ABSTRACT
Systemic sclerosis is a challenging diagnosis for clinicians and pathologists alike due to its protean manifestations and often insidious onset, particularly in cases without significant titres of auto-antibodies. Herein we present a case of a female in her sixties who died rapidly following a clinical diagnosis of pneumatosis intestinalis and respiratory failure of unclear etiology. Recently revised clinical diagnostic criteria were applied to the clinical history and postmortem findings to reach an unexpected diagnosis of systemic sclerosis. The diagnosis of systemic sclerosis at autopsy has important medicolegal implications largely related to premature death due to delayed treatment or poor post-operative outcome. Moreover, familial clustering of this disease underscores the importance of maintaining a high index of suspicion in the postmortem setting.
Subject(s)
Forensic Pathology/methods , Pneumatosis Cystoides Intestinalis/pathology , Scleroderma, Systemic/pathology , Autopsy , Cause of Death , Female , Humans , Middle Aged , Tomography, X-Ray ComputedABSTRACT
Pathologic processes involving the urachus are usually related to inflammatory or sinofistular conditions. Neoplasms rarely arise within this structure, and when they do occur, they are typically epithelial, with mucinous adenocarcinoma being the most common. Mesenchymal lesions, both benign and malignant, have rarely been described in this location. We report the case of a 66-year-old white man who presented with a primary urachal malignant fibrous histiocytoma and died of metastatic disease 20 months after the initial diagnosis. This is an unusual case of malignant fibrous histiocytoma arising in a urachal remnant.
Subject(s)
Histiocytoma, Benign Fibrous/pathology , Urachus/pathology , Urinary Bladder Neoplasms/pathology , Abdominal Neoplasms/secondary , Aged , Biomarkers, Tumor/analysis , Combined Modality Therapy , Cystectomy , Fatal Outcome , Histiocytoma, Benign Fibrous/chemistry , Histiocytoma, Benign Fibrous/radiotherapy , Histiocytoma, Benign Fibrous/secondary , Histiocytoma, Benign Fibrous/surgery , Humans , Male , Neoplasm Recurrence, Local , Radiotherapy, Adjuvant , Retroperitoneal Neoplasms/secondary , Urachus/surgery , Urinary Bladder Neoplasms/chemistry , Urinary Bladder Neoplasms/radiotherapy , Urinary Bladder Neoplasms/surgery , Urinary DiversionABSTRACT
Patients with renal cell carcinoma (RCC) often remain asymptomatic until the disease has progressed beyond the confines of the kidney, as almost one third of patients have metastatic disease at the time of diagnosis.1 The average survival for a patient presenting with metastatic renal cell carcinoma is 4 months and only 10% will be alive at 1 year.2 Many different treatment combinations have tried for metastatic disease, but with limited results. Some success has been shown in smaller studies using nephrectomy and Bacillus Calmette-Guerin (BCG) immunotherapy but these results have not been followed by larger randomized control trials.3 We report a case metastatic renal cell carcinoma with an unusually long survival and regression of metastases from 2 uncommon sites.
