Demystifying NUT Midline Carcinoma: Radiologic and Pathologic Correlations of an Aggressive Malignancy

Bair, Ryan J.; Chick, J.; Chauhan, Nikunj Rashmikant; French, Christopher A.; Madan, Rachna

doi:10.2214/ajr.13.12401

Cited by 37 publications

(55 citation statements)

References 37 publications

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“…Selected radiographic, pathologic and/or molecular features of cases 3, 5, 6, 7 and 8 have been included in previous reports. 1,13,15,16 The mean age of the patients at the time of diagnosis was 41 years (median 30, range 21-68). Clinical history was available for 7 patients.…”

Section: Resultsmentioning

confidence: 99%

Primary Pulmonary NUT Midline Carcinoma: Clinical, Radiographic, and Pathologic Characterizations

Sholl

Nishino

Pokharel

et al. 2015

Journal of Thoracic Oncology

108

145

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NUT midline carcinoma (NMC) is a poorly differentiated tumor typically driven by a t(15;19) rearrangement leading to a NUT fusion event. This rare and uniformly fatal tumor arises in multiple organ sites, however the clinical, radiographic, and pathologic characteristics of primary pulmonary NMC are poorly defined. We identified eight cases of primary pulmonary NMC in our consult practice over four years and, using a NUT immunohistochemistry screen, retrospectively identified one additional case from 166 (0.6%) consecutive in-house biopsies of lung carcinomas lacking glandular differentiation. Eight cases had available clinical and radiographic data and shared a remarkable degree of similarity. The median age at presentation was 30 (range 21-68). Six patients had little or no smoking history. All complained of one to three months of cough at presentation. Computed tomography scans showed a large, centrally-located primary mass with confluent involvement of mediastinal lymph nodes, pleural disease, and sparing of the contralateral lung. Lytic bone metastases were common but brain metastases were absent in all cases. Pathologically, all cases showed primitive-appearing round to epitheloid cells growing in nests and sheets. All tumors expressed keratin, p63 or p40, and NUT protein. Eight cases had a FISH-proven BRD4-NUT or BRD3-NUT rearrangement; one case was presumed to have a NUT-variant fusion event. Median overall survival was 2.2 months. Despite the rarity of primary pulmonary NMC, it is important to recognize this entity in order to counsel patients regarding outcome and to identify candidates for targeted BRD inhibitors currently in clinical trials.

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Section: Resultsmentioning

confidence: 99%

Primary Pulmonary NUT Midline Carcinoma: Clinical, Radiographic, and Pathologic Characterizations

Sholl

Nishino

Pokharel

et al. 2015

Journal of Thoracic Oncology

108

145

View full text Add to dashboard Cite

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“…CT images are most frequently characterized by hypoattenuation and heterogenous enhancement of an infiltrative or destructive appearing mass. Occasional intralesional calcifications have been reported [7]. Despite the frequent evidence of disregard for normal tissue boundaries, the current case displayed some evidence circumscription and osseous remodeling.…”

Section: Diagnosismentioning

confidence: 49%

“…MR images also characteristically demonstrate a heterogenous mass with hypointense T1 and hyperintense T2 signalling, although the current case was only mildly hyperintense on both. MRI is the preferred modality with regards to delineation of tumor margins and for treatment planning [7]. The non-specific radiographic features of NMC necessitate the development of a broad differential diagnosis with primary consideration given to aggressive malignancies, including sinonasal undifferentiated carcinoma, neuroendocrine carcinoma, melanoma, rhabdomyosarcoma, Ewing sarcoma family tumors and hematologic malignancies.…”

Section: Diagnosismentioning

confidence: 99%

NUT Midline Carcinoma of the Nasal Cavity

Edgar

Caruso

Kim

et al. 2016

Head and Neck Pathol

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Nuclear protein in testis (NUT) midline carcinoma (NMC) is a rare, aggressive, poorly differentiated form of squamous cell carcinoma caused by a chromosomal rearrangement of the NUT gene on chromosome 15. These tumors have a predilection for midline and paramidline structures of the upper aerodigestive tract and mediastinum and can affect patients across a broad age range, including children. In the current example, a 53 year old male presented with a mass originating in the left nasal cavity. The clinical, radiographic, and morphologic features of NMC are discussed.Keywords NUT midline carcinoma Á Undifferentiated carcinoma Á Nasal Á Nasal cavity Á Sinonasal Á Nuclear protein of testis Á Radiology HistoryA 53 year old male presented with a 9 month history of left nasal congestion, rhinorrhea, and sinus tenderness. The patient also noted recurrent epistaxis lasting longer than 30 min 6 months prior to presentation. Additionally, patient reported recent onset of left facial numbness in the V 2 distribution of the trigeminal nerve. He denied fevers and weight loss, but did note drenching night sweats. Radiographic FeaturesSinus CT examination revealed a soft tissue mass which completely obstructed the left nasal cavity. Coronal views delineated osseous expansion with erosion and destruction of the nasal septum and medial wall of the left maxillary sinus (Fig. 1a). There was opacification of left ethmoid air cells and the left maxillary sinus. Corresponding MR images demonstrated an 8.4 9 3.3 9 2.3 cm mass centered in the left nasal cavity (Fig. 1b). The mass was heterogeneously enhancing on postcontrast T1-weighted images and displayed predominantly isointense signaling with T2-weighted studies. The mass measured 8.4 cm in anterior-posterior dimension, including a pedunculated component that extended past the choana into the nasopharynx (Fig. 2). No intracranial extension or erosion into the orbits or cribriform was present. Brain and neck MRI evaluation revealed no evidence of brain metastasis or cervical lymphadenopathy. TreatmentFollowing an incisional biopsy, the patient underwent functional endoscopic sinus surgery including a left maxillary antrostomy, total ethmoidectomy, sphenoidotomy,

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“…Adjunctive MRI is performed if there is a concern for the chest wall, vascular, or cardiac invasion. [4] The signal intensity on T2-weighted MRIs is that of a cellular neoplasm, but imaging characteristics are otherwise indistinguishable from other high-grade neoplasms such as lymphoma or sarcoma. [5] FDG-PET/CT is the preferred modality for guiding biopsy to viable tissue, staging and assessment of metastatic disease, evaluation of disease treatment response, and restaging with assessment of disease extent and severity over time.…”

Section: Discussionmentioning

confidence: 99%

“…[5] FDG-PET/CT is the preferred modality for guiding biopsy to viable tissue, staging and assessment of metastatic disease, evaluation of disease treatment response, and restaging with assessment of disease extent and severity over time. [4]…”

Section: Discussionmentioning

confidence: 99%

Nuclear protein in testis midline carcinoma presenting in an infant as a pericardial mass with staging by 18F-fluorodeoxyglucose-positron emission tomography/computed tomography

et al. 2017

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Nuclear protein in testis (NUT) midline carcinoma (NMC) is a rare, aggressive, lethal, epithelioid, poorly differentiated cancer first described in Japan in 1991, unique in that is defined genetically rather than by histological tissue of origin. It usually arises in the body midline and presents as a mass with metastasis. An infant presenting with pneumonia was found to have a pericardial mass, NMC resected, and subsequent staging positron emission tomography (PET) showing residual mediastinal tumor and midline abdominal metastases. Fewer than 100 cases of NMC have been reported in the literature, and PET appears to be the imaging modality of choice in complete staging and evaluation of treatment response.

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Demystifying NUT Midline Carcinoma: Radiologic and Pathologic Correlations of an Aggressive Malignancy

Cited by 37 publications

References 37 publications

Primary Pulmonary NUT Midline Carcinoma: Clinical, Radiographic, and Pathologic Characterizations

Primary Pulmonary NUT Midline Carcinoma: Clinical, Radiographic, and Pathologic Characterizations

NUT Midline Carcinoma of the Nasal Cavity

Nuclear protein in testis midline carcinoma presenting in an infant as a pericardial mass with staging by 18F-fluorodeoxyglucose-positron emission tomography/computed tomography

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