A case of anaplastic lymphoma kinase (ALK)-positive ciliated muconodular papillary tumor (CMPT) of the lung.

Ciliated muconodular papillary tumor (CMPT) is a rare papillary tumor that arises in the peripheral lung fields and is associated with the proliferation of ciliate d and goblet cells and increased mucin production. We report a case of CMPT involving the rearrangement of the anaplastic lymphoma kinase (ALK) gene. The patient was an 84-year-old Japanese female who had exhibited a small nodular shadow on chest computed tomography during a regular checkup 10 years ago. She underwent a partial resection of segment S10 of the right lung. The cut surface of the surgical specimen revealed a well-circumscribed, jelly-like mass measuring 8 × 8 × 10 mm. Histologically, the tumor was composed of a mixture of ciliated, goblet, and basal cells arranged in a papillary pattern together with pools of mucin. A diagnosis of CMPT was made. The lung tumor cells were subjected to fluorescent in situ hybridization and highly sensitive immunohistochemical staining for the ALK protein, both of which produced positive results. CMPT usually follows a favorable course, but the exact nature of this tumor; i.e., whether it is benign or malignant, has not been established. This is the first reported case of an ALK-positive CMPT.

Simultaneous nodular lymphocyte-predominant Hodgkin lymphoma with papillary thyroid carcinoma: a case report.

We herein report the case of a 48-year-old man diagnosed with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL, Stage IA) and papillary thyroid carcinoma (PTC, Stage I). Total thyroidectomy, left modified neck dissection and biopsy of the right cervical lymph node were performed. Postoperatively, NLPHL treatment was prioritized, and external radiation (30.6 Gy) was applied to the right neck. PTC was considered a high-risk category for recurrence due to extranodal invasion of lymph node metastasis, and radioactive iodine therapy (ablative dose, 1110 MBq) was administered. Both PTC and NLPHL showed no recurrence 18 months after surgery.

Angiomatoid fibrosis histiocytoma in the pulmonary artery: A case report.

Angiomatoid fibrosis histiocytoma (AFH) is a rare neoplastic disease. Only one report has demonstrated an intraluminal tumor of the pulmonary artery (PA) corresponding to AFH to date. We describe the case of AFH with EWSR1-CREB1 fusion occurring in the ascending artery. A 42-year-old man exhibited an abnormal nodule on chest computed tomography (CT) during checkup. It revealed an intraluminal mass in the ascending artery with significant metabolic uptake in positron emission tomography (PET)/CT. Therefore, right upper lobectomy with wedge resection of the PA trunk was performed. Histologically, the tumor was multinodular and surrounded by a dense lymphoplasmacytic cuff. Each nodule was composed of myxoid stroma and comprised ovoid or spindle cell fascicles with mild atypia. Fluorescent in situ hybridization (FISH) analysis confirmed EWSR1-CREB1 fusion. A diagnosed as AFH was made. This report widens the spectrum of differential diagnoses of primary tumors occurring in the PA.

A resected case of recurrent ITPN in the remnant pancreas after pancreatoduodenectomy.

BACKGROUND: Since intraductal tubulopapillary neoplasm (ITPN) is a rare disease, the clinical features of ITPN, especially the characteristics related to recurrence, have not been revealed. We performed a total remnant pancreatectomy for a patient whose ITPN recurred 16 months after pancreatoduodenectomy (PD). We report useful findings to clarify how ITPN reoccurs based on this experience and previously reported cases. CASE PRESENTATION: A 61-year-old male patient was diagnosed with pancreatic cancer and underwent PD. However, a postoperative pathologic examination diagnosed ITPN with invasive cancer. After receiving adjuvant chemotherapy, he was hospitalized for pancreatitis 16 months after the operation. He was diagnosed as having recurrence near the pancreato-jejunal anastomosis based on detailed examinations and underwent a remnant total pancreatectomy. From the results of the histopathological examination, he was found to have a recurrence of ITPN as a polypoid mass without invasion distant from the surgical stump of the first operation. Furthermore, tumor cells floating in the main pancreatic duct distant from the main tumor were observed at three locations. REVIEW OF THE LITERATURE: Including our case, five cases of recurrence in the remnant pancreas after surgery for ITPN have been reported. Recurrence in the main pancreatic duct was observed in four of these five cases. The primary tumor, which recurred in the remnant pancreas after surgery, was characterized as being relatively small and less invasive; however, Ki-67 labeling index was high. In immunohistochemical examination, the expression of MUC6, which is not one of characteristics of ITPN, tended to be positive. CONCLUSION: In this case, tumor cells were floating inside the pancreatic duct at several locations. From the results of this case and a review of previous reports, the cause of ITPN recurrence in this case seemed to be due to tumor cells leaving the tumor and implanting into the pancreatic duct.

Pulmonary tumor with notochordal differentiation: report of 2 cases suggestive of benign notochordal cell tumor of extraosseous origin.

Intraosseous benign notochordal cell tumor (BNCT) is a lesion postulated to be of notochordal cell origin. BNCT has recently been recognized as a potential precursor of classic chordoma, a rare malignant neoplasm usually presenting in the sacrococcygeal region, skull base, or mobile spine. Extra-axial chordoma is extremely rare, and only 2 cases of pulmonary chordoma have been reported previously. We describe herein 2 cases of hitherto-unreported lung tumors that were diagnosed as BNCT. The patients were a middle-aged asymptomatic man and woman who were each incidentally found to have a 15-mm pulmonary nodule on computed tomography. They underwent surgical resection of the tumors under a diagnosis of probable benign tumor of uncertain nature. Histopathologically, both tumors showed solid sheets of peculiar adipocyte-like univacuolated cells, multivacuolated cells, and less vacuolated cells with small, round nuclei and mildly eosinophilic cytoplasm. Mitosis was absent. These features were typical of BNCT. Immunohistochemically, the tumor cells in both cases were positive for brachyury, a transcription factor essential for notochordal cell differentiation and for other markers of notochordal cells including cytokeratins, vimentin, and S-100 protein. Postoperatively, extensive radiographic examination of the whole body revealed no evidence of a primary tumor elsewhere, and both patients are alive and well, with no evidence of disease 1 year after surgery. These 2 cases raise the possibility of a new explanation for the histogenesis of extra-axial chordomas: BNCT may be a precursor lesion of not only conventional axial chordoma but also of extra-axial chordoma.