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Malignant Peripheral Nerve Sheath Tumor¹

¹ From the Departments of Radiology (P.A.H., H.R.F., S.M.) and Pathology (P.C.), St Vincent’s Catholic Medical Center, 355 Bard Ave, Staten Island, NY 10310. Received October 15, 2002; revision requested November 25 and received December 10; accepted December 16. Address correspondence to P.A.H. (e-mail: phrehorovich@yahoo.com).

View larger version (105K): [in a new window]   Figure 1a.  (a) CT scan obtained with oral and intravenous contrast material shows the large, heterogeneous MPNST (*) in the midpelvis encasing the iliac vessels (small arrowheads). The MPNST was isolated in the abdomen and was not connected to the plexiform neurofibromas that arose from the sacral plexus. The surrounding intestine (small arrows) is displaced peripherally; however, there is no evidence of obstruction. Low-attenuation lesions (large arrows) are seen posterior to the psoas muscles bilaterally as well as within the right psoas muscle and represent neurofibromas. A cutaneous lesion (large arrowhead) is present in the right anterolateral soft tissues. (b) Postmortem specimen shows the MPNST (*), which is large and yellow-tan with some scattered areas of hemorrhage on the surface. An adherent vessel is noted (arrowheads), as well as a dark grayish portion of psoas muscle (arrow) on the far left side.

View larger version (132K): [in a new window]   Figure 1b.  (a) CT scan obtained with oral and intravenous contrast material shows the large, heterogeneous MPNST (*) in the midpelvis encasing the iliac vessels (small arrowheads). The MPNST was isolated in the abdomen and was not connected to the plexiform neurofibromas that arose from the sacral plexus. The surrounding intestine (small arrows) is displaced peripherally; however, there is no evidence of obstruction. Low-attenuation lesions (large arrows) are seen posterior to the psoas muscles bilaterally as well as within the right psoas muscle and represent neurofibromas. A cutaneous lesion (large arrowhead) is present in the right anterolateral soft tissues. (b) Postmortem specimen shows the MPNST (*), which is large and yellow-tan with some scattered areas of hemorrhage on the surface. An adherent vessel is noted (arrowheads), as well as a dark grayish portion of psoas muscle (arrow) on the far left side.

View larger version (122K): [in a new window]   Figure 2a.  (a) CT scan obtained with oral and intravenous contrast material shows low-attenuation neurofibromas (large arrows) that arise from the neuroforamina bilaterally and have the characteristic "dumbbell" appearance, which represents a partly intradural and partly extradural lesion. A low-attenuation mass (*) is noted posterior to the aorta, displacing the vessel anteriorly. This mass represents the uppermost portion of the MPNST. A subcutaneous neurofibroma is noted (small arrow). (b) CT scan obtained caudal to a shows fusiform, nodular, low-attenuation lesions (arrowheads) in the presacral region. The lesions arise from the sacral foramina and surround the rectum and distal sigmoid colon (between arrows); they represent numerous plexiform neurofibromas. (c) Autopsy specimen of the thoracic segment of the spinal cord shows nodular enlargement of the spinal roots, an appearance characteristic of plexiform neurofibromas (arrows) that diffusely infiltrate along an entire nerve trunk, the so-called dumbbell appearance.

View larger version (115K): [in a new window]   Figure 2b.  (a) CT scan obtained with oral and intravenous contrast material shows low-attenuation neurofibromas (large arrows) that arise from the neuroforamina bilaterally and have the characteristic "dumbbell" appearance, which represents a partly intradural and partly extradural lesion. A low-attenuation mass (*) is noted posterior to the aorta, displacing the vessel anteriorly. This mass represents the uppermost portion of the MPNST. A subcutaneous neurofibroma is noted (small arrow). (b) CT scan obtained caudal to a shows fusiform, nodular, low-attenuation lesions (arrowheads) in the presacral region. The lesions arise from the sacral foramina and surround the rectum and distal sigmoid colon (between arrows); they represent numerous plexiform neurofibromas. (c) Autopsy specimen of the thoracic segment of the spinal cord shows nodular enlargement of the spinal roots, an appearance characteristic of plexiform neurofibromas (arrows) that diffusely infiltrate along an entire nerve trunk, the so-called dumbbell appearance.

View larger version (114K): [in a new window]   Figure 2c.  (a) CT scan obtained with oral and intravenous contrast material shows low-attenuation neurofibromas (large arrows) that arise from the neuroforamina bilaterally and have the characteristic "dumbbell" appearance, which represents a partly intradural and partly extradural lesion. A low-attenuation mass (*) is noted posterior to the aorta, displacing the vessel anteriorly. This mass represents the uppermost portion of the MPNST. A subcutaneous neurofibroma is noted (small arrow). (b) CT scan obtained caudal to a shows fusiform, nodular, low-attenuation lesions (arrowheads) in the presacral region. The lesions arise from the sacral foramina and surround the rectum and distal sigmoid colon (between arrows); they represent numerous plexiform neurofibromas. (c) Autopsy specimen of the thoracic segment of the spinal cord shows nodular enlargement of the spinal roots, an appearance characteristic of plexiform neurofibromas (arrows) that diffusely infiltrate along an entire nerve trunk, the so-called dumbbell appearance.

View larger version (96K): [in a new window]   Figure 3a.  (a) CT scan obtained at the midthorax shows a low-attenuation lesion (large arrow) with a small, central region of high attenuation (small arrow) in the left posterior subcutaneous tissues. The appearance of the lesion is analogous to the CT target sign. Note the subpleural lesions (arrowheads) in the left posterolateral region. (b) Photograph of the thoracic cavity exposed at autopsy shows numerous paraspinal (arrows) and subpleural (arrowheads) nodular masses of various sizes, which are compatible with plexiform neurofibromas.

View larger version (155K): [in a new window]   Figure 3b.  (a) CT scan obtained at the midthorax shows a low-attenuation lesion (large arrow) with a small, central region of high attenuation (small arrow) in the left posterior subcutaneous tissues. The appearance of the lesion is analogous to the CT target sign. Note the subpleural lesions (arrowheads) in the left posterolateral region. (b) Photograph of the thoracic cavity exposed at autopsy shows numerous paraspinal (arrows) and subpleural (arrowheads) nodular masses of various sizes, which are compatible with plexiform neurofibromas.

View larger version (143K): [in a new window]   Figure 4a.  (a) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) of the MPNST shows a whorling pattern of spindle cells with irregular nuclei and perivascular accentuation (arrows), an appearance characteristic of MPNST. Malignant tumors demonstrate higher cellularity and more evident nuclear pleomorphism than their benign counterparts. (b) High-power photomicrograph (original magnification, x250; hematoxylin-eosin stain) of a neurofibroma shows fusiform, wavy cells with fine collagenous fibers. Neurofibromas are benign tumors of nerves and typically demonstrate hypocellular smears with loose tissue fragments, a fibrillar ground substance, and clusters of spindle cells.

View larger version (144K): [in a new window]   Figure 4b.  (a) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) of the MPNST shows a whorling pattern of spindle cells with irregular nuclei and perivascular accentuation (arrows), an appearance characteristic of MPNST. Malignant tumors demonstrate higher cellularity and more evident nuclear pleomorphism than their benign counterparts. (b) High-power photomicrograph (original magnification, x250; hematoxylin-eosin stain) of a neurofibroma shows fusiform, wavy cells with fine collagenous fibers. Neurofibromas are benign tumors of nerves and typically demonstrate hypocellular smears with loose tissue fragments, a fibrillar ground substance, and clusters of spindle cells.