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Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review.

Dong M, Liu J, Song Z, Li X, Shi T, Wang D, Ren D, Chen J - Medicine (Baltimore) (2015)

Bottom Line: As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy.We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare.Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease.

View Article: PubMed Central - PubMed

Affiliation: From the Department of Lung Cancer Surgery, Tianjin Lung Cancer Institute (MD, JL, ZS, XL, DR, JC); Department of Pathology; Tianjin Medical University General Hospital, Heping District, Tianjin, China (TS, DW).

ABSTRACT
Primitive neuroectodermal tumors (PNETs) arising directly from the lung are extremely rare but particularly aggressive neoplasms. Although thoracic PNET usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.We present the case of a 16-year-old male with PNET diagnosed following histologic and immunohistochemical examination of a video-assisted thoracic surgical biopsy. As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy. We attempted to explore the use of targeted pharmacotherapy through high-throughput sequencing in this case. We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare. Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease.

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Related in: MedlinePlus

Histopathology. Hematoxylin–eosin (H&E) staining of the primary pulmonary primitive neuroectodermal tumor; immunohistochemistry staining of the primary tumor with different antibodies, such as anti-CD99, vimentin, chromogranin A (CgA), and friend leukemia integration 1 transcription factor (Fli-1). The images for the rest negative immunohistochemistry staining of thyroid transcription factor 1 (TTF-1), cytokeratin 7 (CK7), cytokeratin 19 (CK19), HMB45, protein S-100, epithelial membrane antigen (EMA), smooth muscle actin (SMA), CD34, CD20, CD56, CD31, leukocyte common antigen (LCA), and Desmin were not shown.
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Figure 2: Histopathology. Hematoxylin–eosin (H&E) staining of the primary pulmonary primitive neuroectodermal tumor; immunohistochemistry staining of the primary tumor with different antibodies, such as anti-CD99, vimentin, chromogranin A (CgA), and friend leukemia integration 1 transcription factor (Fli-1). The images for the rest negative immunohistochemistry staining of thyroid transcription factor 1 (TTF-1), cytokeratin 7 (CK7), cytokeratin 19 (CK19), HMB45, protein S-100, epithelial membrane antigen (EMA), smooth muscle actin (SMA), CD34, CD20, CD56, CD31, leukocyte common antigen (LCA), and Desmin were not shown.

Mentions: In order to establish the pathological diagnosis, a left-sided video-assisted thoracoscopy (VAT) was performed and 6 nodules in the lingual segment were removed. During the operation, a number of nodules were present on the surface of both left lung lobes with protrusion, but under the visceral pleura. As shown in Figure 2, histopathologic examination showed tumor cells that exhibited small, round, and mild morphology; partial cells with more cytoplasm; and partial cells with transparent cytoplasm. The majority of cells exhibited relatively consistent nuclear size. Immunostaining was positive for the expression of CD99 and vimentin and was negative for the expression of chromogranin A, thyroid transcription factor 1, cytokeratin 7, cytokeratin 19, human melanoma black 45 (HMB45), protein S-100, epithelial membrane antigen, smooth muscle actin (SMA), CD34, CD20, CD56, CD31, leukocyte common antigen, friend leukemia integration 1 transcription factor, and Desmin. In view of the foregoing, a diagnosis of primary lung PNET was made.


Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review.

Dong M, Liu J, Song Z, Li X, Shi T, Wang D, Ren D, Chen J - Medicine (Baltimore) (2015)

Histopathology. Hematoxylin–eosin (H&E) staining of the primary pulmonary primitive neuroectodermal tumor; immunohistochemistry staining of the primary tumor with different antibodies, such as anti-CD99, vimentin, chromogranin A (CgA), and friend leukemia integration 1 transcription factor (Fli-1). The images for the rest negative immunohistochemistry staining of thyroid transcription factor 1 (TTF-1), cytokeratin 7 (CK7), cytokeratin 19 (CK19), HMB45, protein S-100, epithelial membrane antigen (EMA), smooth muscle actin (SMA), CD34, CD20, CD56, CD31, leukocyte common antigen (LCA), and Desmin were not shown.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4504587&req=5

Figure 2: Histopathology. Hematoxylin–eosin (H&E) staining of the primary pulmonary primitive neuroectodermal tumor; immunohistochemistry staining of the primary tumor with different antibodies, such as anti-CD99, vimentin, chromogranin A (CgA), and friend leukemia integration 1 transcription factor (Fli-1). The images for the rest negative immunohistochemistry staining of thyroid transcription factor 1 (TTF-1), cytokeratin 7 (CK7), cytokeratin 19 (CK19), HMB45, protein S-100, epithelial membrane antigen (EMA), smooth muscle actin (SMA), CD34, CD20, CD56, CD31, leukocyte common antigen (LCA), and Desmin were not shown.
Mentions: In order to establish the pathological diagnosis, a left-sided video-assisted thoracoscopy (VAT) was performed and 6 nodules in the lingual segment were removed. During the operation, a number of nodules were present on the surface of both left lung lobes with protrusion, but under the visceral pleura. As shown in Figure 2, histopathologic examination showed tumor cells that exhibited small, round, and mild morphology; partial cells with more cytoplasm; and partial cells with transparent cytoplasm. The majority of cells exhibited relatively consistent nuclear size. Immunostaining was positive for the expression of CD99 and vimentin and was negative for the expression of chromogranin A, thyroid transcription factor 1, cytokeratin 7, cytokeratin 19, human melanoma black 45 (HMB45), protein S-100, epithelial membrane antigen, smooth muscle actin (SMA), CD34, CD20, CD56, CD31, leukocyte common antigen, friend leukemia integration 1 transcription factor, and Desmin. In view of the foregoing, a diagnosis of primary lung PNET was made.

Bottom Line: As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy.We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare.Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease.

View Article: PubMed Central - PubMed

Affiliation: From the Department of Lung Cancer Surgery, Tianjin Lung Cancer Institute (MD, JL, ZS, XL, DR, JC); Department of Pathology; Tianjin Medical University General Hospital, Heping District, Tianjin, China (TS, DW).

ABSTRACT
Primitive neuroectodermal tumors (PNETs) arising directly from the lung are extremely rare but particularly aggressive neoplasms. Although thoracic PNET usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.We present the case of a 16-year-old male with PNET diagnosed following histologic and immunohistochemical examination of a video-assisted thoracic surgical biopsy. As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy. We attempted to explore the use of targeted pharmacotherapy through high-throughput sequencing in this case. We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare. Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease.

Show MeSH
Related in: MedlinePlus