A rare type of diffuse carcinoma of the pancreas with unusual metastases

1936 ◽  
Vol 42 (1) ◽  
pp. 203-207 ◽  
Author(s):  
Rupert A. Willis
2020 ◽  
Vol 11 (3) ◽  
pp. 3-8
Author(s):  
Apostolos Pourtsidis ◽  
Smaragda Papachristidou ◽  
Orthodoxos Achilleos ◽  
Darius Mirza ◽  
Marina Servitzoglou ◽  
...  

Abstract Introduction Pancreatic tumors are reported rarely in childhood and represent an extremely rare entity in Pediatric Oncology. One of the least common types of pediatric pancreatic tumor is acinar cell carcinoma (ACC). We aim to present a rare case of ACC and the difficulties we faced during diagnosis and treatment. Patient and Methods An 8-year old girl presented with jaundice. Workup revealed a tumor originating from the head of the pancreas with multiple metastatic lesions in her liver. Evaluation of tumor markers revealed elevated levels of AFP. Pathology report was indicative of acinar cell carcinoma of the pancreas. Results After consulting the EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors), chemotherapy was initiated. Partial response was observed after the first 4 courses with decrease of AFP levels. While planning her surgery, AFP elevated and a second-line course of chemotherapy was administered. Our patient underwent Whipple’s Duodenopancreatectomy with partial metastasectomy. Although the postoperative period was uneventful, AFP continued to rise even after postoperative chemotherapy was administered. There were signs of metastatic disease progression. Our patient received a third-line regimen with no improvement. She received local radiotherapy and a next-line chemotherapy course. Local relapse and metastatic disease progression placed our patient in palliative care. She passed away nine months after the initial diagnosis. Conclusions Acinar cell carcinoma of the pancreas is a rare type of pediatric cancer with very challenging diagnosis and treatment. Cooperation at the European level and multicenter management of those rare cases is vital for the optimum outcome.


Author(s):  
P.K. Simons

Glycogenosis is defined as any condition in which the tissue concentration of glycogen is increased. There are currently ten recognized variants of glycogenosis that are heritable inborn errors of metabolism. The specific enzymatic defect in each of the variants is known or at least suspected. In all cases, the enzymatic defect prevents the proper metabolism or formation of the glycogen molecule. The clinical and histologic differences between the types of glycogenosis is important to a proper diagnosis after the presence of such a condition is realized. This study was initiated to examine the ultrastructure of the rare Type IV Glycogenosis (Amylopectinosis) of which there is very little morphologic characterization in the literature.Liver tissue was obtained by needle biopsy from a 12-month-old Oriental female who was originally admitted to the hospital after observation of poor development, loss of appetite, and hepatomegaly. The majority of the tissue was fixed for light microscopy in neutral buffered formalin and processed using routine and special staining procedures (reticulin, trichrome, iron, copper, PAS, PAS-diastase and PAS-pectinase.


2019 ◽  
Vol 11 (1) ◽  
pp. 81-83
Author(s):  
Md Mustafizur Rahman ◽  
Nadim Ahmed ◽  
Sami Ahmad ◽  
Shoaeb Imtiaz Alam ◽  
Mohammad Rashedul Hassan ◽  
...  

Paget’s disease of the breast is a rare type of cancer of the nipple–areola complex and that is often associated with an underlying in situ or invasive carcinoma. It is often misdiagnosed as eczema of breast and treatment is delayed. Here we present a case where a 30 year old female presented with itching ulceration and destruction of her left nipple. She was treated initially by local physicians by applying local ointments but as her condition did not improve she was admitted to department of surgery Shaheed Suhrawardy Medical College hospital where she was diagnosed as Paget’s disease with infiltrating ductal cell carcinoma. She underwent modified radical mastectomy with axillary clearance and referred to oncology department for further management. J Shaheed Suhrawardy Med Coll, June 2019, Vol.11(1); 81-83


2001 ◽  
Vol 45 (1) ◽  
pp. 29 ◽  
Author(s):  
Jong Young Oh ◽  
Kyung Jin Nam ◽  
Jong Cheol Choi ◽  
Suck Bin Suh ◽  
Ki Nam Lee ◽  
...  

2017 ◽  
Vol 26 (2) ◽  
pp. 183-187
Author(s):  
George P. Christophi ◽  
Yeshika Sharma ◽  
Quader Farhan ◽  
Umang Jain ◽  
Ted Walker ◽  
...  

Background: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits. This is the first report documenting ECD manifesting as segmental colitis and causing cytokine-release syndrome.Case presentation: A 68-year old woman presented with persistent fever without infectious etiology and hematochezia. Endoscopy showed segmental colitis and pathology revealed infiltration of large foamy histiocytes CD3-/CD20-/CD68+/CD163+/S100- consistent with ECD. The patient was empirically treated with steroids but continued to have fever and developed progressive distributive shock.Conclusion: This case report describes the differential diagnosis of infectious and immune-mediated inflammatory and rheumatologic segmental colitis. Non-Langerhans histiocytosis and ECD are rare causes of gastrointestinal inflammation. Prompt diagnosis is imperative for the appropriate treatment to prevent hemodynamic compromise due to distributive shock or gastrointestinal bleeding. Importantly, gastrointestinal ECD might exhibit poor response to steroid treatment and other potential treatments including chemotherapy, and biologic treatments targeting IL-1 and TNF-alpha signaling should be considered.Abbreviations: AFB: acid-fast bacilli; ECD: Erdheim-Chester Disease; IBD: inflammatory bowel disease; PASD: periodic acid-Schiff with diastase; TB: tuberculosis


Choonpa Igaku ◽  
2018 ◽  
Vol 45 (3) ◽  
pp. 301-309 ◽  
Author(s):  
Sachiko KOBAYASHI ◽  
Shigeyuki HASUO ◽  
Motoi MIYAKOSHI ◽  
Tomohiro NAKATANI ◽  
Yukie NAKAJIMA ◽  
...  

2015 ◽  
Vol 15 (1) ◽  
Author(s):  
Xiang Zhu ◽  
Huijuan Yong ◽  
Li Zhang ◽  
Yonghui Huang ◽  
Jie Zheng ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document