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| Discussion |
Case Report: A 35-year-old lady presented with a lump in the right upper quadrant of the abdomen since one year. The lump had gradually increased in the size over the period. On clinical examination, a firm, non tender swelling was detected which moved with respiration. Clincally, the lesion was suspected to be originating from the liver. Sonography of the abdomen revealed a large mass superior to the right kidney and separate from the liver. It was thought to be of adrenal origin. The patient then underwent CT and MRI of the abdomen. .
Plain and contrast enhanced CT scans of the abdomen showed a large hypodense mass lesion in the right upper abdomen displacing the liver and the IVC. Post contrast scans showed peripheral enhancement with central necrotic component. No calcification or hemorrhage was seen
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MRI revealed a large soft tissue mass with cystic lesion arising from the right dome of the diaphragm and placed inferior to the diaphragm. T2W images revealed intermediate signal intensity peripherally and hyperintese signal in the centre. T1W images showed hypointense signal.. The fat planes with the kidney were well preserved. The mass was seen displacing the IVC anteriorly and the liver inferiorly. Right sided pleural effusion and consolidation was seen.
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The patient was operated and a mass arising from inferior surface of diaphragm was resected.
Hisopathology showed an inflammatory myofibroblastic tumour of the diaphragm.
DISCUSSION:
Inflammatory myofibroblastic tumor is sometimes known as inflammatory pseudotumor . The tumor is more common in women. It is a lesion of unknown etiology that has been reported in numerous anatomic sites. It has been reported to arise from the diaphragm, heart and many extra-thoracic locations such as the liver, spleen, testes, epididymis, spermatic cord, bladder, salivary glands, spinal meninges and orbit. By definition, the tumor is composed predominantly of spindle cell proliferation with a variable inflammatory component. These spindle cells are now known to be myofibroblasts and this is the reason for the current designation for this disease. Its histological elements are myofibroblasts, histiocytes, plasma cells, and lymphocytes. Presence of these features suggests that this is a fibro-inflammatory lesion with abnormal extension of the reparative healing process after an unknown insult. The term “inflammatory” may not be applicable to all tumors since some investigators have demonstrated the presence of chromosomal abnormalities and documented cases showing aggressive behavior supporting the theory that at least some of these tumors are true neoplasms. The characteristics of this tumour include its unusual presentation as in the case reported herein. This tumour may have variable physical consistency. It can be of semi-solid gelatinous consistency or present as a solitary nodule and solitary or multiple calcified fibrous nodules . The case of inflammatory myofibroblastoma presented in this case report is unique due to its presentation with a long-standing history. This only adds to the diagnostic dilemma related with this tumour. Surgical treatment can be challenging in non-solid tumours, as complete removal can be difficult - resulting in a high risk of local recurrence.
Radiological findings may be solid enhancing mass lesion or soft tissue lesion with cystic areas within. The mass is slow growing and displaces the surrounding structures.