scholarly journals Angiosarcoma Secondary to a Paget’s Disease: A Rare Clinical Case With 5 Years of Follow-Up and Review of the Literature

2020 ◽  
pp. 1-4
Author(s):  
M. Silvia Spinelli ◽  
Andrea Di Bernardo ◽  
Fabio Sciancalepore ◽  
Fabio L. Giardina ◽  
M. Silvia Spinelli ◽  
...  

Paget's disease is characterized by disorganized bony tissue due to altered equilibrium of bone formation and resorption. Its malignant transformation is well known and is usually described to an osteosarcoma, often with bad prognosis. In this paper, we present a case clinic of an angiosarcoma from a Paget’s disease of a pelvic bone. The patient underwent a surgical procedure of tumor resection with wide margins (Enneking zone 2-3). The follow-up of 5 years in this case is unique in the literature.

2008 ◽  
Vol 102 (4) ◽  
pp. 485-488 ◽  
Author(s):  
Zhong Wang ◽  
Mujun Lu ◽  
Guo-Qin Dong ◽  
Yue-Qing Jiang ◽  
Mei-Sui Lin ◽  
...  

1988 ◽  
Vol 74 (2) ◽  
pp. 183-190 ◽  
Author(s):  
Pietro Muretto ◽  
Vincenzo Polizzi ◽  
M. Pia Staccioli

A case of Paget's disease and gynecomastia in a 70-year-old man is reported. Paget's disease was connected to an intraductal carcinoma, and the immunohistochemical study revealed similar positivity for cytokeratin A, carcinoembryonic antigen and epithelial membrane antigen in Paget cells and intraductal neoplastic cells whereas Paget cells resulted negative for cytokeratin B and C. The study using monoclonal anti-cytokeratin A (35 βH11), B (34 βE12) and C (34 βB4) could represent a good tool, supporting the theory of a ductal origin of Paget cells. A review of the literature has shown the rarity of Paget's disease in the male breast and revealed only two previous reports with an associated gynecomastia, in 2 patients with Klinefelter's syndrome and infiltrating breast carcinoma.


2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Yu-Chen Wang ◽  
Anna Fen-Yau Li ◽  
Shung-Haur Yang ◽  
Hsiu-Hsun Ma ◽  
Wen-Yih Liang

Aim. To determine the incidence, prognosis, and immunophenotypes (CK7, CK20, CDX2, and GCDFP-15) of primary or secondary perianal Paget’s diseases (PPDs). Methods. Twenty-three PPD patients were recruited, including 10 primary and 13 secondary PPDs. Immunophenotypes of PPD were analyzed. Results. In 23 PPD patients, 14 (60.9%) were male and the median age was 75 years. Three (13.0%, 2 primary and 1 secondary PPDs) had recurrence and two (8.7%, both primary PPDs) had invasive PPDs. The colorectal cancers (CRCs) in secondary PPD cases were located in anorectal area for 9 patients while 4 were located in the rectum; 5, 2, 4, and 2 were in stages I, II, III, and in uncertain stage, respectively. The distant metastasis rates of CRC in the secondary PPD patients during follow-up were 40% (2/5), 0% (0/2), and 50% (2/4) for stages I, II, and III, respectively. Other synchronous or metachronous malignancies included cholangiocarcinoma, urothelial carcinoma, anorectal small-cell carcinoma, and unknown hepatic malignancy. One primary PPD patient died from the metastases of invasive Paget’s disease while 3 secondary PPD patients died from the metastases of CRCs during follow-up. Immunohistochemical staining showed CK7 (7/10 and 6/13), CK20 (6/10 and 10/13), CDX2 (6/10 and 12/13), and GCDFP-15 (3/10 and 0/13) positivities in primary and secondary PPD patients, respectively. The immunophenotypes were not statistical significantly related to synchronous CRC (P=0.402, 0.650, 0.127, and 0.068 for CK7, CK20, CDX2, and GCDFP-15, respectively). Conclusions. The incidence of concurrent CRC in PPD patients is not low. An adequate survey for CRC should be considered for PPD patients at initial diagnosis. In this series of study, stage I CRC with PPD would have a higher metastatic rate, thus indicating aggressive treatment and follow-up. The CK7, CK20, CDX2, and GCDFP-15 immunostaining results for the PPD patients were not predictive of primary or secondary type.


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