Giant variant of acquired perforating dermatosis, clinically masquerading as a sarcoma: A report of a rare case

Acquired perforating dermatosis is a group of disease characterized by transepidermal elimination of altered dermal constituents of unknown pathogenesis. The giant variant was first described in 2006, as an emerging entity with seven reported cases to date. Here is an 83-year-old male presented with a 4-year history of gradually enlarging soft tisssue mass with ulcerartions at the left knee joint. Imaging revealed an extra-articular, single, heterogeneous, multinodular mass, suspicious for a soft tissue sarcoma. Wide local excision of the mass showed fleshy, hemorrhagic nodules communicating with epidermal ulcers. Microscopy showed cystic spaces straddling dermis and subcutis, containing eosinophilic, amorphous, granular material extruding through epidermal craters, surrounded by exuberant myofibroblastic proliferation. Trichrome and van-Gieson stains confirmed that the extruded material is collagen and the histology was compatible with the giant variant of acquired perforating collagenosis. Awareness of histological appearance prevents misdiagnosis and overtreatment of this entity, masquerading as a sarcoma clinically.

Prognostic indices predictive of short-term disease-free survival of breast carcinoma patients receiving primary surgical treatment in Sri Lanka

Background: Breast carcinoma (BCa) is the commonest malignancy among women worldwide and in Sri Lanka. Several prognostic indices are described for BCa. Aims: To assess clinicopathological features and prognostic indices derived from routine clinical, histopathological and immunohistochemical (IHC) data, in a cohort of patients undergoing primary surgery for BCa and to determine their prognostic impact on short-term disease free survival. Setting and Design: This is a bidirectional cohort study of 208 women undergoing primary surgery for BCa at the National Hospital of Sri Lanka, from 2012-2014, excluding post-neoadjuvant chemotherapy cases. Material and Methods: Clinical details, tumor size and nodal status were obtained from histopathology reports. Histopathology and estrogen/progesterone receptor and HER2 status were reviewed. Molecular subtype based on IHC was determined. Nodal ratio (number of positive nodes/total number retrieved) and Nottingham prognostic index were calculated. Follow up information was obtained by patient interviews and record review. Statistical Analysis: Data was analyzed by univariate and multivariate Cox regression using SPSS19.0. Results: Mean follow-up duration was 27.16 months (0.5-52 months, s = 9.35 months). 174 (82.9%) remained disease free with 19 (9%) deaths. Thirteen (6.2%) survived with metastasis and 4 (1.9%) with recurrences. On univariate Cox regression, tumor, nodal and TNM stages, nodal ratio and lymphovascular invasion (LVI) were predictive of disease free survival (DFS) (P = 0.001, P = 0.021, P = 0.022, P = 0.002, P = 0.018). On multivariate analysis TNM stage and LVI were predictive of DFS. Conclusion: TNM stage and LVI were the most important predictors of short-term disease free survival in this study population, confirming that early detection of BCa at a lower stage has a significant impact on short-term outcomes.