastatic Spiradenocarcinoma Occurring in an 8-Year-Old Boy

Authors

  • Jayson R. Miedema M.D.,

    Corresponding author
    1. Department of Pathology, University of North Carolina, Chapel Hill, North Carolina
    • Address correspondence to Jayson R. Miedema, M.D., Department of Surgical Pathology, University of North Carolina, Women's and Children's Hospital, 3rd Floor, Room 30149, 101 Manning Drive, Chapel Hill, NC 27514, or e-mail: jmiedema@unch.unc.edu.

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  • Eric Burgon M.D., D.S.,

    1. School of Medicine, University of North Carolina, Chapel Hill, North Carolina
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  • Craig Burkhart M.D.,

    1. Department of Dermatology, University of North Carolina, Chapel Hill, North Carolina
    2. Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, North Carolina
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  • Karyn Stitzenberg M.D.,

    1. Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, North Carolina
    2. Department of Surgery, University of North Carolina, Chapel Hill, North Carolina
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  • John Hipps M.D.,

    1. Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, North Carolina
    2. Department of Pediatric Hematology/Oncology, University of North Carolina, Chapel Hill, North Carolina
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  • Daniel Zedek M.D.

    1. Department of Pathology, University of North Carolina, Chapel Hill, North Carolina
    2. Department of Dermatology, University of North Carolina, Chapel Hill, North Carolina
    3. Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, North Carolina
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Abstract

We recently saw the case of an 8-year-old boy with histologic findings of spiradenocarcinoma. Malignant adnexal tumors in children are exceedingly rare, and cases of spiradenocarcinoma in children are absent in the literature. We report the case of an 8-year-old boy with metastatic spiradenocarcinoma. A biopsy 2 years before his presentation at our institution was interpreted as a benign lesion, but when the lesion regrew, a repeat biopsy was performed that demonstrated ominous findings, prompting a reexcision. This reexcision demonstrated an area with significant necrosis, many mitoses, and cellular pleomorphism apparently arising out of a sharply demarcated, adjacent, lower-grade area. The histologic features of this patient's biopsies were those of spiradenocarcinoma, potentially arising out of a preexisting spiradenoma, a finding that has not been documented previously in this age group. Staging studies demonstrated multiple bilateral pulmonary nodules, which were confirmed using thoracoscopic biopsy to be metastatic disease. His chemotherapy regimen has included several cycles of cisplatin and 5-flourouracil, with ongoing disease progression of pulmonary disease. It has been 16 months since he presented to our institution (>3 years since the appearance of his initial lesion). Imaging studies demonstrate a slowly increasing size and number of pulmonary lesions. The long-term prognosis is guarded.

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