NUT midline carcinoma as a primary lung tumor: a case report

Cao, Jianzhu; Chen, Donghong; Yang, Fan; Yao, Jiayi; Zhu, Weipeng; Zhao, Chenyang

doi:10.21037/jtd.2017.11.50

Cited by 15 publications

(12 citation statements)

References 19 publications

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“…Importantly the patient has demonstrated signs of improved quality of life since beginning treatment, such as the ability to exercise again. The median survival time for NMC patients from diagnosis is reported to be less than 7 months, and there is a greater than 80% likelihood of death within the first year of diagnosis for adult patients [1] [18] [19]. So far as current literature suggests, there appears only two cases of NMC patients reporting longer survival times than the patient presented here.…”

Section: Discussionmentioning

confidence: 53%

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Prolonged Disease Stabilization and Tolerability in a Nuclear Protein in Testis Midline Carcinoma Patient Treated with Dual Histone Deacetylase and Phosphoinositide 3-Kinase Inhibitor CUDC-907

Münster¹,

Wu²,

McMahon³

et al. 2018

CRCM

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Introduction: Nuclear protein in testis midline carcinoma (NMC) is a rare and extremely aggressive carcinoma (median survival < 7 months) with no effective treatment options. CUDC-907 is a novel small molecule inhibitor of histone deacetylase (HDAC) and phosphoinositide 3-kinase (PI3K) enzymes currently being investigated in multiple tumor types, including NMC. Case Report: A 61-year old female NMC patient enrolled on study CUDC-907-102 (NCT02307240) after rapidly progressing through two prior treatments. The patient's assessable sites of disease consisted of right pleural effusion, right hilar soft tissue, and segment IV liver. Treatment was well tolerated with toxicities primarily consisting of manageable diarrhea and thrombocytopenia. The patient remains on active treatment after more than 32 months of stable disease. Discussion: Dysregulation of MYC in NMC is believed to play a central role in pathogenesis. CUDC-907 has demonstrated potent suppression of MYC expression and anti-tumor activity in preclinical NMC models, providing a mechanistic rationale for the prolonged disease stabilization observed here. The treatment of additional NMC patients with CUDC-907 is needed to further evaluate this promising report. Conclusion: This case demonstrates a rare success in the treatment of a devastating disease using only a novel small molecule, warranting further investigation of CUDC-907 in NMC.

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Section: Discussionmentioning

confidence: 53%

“…Presently there is no standard of care treatment in NMC due to the rarity and fulminant nature of the disease [18]. BET inhibitors have been shown to competitively inhibit BRD-NUT chromatin interactions, down regulate MYC and MYC-dependent gene expression, and promote cellular differentiation [22].…”

Section: Discussionmentioning

confidence: 99%

Prolonged Disease Stabilization and Tolerability in a Nuclear Protein in Testis Midline Carcinoma Patient Treated with Dual Histone Deacetylase and Phosphoinositide 3-Kinase Inhibitor CUDC-907

Münster¹,

Wu²,

McMahon³

et al. 2018

CRCM

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show abstract

“…This could not be performed in the presented case due to the diffuse metastatic disease. Considering that there exists no standard treatment for NUT midline carcinoma until now [ 5 , 27 ], the case was discussed interdisciplinary. A palliative systemical chemotherapy was started with nab-paclitaxel and carboplatin.…”

Section: Discussionmentioning

confidence: 99%

NUT midline carcinoma in a young pregnant female: a case report

et al. 2020

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Introduction The NUT midline carcinoma is a rare tumor mostly reported in the midline of upper aerodigestive tract and mediastinum. Children as well as adolescents are affected without a gender distribution. A standard treatment is not established. So far, there exists no reported case of a pregnant female suffering from NUT midline carcinoma with musculoskeletal manifestation. Case presentation A 34-year-old woman was referred to our outpatient clinic by the general practitioner during her 31st week of pregnancy suffering from shoulder pain and dyspnea. So far, dyspnea was interpreted as a typical pregnancy-related symptom. However, a chest X-ray showed a tumor mass in the right lung in close relation to the scapula. Further examinations found metastases in different areas of the body. No pregnancy-related complications were detected by obstetric examination. After an interdisciplinary perinatal case discussion, cesarean section was directly followed by an open biopsy of the right side scapula tumor lesion. A NUT midline carcinoma was diagnosed by immunohistochemistry. Due to disseminated tumor disease in multiple non-resectable locations, a palliative systemical chemotherapy was started by the oncological outpatient clinic. Conclusion This report presents the case of the very rare NUT midline carcinoma under pregnancy which made interdisciplinary case discussions indispensable for therapy planning.

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“…To the best of our knowledge, primary pulmonary NCs are rare, with approximately 32 cases reported to date 12‐30 . Because NCs are aggressive tumors that usually present at advanced stages, cytology is likely to be the primary diagnostic modality.…”

Section: Introductionmentioning

confidence: 99%

Cytomorphology of primary pulmonary NUT carcinoma in different cytology preparations

Dutta

Nambirajan

Mittal

et al. 2020

Cancer Cytopathology

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Background NUT carcinoma (NC) is an aggressive neoplasm that often presents with alarge tumor burden and metastases; cytology is frequently one of the primary diagnostic modalities. Primary pulmonary NCs are very rare and cytology descriptions are limited. The current study was performed to analyze the cytomorphological features of primary pulmonary NCs in different cytology samples and preparations. Methods A total of 15 cytology specimens from 10 patients with primary pulmonary NCs diagnosed primarily on histology were retrieved and reviewed. Results Fifteen cytology samples, including aspirates from primary (5 samples) and metastatic (5 samples) sites, sputum (1 sample), and effusions (4 samples), that were prepared as direct smears, centrifuged smears, and cell blocks were reviewed. Aspirate smears from all cases were cellular and demonstrated fragments and cohesive clusters of primitive tumor cells with scant cytoplasm, ovoid nuclei with coarse granular chromatin, and consistently conspicuous single nucleoli in a frequently neutrophil‐rich necrotic background with dispersed bare tumor nuclei. In fluid cytology, tight, 3‐dimensional tumor clusters and singly lying tumor cells were observed. Squamous differentiation in the form of sheets and singly lying polygonal tumor cells with abundant dense cytoplasm was noted focally in rare cases. The diagnoses during original sign‐outs were poorly differentiated carcinoma, poorly differentiated squamous cell carcinoma, and malignant small round cell tumor. NUT‐1 (NUT family member 1 protein) immunocytochemistry performed on cell blocks demonstrated characteristic speckled nuclear staining in tumor cells. Conclusions Pulmonary NC presents as a poorly differentiated carcinoma with focal to absent squamous differentiation on cytology. Cellular fragments of primitive tumor cells with conspicuous nucleoli should raise suspicion of NUT carcinoma and prompt ancillary testing.

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NUT midline carcinoma as a primary lung tumor: a case report

Cited by 15 publications

References 19 publications

Prolonged Disease Stabilization and Tolerability in a Nuclear Protein in Testis Midline Carcinoma Patient Treated with Dual Histone Deacetylase and Phosphoinositide 3-Kinase Inhibitor CUDC-907

Prolonged Disease Stabilization and Tolerability in a Nuclear Protein in Testis Midline Carcinoma Patient Treated with Dual Histone Deacetylase and Phosphoinositide 3-Kinase Inhibitor CUDC-907

NUT midline carcinoma in a young pregnant female: a case report

Cytomorphology of primary pulmonary NUT carcinoma in different cytology preparations

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