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Presented by George Netto, M.D. and prepared by Doreen Nguyen, M.D.
Case 2: A 30 year-old male with Neurofibromatosis Type 1
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Question 1 of 1
1. Question
Week593: Case 2
A 30 year-old male with Neurofibromatosis Type 1 presented with gastrointestinal bleeding and symptoms of severe anemia. He was found to have a 4 cm mass in his jejunum.images/D Nguyen/8-4-14/case 2/2X_T2mucosa_450 pixels.jpg
images/D Nguyen/8-4-14/case 2/2X_necrosis_450 pixels.jpg
images/D Nguyen/8-4-14/case 2/4X_2_450 pixels.jpg
images/D Nguyen/8-4-14/case 2/20X_blunt ends_450 pixels.jpg
images/D Nguyen/8-4-14/case 2/20X_mitosis_450 pixels.jpgCorrect
Answer: Gastrointestinal Stromal Tumor (GIST)
Histology: The mesenchymal tumor is composed of anastomosing bundles of somewhat uniform spindle cells. The neoplastic cells contain ovoid nuclei with only mild nuclear atypia and rare identifiable mitotic figures (1 per 50 HPF). Areas of necrosis are present involving 15% of the tumor volume. Positivity for c-kit and DOG-1 (not shown) confirm the diagnosis of GIST.
The tumor cells were negative for S100 and Smooth Muscle Actin (SMA). The current GIST was assigned a low malignant potential based on the size of the tumor (>2cm but <5cm) and the low mitotic rate (<5 mitoses per 50 HPF).
Discussion: GIST is the most common gastrointestinal mesenchymal tumor in NF-1 patients. Small intestinal location is the most frequent. NF-1 associated intestinal GIST tumors occur a decade earlier than sporadic GISTs. Like our case, c-kit is strongly positive in the majority of such tumors. The majority of NF-1 associated intestinal GISTs are also small in size and mitotically inactive portending a good prognosis. Multiple tumors are not uncommon in this setting. NF-1 associated GISTs lack C-Kit or PDGFRA mutations.
Incorrect
Answer: Gastrointestinal Stromal Tumor (GIST)
Histology: The mesenchymal tumor is composed of anastomosing bundles of somewhat uniform spindle cells. The neoplastic cells contain ovoid nuclei with only mild nuclear atypia and rare identifiable mitotic figures (1 per 50 HPF). Areas of necrosis are present involving 15% of the tumor volume. Positivity for c-kit and DOG-1 (not shown) confirm the diagnosis of GIST.
The tumor cells were negative for S100 and Smooth Muscle Actin (SMA). The current GIST was assigned a low malignant potential based on the size of the tumor (>2cm but <5cm) and the low mitotic rate (<5 mitoses per 50 HPF).
Discussion: GIST is the most common gastrointestinal mesenchymal tumor in NF-1 patients. Small intestinal location is the most frequent. NF-1 associated intestinal GIST tumors occur a decade earlier than sporadic GISTs. Like our case, c-kit is strongly positive in the majority of such tumors. The majority of NF-1 associated intestinal GISTs are also small in size and mitotically inactive portending a good prognosis. Multiple tumors are not uncommon in this setting. NF-1 associated GISTs lack C-Kit or PDGFRA mutations.
