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Pulmonary epithelioid inflammatory myofibroblastic sarcoma with multiple bone metastases: case report and review of literature.

Fu X, Jiang J, Tian XY, Li Z - Diagn Pathol (2015)

Bottom Line: Fluorescence in situ hybridization (FISH) assay revealed the presence of rearrangement of ALK gene.The second surgical treatment was performed to remove the intraspinal lesion.To the best of our knowledge, it may be the first case of an EIMS arising in lung.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, 151, Yanjiang Road, Guangzhou, 510120, China.

ABSTRACT
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumor with distinctive morphological features and malignant clinical behavior. Only a few such cases have been described in the literature. We report here a case of unusual pulmonary EIMS with multiple bone metastases. A 21-year-old Chinese male patient presented with complaints of general fatigue and rapid weight loss, and a huge tumor arising in the left lower lobe of lung was detected by chest computed tomography. The mass of lung was totally resected. Microscopically, the tumor cells were rounded and epithelioid in shape. Myxoid stroma and inflammatory infiltration was also present. The tumor cells were immunopositive to anaplastic lymphoma kinase (ALK) in smooth cytoplasmic pattern. Fluorescence in situ hybridization (FISH) assay revealed the presence of rearrangement of ALK gene. Three months after lung surgery, there were multiple bone metastases and intraspinal mass found by positron emission tomography. The second surgical treatment was performed to remove the intraspinal lesion. The histological and immunohistochemical features of intraspinal mass were similar to those of pulmonary tumor. The diagnosis of pulmonary EIMS with multiple bone metastases was made. To the best of our knowledge, it may be the first case of an EIMS arising in lung. Awareness of EIMS in respiratory tract and its distinctive features is important for pathologists to avoid a diagnostic pitfall caused by histologic similarities to other ALK-positive tumors. ALK inhibitor is a promising treatment for this aggressive tumor regardless of its potential acquired resistance.

No MeSH data available.


Related in: MedlinePlus

Postoperative micrographs of pulmonary and intraspinal masses. a Pulmonary tumor was non-encapsulated and observed to infiltrating the surrounding lung tissue. b In some areas of pulmonary tumor, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. c The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. d In other areas, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli. Nuclear atypia and mitotic figures were observed. e Necrosis could be found in epithelioid areas of pulmonary tumor. f The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. a HE staining with original magnification × 100; b-f, HE staining with original magnification × 400)
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Fig2: Postoperative micrographs of pulmonary and intraspinal masses. a Pulmonary tumor was non-encapsulated and observed to infiltrating the surrounding lung tissue. b In some areas of pulmonary tumor, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. c The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. d In other areas, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli. Nuclear atypia and mitotic figures were observed. e Necrosis could be found in epithelioid areas of pulmonary tumor. f The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. a HE staining with original magnification × 100; b-f, HE staining with original magnification × 400)

Mentions: The surgical specimens was received and routinely fixed in 10 % neutral buffered formalin after tumor resection. Four micrometer thick sections were cut and stained with H&E. On microscopic examination, the pulmonary and intraspinal masses exhibited similar histological appearance. Both tumors were non-encapsulated and had an infiltrative margin. The pulmonary tumor was composed of two areas with distinct histological appearance. In one area, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. The microscopical appearance of this area was consistent with the histological features of conventional IMT (Fig. 2a-c). However, in other area, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli and eosinophilic or pale cytoplasm. Nuclear atypia, mitotic figures (3/10 high power field) and focal necrosis were observed. Background of tumor in this area showed myxoid change with inflammatory cells infiltration, mainly neutrophils, eosinophils and lymphocytes. The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. The spindle cell component was not present in intraspinal tumor (Fig. 2d-f).Fig. 2


Pulmonary epithelioid inflammatory myofibroblastic sarcoma with multiple bone metastases: case report and review of literature.

Fu X, Jiang J, Tian XY, Li Z - Diagn Pathol (2015)

Postoperative micrographs of pulmonary and intraspinal masses. a Pulmonary tumor was non-encapsulated and observed to infiltrating the surrounding lung tissue. b In some areas of pulmonary tumor, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. c The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. d In other areas, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli. Nuclear atypia and mitotic figures were observed. e Necrosis could be found in epithelioid areas of pulmonary tumor. f The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. a HE staining with original magnification × 100; b-f, HE staining with original magnification × 400)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4504132&req=5

Fig2: Postoperative micrographs of pulmonary and intraspinal masses. a Pulmonary tumor was non-encapsulated and observed to infiltrating the surrounding lung tissue. b In some areas of pulmonary tumor, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. c The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. d In other areas, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli. Nuclear atypia and mitotic figures were observed. e Necrosis could be found in epithelioid areas of pulmonary tumor. f The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. a HE staining with original magnification × 100; b-f, HE staining with original magnification × 400)
Mentions: The surgical specimens was received and routinely fixed in 10 % neutral buffered formalin after tumor resection. Four micrometer thick sections were cut and stained with H&E. On microscopic examination, the pulmonary and intraspinal masses exhibited similar histological appearance. Both tumors were non-encapsulated and had an infiltrative margin. The pulmonary tumor was composed of two areas with distinct histological appearance. In one area, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. The microscopical appearance of this area was consistent with the histological features of conventional IMT (Fig. 2a-c). However, in other area, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli and eosinophilic or pale cytoplasm. Nuclear atypia, mitotic figures (3/10 high power field) and focal necrosis were observed. Background of tumor in this area showed myxoid change with inflammatory cells infiltration, mainly neutrophils, eosinophils and lymphocytes. The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. The spindle cell component was not present in intraspinal tumor (Fig. 2d-f).Fig. 2

Bottom Line: Fluorescence in situ hybridization (FISH) assay revealed the presence of rearrangement of ALK gene.The second surgical treatment was performed to remove the intraspinal lesion.To the best of our knowledge, it may be the first case of an EIMS arising in lung.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, 151, Yanjiang Road, Guangzhou, 510120, China.

ABSTRACT
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumor with distinctive morphological features and malignant clinical behavior. Only a few such cases have been described in the literature. We report here a case of unusual pulmonary EIMS with multiple bone metastases. A 21-year-old Chinese male patient presented with complaints of general fatigue and rapid weight loss, and a huge tumor arising in the left lower lobe of lung was detected by chest computed tomography. The mass of lung was totally resected. Microscopically, the tumor cells were rounded and epithelioid in shape. Myxoid stroma and inflammatory infiltration was also present. The tumor cells were immunopositive to anaplastic lymphoma kinase (ALK) in smooth cytoplasmic pattern. Fluorescence in situ hybridization (FISH) assay revealed the presence of rearrangement of ALK gene. Three months after lung surgery, there were multiple bone metastases and intraspinal mass found by positron emission tomography. The second surgical treatment was performed to remove the intraspinal lesion. The histological and immunohistochemical features of intraspinal mass were similar to those of pulmonary tumor. The diagnosis of pulmonary EIMS with multiple bone metastases was made. To the best of our knowledge, it may be the first case of an EIMS arising in lung. Awareness of EIMS in respiratory tract and its distinctive features is important for pathologists to avoid a diagnostic pitfall caused by histologic similarities to other ALK-positive tumors. ALK inhibitor is a promising treatment for this aggressive tumor regardless of its potential acquired resistance.

No MeSH data available.


Related in: MedlinePlus