Malignant Peripheral Nerve Sheath Tumor Arising from an Adrenal Ganglioneuroma in a 6-Year-old Boy
Drexel University, Filadelfia, Pennsylvania, United StatesPediatric and Developmental Pathology (Impact Factor: 0.87). 05/2004; 7(3):277-84. DOI: 10.1007/s10024-004-8084-9
This report describes the youngest patient to develop a malignant peripheral nerve sheath tumor arising from a ganglioneuroma (MPNST ex Ganglioneuroma). The patient, a 6-year-old boy, was never irradiated and had no history or stigmata of neurofibromatosis. The report also includes a review of the previously published related cases, and an analysis of the immunohistochemistry and electron microscopy data available to date on the subject.
Article: [Surgical education].
Article: Schwannoma of the Adrenal Gland[Show abstract] [Hide abstract]
ABSTRACT: Primary schwannomas of the adrenal gland are extremely uncommon. We report the clinical and pathologic features of two cases, occurring in a 73-year-old man and a 26-year-old woman, both of whom presented with abdominal pain and an adrenal mass on imaging studies. Both tumors were characterized by a proliferation of cytologically bland spindle cells. One case exhibited alternating compact Antoni A areas and less cellular Antoni B areas consistent with conventional schwannoma, whereas the other case was hypercellular and composed predominantly of Antoni A areas consistent with cellular schwannoma. Reactive inflammatory cells and lymphoid aggregates were present throughout both tumors. The diagnosis of schwannoma was supported by positive immunoreactivity for S-100 protein and collagen IV and absence of reactivity for keratin, muscle related antigens, and CD34 in both cases. Electron microscopy was also performed in the case of cellular schwannoma, which showed ultrastructural features confirming the diagnosis. Both cases had a favorable outcome without evidence of recurrence or metastasis. Because of its rarity, schwannoma occurring at this particular site can pose problems in diagnosis and should be distinguished from other spindle cell lesions of the adrenal gland.
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ABSTRACT: An 11-year-old girl presented with a homogeneous right retroperitoneal tumor 5 cm in diameter. Nonfunctioning benign neurogenic tumor was suspected, and a laparoscopic resection was performed. The inferior vena cava ran just behind the tumor, and there were small vessels between the tumor and inferior vena cava. To create a space behind the tumor, we suspended the tumor by using a traction suture, and to obtain adequate hemostasis, we used a vessel-sealing device. The tumor was safely removed and the pathological diagnosis was benign ganglioneuroma. The postoperative recovery was uneventful, and the patient left her bed and started a regular diet on the postoperative day 1. Laparoscopic surgery is a safe, feasible method for the diagnosis and treatment of benign retroperitoneal tumors, such as ganglioneuroma.
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