scholarly journals Pulmonary Tumor With Notochordal Differentiation: A Case Report and Morphologic, Immunohistochemical and Molecular Study of Benign Notochordal Cell Tumor Originating in the Lung

Author(s):  
Kai Song ◽  
Xiaojing Ma ◽  
Jinghong Xu ◽  
Lirong Chen

Abstract Background: Extraosseous benign notochordal cell tumor is extremely rare, and there are only five reported cases worldwide. The presented case of pulmonary primary benign notochordal cell tumor is the sixth case, but the first to report the deletion mutation of EGFR gene exon 19. Case presentation: The patient was a 50-year-old asymptomatic woman, who had been followed up for 3 years for a nodule in the right lung. Six months after the wedge resection, the patient is alive without evidence recurrence or metastasis. The tumor was 7 millimeters in diameter and was well demarcated. The tumor was consisted of a sheet of large round vacuolated cells with small and bland nuclei. No connective tissue containing blood vessels or inflammatory cell infiltration was detected in the stroma. The tumor were positive for CK AE1/AE3, Vimentin, S100 and Brachyury. Deletion mutation of EGFR gene exon 19 was first detected and reported. Conclusions: We firstly reported the EGFR mutation of pulmonary primary benign notochordal cell tumor. Due to the rarity of this tumor, more reports are need to explore pathological characteristics, especially the molecular characteristics, in order to better understand the nature of tumors

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Kai Song ◽  
Xiaojing Ma ◽  
Jinghong Xu ◽  
Lirong Chen

Abstract Background Extraosseous benign notochordal cell tumor is extremely rare, and there are only five reported cases worldwide. The presented case of pulmonary primary benign notochordal cell tumor is the sixth case, but the first to report the deletion mutation of EGFR gene exon 19. Case presentation The patient was a 50-year-old asymptomatic woman, who had been followed up for 3 years for a nodule in the right lung. After ten months of the wedge resection, the patient is alive without evidence of recurrence or metastasis. The tumor was 7 mm in diameter and was well demarcated. The tumor was consisted of a sheet of large round vacuolated cells with small and bland nuclei. No connective tissue containing blood vessels or inflammatory cell infiltration was detected in the stroma. The tumor was positive for CK AE1/AE3, Vimentin, S100 and Brachyury. EGFR gene mutation and amplification were not detected. Conclusions We firstly reported the positive immunohistochemical staining for EGFR and the negative molecular results of EGFR gene of pulmonary primary benign notochordal cell tumor. Due to the rarity of this tumor, more reports are needed to explore pathological characteristics, especially the molecular characteristics, in order to better understand the nature of tumors.


2016 ◽  
Vol 183 ◽  
pp. 441-443 ◽  
Author(s):  
Bagher Eftekhari-Sis ◽  
Maryam Anvari Aliabad ◽  
Farrokh Karimi

2020 ◽  
Vol 133 (5) ◽  
pp. 1355-1359
Author(s):  
Maria Peris-Celda ◽  
Laura Salgado-Lopez ◽  
Carrie Y. Inwards ◽  
Aditya Raghunathan ◽  
Carrie M. Carr ◽  
...  

Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors’ knowledge, have not been previously documented at this anatomical site.An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.


ORL ◽  
2021 ◽  
pp. 1-6
Author(s):  
Qingjiao Li ◽  
Xiaolu Yuan

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typically positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break apart involving the <i>Ewing sarcoma</i> (<i>EWS</i>) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and <i>EWS</i> gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.


Open Medicine ◽  
2015 ◽  
Vol 10 (1) ◽  
Author(s):  
Ryszard Pogorzelski ◽  
Sadegh Toutounchi ◽  
Patryk Fiszer ◽  
Ewa Krajewska ◽  
Izabela Łoń ◽  
...  

AbstractIntroduction: We present a case of a 29-year-old patient treated due to fully symptomatic pheochromocytoma of the right adrenal gland. Case presentation: Patient was operated on and an open right-sided adrenalectomy was performed. At the time of the surgery, a rupture of the tumor capsule occurred. Five years post-operatively, a recurrence of the symptoms of chromaffin-cell tumor was noted. After the exact localization of the multiple recurrences, the patient was reoperated on. Conclusion: The case of pheochromocytoma is presented due to the possibility of chromaffin-cell seeding into the peritoneum, with no signs of distal metastases so far.


Oncotarget ◽  
2017 ◽  
Vol 8 (67) ◽  
pp. 111246-111257 ◽  
Author(s):  
Jian Su ◽  
Wenzhao Zhong ◽  
Xuchao Zhang ◽  
Ying Huang ◽  
Honghong Yan ◽  
...  

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Monet E. Meter ◽  
David J. Nye ◽  
Christian R. Galvez

Introduction. It is rare for actinic or squamous cell carcinoma (SCC) in situ to metastasize. Case Presentation. A 67-year-old male had a significant medical history including severe psoriatic arthritis treated with UVB, methotrexate, and rapamycin. He had twenty-five different skin excisions of actinic keratosis four of which were invasive SCC. Our patient developed shortness of breath necessitating a visit to the emergency department. A CT scan of his chest revealed a mass in the right lower lung. A subsequent biopsy of the mass revealed well-differentiated SCC. He underwent thoracoscopic surgery with wedge resection of the lung lesion. Discussion. Actinic keratosis (AK) is considered precancerous and associated with UV exposure. It exists as a continuum of progression with low potential for malignancy. The majority of invasive SCCs are associated with malignant progression of AK, but only 5–10% of AKs will progress to malignant potential. Conclusion. In this case, a new finding of lung SCC in the setting of multiple invasive actinic cutaneous SCC associated with a history of extensive UV light exposure and immunosuppression supports a metastatic explanation for lung cancer.


2020 ◽  
Vol 63 (2) ◽  
pp. 91-101
Author(s):  
Elham Amini ◽  
Fatemeh Nasrollahi ◽  
Ali Sattarian ◽  
Mahboobeh Haji Moradkhani ◽  
Sohrab Boozarpour ◽  
...  

Hedera, with 12 extant species, is a genus of evergreen climbers native to Europe, north Africa, and south Asia. In this study, the micromorphological, anatomical structure and molecular evidences of 11 populations from two species of Hedera (H. helix and H. pastuchovii) have been considered to evaluate the relationships in Hedera. In total, seven quantitative and qualitative characters of pollen were selected and measured. Based on this study, the anticlinal wall and surface sculpturing of seed support for separation of two species of Hedera. Micromorphology of epidermis illustrated two types of epidermal cells: puzzle-shaped and polygonal cells. Using nuclear (nrDNA ITS) marker, we reconstructed phylogenetic relationships within two species of Hedera. This data set was analyzed by phylogenetic methods including Bayesian inference, maximum likelihood, and maximum parsimony. In phylogenetic analyses, all members of two species formed a well-supported clade (PP = 1; ML/BS = 100/100) and divided into two major clades (A and B). Neighbor Net diagram demonstrated separation of the studied populations. The results showed that these taxa differ in taxonomically important micromorphological, anatomical and molecular characteristics and these data provide reliable evidence for separation of these two species.


Author(s):  
Eliandro de Souza Freitas ◽  
Francisco Amadis Batista Ferreira ◽  
Brendo Vinícius Rodrigues Louredo ◽  
Milena Gomes Melo Leite ◽  
José da Cruz Luna Neto ◽  
...  

Giant Cell Tumor of Bone is an uncommon aggressive benign neoplasm that frequently affecting the epiphysis of long bones especially around the knee. This is a singular rare case of GCTB in the right maxillary sinus implicated in a process coincidentally or casually with another type of lesion primarily developed.


2017 ◽  
Vol 1 (2) ◽  
pp. 43
Author(s):  
Burçin TUNA ◽  
Sermin ÖZKAL ◽  
Ali BALCI

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