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Presented by Peter Illei, M.D. and prepared by Priya Banerjee, M.D.
Case 1: 44 y.o. male patient with a 4.5 cm pedunculated endotrachial mass that was endoscopically resected.
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1. Question
Week 371: Case 1
44 y.o. male patient with a 4.5 cm pedunculated endotrachial mass that was endoscopically resected. The biopsy site showed no lesional tissue on repeat biopsy 6 weeks after the initial resection.images/11_3_08 IMT_1.jpg
images/11_3_08 IMT_2.jpg
images/11_3_08 IMT_3.jpg
images/11_3_08 IMT_4.jpgCorrect
Answer: Inflammatory myofibroblastic tumor (IMT)
Histology: see below
Discussion: Histologically, this is a submucosal mass composed of an almost solid proliferation of plasma cells, with readily identifiable bi-nucleate cells with prominent nucleoli. On H&E staining alone, it would seem to be an extra-osseous plasmacytoma. The plasma cells are highlighted by CD138, but they are polyclonal in origin as seen on kappa and lambda light chain stains (kappa:lambda ratio of approximately 3:1). CD3 and CD20 stains are positive in the scattered background T and B-cells more in keeping with a reactive lymphocytic population. Additionally, there appears to be a fibrovascular supporting network to this submucosal lesion, which also contains larger cells that are ALK-1 positive. This lesion is negative for cytokeratin (AE1/AE3, CAM 5.2), CD23, CD15, CD30, HHV-8, EBER and desmin. This histological and mmunohistochemical pattern favors a pulmonary inflammatory myofibroblastic tumor (plasma cell granuloma). Additional immunohistochemical stains demonstrate that the plasma cells Are EMA and MUM-1 positive. A c-kit is negative and highlights associated mast cells. CD56 highlights scattered plasma cells, and may represent a small proportion of NK cells. Fluorescent in situ hybridization (FISH) demonstrated a split signal for the ALK translocation [t(2;5)] in the large atypical cells that were positive by immunohistochemistry. Pulmonary IMT-s are slowly growing tumors that are more common in young adults (>50% occurs in patients less than 40). They usually behave in a benign fashion and are of unknown etiology. The tumors can be asymptomatic or present with obstructive symptoms if arising in a larger airway. They are usually well circumscribed variably sized (1.0 – 36 cm) tumors that may present as a coin lesion on chest X-ray. There are two variants, the plasma cell granuloma variant and the fibrohistiocytic variant. Mitotic figures may be seen and the tumor may become extensively fibrotic. Complete surgical resection is the treatment of choice. Occasional recurrent or malignant IMT-s have also been described.
Incorrect
Answer: Inflammatory myofibroblastic tumor (IMT)
Histology: see below
Discussion: Histologically, this is a submucosal mass composed of an almost solid proliferation of plasma cells, with readily identifiable bi-nucleate cells with prominent nucleoli. On H&E staining alone, it would seem to be an extra-osseous plasmacytoma. The plasma cells are highlighted by CD138, but they are polyclonal in origin as seen on kappa and lambda light chain stains (kappa:lambda ratio of approximately 3:1). CD3 and CD20 stains are positive in the scattered background T and B-cells more in keeping with a reactive lymphocytic population. Additionally, there appears to be a fibrovascular supporting network to this submucosal lesion, which also contains larger cells that are ALK-1 positive. This lesion is negative for cytokeratin (AE1/AE3, CAM 5.2), CD23, CD15, CD30, HHV-8, EBER and desmin. This histological and mmunohistochemical pattern favors a pulmonary inflammatory myofibroblastic tumor (plasma cell granuloma). Additional immunohistochemical stains demonstrate that the plasma cells Are EMA and MUM-1 positive. A c-kit is negative and highlights associated mast cells. CD56 highlights scattered plasma cells, and may represent a small proportion of NK cells. Fluorescent in situ hybridization (FISH) demonstrated a split signal for the ALK translocation [t(2;5)] in the large atypical cells that were positive by immunohistochemistry. Pulmonary IMT-s are slowly growing tumors that are more common in young adults (>50% occurs in patients less than 40). They usually behave in a benign fashion and are of unknown etiology. The tumors can be asymptomatic or present with obstructive symptoms if arising in a larger airway. They are usually well circumscribed variably sized (1.0 – 36 cm) tumors that may present as a coin lesion on chest X-ray. There are two variants, the plasma cell granuloma variant and the fibrohistiocytic variant. Mitotic figures may be seen and the tumor may become extensively fibrotic. Complete surgical resection is the treatment of choice. Occasional recurrent or malignant IMT-s have also been described.
