Acral Myxoinflammatory Fibroblastic Sarcoma Research Articles

Superficial Acral Fibromyxoma on the Palm

relatively new entity, most likely representing a fibrohistiocytic neoplasm. This tumor presents as a slowly growing solitary nodule, usually confined to the dermis and subcutaneous tissue. It has a distinct predilection for finger nails and toe nails of young to middle-aged adults. Here, we report a case of SAFM occurring on the palm, which is very rare site. A 58-year-old Korean woman presented with a history of a nodular mass on the palm of her right hand (Fig. 1). The tumor had been present for several years and had gradually increased in size. There were no subjective symptoms, such as pain. The patient underwent shave excision of this tumor. The lesion measured 0.7×0.7 cm. It was domeshaped, moderately circumscribed, firm, and had a uniform consistency. Histological examination showed a moderately cellular proliferation of spindled fibroblast-like and stellate cells embedded in myxoid matrix (Fig. 2A, B). The tumor cells were arranged in a loose storiform and focally fascicular pattern. Nuclear pleomorphism or mitotic activity was not present. Immunohistochemically, tumor cells were positive for CD99, negative for CD34 and epithelial membrane antigen (EMA) (Fig. 2C∼E). At 6 months follow-up, there was no recurrence of the tumor. SAFM typically is detected in adulthood as a solitary slow-growing mesenchymal mass involving the periungual and subungual regions of the fingers and toes. Hollmann et al. 2 reported 124 cases of digital fibromyxoma; most tumors occurred on the digits, with the majority growing in close proximity to the nail (97% on fingers, 96% on toes). Occurrence on the palm is very rare. The histopathological characteristics of these tumors are distinctive, showing a moderately cellular dermal proliferation of spindle-shaped fibroblast-like cells in a myxoid or collagenous matrix. The differential diagnosis for SAFM includes a fibrous histiocytoma variant, dermatofibrosarcoma, cutaneous myxoma, acquired fibrokeratoma, sclerosing perineurioma, and the acral myxoinflammatory fibroblastic sarcoma. Immunohistochemical results of SAFM are controversial. Tumor cells have been reported positive for CD34 (21/23, 91.3%), EMA (18/25, 72%), and CD99 (11/13, 84.6%) in one study 1 , but positive for only CD34 (42/61, 68.8%) and

Acral Myxoinflammatory Fibroblastic Sarcoma Successfully Treated Using Mohs Micrographic Surgery

Acral Myxoinflammatory Fibroblastic Sarcoma Successfully Treated Using Mohs Micrographic Surgery

Acral myxoinflammatory fibroblastic sarcoma: A case report and literature review

Acral myxoinflammatory fibroplastic sarcoma is an extremely rare soft-tissue sarcoma. It typically presents as an inflammatory mass in the distal extremities of adult patients. The authors present a review of the available literature as well as a discussion on the surgical management of a patient with acral myxoinflammatory fibroplastic sarcoma who originally requested conservative management but ultimately required a two-digit ray amputation after local recurrence.

The Contribution of Electron Microscopy to the Characterization of Soft Tissue Fibrosarcomas

In recent years, with the application of immunohistochemical and cytogenetic methods, numerous lesions formerly diagnosed as fibrosarcoma were reclassified as other malignant soft tissue tumors, and therefore conventional fibrosarcoma has largely become a diagnosis of exclusion. On the other hand, several new entities belonging to the group of fibrosarcomas have been characterized, including low-grade fibromyxoid sarcoma / hyalinizing spindle cell tumor with giant rosettes, sclerosing epithelioid fibrosarcoma, acral myxoinflammatory fibroblastic sarcoma, and the epithelioid variant of myxofibrosarcoma. Electron microscopy has contributed to the identification of the fibroblastic phenotype in these fibrosarcoma variants and still retains a central role in the differential diagnosis of these soft tissue sarcomas, thus helping to render specific diagnoses and to broaden the spectrum of fibrosarcoma variants.

Acral myxoinflammatory fibroblastic sarcoma: A case report and literature review

Acral myxoinflammatory fibroblastic sarcoma: A case report and literature review

Myxoinflammatory fibroblastic sarcoma of the thigh: A morphologic diversity

Myxoinflammatory fibroblastic sarcoma (MIFS)/acralmyxoinflammatory fibroblastic sarcoma (AMFS) is a rare, painless, low-grade neoplasm which commonly occurs in the extremities. It has a distinctive morphology and can be a diagnostic challenge, simulating inflammatory conditions as well as neoplastic conditions. They are low-grade sarcomas with a protracted clinical course, a high rate of local recurrence and a low rate of metastasis. We report a case of proximal MIFS in a 50-year-old woman who presented with a mass in the thigh.

Acral myxoinflammatory fibroblastic sarcoma fine needle aspiration: A case report

Cytological diagnosis of low grade sarcomas can be a daunting task, owing to the varied cytomorphological appearances possible. We report a case of acral myxoinflammatory fibroblastic sarcoma (AMIFS) in a woman who presented with a longstanding mass on the dorsum of her left foot. The diagnosis was suggested by fine needle aspiration cytology and established by wide excision. Microscopic examination showed that fine needle aspirate smears of this lesion contained the characteristic features seen in the surgical excision of this AMIFS: myxoid material, spindled to epithelioid cells with variably prominent nucleoli, nuclear pseudoinclusions, bipolar cytoplasmic extensions, globules of extracellular material, and bizarre virocyte or ganglion-like giant cells.

Management of acral myxoinflammatory fibroblastic sarcoma

Acral myxoinflammatory fibroblastic sarcoma (AMFS) is a rare, low-grade sarcoma that commonly affects the distal extremities. From the published cases, therapy for AMFS to date has been comprised of excision or amputation, with limited use of radiotherapy (RT) or chemotherapy. In this report, the outcome of 17 patients with AMFS treated at the study institution was reported. A retrospective review of all cases of AMFS identified in the Sarcoma Database in the Department of Radiation Oncology at the study institution was conducted. Treatment records and data from follow-up visits of patients were reviewed. Seventeen patients were identified. All the patients underwent surgical resection (15 excisions and 2 amputations). Positive surgical margins after excisions were noted in 5 patients and were widely positive in 1 patient. Of the 17 patients, 14 patients received some form of RT. The average total dose was 56.4 Gray (Gy). Eight patients received preoperative RT alone, 5 patients received preoperative RT and postoperative RT, and 1 patient received preoperative RT and intraoperative RT. Median follow-up was 24.5 months. One patient presented with recurrent disease and was treated with resection, and both pre- and postoperative RT. He was free of disease 23 months after his last treatment. No local recurrence was noted in the remaining patients. Of the 14 patients undergoing preoperative RT, complete pathologic necrosis or no tumor was noted in 1 of the patients. No metastatic disease was observed in any of the patients. There was no significant radiation toxicity observed in any of the patients. Data were consistent with local control of distal extremity sarcomas with resection and RT, suggesting that limb-sparing surgery with this treatment combination is an appropriate option in the limb-sparing control of patients with AMFS, even those with positive surgical margins.

Acral myxoinflammatory fibroblastic sarcoma

Acral myxoinflammatory fibroblastic sarcoma is a rare low-grade malignant soft tissue tumor, usually observed in the extremities of middle-aged patients. We report a case involving the third finger of the left hand of a middle-aged man. The tumor showed a nodular architecture, with cellular areas, occasional foci of hyalinized fibrosis, and hypocellular areas with a myxoid background. Various neoplastic cells were identified including spindled or rounded epithelioid cells and occasional bizarre giant cells, morphologically mimicking ganglion cells. Tumor cells were strongly immunoreactive for vimentin and variably positive for CD68 and CD34. The tumor was completely removed, without further treatment.

A rare case of acral myxoinflammatory fibroblastic sarcoma at the hand

One case of acral myxoinflammatory fibroblastic sarcoma is presented. Most of the soft tissue tumefactions of the distal extremities are benign. Sarcomas are rare and usually high grade. Acral myxoinflammatory fibroblastic sarcoma is a unique low-grade tumor described by Kindblom in 1998. This neoplasm has a propensity for digits, presents a low rate of metastasis and is characterized by a high rate of local multiple and aggressive recurrence. The clinical presentation, the frequent association with tenosynovitis and the extensive inflammatory infiltrate can lead to misdiagnosis by both the surgeon and the pathologist. Correct and early diagnosis depends on a good knowledge of histological and immunohistochemical findings. It is important to avoid recurrences recurring to an immediate wide excision of the tumor.

Radiation Therapy in the Management of Acral Myxoinflammatory Fibroblastic Sarcoma

Radiation Therapy in the Management of Acral Myxoinflammatory Fibroblastic Sarcoma

Acral Myxoinflammatory Fibroblastic Sarcoma in a Renal Transplant Patient: A Case Report

Herein, we have reported a case of a 62-year-old patient who presented at 10 months after renal transplantation with a nodular mass on the dorsum of his foot. Local excision was performed and an acral myxoinflammatory fibroblastic sarcoma was diagnosed. This is a rare, low-grade sarcoma with a high rate of local recurrence, sometimes leading to amputation. Metastasis to the lungs and liver has been reported, but is rare. Since our patient received triple immunosuppressive therapy, our major concern was a much more aggressive behavior. However, more than 2 years after excision of the tumor, the patient is still free of disease.

Acral myxoinflammatory fibroblastic sarcoma: case series and immunohistochemical analysis

Acral myxoinflammatory fibroblastic sarcoma (AMFS) is a rare, low-grade neoplasm most often occurring on the extremities of adults. It consists of mixed inflammatory infiltrates with nodules of epithelioid, spindled and bizarre-appearing cells within a fibrosclerotic-to-myxoid stroma. AMFS frequently recurs, but only rarely metastasizes. A retrospective analysis of all cases of AMFS seen in the past 4 years from the Stanford University Laboratory of Surgical Pathology and collaborating institutions was performed. We sought to better characterize the clinicopathologic characteristics of this rare tumor. Immunohistochemical stains, including CD34, epithelial membrane antigen (EMA), epidermal growth factor receptor (EGFR), CD117, CD163, Ki67 and p53, were also performed. Eighteen cases were analyzed, and clinical information was available on 13 of them. The mean age at diagnosis was 48 years old, 10/13 (77%) occurred on the distal extremities and diameter of the lesions ranged from 1.0 to 10.0 cm. Treatment included wide local or radical excision and local recurrences were not reported. Many of the lesions were multinodular. Histologic characteristics included the presence of fibrosclerotic and myxoid stroma, sheets of spindled to round epithelioid cells, Reed-Sternberg or virocyte-like cells, lipoblast-like cells and rare mitotic figures. In most cases, CD34, EGFR and CD163 were diffusely positive. EMA and CD117 were weakly positive in some cases. Ki67 labeled < 10% of cells, and staining with P53 was variable. Because AMFS may be mistaken for lymphoma, infection or tumors with higher metastatic potential, correct diagnosis is important to avoid unnecessary procedures and allow for proper clinical management. EGFR positivity suggests possible therapeutic use in aggressive cases.

Acral myxoinflammatory fibroblastic sarcomas: are they all low‐grade neoplasms?

Acral myxoinflammatory fibroblastic sarcoma (AMIFS) is a low-grade sarcoma that presents mostly in distal extremities of middle-aged patients. The clinicopathologic features, immunohistochemical profile and follow-up data of five cases (three men and two women; age 39-65 years) are presented. The tumors presented as a slow-growing, poorly circumscribed, subcutaneous masses in the hands (three), foot (one) and calf (one), with dermal involvement in two cases. They had myxoid and hyaline stroma with dense acute and chronic inflammation. Spindle cells, large bizarre ganglion-like cells and multivacuolated cells were seen. Variable reactivity in lesional cells were noted for vimentin, Alpha-1-antitrypsin (A1AT), factor XIIIa, CD68, CD95, CD117, Alpha-1-antichymotrypsin (A1ACT), CD34, AE1/3, S-100 protein, EBER, CD63 and CD15. MIB-1 showed 5-30% nuclear labeling. They were negative for cytokeratin AE1/3, smooth muscle actin, CD30, ALK-1, EMA, desmin, CMV, HMB-45 and Melan-A. Follow up ranged from 2 weeks to 95 months (mean 54). One patient was lost to follow up; three underwent excision and one patient had below the knee amputation. Two patients developed metastases (one died of disease), and two patients are alive without evidence of disease. AMIFS are rare tumors that may involve joints and tendons leading to clinical diagnosis of ganglion cyst or tenosynovitis.

Fine needle aspiration cytology of an acral myxoinflammatory fibroblastic sarcoma: case report with cytological and cytogenetic findings

Fine needle aspiration cytology of an acral myxoinflammatory fibroblastic sarcoma: case report with cytological and cytogenetic findings

Acral Myxoinflammatory Fibroblastic Sarcomas: MRI Findings in Four Cases

Acral myxoinflammatory fibroblastic sarcoma is a rare, recently described, low-grade sarcoma that involves mainly the distal extremities. The purpose of this study is to report the MRI findings in four cases of acral myxoinflammatory fibroblastic sarcoma. Acral myxoinflammatory fibroblastic sarcomas may present with various MRI patterns that probably reflect their variable histologic composition. Differential diagnosis with other benign conditions, especially with ganglion cysts and giant cell tumors of the tendon sheath, may be difficult. We report tumoral invasion of the bone in one case, which to our knowledge has not been previously described.

Acral myxoinflammatory fibroblastic sarcoma: report of a case

患者男,30岁.因右足背无痛性肿块半年于2006年3月15日入院就诊.门诊体检:右足背可扪及一肿块,大小5 cm×4 cm,质中,活动欠佳.行肿块切除术,术中肿块与周围组织粘连,不易完全剥除。

Sarcome fibroblastique myxoinflammatoire acral : à propos de deux cas

Acral myxoinflammatory fibroblastic sarcoma is a rare low-grade malignant soft tissue tumor, usually observed in the extremities of middle-aged adults. We report two cases which occurred in the thumb and knee of middle-aged women. Both tumors showed a multinodular architecture, with cellular areas, occasional foci of hyalinized fibrosis, and hypocellular areas with a myxoid background. Various neoplastic cells were identified including spindled or rounded epithelioid cells and occasional bizarre giant cells, morphologically mimicking Reed-Sternberg cells or ganglion cells. Tumor cells were strongly immunoreactive for vimentin, and variably positive for CD68 and CD34. Both tumors were completely resected and patients were free of disease without any further treatment after a mean follow-up of 14 months.

Case Reports: Acral Myxoinflammatory Fibroblastic Sarcoma

During the past 2 years, we treated five patients with acral myxoinflammatory fibroblastic sarcoma at our institution. Four patients presented with a firm, painless mass in the hand that appeared over several months. One patient discovered a painless mass in his shoulder region. The five patients initially were diagnosed as having benign conditions and treated with intralesional or marginal excision by referring physicians, only to have the lesion reappear as sarcoma. Each patient was treated with wide resection of the tumor bed. Acral myxoinflammatory fibroblastic sarcoma is a rare, but increasingly recognized sarcoma of the distal extremities, which often is confused with benign lesions. Surgeons should be familiar with this tumor's clinical, radiographic, and histologic appearances as it has a high rate of recurrence and can metastasize.

Low‐grade fibromyxoid sarcoma arising in the big toe

Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor. Reported herein is a case of LGFMS arising in the big toe. The patient was a 58-year-old man who underwent excision of the tumor. The tumor was well-demarcated. Histologically, there were proliferating spindle-shaped tumor cells arranged in a whorled growth pattern, and the stroma showed hyalinized collagen bundles and a myxoid matrix. Nuclear mitotic figures were conspicuous in part. A large rosette-like structure with hyalinized stroma was found, which is characteristic of LGFMS. The differential diagnosis included tumor occurrence in adults; tending to arise in distal extremities; and having bland fibromyxoid histological features, such as fibroma of tendon sheath, low-grade myxofibrosarcoma and acral myxoinflammatory fibroblastic sarcoma. It was not possible to detect the FUS/CREB3L2 and FUS/CREB3L1 fusion genes from the formalin-fixed and paraffin-embedded tissue, although the histological features of the present case were typical of LGFMS. LGFMS may become more common with time, and unique cases may accumulate.