title: Pheochromocytoma with histologic transformation to composite type, complicated by watery diarrhea, hypokalemia, and achlorhydria syndrome.
abstract: OBJECTIVE
  To describe the rare occurrence of histologic transformation of a pheochromocytoma to a composite type of tumor during a long-term follow-up, which was complicated by watery diarrhea, hypokalemia, and achlorhydria syndrome.
  
  
  METHODS
  We report the case of a 12-year-old girl who presented with headache, hypertension, and elevated catecholamine levels in the blood and urine. A tumor was found in the right adrenal gland and resected. When she was 15 years of age, multiple metastatic nodules were found in the lung and liver. Intensive chemotherapy was ineffective, and she underwent follow-up with conservative therapy. At 25 years of age, she complained of diarrhea. Laboratory studies revealed hypokalemia and an increase in the level of serum vasoactive intestinal polypeptide (VIP). A year later, she died of extensive metastatic disease. The primary and recurrent tumors at autopsy were histologically examined.
  
  
  RESULTS
  The primary tumor was pure pheochromocytoma, and the tumors at autopsy were a composite type of pheochromocytoma and ganglioneuroma. Only a few VIP-positive cells were found in the primary tumor, whereas both pheochromocytoma and ganglioneuroma cells of composite tumors were frequently positive for VIP.
  
  
  CONCLUSIONS
  Our case showed histologic transformation from pheochromocytoma to a composite type of tumor during a 14-year clinical course, which was associated with additional hormone production and a change in symptoms. Careful attention should be paid to the alteration of endocrine symptoms and hormone levels during prolonged follow-up of pheochromocytoma in young patients.
authors.affiliation: Department of Pathology and Molecular Medicine, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Kikuchi
authors.forename: Ryuichi
authors.initials: R
authors.lastname: Wada
authors.forename: Satoru
authors.initials: S
authors.lastname: Sakihara
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Suda
authors.forename: Soroku
authors.initials: S
authors.lastname: Yagihashi
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D000315:Adrenalectomy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002395:Catecholamines / Q000097:blood / Q000652:urine; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D002648:Child; D003131:Combined Modality Therapy; D019008:Drug Resistance, Neoplasm; D017809:Fatal Outcome; D005260:Female; D005729:Ganglioneuroma / Q000097:blood / Q000188:drug therapy / Q000473:pathology / Q000652:urine; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000556:secondary; D008175:Lung Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000556:secondary; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D018193:Neoplasms, Complex and Mixed / Q000097:blood / Q000188:drug therapy / Q000473:pathology* / Q000652:urine; D010166:Palliative Care; D010673:Pheochromocytoma / Q000188:drug therapy / Q000473:pathology* / Q000556:secondary / Q000601:surgery; D014660:Vasoactive Intestinal Peptide / Q000097:blood; D003969:Vipoma / Q000097:blood / Q000188:drug therapy / Q000473:pathology* / Q000652:urine
chemical_list: D002395:Catecholamines; D014660:Vasoactive Intestinal Peptide