title: Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.
abstract: Adrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old man is reported here. After adequate preoperative control, left adrenalectomy was performed simultaneously with resection of the ipsilateral kidney for spontaneous rupture of the left adrenal tumor. Pathological findings demonstrated pheochromocytoma and malignant peripheral nerve sheath tumor in a ruptured adrenal tumor. To date, there have been only four reported cases of composite pheochromocytoma with malignant peripheral nerve sheath tumor, so the present case is only the fifth case in the world. Despite the very poor prognosis of patients with pheochromocytoma and malignant peripheral nerve sheath tumors reported in the literature, the patient remains well without evidence of recurrence or new metastatic lesions at 36 months postoperatively.
authors.affiliation: Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Namekawa
authors.affiliation: Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address: p-cats@pd6.so-net.ne.jp.
authors.forename: Takanobu
authors.initials: T
authors.lastname: Utsumi
authors.affiliation: Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Imamoto
authors.affiliation: Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Kawamura
authors.affiliation: Department of Diagnostic Pathology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Oide
authors.affiliation: Department of Clinical Cell Biology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Tanaka
authors.affiliation: Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Nihei
authors.affiliation: Department of Urology, Toho University, Sakura Medical Center, Sakura, Japan.
authors.forename: Hiroyoshi
authors.initials: H
authors.lastname: Suzuki
authors.affiliation: Department of Diagnostic Pathology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Yukio
authors.initials: Y
authors.lastname: Nakatani
authors.affiliation: Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan.
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Ichikawa
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D006801:Humans; D008297:Male; D018193:Neoplasms, Complex and Mixed / Q000175:diagnosis* / Q000601:surgery; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000601:surgery; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000601:surgery
keywords: adrenal tumor; adrenalectomy; malignant peripheral nerve sheath tumor; pheochromocytoma; spontaneous rupture