title: Metastatic spiradenocarcinoma occurring in an 8-year-old boy.
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.
authors.affiliation: Department of Pathology, University of North Carolina, Chapel Hill, North Carolina.
authors.forename: Jayson R
authors.initials: JR
authors.lastname: Miedema
authors.forename: Eric
authors.initials: E
authors.lastname: Burgon
authors.forename: Craig
authors.initials: C
authors.lastname: Burkhart
authors.forename: Karyn
authors.initials: K
authors.lastname: Stitzenberg
authors.forename: John
authors.initials: J
authors.lastname: Hipps
authors.forename: Daniel
authors.initials: D
authors.lastname: Zedek
mesh_terms: D000230:Adenocarcinoma / Q000188:drug therapy / Q000378:metabolism / Q000556:secondary*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D002648:Child; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000556:secondary*; D008297:Male; D009363:Neoplasm Proteins / Q000378:metabolism; D012878:Skin Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D013544:Sweat Gland Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins