This uncommon, low grade sarcoma was first described by Montgomery, Devaney, Giordano and Weiss as recently as April, 1998 when they reported 51 cases collected from Sharon Weiss’ consultation files. They named the tumor descriptively, but verbosely, “inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells.” Four month later, the Kindbloms, working in Sweden, reported 44 cases of an identical tumor collected from The Armed Forces Institute of Pathology In Washington, DC and the Sahlgrenska University Hospital in Gothenburg, Sweden. They named it acral myxoinflammatory fibroblastic sarcoma. The sex incidence was equal; the age at diagnosis ranged from 20 to 91, with a median in the fifth decade; tumors were painless and had a long history (median 1 year); were located subcutaneously in the hands, wrists ,feet , and measured from 1 to 6 cm. Clinically they were thought to be ganglion cysts, tenosyonovitis, or giant cell tumors of tendon sheath. Initial histologic diagnoses often included pigmented villonodular tenosynovitis or various reactive fibroinflammatory processes. Histologically, there were usually more inflammatory cells that are present in the seminar slide, the tumor cells stained positively for vimentin and CD 68 but were negative for other stains.
Other workers have confirmed the overall accuracy of the original observations with the additional observations that this tumor can occur outside the acral regions on the forearm, arm and thigh and may also behave in a high grade fashion with recurrences and metastases as early as 3 months after the first excision. Before the entity was described, cases were probably diagnosed as peculiar inflammations or low grade myxoid malignant fibrous histiocytomas. The entity is probably much commoner than one would expect with such a recently described tumor.
Prognosis：Combining the two series, 30 of 63 patients with follow up (Recurrences: 48%) experienced one or more recurrences over periods as long as 10 years and two tumors (Metastasis: 3%) metastasized but there were no deaths caused by tumor.
Treatment：Treatment included surgery, radiotherapy and chemotherapy.
This uncommon, low grade sarcoma was first described by Montgomery, Devaney, Giordano and Weiss as recently as April, 1998 when they reported 51 cases collected from Sharon Weiss’ consultation files. They named the tumor descriptively, but verbosely, “inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells.” Four month later, the Kindbloms, working in Sweden, reported 44 cases of an identical tumor collected from The Armed Forces Institute of Pathology In Washington, DC and the Sahlgrenska University Hospital in Gothenburg, Sweden. They named it acral myxoinflammatory fibroblastic sarcoma. The sex incidence was equal; the age at diagnosis ranged from 20 to 91, with a median in the fifth decade; tumors were painless and had a long history (median 1 year); were located subcutaneously in the hands, wrists ,feet , and measured from 1 to 6 cm. Clinically they were thought to be ganglion cysts, tenosyonovitis, or giant cell tumors of tendon sheath. Initial histologic diagnoses often included pigmented villonodular tenosynovitis or various reactive fibroinflammatory processes. Histologically, there were usually more inflammatory cells that are present in the seminar slide, the tumor cells stained positively for vimentin and CD 68 but were negative for other stains.
Other workers have confirmed the overall accuracy of the original observations with the additional observations that this tumor can occur outside the acral regions on the forearm, arm and thigh and may also behave in a high grade fashion with recurrences and metastases as early as 3 months after the first excision. Before the entity was described, cases were probably diagnosed as peculiar inflammations or low grade myxoid malignant fibrous histiocytomas. The entity is probably much commoner than one would expect with such a recently described tumor.
Prognosis：Combining the two series, 30 of 63 patients with follow up (Recurrences: 48%) experienced one or more recurrences over periods as long as 10 years and two tumors (Metastasis: 3%) metastasized but there were no deaths caused by tumor.
Treatment：Treatment included surgery, radiotherapy and chemotherapy.
