scholarly journals Undifferentiated carcinoma with osteoclastic giant cells of pancreas: Case report and brief literature review

2021 ◽  
Vol 6 (4) ◽  
pp. 307-310
Author(s):  
Jyoti Parag Dekate ◽  
Manjula V Kelkeri ◽  
Gowri Garudadri

Undifferentiated carcinoma with osteoclastic giant cells is a distinctive rare neoplasm involving pancreas and rarely bile ducts. This neoplasm shows characteristic histologic features with variable admixture of mononuclear histiocytic cells, non- neoplastic osteoclastic giant cells and neoplastic mononuclear cell component. Though, this tumor has been shown to share genetic alterations with pancreatic ductal adenocarcinoma, clinically, it behaves unpredictably with a substantial proportion of patient showing prolonged survival period. We present a case of this rare pancreatic tumour with brief literature review discussing the key pathologic features, immunophenotype, genetic profile and clinical behavior. Undifferentiated carcinoma with osteoclastic giant cells is a rare neoplasm in pancreas which is believed to be of epithelial origin and shares genetic aberrations with ductal adenocarcinoma. Despite this, these tumours have better prognosis with prolonged survival period.

2017 ◽  
Vol 243 (2) ◽  
pp. 148-154 ◽  
Author(s):  
Claudio Luchini ◽  
Antonio Pea ◽  
Gemma Lionheart ◽  
Andrea Mafficini ◽  
Alessia Nottegar ◽  
...  

2021 ◽  
Vol 19 (3) ◽  
pp. 247-252
Author(s):  
Robert J. Besaw ◽  
Adrienne R. Terra ◽  
Grace L. Malvar ◽  
Tobias R. Chapman ◽  
Lauren M. Hertan ◽  
...  

Undifferentiated carcinoma with osteoclast-like giant cells (UCOGC) of the pancreas is a rare and potentially aggressive variant of pancreatic ductal adenocarcinoma. Data on this disease are sparse, and despite genetic similarities to pancreatic ductal adenocarcinoma, UCOGC clinical outcomes can be markedly different. We report on a female patient aged 62 years who presented with UCOGC with pulmonary metastases initially treated with 2 lines of cytotoxic chemotherapy. After rapid disease progression with both cytotoxic treatments, the patient’s tissue was sent for next-generation sequencing, which revealed a high tumor mutation burden (32 mutations per megabase), as well as somatic mutations in BRAF, NF1, PIK3CA, CDKN2A, TERT, and TP53. Pancreatic cancers have previously demonstrated suboptimal responses to immunotherapeutic approaches. However, given the high tumor mutation burden and distinctiveness of the tumor class, the patient began third-line pembrolizumab monotherapy after palliative radiation to the rapidly progressing and painful abdominal mass from her primary tumor. She had a marked response in her primary UCOGC tumor and metastatic sites, and she remains on pembrolizumab monotherapy with ongoing response after 32 months of therapy. Recent evidence showing significant PD-L1 enrichment on neoplastic cells of undifferentiated carcinomas (including UCOGC) may indicate a role for immunotherapeutic approaches in these patients. Rare cancers such as UCOGC and other undifferentiated carcinomas may benefit from next-generation sequencing to inform treatment decisions when standards of care are absent, as in this report.


2010 ◽  
Vol 3 (4) ◽  
pp. 195-203 ◽  
Author(s):  
Yasuhiro Togawa ◽  
Akihiko Tonouchi ◽  
Tsuyoshi Chiku ◽  
Wataru Sano ◽  
Tomoko Doki ◽  
...  

2019 ◽  
Vol 06 (01) ◽  
pp. 6-21
Author(s):  
Andreea Rusu ◽  
Simona Eliza Giusca ◽  
Delia Gabriela Ciobanu Apostol ◽  
Lidia Ionescu ◽  
Irina Draga Caruntu

Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.


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