scholarly journals Primary Extra Mammary Paget’s Disease of Vulva, With Apocrine Adenocarcinoma, Signet Ring Cell Differentiation and Distant Metastasis

Author(s):  
Ruchi Rathore ◽  
Deepika Yadav ◽  
Shipra Agarwal ◽  
Pankhuri Dudani ◽  
Kaushal K Verma ◽  
...  

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease. Case report: We hereby report a case of primary EMPD with invasive carcinoma and distant metastasis in a 59-year-old female who presented with erythematous indurated plaque over vulva. Histopathology revealed Paget cell infiltration throughout the epidermis with invasive carcinoma in dermis and liver metastasis on CECT. The immunohistochemical expressions of CK7, CK20, GCDFP-15, CEA, p40, CDX 2, Her-2/ neu, AR, ER, were examined to explicate the cellular differentiation of this carcinoma. According to the histological assessment, this case was diagnosed as primary EMPD with apocrine adenocarcinoma, signet ring cell differentiation, vulva. Conclusion: Owing to poor prognosis, a high index of clinical suspicion along with histological and immunohistochemical assessment is of utmost importance in arriving at final diagnosis.  

2020 ◽  
Vol 6 (3) ◽  

Primary signet ring cell carcinoma (PSRCC) of the breast is a rarely diagnosed neoplasm. We present a 76-year-old woman with a tumor formation in the left mammary gland, who has been self-medicating for a year. Pathohistological and immunohistochemical analysis proved rare primary invasive ductal carcinoma with focal (over 90%) signet ring cell differentiation, size 4 cm / 3.5 cm / 2 cm, moderately differentiated (G2). Complex oncological treatment, including radical mastectomy with axillary dissection, 6 courses of systemic adjuvant chemotherapy, radiotherapy of the chest wall and regional lymph nodes with TD 46 Gy and antiestrogenic hormone therapy, was performed. The diagnosis and the differential diagnosis of this rare tumor require precise pathohistological and immunohistochemical analysis. The prognosis and complex treatment depend on the clinical stage, hormonal and HER2 status. In locally advanced PSRCC of the breast with moderately differentiation, the combination of surgery, systemic chemotherapy, postoperative radiotherapy and antiestrogenic hormone therapy achieves long-term local tumor control without distant metastases for nearly two years.


Author(s):  
Sayali Y. Pangarkar ◽  
Akshay D. Baheti ◽  
Kunal A. Mistry ◽  
Amit J. Choudhari ◽  
Vasundhara R. Patil ◽  
...  

Abstract Background Presence of extramural venous invasion (EMVI) is a poor prognostic factor for rectal cancer as per literature. However, India-specific data are lacking. Aim The aim of the study is to determine the prognostic significance of EMVI in locally advanced rectal cancer on baseline MRI. Materials and Methods We retrospectively reviewed 117 MRIs of operable non-metastatic locally advanced rectal cancers in a tertiary cancer institute. Three dedicated oncoradiologists determined presence or absence of EMVI, and its length and thickness, in consensus. These patients were treated as per standard institutional protocols and followed up for a median period of 37 months (range: 2–71 months). Kaplan-Meier curves (95% CI) were used to determine disease-free survival (DFS), distant-metastases free survival (DMFS), and overall survival (OS). Univariate analysis was performed by comparing groups with log-rank test. Results EMVI positive cases were 34/114 (29%). More EMVI-positive cases developed distant metastasis compared with EMVI-negative cases (14/34–41% vs. 22/83–26%). The difference, however, was not statistically significant (p = 0.146). After excluding signet-ring cell cancers (n = 14), EMVI showed significant correlation with DMFS (p = 0.046), but not with DFS or OS. The median thickness and length of EMVI was 6 and 14 mm, respectively in patients who developed distant metastasis, as compared with 5 and 11 mm in those who did not, although this difference was not statistically significant. Conclusion EMVI is a predictor of distant metastasis in locally advanced non-metastatic, non-signet ring cell rectal cancers. EMVI can be considered another high-risk feature to predict distant metastasis.


2007 ◽  
Vol 212 (3) ◽  
pp. 278-286 ◽  
Author(s):  
ME Börger ◽  
MJEM Gosens ◽  
JWM Jeuken ◽  
LCLT van Kempen ◽  
CJH van de Velde ◽  
...  

1997 ◽  
Vol 19 (5) ◽  
pp. 500
Author(s):  
B C Bastian ◽  
H Kutzner ◽  
T SB Yen ◽  
P E LeBoit

Sign in / Sign up

Export Citation Format

Share Document