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

Perkins, Crystal; Pucar, Darko; McDonough, Colleen Hope; Williams, Hadyn T.

doi:10.4103/1450-1147.207284

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Studies on NC indicated that 18 FDG-PET/CT was useful in determining its staging, the biopsy site (avoiding necrotic tissue), and therapeutic efficacy [ 16 , 17 ]. In the current case, disappearance of ileal metastasis was evident, so local treatment was considered.…”

Section: Discussionmentioning

confidence: 99%

NUT carcinoma of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors: a case report

et al. 2018

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BackgroundNuclear protein in testis (NUT) carcinoma (NC) is a rare epithelial malignancy characterized by rearrangement of the NUT gene on chromosome 15. If NC is not suspected, it is often diagnosed as other malignancies. We present the case of NC of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors (ESFT).Case presentationA 49-year-old male presented with epistaxis and pain in the left eye. The patient had a tumor in the left nasal cavity at initial visit and it was biopsied. Firstly, the man was diagnosed with ESFT based on a histopathological examination. The tumor markedly responded to standard cytotoxic chemotherapy for ESFT with distant metastasis. After the start of therapy, a chromosomal analysis revealed an atypical translocation in ESFT and additional immunostaining was positive for anti-NUT antibody. Ultimately, the patient was definitively diagnosed with NC. He received multidisciplinary therapy and symptoms were temporarily relieved. However, he died 9 months after the diagnosis of NC.ConclusionsWhen a pathologically undifferentiated tumor is evident along the midline of the body, NC must be included in the differential diagnosis, and immunohistochemical staining or genetic testing/chromosomal analysis needs to be performed.

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Section: Discussionmentioning

confidence: 99%

NUT carcinoma of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors: a case report

et al. 2018

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“…Chest CT 18 F-fluorodeoxyglucose positron emission tomography/ CT (FDG-PET/CT) has been proposed in various case reports as the imaging modality of choice for distant metastatic assessment. [34][35][36][39][40][41][42][43][44] On the basis of this literature, and despite the lack of cohort studies specifically assessing the additional value of FDG-PET/ CT to standard imaging, we recommend performing it whenever possible, both for the initial staging and for monitoring treatment response (level V; grade B).…”

Section: Distant Metastasismentioning

confidence: 99%

NUT Carcinoma in Children and Adolescents: The Expert European Standard Clinical Practice Harmonized Recommendations

Lemelle¹,

Flaadt²,

Fresneau

et al. 2022

Journal of Pediatric Hematology/Oncology

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Background and Aims: Nuclear protein of the testis (NUT) carcinoma (NC) is a rare and highly aggressive tumor mainly occurring in adolescents and young adults, defined by the presence of a somatic NUTM1 rearrangement. The aim is to establish internationally harmonized consensus recommendations for the diagnosis and treatment of adolescents and young adults with NC in the framework of the European Reference Network for Paediatric Oncology. Methods: The European Cooperative Study Group for Pediatric Rare Tumors developed recommendations according to the Consensus Conference Standard Operating procedure methodology and reviewed by external “experts.” No evidence of level I to II exists. Recommendations were developed based on published prospective (level III), but more frequently retrospective series (level IV), case reports (level V), and personal expertise (level V). In addition, “strength” of recommendations were categorized by grading (grade A to E). Results: Histology is mandatory for the diagnosis of NC, including immunolabeling with anti-NUT antibodies and molecular biology (NUTM1 rearrangement) (level V; grade A). Treatment of NC usually combines aggressive approaches in multimodal regimens. Chemotherapy should be considered as first-line treatment (neoadjuvant vincristine-adriamycin-ifosfamide/cisplatin-adriamycin-ifsofamide or vincristine-doxorubicin-cyclophosphamide/ifosfamide-etoposide) for unresectable or metastatic tumor (ie, 3 courses), rapidly followed by local treatment (level IV; grade B). Referral to a specialized surgical oncology center is highly recommended (level V; grade A). In localized NC, a complete microscopic surgical resection should be attempted whenever and as soon as possible, followed by primary irradiation (60 to 70 Gy) and involved lymph nodes area (level IV; grade B). For head and neck tumors, a systematic neck dissection might be considered, even if N0 (level V; grade C). Adjuvant postirradiation chemotherapy is recommended, for a total of 9 to 12 courses (level IV; grade B). For first-line resected tumors, concomitant adjuvant chemotherapy to radiotherapy may be discussed (level IV; grade B). Targeted therapies and immunotherapeutic regimens should be delivered in the setting of prospective trials (level V; grade B). Conclusions: This project leads to a consensus strategy based on international experience with this very rare disease.

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Clinical imaging of primary pulmonary nucleoprotein of the testis carcinoma

Huang

Zhang²,

Yang³

et al. 2023

Front. Med.

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ObjectivePrimary pulmonary nucleoprotein of the testis (NUT) carcinoma is very rare in the clinic. In this study, the clinicopathological manifestations and imaging features of the primary pulmonary NUT carcinoma were investigated to improve the diagnosis of this disease.MethodsSix patients with pathologically diagnosed pulmonary NUT carcinoma were analyzed, including three males and three females, aged 19–64 (49.00 ± 16.40) years, with clinical manifestations of cough in two cases, hoarseness in one case, blood in sputum in one case, chest pain in one case, and physical examination findings in one case, with a disease duration of 5 days to 4 months. The clinical and imaging data including CT and PET/CT were retrospectively analyzed. Further literature reviews were analyzed in both pulmonary and extrapulmonary NUT carcinoma cases who performed 18F-FDG PET/CT.ResultsMost of the patients with pulmonary NUT carcinomas presented as heterogeneous lobulated masses (83.33%), four cases (66.67%) were located in the upper lobe of the left lung, one case (16.67%) in the middle lobe of the right lung, and one case (16.67%) in the lower lobe of the right lung, with the maximum diameter ranging from 1.30 to 8.90 cm and the median of 3.55 cm, most of them were irregularly shaped, with more lobulated margins and more heterogeneous density (83.33%), and the enhancement was mild. PET/CT showed increased 18F-FDG uptake in the lesion and metastatic areas. Both the pulmonary NUT patients in this study and literature reviews showed the SUVmax of the tumor ranged from 5 to 40 with an average value of 12.8, whereas that of extrapulmonary lesions had a range of SUVmax at 4.5–64.1 and a mean of 13.8.ConclusionIn patients with central lung masses, rapid disease progression, and poor response to initial treatment, the possibility of NUT cancer should be considered and anti-NUT monoclonal antibody immunohistochemical staining, combined with genetic detection, if necessary, should be performed as soon as possible. CT and PET/CT imaging are essential for the staging, management, treatment response assessment, and monitoring of pulmonary NUT cancer.

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

Cited by 3 publications

References 7 publications

NUT carcinoma of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors: a case report

NUT carcinoma of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors: a case report

NUT Carcinoma in Children and Adolescents: The Expert European Standard Clinical Practice Harmonized Recommendations

Clinical imaging of primary pulmonary nucleoprotein of the testis carcinoma

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