Ciliated Muconodular Papillary Tumor Research Articles

Ciliated Muconodular Papillary Tumors of the Lung Harboring STRN::ALK Fusion: Case Report and Review of the Literature.

Ciliated muconodular papillary tumor (CMPT) is a rare pulmonary tumor, typically occurring in middle-aged and elderly individuals. The molecular mutation spectrum of CMPT remains insufficiently explored. Commonly known driver gene alterations include KRAS, BRAF, EGFR, and ALK rearrangement. This report details the clinicopathological features of 2 patients presenting with CMPT as pulmonary nodules during clinical examinations. Microscopic analysis revealed tumors with glandular or papillary structures, consisting of mucinous cells, ciliated columnar cells, and basal cells. Notably, both patients exhibited STRN::ALK fusion, a finding not previously associated with CMPT. STRN::ALK fusion serves as a target for therapy in various tumors, including non-small cell lung cancer, thyroid cancer, and colon cancer. Consequently, we conducted a review of relevant literature, summarizing the clinicopathological and molecular characteristics of CMPT to facilitate further research. Our insights enhance the understanding of this uncommon tumor and contribute to the expansion of its molecular alteration spectrum.

Computed tomography (CT) characteristics and pathologic basis of ciliated muconodular papillary tumors of the lung.

Ciliated muconodular papillary tumor (CMPT) is a rare pulmonary tumor with papillary architecture. Most studies have focused on the clinicopathological features of CMPT, while computed tomography (CT) characteristics have rarely been systematically described. A cohort of 27 patients with surgically resected CMPT were identified. Clinical and demographic features were recorded. Preoperative CT images of the CMPTs and the corresponding histopathological basis were also retrospectively analyzed. All of the tumors appeared as solitary nodules. Pure ground glass, part-solid nodules and solid nodules were detected in 2/27 (7.4%), 17/27 (63.0%), and 8/27 (29.6%) patients, respectively. Twenty-one tumors (77.8%) were located in the lower lobe. The average tumor size was 1.21±0.74 (range, 0.44-3.46) cm. Eighteen (66.7%) of the 27 patients had tumors with well-defined margins and lobulated contours. Fifteen patients (55.6%) had air bronchograms in the tumor, and 19 patients (70.4%) had air-containing space. There were two patients whose tumor size was enlarged and accompanied by an increase in solid components, and one patient simply had an increase in tumor size at the preoperative follow-up duration. Notably, one patient with solid tumor components was finally diagnosed with CMPT accompanied by adenocarcinoma. CMPTs of the lung mostly manifest as solitary, lobulated, well-defined tumors with air-containing spaces on CT and often occur in the periphery of the pulmonary lower lobe. When CT findings meet these criteria, the possibility of CMPT should be considered. Additionally, CMPT can coexist with adenocarcinoma. Further investigation will contribute significantly to the biological properties of CMPT and its relationship to the potential for malignant transformation.

A surgical case of ciliated muconodular papillary tumor of the lung

Ciliated muconodular papillary tumor (CMPT) is a rare type of tumor with both benign and malignant characteristics. Herein, we report the surgical case of a 65-year-old man with CMPT. Chest computed tomography revealed a solitary cavitary lesion with a maximum diameter of 11-mm in S10b of the right lower lung. A thoracoscopic lung wedge resection was subsequently performed. On microscopic examination, the tumor was composed of highly columnar cells with tubular-to-papillary and cystic growth patterns. On immunostaining, it was positive for the epidermal growth factor receptor. The tumor was diagnosed as a CMPT, exhibiting no recurrence after two years of follow-up.

Ciliated muconodular papillary tumor of the lung with cavitary change: A case report with 11‐year preoperative follow‐up

Ciliated muconodular papillary tumor (CMPT) is a rare benign lung tumor characterized by ciliated columnar cells, mucous cells, and basal cells. Herein, we report a case of CMPT with 11 years of preoperative follow‐up, depicting the natural course of the tumor and changes in computed tomography (CT) findings. A 39‐year‐old man had a 5‐mm solid pulmonary nodule in the right lower lobe that had slowly grown and transformed into a thin‐walled cavitary lesion. Right lower lobe lobectomy was performed and the tumor was confirmed to be a CMPT. Although it is difficult to diagnose CMPT with CT findings alone, CMPT should be considered as a possible diagnosis when a slowly growing nodule undergoes cavitary changes.

A clinicopathologic and molecular analysis of five cases of bronchiolar adenoma with rare mutations.

Bronchiolar adenoma (BA) is a rare benign lung tumor that shows proliferation of bland bronchiolar-type epithelium containing a continuous layer of basal cells. This tumor entity has been newly added to the recent World Health Organization (WHO) classification 5th edition. This entity encompasses a spectrum of lesions: the classic ciliated muconodular papillary tumor (CMPT) and the non-classic CMPT. Although BA is reported to have driver mutations including BRAF V600E, EGFR, and KRAS, the molecular profile of BA is still incompletely understood. Five resected BAs at our institutions were analyzed. The BA lesions were subdivided into two groups: three proximal-type BAs and two distal-type BAs. NRAS codon 12/13 mutation and EML4 exon 20-ALK exon 20 fusion were found in two of the three proximal-types. BRAF V600E mutation was found in one of the two distal-types. Two cases coexisted with lung adenocarcinoma, with EGFR exon 19 deletion and KRAS mutation, respectively. No recurrence was observed at a median of 12 months (range 2-84 months) of follow-up. BA has uncommon variants of mutation seen in lung adenocarcinoma. NRAS mutation and ALK fusion partner has not been reported previously. The present cases may reinforce the distinctive biology of BA from lung adenocarcinoma.

Resection of Synchronous Multiple Ciliated Muconodular Papillary Tumor: a Case Report

Resection of Synchronous Multiple Ciliated Muconodular Papillary Tumor: a Case Report

Primary ciliated muconodular papillary tumor: A rare pulmonary disease and literature review of 65 cases.

A ciliated muconodular papillary tumor (CMPT) or bronchiolar adenoma (BA) is a rather rare and unique type of lung tumor characterized by tripartite cellular components with a papillary‐predominant structure including ciliated columnar cells, mucinous cells, and basal cells. Here, we present the case of a 64‐year‐old woman who was diagnosed with CMPT in our center. In addition to reporting the clinicopathological characteristics of this case, we also conducted whole exome sequencing (WES) to explore the underlying mechanism. According to current evidence, CMPTs tends to be benign or of low grade malignancy. However, this requires further validation.

Ciliated muconodular papillary tumor/bronchiolar adenoma of the lung

Ciliated muconodular papillary tumor (CMPT) is an uncommon tumor of the lung, usually detected incidentally in the peripheral lung fields on computed tomography. It is most commonly diagnosed in patients between the sixth and last decades of life. The canonical microscopic feature is a tripartite mixture of ciliated columnar, mucous, and basal cells with papillary architecture. However, studies have shown wide morphological variations in this tumor, such as a lack of ciliated columnar or mucous cells and flat instead of papillary architecture. Hence, a new umbrella term, bronchiolar adenoma (BA), has been proposed to encompass all tumors comprising a uniform layer of basal cells underneath the luminal epithelium displaying bronchiolar differentiation. Recent molecular studies on CMPT/BA discovered a high frequency of driver gene alterations, including mutations in the BRAF, EGFR, AKT1, KRAS, and HRAS genes, as well as ALK gene rearrangements. The tumor follows an indolent clinical course, as no recurrences or metastases have been reported in patients who underwent surgical resection. In this article we review the clinical, radiological, and pathological features, and molecular characteristics of CMPTs/BAs.

The clinical characteristics and surgical management of ciliated muconodular papillary tumor

Objective: To investigate the clinical characteristics and surgical management based on the clinical manifestation, pathological feature and the medical imaging finding of ciliated muconodular papillary tumor (CMPT). Methods: The data of clinical manifestation, pathological feature and the medical imaging finding of 15 patients with CMPT who received surgical treatment from January 2017 to April 2019 were collected and retrospectively analyzed. Results: CMPT generally occurred in the elderly people. Most of the diameter of the tumor was less than 1 cm, while the diameters of other 3 patient were 1~2 cm. The computed tomography (CT) scan of 9 patients displayed solid nodule, while 4 displayed ground glass opacity (GGO), and other 2 showed no significant abnormal. Thirteen patients received minimally invasive video-assisted thoracoscopic surgery (VATS), the other 2 received open surgery. Eight patients received lobectomy, 3 received thoracoscopic anatomical partial-lobectomy, 4 received wedge resection. The frozen section diagnostic results of 8 patients were adenocarcinoma, including 3 mucinous adenocarcinoma. Other 5 patients were diagnosed as CMPT and 2 were reported as infection nodule. During the perioperative period, 2 patients occurred cardiac arrhythmia, 1 occurred pulmonary infection. None of the patients had local recurrence or distant metastasis during the follow-up. Conclusions: CMPT usually presents as solitary peripheral lung nodules without obvious symptoms. Most CMPTs are incidentally detected by routine CT scan. Some of the cases are accompanied by primary lung cancer probably. Surgical treatment is the major therapy for CMPT. The imaging feature of CT scam usually shows a solid nodule or a GGO locates in peripheral pulmonary. Frozen section diagnosis for CMPTs can be easily confused with adenocarcinoma or mucinous adenocarcinoma.

Ciliated Muconodular Papillary Tumor of the Lung: a Surgical Case Report

We describe a surgical case of ciliated muconodular papillary tumor (CMPT) of the lung. A 48-year-old man presented to our hospital complaining of right chest pain. Computed tomography (CT) revealed a part-solid grand glass nodule (GGN) in the superior segment of the lower lobe (S6) of the right lung. The maximum diameter of the GGN was 7 mm, with the solid portion having a 5 mm diameter. On follow-up CT, performed 14 months after the first examination, the size of the mass was stable, with almost the same extent of pleural indentation. A CT scan obtained 12 years previously had shown no evidence of a tumor. Considering the continued growth of the lesion, an adenocarcinoma was suspected, and a wedge resection of the right lower lobe was performed. The intra-operative pathological diagnosis by frozen section revealed a dysplastic epithelium. Thus, we did not proceed with lobectomy and lymph node dissection. Postoperative pathological diagnosis of CMPT was confirmed. For comparison, we included an overview of 41 cases of CMPT previously published in 17 studies. In these cases, computed tomography was performed over the follow-up period in 13 cases (31.7%). Tumor growth, which mimicked malignancy, was observed in 9 of 13 cases (69.2%). Since it is usually difficult to make a diagnosis pre- and intra-operatively, most cases of CMPT are pathologically diagnosed postoperatively. During surgery for small peripheral pulmonary tumor like lung cancer, which is not definitive in intra-operative pathological diagnosis, CMPT should be considered as one of differential diagnoses.

Ciliated muconodular papillary tumor of the lung: 18F-FDG PET/CT findings of 15 cases.

Ciliated muconodular papillary tumor (CMPT) is a rare lung tumor that was first reported in 2002. This study assessed 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) findings of CMPT of the lung. FDG PET/CT findings of 15 patients (eight men and seven women; median age, 67years) with surgically resected CMPTs were retrospectively analyzed. Size, location, and maximum standardized uptake values (SUVmax) of CMPTs were measured. Histopathological features of the resected tumors were assessed and compared with the FDG PET/CT findings. CMPTs were detected as a small pulmonary nodule in all 15 patients. Twelve of 15 tumors were found in the lower lobe of the lung. Mean maximal diameter of the tumors was 9mm (range 6-14mm). All but one tumor showed low FDG uptake, with a SUVmax ranging from 0.57 to 1.35. The remaining tumor showed moderate FDG uptake, with a SUVmax of 3.67. Pathologically, tumors with low FDG uptake contained various amounts of mucin and no or only a small amount of lymphocyte infiltration. In contrast, the tumor with moderate FDG uptake had a large cellular component and prominent lymphocyte infiltration. CMPT typically shows low FDG uptake.

Ciliated Muconodular Papillary Tumor of the Lung: Thin-Section CT Findings of 16 Cases.

OBJECTIVE. The purpose of this article was to assess thin-section CT features of ciliated muconodular papillary tumors (CMPTs) of the lung and correlations between radiologic and pathologic findings. MATERIALS AND METHODS. Thin-section CT findings of 16 patients (10 men and six women; mean age, 70.7 years) with surgically resected CMPTs were retrospectively analyzed. Size, location, and internal characteristics of the tumors were evaluated. The amount of mucin in the tumors was assessed histopathologically and compared with CT findings. Tumor growth speed was calculated on the basis of size changes on thin-section CT. RESULTS. In all 16 patients, tumors were detected as a solitary pulmonary nodule. Thirteen tumors (81.3%) were located in the lower lobes, and 10 (62.5%) were adjacent to the pleura. Mean maximal diameter of the tumors was 9.1 mm (range, 6-14 mm). One tumor (6.3%) presented as a pure ground-glass nodule (GGN), seven (43.8%) as dense GGNs, and eight (50.0%) as solid nodules. Pathologically, the pure GGN and five of seven dense GGNs had a large amount of mucin, whereas seven of eight solid nodules had an intermediate or small amount of mucin. The mean annual tumor growth rate (in diameter) was 0.49 mm/y. CONCLUSION. CMPTs appear as solitary, small, and peripheral pulmonary nodules with very slow growth rates. CMPTs appearing as pure GGNs and dense GGNs tend to contain more mucin than CMPTs appearing as solid nodules.

A rare case of contralateral ciliated muconodular papillary tumor after limited surgery

Ciliated muconodular papillary tumor (CMPT) was a rare tumor with low-grade malignancy. The histopathogenesis of CMPT remains unclear. Three typical components are ciliated columnar cells, goblet cells, and basal cells. Limited resection is sufficient for CMPT. No recurrences or metastases have been reported. Herein, we reported a 73-year-old man who presented with right lower lobe partial sold nodule. He received partial resection for the right lower lobe tumor. CMPT was confirmed. However, contralateral left lower lobe ground-glass opacity (GGO) was found 3 months later. He received partial resection for the left lower lobe GGO. CMPT was diagnosed again. Double primary CMPTs were recognized because morphologic patterns were different. At his 2-year follow-up, there was no local recurrence or metastasis.

A Case of Pulmonary Mucoepidermoid Carcinoma with Cilia That Was Difficult to Differentiate from Ciliated Muconodular Papillary Tumor

A Case of Pulmonary Mucoepidermoid Carcinoma with Cilia That Was Difficult to Differentiate from Ciliated Muconodular Papillary Tumor

Clinicopathological features and prognosis of ciliated muconodular papillary tumor

BackgroundsThe pulmonary ciliated muconodular papillary tumor (CMPT) is a very rare tumor with only several case reports in published literatures, and its clinicopathological features, standard treatment methods and prognosis has not been well defined.MethodsTwo cases of CMPT diagnosed and treated in our hospital and 39 cases reported in the published literature were analyzed retrospectively.ResultsThe cohort of 41 CMPT patients comprised of 20 males and 21 females, aged 9–84 years. The diameter of the primary tumor was 0.3–4.5 cm. Most of these lesions were subsolid nodules, as observed on computed tomography and easily misdiagnosed as early lung adenocarcinoma. Tumors of 26 patients were stained by immunohistochemistry method, which revealed that CK7, CEA, and TTF-1 were positive and CK20 was negative in most patients. The results of gene alternation demonstrated mutations in EGFR, KRAS, and BRAF and ALK rearrangements in CMPT. All the patients underwent surgical treatment and did not receive postoperative adjuvant therapy. The follow-up duration was 0–120 months, and no case of tumor recurrence was found until the final follow-up.ConclusionsThe incidence of CMPT was low and rate of image misdiagnosis high. Immunohistochemistry is helpful for accurate diagnosis of CMPT. Sub-lobectomy may be proper and adjuvant treatment should be avoided since the disease is now prone to benign lesions. Furthermore, since the biological behavior of this tumor is not yet fully elucidated, additional case data are essential for accurate conclusions.

Expanding the Spectrum of Ciliated Muconodular Papillary Tumor of the Lung: A Case Showing Nonpapillary and Purely Glandular Morphology

Abstract We report an unusual case of a subpleural bronchiolar/glandular proliferation in a 76-year-old Hispanic woman with microscopic features reminiscent of those of the so-called ciliated muconodular papillary tumor (CMPT). The lesion presented radiologically as a solitary subpleural nodule measuring 2.7 × 1.2 cm with low standard uptake value (SUV) of 1.4. Microscopically, it was circumscribed but unencaspulated and consisted of mucous-filled spaces lined by cytologically bland cuboidal to low columnar cells, including goblet cells and ciliated epithelium, with underlying nodular collections of basaloid cells. The lining cells stained diffusely for CK7 but only focally for TTF-1 and CEA, whereas the basal cells expressed CK5/6. Ki-67 showed a proliferative index of less than 1%. Target gene mutation analysis with a 50-cancer-related-gene panel using next-generation sequencing showed the bronchiolar proliferation to harbor an EGFR in frame exon 20 insertion mutation with 30% allele frequency, which supports a neoplastic diagnosis. Accordingly, given the mutually exclusive nature of driver mutations, a BRAF V600E mutation was absent by both immunohistochemical and next-generation sequencing analysis. Microscopic evidence of metastatic spread was not seen in 8 regional lymph nodes examined. Given the histomorphology and immunohistochemical findings, the mutational profile and the reported heterogeneous microscopic patterns of CMPTs, including glandular component, we suggest the tumor herein described be a glandular variant of CMPT. We therefore suggest that the term ciliated muconodular tumor may be more appropriate for this group of lesions.

Ciliated muconodular papillary tumor of the lung harboring ALK gene rearrangement: Case report and review of the literature

Ciliated muconodular papillary tumor (CMPT) of the lung is an extremely rare peripheral tumor of the lung. The pathogenesis of CMPT is still unknown, and its nature as a benign tumor or reactive process is still open to discussion. Recent studies have identified BRAF, EGFR and AKT1 mutations in CMPT, which would support a true neoplastic process. Here for the first time, we report a case of morphologically typical CMPT harboring ALK gene rearrangement to further provide convincing evidence that CMPT is a neoplastic process rather than a reactive lesion. The patient was a 59-year-old woman, characterized by a circumscribed tubulopapillary tumor consisting of ciliated columnar cells, mucous cells, and basal cells, accompanied with peripheral abundant extracellular mucin. The tubulopapillary architecture with abundant extracellular mucin is mimicking adenocarcinoma. The tumor cells were immunoreactive for cytokeratin 7, thyroid transcription factor-1, whereas p40 and p63 highlighted the presence of basal cells. The ALK gene rearrangement was detected using fluorescence in situ hybridization and Ventana immunohistochemistry platform. To our knowledge, this is the first study to confirm CMPT harboring ALK gene rearrangement.

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.

Ciliated muconodular papillary tumor of the lung: The risk of false-positive diagnosis in frozen section

Ciliated muconodular papillary tumor (CMPT) is newly-defined low-grade malignant tumor, characterized as papillary tumor consisting of ciliated columnar, goblet, and basal cells. We present one case of pulmonary peripheral neoplasm misdiagnosed as mucinous adenocarcinoma. Pathologic findings showed, centrally, cystic papillary growth consisting of ciliated columnar, goblet, and basal cells, and peripherally, some tumor cells spread along the adjacent alveolar walls, in a lepidic fashion, and floating in pool of mucin. Tumor cells had bland nuclei, and no mitotic activity was observed. This had been initially misdiagnosed as mucinous adenocarcinoma in frozen section. We reviewed previous articles as well as permanent and frozen slides. In conclusion, in order to reduce the possibility of false positives, it is important to be aware that CMPT is a rare form of peripheral mucin-producing tumor with characteristic histologic findings.

Ciliated Muconodular Papillary Tumor of the Lung: A Newly Defined Low-grade Malignant Tumor with CT Findings Reminiscent of Adenocarcinoma

A ciliated muconodular papillary tumor has been reported to be a peripheral low-grade malignant tumor, consisting of ciliated columnar cells and goblet cells with basaloid cell proliferation. Although ciliated muconodular papillary tumors have not yet been classified according to the World Health Organization classification, they can pose diagnostic and therapeutic problems. Here we report a resected case of ciliated muconodular papillary tumor with computed tomography findings reminiscent of adenocarcinoma, showing a small irregular nodule adjacent to the intersegment pulmonary vein. There was no uptake of F-18 fluorodeoxyglucose positron emission tomography. The patient underwent surgical resection, and a lobectomy was performed because intraoperative needle biopsy suggested neoplastic proliferation. No EGFR mutations were detected. No recurrence was noted during 24-month follow-up after lobectomy.