Want to take quizzes and track your credits?
A young man in his early 20s presented with a 1-day history of pleuritic chest pain and shortness of breath, with no fever or cough. He smoked approximately 10 cigarettes per day for 4 years. Results of the physical examination were unremarkable. Radiography of the chest revealed an ill-defined consolidation in the right lower lung field. Laboratory investigations were significant for elevated levels of lactate dehydrogenase (273 U/L; reference range, 135-225 U/L [to convert to microkatals per liter, multiply by 0.0167]), C-reactive protein (125.3 mg/L; reference range, 0.0-4.9 mg/L [to convert to nanomoles per liter, multiply by 9.524]), and alkaline phosphatase (217 U/L; reference range, 40-129 U/L [to convert to microkatals per liter, multiply by 0.0167]). Computed tomography of the chest confirmed the presence of a dense consolidation involving most of the right middle lobe with right hilar and subcarinal lymphadenopathy (Figure, A). He underwent endobronchial ultrasonography-guided transbronchial needle aspiration of a fleshy, whitish endobronchial lesion obstructing both uptakes to the medial and lateral segments. Histologic analysis revealed a poorly differentiated malignant neoplasm (Figure, B). Most of the cells were relatively monotonous, with enlarged vesicular nuclei, prominent nucleoli, and a high ratio of nucleus to cytoplasm. Foci of abrupt squamous differentiation, including keratinization and intercellular bridges, were identified. Immunohistochemical staining showed tumor cells to be strongly and diffusely positive for keratin AE1/AE3, cytokeratin 5/6, and p63. However, findings were negative for S-100, melan A, placental alkaline phosphatase, CD56, thyroid transcription factor-1, terminal deoxynucleotidyl transferase, CD45, and cytokeratin 7. A positron emission tomography–computed tomography scan showed a fludeoxyglucose-enhanced avid right middle lobe mass, a large right-sided pleural effusion, and fludeoxyglucose-enhanced avid mediastinal and right hilar lymph nodes, with extensive skeletal metastases to both proximal humeri and scapulae, the sacrum, spine, iliac bones, and both hips.
Please finish quiz first before checking answer.
Read the answer below and download your certificate.
Read the discussion below and retake the quiz.
D. Nuclear protein in testis midline carcinoma
Nuclear protein in testis (NUT) midline carcinoma (NMC) is a rare aggressive human cancer that presents as a poorly differentiated carcinoma originating from midline locations and does not arise from any specific tissue type or organ.1 NUT midline carcinoma is driven by a rearrangement of the NUT gene (OMIM 608963) located on chromosome 15. This rearrangement commonly occurs between the NUT gene and a member of the BET genes family, most commonly BRD4 (OMIM 608749), resulting in the formation of the fusion oncogene BRD4-NUT.2,3 NUT midline carcinoma is extremely aggressive, with most cases presenting at an advanced stage as widely metastatic and unresectable disease when diagnosed,3,4 and has a highly lethal course.5
Sign in to take quiz and track your certificates
JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC
Corresponding Author: Radowan Elnair, MD, Department of Internal Medicine, University of South Dakota Sanford School of Medicine, 1400 W 22nd St, Sioux Falls, SD 57105 (firstname.lastname@example.org).
Published Online: August 9, 2018. doi:10.1001/jamaoncol.2018.2648
Conflict of Interest Disclosures: None reported.
Additional Contributions: Jennifer Boland Froemming, MD (Mayo Clinic, Rochester, Minnesota), and Christopher French, MD (Brigham and Women’s Hospital, Boston, Massachusetts), assisted with and contributed to establishing the diagnosis. They received no compensation for this work.
You currently have no searches saved.