title: Nonfunctioning Adrenocortical Carcinoma in Pediatric Acute Lymphoblastic Leukemia: A Case Report of a Rare Multiple Primaries Combination.
abstract: Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin. Mitotane was added when a pulmonary metastasis was discovered and then stopped after the patient suffered from an arachnoid cyst and speech difficulties. The ALL protocol was resumed from week 34 of the continuation phase. Progression of pulmonary nodules was noted after week 45. A pulmonary metastectomy was performed. The ALL protocol was resumed up to week 51 with a good response as proven by assessment of minimal residual disease. A further recurrence was diagnosed at the abdominal tumor bed with a paravertebral mass and a pulmonary nodule. The patient was assigned to palliative treatment and died after a 32-month survival. Such rare associations need more extensive discussions of the best possible management in scientific literature.
authors.affiliation: *Research Department ‡Pediatric Oncology Department ∥Pathology Department, Children's Cancer Hospital Egypt §National Cancer Institute, Cairo University ¶Cairo University Hospitals †Armed Forces College of Medicine-Egypt (AFCM), Cairo, Egypt #Charité-Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: Wafaa M
authors.initials: WM
authors.lastname: Rashed
authors.forename: Wael
authors.initials: W
authors.lastname: Zekri
authors.forename: Madiha
authors.initials: M
authors.lastname: Awad
authors.forename: Hala
authors.initials: H
authors.lastname: Taha
authors.forename: Badr
authors.initials: B
authors.lastname: Abdalla
authors.forename: Ahmad S
authors.initials: AS
authors.lastname: Alfaar
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000601:surgery; D000315:Adrenalectomy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002277:Carcinoma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000556:secondary / Q000628:therapy; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D017809:Fatal Outcome; D006801:Humans; D007805:Language Development Disorders / Q000139:chemically induced; D008175:Lung Neoplasms / Q000556:secondary / Q000601:surgery; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D008942:Mitoxantrone / Q000008:administration & dosage / Q000009:adverse effects; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D009392:Nephrectomy; D011013:Pneumonectomy / Q000379:methods; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D005047:Etoposide; D004317:Doxorubicin; D008942:Mitoxantrone; D015122:Mercaptopurine; D002945:Cisplatin; D008727:Methotrexate