Osteosarcomas are highly malignant neoplasms of bone that are challenging to diagnose. These neoplasms often show atypical behavior. In the initial phase they may present as nondescript bony swellings with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease. Similarly, the histological growth pattern of this neoplasm can be quite diverse, presenting with areas that mimic benign myofibroblastic tumors, giant cell granulomatous conditions and partial encapsulation. The final diagnosis of an osteosarcoma is often reached after thorough sampling and examination of multiple biopsy specimens. All these clinical features and histological diagnostic difficulties were encountered in a case of osteosarcoma affecting the right mandible of a 62-year-old Chinese woman described here. The diagnostic lessons accrued from this case are discussed.
A 30-year-old ex-smoker with a background history of childhood asthma presented with worsening shortness of breath despite receiving high doses of oral corticosteroid for pemphigus vulgaris which was diagnosed 5 years earlier. A high-resolution CT examination of the thorax reported non-specific bronchiectatic changes and revealed an incidental suprarenal mass. A subsequent CT scan confirmed a large adrenal mass with areas of necrosis and calcification. Serum renin and aldosterone, urinary catecholamine and 5-hydroxyindoleacetic acid were within normal limits. Surgical intervention was delayed due to difficulty in optimising preoperative respiratory functions. He finally underwent a midline laparotomy for removal of the tumour. Histopathological examinations revealed extrapulmonary inflammatory myofibroblastic tumour arising from the periadrenal soft-tissue, with presence of normal adrenal gland. He showed immediate improvements of his asthmatic symptoms and pemphigus vulgaris following the surgery. His oral steroid was rapidly reduced and he achieved complete remission 2 months later.
Inflammatory myofibroblastic tumour is rare but more common in children. It shows an immunophenotypic features of myofibroblastic differentiation, hence bearing neoplastic potential. The diagnosis is challenging especially if it involves rectum. Surgical resection is the mainstay of treatment if clinically obstructed. A 65-year-old gentleman presented with intestinal obstruction, which then followed by a hartmann's procedure. Final diagnosis is a rare case of inflammatory myofibroblastic tumour of the rectum. We discuss its genetic involvement with a literature review.