a 35-year - old man visited our hospital presenting with a palpable mass on his left lateral chest wall , which was movable , rubbery , and non - tender . a gun biopsy assisted by ultrasonography
was performed , but failed to confirm a specific diagnosis ; therefore , we performed a local excision .
the mass was 321 cm , non - adhesive , round , and pink ; the cut sections evidenced well - demarcated , gray - white gelatinous masses .
a microscopic examination revealed a tumor in the subcutaneous tissues without invasion to the adjacent structures ( fig .
1 ) . the specimen exhibited a mixture of myxoid , hyaline , and inflammatory zones ( fig .
2 ) . scattered and enlarged tumor cells , each with a large eosinophilic nucleus resembling a virocyte , were observed , consistent with a diagnosis of myxoinflammatory fibroblastic sarcoma ( mifs ) ( fig .
an immunohistochemical study revealed that the tumor cells had a strong positivity for vimentin and focal positivity for cd34 and cd68 , confirming the diagnosis of mifs .
a microscopic histological analysis of the specimen revealed that the mass was abutting to the resection margin in a series of multifocal areas .
thus , an additional wide excision was advised for an adequate tumor - free margin . on combined positron emission tomography and computed tomography ,
fluoro-2-deoxy - d - glucose uptake was increased at the left chest wall , probably due to a previous procedure ; multiple nodules were also observed in the right lung , suggesting possible lung metastasis . after administering general anesthesia and single - lung ventilation , a wedge resection of the right upper lung , including the nodules ,
subsequently , a wide left chest wall excision was performed , including partial resections of the 5th , 6th , and 7th ribs . the tumor free margin of the subcutaneous and muscle layers was 4 cm , and the skin margin was 2 cm .
mifs , or acral mifs , is a low - grade , malignant tumor that has only recently been defined pathologically .
about 200 cases have been reported since it was first described in 1998 by montgomery et al . .
in 80% of cases , the lesion involves the subcutaneous tissues of the hands or feet , but it can be also be found on the elbows , thighs , or shoulders ; thus the term " acral " was dropped in the 2002 world health organization classification .
clinically , mifs usually presents as a slow - growing , solitary , and painless mass that can sometimes infiltrate extensively into the surrounding soft tissues .
grossly , it is usually visible as an ill - defined , multinodular mass , with myxoid areas , within the subcutaneous tissue , and has an irregular border and invasive margin . necrosis or hemorrhage may also occur .
the tumors are 1 - 8 cm in size and discharge a clear , jelly - like substance when compressed
. the simple x - ray imaging of the affected area did not provide any specific findings .
a magnetic resonance imaging also provided no pathognomonic patterns because of the mass ' varied histological composition ; however , the images did exhibit low signal intensity on the t1-weighted image and high signal intensity on the t2-weighed image .
histologically , the mass was composed of dense , mixed acute and chronic inflammatory cells , and the hypocellular area was composed of hyaline and myxoid zones .
characteristic ganglion - like , lipoblast - like , and polygonal spindle cells that contained bizarrely shaped nuclei and inclusion - like nucleoli were present in an abundant eosinophilic cytoplasm .
a mitotic division was generally rare , and necrosis was often evident . upon the immunohistochemical study ,
vimentin was always positive , cd68 and cd34 exhibited variable positivity , and hmb45 and cd45 were always negative .
the differential diagnosis can be broad and varied depending on whether the inflammatory , myxoid , or bizarre atypical component predominates within the lesion .
tenosynovitis , ganglion cyst , and myxoma ( for the inflammatory and benign entities ) , and myxoid malignant fibrous histiocytoma , myxoid liposarcoma , and epithelioid sarcoma ( for the malignant tumors ) are the main components of the histopathological differential diagnosis of a giant cell tumor in the tendon sheath or of a myxoid origin . because of the difficulty of an accurate diagnosis from the lesion 's clinical presentation or radiological characteristics , the diagnosis is often delayed and a surgical excision is more likely to be suboptimal .
the local recurrence rate of the tumor is high ( range , 22% to 67% ) when a local excision alone has been performed , usually being reported between 3 months and 5 years postoperatively .
the distant metastasis rate is low ( 6.5% ) ; however , cases have reported metastasis to the lung , neck , and skull .
one tumor - related death has been reported . although the treatment process has not been well - defined , a complete excision with clear margins is recommended .
postoperative radiotherapy may be a good additional therapy for patients who have a large tumor or a positive resection margin .
premalata et al .   reported upon a mifs of the upper back , over the scapula .
they performed a wide excision of the tumor , without rib resection , and reported no recurrence within 10 months after surgery .
we performed a wider local excision with a tumor free margin of 4 cm , including an adjacent rib resection and the reconstruction of the chest wall , in accordance with the guidelines for a malignant tumor of the chest wall . in summary , mifs is a very rare tumor that predominantly affects the distal extremities .
we report the first case of mifs in korea , and the second such tumor to be described in the chest wall .
 myxoinflammatory fibroblastic sarcoma ( mifs ) is a recently defined rare tumor . 
 it is mainly found in the upper and lower extremities of adults . due to its high local recurrence rate and low metastatic rate , 
 it is classified as a low grade - malignancy . 
 accurate diagnosis and early , wide excision are important for prognosis . herein , we report a case of mifs in a 35-year - old male patient that presented in an unusual location , the left chest wall . to our knowledge 
 , this is the first reported case of mifs in korea and the second case to be reported within the global scientific literature involving the chest wall . 
CASE REPORT
DISCUSSION