Gastrointestinal Stromal Tumors in Patients with Neurofibromatosis: Imaging Features with Clinicopathologic Correlation
Angela D. Levy1,2,
Nandini Patel1,3,
Robert M. Abbott1,2,4,
Nancy Dow5,
Markku Miettinen6 and
Leslie H. Sobin5
1 Department of Radiologic Pathology, Armed Forces Institute of Pathology,
Alaska and Fern Streets, NW, Washington, DC 20306-6000.
2 Department of Radiology and Nuclear Medicine, Uniformed Services University of
the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814-4799.
3 Present address: Department of Medical Education, Washington Hospital Center,
Washington, DC 20010-2975.
4 Department of Radiology, University of Maryland School of Medicine, Baltimore,
MD 21201-1544.
5 Department of Hepatic and Gastrointestinal Pathology, Armed Forces Institute
of Pathology, Washington, DC 20306-6000.
6 Department of Soft Tissue Pathology, Armed Forces Institute of Pathology,
Washington, DC 20306-6000.
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Fig. 1A. —Unresectable duodenal gastrointestinal stromal tumor in
52-year-old woman (patient 3 in Table
1) who presented with anemia, gastrointestinal bleeding, and
history of neurofibromatosis 1. and B, Preoperative IV (A) and
oral (B) contrast–enhanced CT scans show rim-enhancing cavitary
mass (arrows, A) in fourth portion of duodenum. Calcification
(arrowhead, B) is present in inferior portion of mass.
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Fig. 1B. —Unresectable duodenal gastrointestinal stromal tumor in
52-year-old woman (patient 3 in Table
1) who presented with anemia, gastrointestinal bleeding, and
history of neurofibromatosis 1. Preoperative IV (A) and oral (B)
contrast–enhanced CT scans show rim-enhancing cavitary mass
(arrows, A) in fourth portion of duodenum. Calcification
(arrowhead, B) is present in inferior portion of mass.
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Fig. 1C. —Unresectable duodenal gastrointestinal stromal tumor in
52-year-old woman (patient 3 in Table
1) who presented with anemia, gastrointestinal bleeding, and
history of neurofibromatosis 1. Image from postoperative small-bowel barium
examination shows unresectable gastrointestinal stromal tumor
(arrows) in fourth portion of duodenum as mural mass with barium
collection in central cavity (arrowhead) and fistulous connection to
duodenal lumen.
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Fig. 2A. —Jejunal ganglioneuroma, gastrointestinal stromal tumor, and
adenocarcinoma in 52-year-old man (patient 6 in
Table 1) who presented with
signs and symptoms of small-bowel obstruction and known history of
neurofibromatosis 1. IV and oral contrast–enhanced CT scans show left
adrenal pheochromocytoma (white arrow, A); subcutaneous
neurofibroma (curved arrow, B); plexiform neurofibroma in
right psoas muscle (black arrow, B); and homogeneous
nonenhancing oval mass (arrowheads, B and C) that was
jejunal ganglioneuroma and that led to intussusception. D, Photograph
of opened resected surgical specimen shows jejunal ganglioneuroma
(arrowheads), adenocarcinoma (black arrow), and exophytic
gastrointestinal stromal tumor (white arrow).
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Fig. 2B. —Jejunal ganglioneuroma, gastrointestinal stromal tumor, and
adenocarcinoma in 52-year-old man (patient 6 in
Table 1) who presented with
signs and symptoms of small-bowel obstruction and known history of
neurofibromatosis 1. IV and oral contrast–enhanced CT scans show left
adrenal pheochromocytoma (white arrow, A); subcutaneous
neurofibroma (curved arrow, B); plexiform neurofibroma in
right psoas muscle (black arrow, B); and homogeneous
nonenhancing oval mass (arrowheads, B and C) that was
jejunal ganglioneuroma and that led to intussusception. D, Photograph
of opened resected surgical specimen shows jejunal ganglioneuroma
(arrowheads), adenocarcinoma (black arrow), and exophytic
gastrointestinal stromal tumor (white arrow).
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Fig. 2C. —Jejunal ganglioneuroma, gastrointestinal stromal tumor, and
adenocarcinoma in 52-year-old man (patient 6 in
Table 1) who presented with
signs and symptoms of small-bowel obstruction and known history of
neurofibromatosis 1. IV and oral contrast–enhanced CT scans show left
adrenal pheochromocytoma (white arrow, A); subcutaneous
neurofibroma (curved arrow, B); plexiform neurofibroma in
right psoas muscle (black arrow, B); and homogeneous
nonenhancing oval mass (arrowheads, B and C) that was
jejunal ganglioneuroma and that led to intussusception. D, Photograph
of opened resected surgical specimen shows jejunal ganglioneuroma
(arrowheads), adenocarcinoma (black arrow), and exophytic
gastrointestinal stromal tumor (white arrow).
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Fig. 2D. —Jejunal ganglioneuroma, gastrointestinal stromal tumor, and
adenocarcinoma in 52-year-old man (patient 6 in
Table 1) who presented with
signs and symptoms of small-bowel obstruction and known history of
neurofibromatosis 1. Photograph of opened resected surgical specimen shows
jejunal ganglioneuroma (arrowheads), adenocarcinoma (black
arrow), and exophytic gastrointestinal stromal tumor (white
arrow).
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Fig. 3A. —Multiple gastrointestinal stromal tumors in 23-year-old woman
(patient 1) with neurofibromatosis 1 who presented with syncope from severe
anemia. Preoperative IV (A) and oral (B) contrast–enhanced
CT scans show proximal jejunal gastrointestinal stromal tumor (arrow,
A) and two large gastrointestinal stromal tumors in mid abdomen. Also,
note mixed solid–cystic gastrointestinal stromal tumor
(asterisk, B) adjacent to predominantly cystic
gastrointestinal stromal tumor.
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Fig. 3B. —Multiple gastrointestinal stromal tumors in 23-year-old woman
(patient 1) with neurofibromatosis 1 who presented with syncope from severe
anemia. Preoperative IV (A) and oral (B) contrast–enhanced
CT scans show proximal jejunal gastrointestinal stromal tumor (arrow,
A) and two large gastrointestinal stromal tumors in mid abdomen. Also,
note mixed solid–cystic gastrointestinal stromal tumor
(asterisk, B) adjacent to predominantly cystic
gastrointestinal stromal tumor.
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Fig. 3C. —Multiple gastrointestinal stromal tumors in 23-year-old woman
(patient 1) with neurofibromatosis 1 who presented with syncope from severe
anemia. Photograph of unopened resected upper jejunum shows innumerable
gastrointestinal stromal tumors (arrows) extending through to serosal
surface of jejunum.
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Fig. 4A. —Jejunal gastrointestinal stromal tumor in 49-year-old man
(patient 5) with neurofibromatosis 1 who was being evaluated for pneumonia.
Axial T1-weighted image shows large well-defined mass in small-bowel
mesentery. Mass has central fluid signal intensity and peripheral low signal
intensity.
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Fig. 4B. —Jejunal gastrointestinal stromal tumor in 49-year-old man
(patient 5) with neurofibromatosis 1 who was being evaluated for pneumonia.
Axial fast spin-echo T2-weighted image shows rim of high-signal tumor and
central higher-signal-intensity fluid.
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Fig. 4C. —Jejunal gastrointestinal stromal tumor in 49-year-old man
(patient 5) with neurofibromatosis 1 who was being evaluated for pneumonia.
Photograph of resected surgical specimen shows marked central hemorrhage and
degeneration within tumor.
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Copyright © 2004 by the American Roentgen Ray Society.
