Aggressive myxoinflammatory fibroblastic sarcoma with multiple site metastases.

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue sarcoma which was initially observed in acral sites and characterised by spindle cells, pleomorphic bizarre cells and distinctive large Reed-Sternberg-like cells admixed with an intense inflammatory cell infiltrates. MIFS manifests as a slow growing often superficial lesion which can be mistaken as infectious or chronic inflammatory process or benign tumours such as nodular fasciitis, giant cell tumour of tendon sheath or synovial pseudocyst. We report a rare presentation of a MIFS in a 38-year-old man with extensive local spread from subcutaneous tissue to the ankle joint and bones as well as multiple synchronous metastases to lung, sixth rib and vertebra. Our case is peculiar for its aggressive clinical behaviour with short duration, fast growth and extensive metastases, a feature infrequent in MIFS.

Predictors of Local Recurrence in Patients With Myxofibrosarcoma.

OBJECTIVES: Myxofibrosarcoma (MFS) is reported to have a higher risk of local recurrence (LR) following definitive surgical excision relative to other soft tissue sarcomas. We reviewed our clinical experience treating MFS to investigate predictors of LR. MATERIALS AND METHODS: We retrospectively reviewed treatment outcomes for MFS patients treated at our institution between 1999 and 2015. A total of 52 patients were identified. Median age was 65 years (range, 21 to 86 y). Site of disease was: upper extremity (27%), lower extremity (46%), trunk (15%), pelvic (8%), and head and neck (4%). Patients had low, intermediate, high-grade, and unknown grade in: 23%, 8%, 67%, and 2% of tumors, respectively. Tumors were categorized as ≤5 cm (35%), >5 cm (56%), or unknown size (9%). In total, 71% received radiotherapy: 19% preoperative, 50% postoperative, and 2% both. All patients underwent surgery. Margins were negative in 71%, close/positive in 21%, and unknown in 8%. In total, 27% of patients received chemotherapy. Univariate Cox regression analysis was utilized to determine associations between clinical and treatment factors with LR. RESULTS: Median follow-up time was 2.9 years (range, 0.4 to 14.3 y). The 3-year actuarial LR, distant metastasis, and overall survival were: 31%, 15%, and 87%, respectively. Predictors of LR were patient age greater than or equal to the median of 65 years (hazard ratio, 13.46, 95% confidence interval, 1.71-106.18, P=0.013), and having close/positive tumor margins (hazard ratio, 3.4, 95% confidence interval, 1-11.53, P=0.049). CONCLUSIONS: In this institutional series of MFS older age and positive/close margins were significantly associated with a higher risk of LR.

Myxoinflammatory Fibroblastic Sarcoma: Review and Update.

Myxoinflammatory fibroblastic sarcoma is a rare soft tissue tumor with most occurring in the distal extremities of adult patients. It has a high rate of local recurrence and a low rate of metastasis. Because it may appear benign on clinical examination, and because the microscopic features are generally underrecognized, it is often inadequately treated and misdiagnosed. In this review, based upon experience and that of the literature, the intent is to highlight salient clinicopathologic features, detail the broad microscopic spectrum including high-grade aggressive variants, review the molecular features, and discuss its relation to hemosiderotic fibrolipomatous tumor.

A rare presentation of myxofibrosarcoma as a Pancoast tumor: a case report.

BACKGROUND: Myxofibrosarcoma is an aggressive soft tissue neoplasm, classified as a variant of malignant fibrous histiocytoma. Most often, it occurs in middle to late adult life peaking in the seventh decade and involving the lower extremities (77%), trunk (12%), and retroperitoneum or mediastinum (8%). We report the first case of thoracic myxofibrosarcoma presenting as a Pancoast tumor. CASE PRESENTATION: A 48-year-old non-tobacco smoking African-American man presented with a slow-growing mass in his neck along with 11 kg weight loss over 9 months. A review of his systems was positive for hoarseness and lowgrade intermittent fever without any shortness of breath or cough. A physical examination revealed a mass on the left side of his neck superior to his sternoclavicular joint measuring 3 × 3 × 1 cm. He had ptosis and miosis of his left eye. His breath sounds were decreased and coarse at the left apex. A neurological examination revealed 3/5 strength in his left upper arm. The remainder of the physical examination was unremarkable. Ultrasound of his neck showed an ill-defined heterogeneous mass lateral to his left thyroid lobe. A computed tomography scan of his chest showed a large multiloculated pleural-based mass in his left lung surrounding the adjacent neurovascular structures. A percutaneous biopsy was non-diagnostic. Subsequently, he underwent a left thoracotomy with biopsy. The mass extended from his anterior mediastinum medially at the level of the pulmonary trunk, superiorly into the superior sulcus and posteriorly into his chest wall. Surgical pathology confirmed the diagnosis of myxofibrosarcoma. CONCLUSIONS: Here we present a case of Pancoast tumor with myxofibrosarcoma as the underlying etiology. Pancoast syndrome generally entails an infiltrating lesion in the superior sulcus presenting with upper extremity pain, atrophy of the hand muscles, and Horner's syndrome. The differential diagnosis of Pancoast syndrome includes inflammatory and infectious etiologies, as well as neoplasms of benign and malignant nature. Of the neoplasms implicated, the most common are non-small cell lung carcinomas; myxofibrosarcoma presenting as a Pancoast tumor has not been reported in the literature.

Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant.

BACKGROUND AND OBJECTIVES: The clinical course of soft tissue myxofibrosarcoma is characterized by a high incidence of recurrences and there is no agreement on how to identify patients at major risk. An epithelioid histological variant has been described, with a possible worse prognosis. We reviewed our series to identify prognostic factors and assess clinical significance of the epithelioid variant. METHODS: We examined the clinico-pathological features of a series of 75 patients affected by soft tissue myxofibrosarcoma at a mean follow-up of 63 months (range 17-132). RESULTS: Disease specific survival and local recurrence free survival were, respectively, 84.8% and 76.8% at 5 years. Seven patients (8.6%) presented with the epithelioid variant with a survival of 62.5%. High grade and epithelioid morphology were negative prognostic factors for patient survival, high grade, and inadequate surgical margins for local recurrence. Radiotherapy had a local protective effect in high grade tumors. CONCLUSIONS: Our experience confirms the difficulties in obtaining wide margins in myxofibrosarcoma and the high rate of recurrence. Local recurrences did not significantly affect survival and a limb-sparing approach can be chosen also in recurrences. Patients affected by the epithelioid variant showed a worse prognosis. Chemotherapy should be considered as adjuvant treatment in this subtype. J. Surg. Oncol. 2016;114:50-55. © 2016 Wiley Periodicals, Inc.

Head and neck myxofibrosarcoma: a case report and review of the literature.

INTRODUCTION: Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, peaking in the seventh decade, and it is mainly encountered in the lower extremities. Myxofibrosarcoma of the head and neck are extremely rare. To the best of our knowledge, only 19 cases have been described in the head and neck so far. This is a literature review and retrospective chart review of our experience in head and neck myxofibrosarcoma treatment in our department. CASE PRESENTATION: In this case report we describe a 35-year-old Caucasian man who presented the first case of myxofibrosarcoma arising from the pterygopalatine fossa. The peculiar anatomical location and the extent in the midcheek region make this case a hard "challenge" for the surgeon, in order to guarantee wide surgical margins of resection. A total right maxillectomy was accomplished by means of the Weber-Ferguson approach, preserving the orbital floor. The excised portion was reconstructed using the free rectus abdominis myocutaneous flap. Postoperative radiotherapy was given to the area adjacent to the lesion, with a total dose of 60 Gy. No relapse occurred in the 27-month postoperative follow-up. CONCLUSIONS: The case described suggests the importance of combined surgical and adjuvant radiotherapy to avoid local and distant recurrences of the tumor. In our opinion, combined surgical and adjuvant radiotherapy followed by close clinical observation to search for a metastatic disease is advisable in all cases. Further studies are needed to confirm the efficacy of combined radio-chemotherapy for head and neck myxofibrosarcoma in terms of long-term disease-free survival.

Myxoinflammatory fibroblastic sarcoma: morphologic and genetic updates.

Myxoinflammatory fibroblastic sarcoma (MIFS) is a malignant mesenchymal neoplasm most frequently arising in the distal extremities of adults, which usually behaves in a low-grade manner but is capable of metastasizing to local and distant sites, rarely leading to death. It is a rare tumor whose unusual morphology can lead to erroneous histologic diagnosis, either as a nonneoplastic (infectious or inflammatory) process or as a variety of neoplastic diseases. While its exact origin is uncertain, ultrastructural studies have shown at least some of the constituent cells to be modified fibroblasts. Distinct and reproducible genetic abnormalities identified in MIFS are translocation t(1;10)(p22:q24), with rearrangements of the TGFBR3 and MGEA5 genes associated with increased levels of FGF8, and formation of marker/ring chromosome 3, with amplification of the VGLL3 locus. Because these genetic abnormalities are shared by both MIFS and hemosiderotic fibrohistiocytic lipomatous tumor, it is thought that these 2 morphologically distinct neoplasms may comprise a spectrum of disease defined by these genetics. We review the literature on MIFS and discuss morphology (including that of MIFS/hemosiderotic fibrohistiocytic lipomatous tumor hybrid lesions), immunohistochemistry, the differential diagnosis, and recent molecular genetic developments.

The management of myxofibrosarcoma - a ten-year experience in a single specialist centre.

The aim of this study was to assess the management of myxofibrosarcoma in a single specialist centre, and examine factors contributing to local recurrence, metastasis and patient survival. Retrospective analysis of the referral, diagnosis, and management were obtained. Outcome measures including local recurrence, metastasis and death were recorded. 30 patients (mean age of 65.8 years) were treated for myxofibrosarcoma with limb salvage surgery between January 2003 and July 2012. 25 patients were treated for primary disease. Mean follow-up was 49 months (range 10-122). Larger tumours were most likely to metastasise (p = 0.041). Tumour size, resection margin and grade did not predict local recurrence or death. Local recurrence developed in eight patients (26.7%) with six subsequently requiring amputation, and four patients (16.7%) developed metastasis. Our results regarding local control and patient survival compare with that of the literature regarding limb salvage for primary disease, but amputation may be required for recurrent disease.

Prognostic factors and outcomes of patients with myxofibrosarcoma.

BACKGROUND: Myxofibrosarcomas (MFS) are a historically heterogeneous group of tumors that exhibit a propensity for local recurrence. The objectives of this study were to analyze the prognostic factors and outcomes of patients with MFS treated at a single institution. METHODS: We retrospectively reviewed the records of 69 consecutive patients with pathologically confirmed MFS of the extremities or superficial trunk who underwent surgery from August 1995 to November 2010. Clinicopathologic features, treatments, and patient outcomes were reviewed. RESULTS: Sixty-nine patients were identified, of whom 38 were men (55%). The median age was 62 years. Sixty-four patients (93%) presented with primary tumors, and 5 patients (7%) presented with locally recurrent tumors. Median tumor size was 6.0 cm, and 44 patients (64%) had grade 3 tumors (FNCLCC [Fédération Nationale des Centres de Lutte Contre le Cancer] classification). Margins were microscopically positive in 14 patients (20%) and negative in 55 patients (80%), including close margins (<1 mm) in 14 patients (20%). Fifty-three patients (77%) received radiotherapy. At a median follow-up of 41 months, there were 11 local (16%) and 11 distant (16%) recurrences. The local and distant 5-year recurrence-free survival rates were 72% and 82%, and the 5-year overall survival was 61%. Increased age (scaled by 0.1; hazard ratio [HR] 1.80, P=0.002) and tumor size (HR 1.12, P=0.004) were negatively correlated with overall survival. Positive/close (<1 mm) margin status (HR 4.34, P=0.030) predicted worsened local recurrence-free survival. CONCLUSIONS: MFS exhibit a propensity for local recurrence, which is predicted by resection with positive or close margins. Aggressive surgery combined with radiotherapy may contribute to more effective local control.

A mass of myxofibrosarcoma in the lung.

Fibrosarcoma is a mesenchymal tumor constituted by malignant fibroblasts. Myxofibrosarcoma is one of the fibrosarcoma variants that mostly develops from dermal/subcutaneous tissues. The most common locations are the limbs, with rare occurrences in the chest, head, and neck. Since, to best of our knowledge, there is no such report in English literature, we hereby present a case of lung myxofibrosarcoma. A 47-year-old man who had chest pain for 4 months was admitted to our clinic. The chest X-ray revealed a homogeneous density in the left upper lung. His chest computed tomography (CT) scan showed a solid mass lesion of 52 x 58 mm in size at the apical segment of the left upper lobe. There was tumor invasion at the second and third ribs. CT-guided fine-needle tru-cut lung biopsy was performed. Histopathological evaluation result was high grade myxofibrosarcoma. Surgery was conducted. Myxofibrosarcoma is a surgically curable disease. However, local recurrences occur in 50% to 60% of the cases. Therefore, chemotherapy and/or radiotherapy is the suggested approach following surgery.

[Palliative sedation therapy for severe dyspnoea]. / Palliative Sedierung zur Symptomkontrolle massiver Dyspnoe.

In this case study we report on a patient with advanced myxofibrosarcoma and lung metastases, suffering from severe dyspnoea. Symptoms were not manageable by means of standard palliative care, which made palliative sedation therapy necessary in order to alleviate dyspnoea. On the basis of this case study, indications, drugs, therapy monitoring and ethical aspects of palliative sedation therapy are discussed.

Left atrium myxosarcoma: an exceptional cardiac malignant primary tumor.

We report the case of a 32-year-old patient with a left atrium myxosarcoma, presenting with congestive heart failure. It is a rare cardiac malignant primary tumor that seems to derive from the same cellular line as myxomas, but the prognosis is very different. These tumors present local recidives and distance metastasis, so the mean survival is about 1 year, independent of any therapeutical option.

Left atrial myxosarcoma with systemic metastasis: a case report.

The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. Echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.

Left atrial myxosarcoma with systemic metastasis: a case report

The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. Echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.

Sarcomas of the jaw.

A retrospective analysis is made of 61 patients with clinical and histological diagnosis of pure sarcomas of the jaw treated between 1950 and 1984. Surgery was the treatment of choice in 41 cases. Biopsy or palliative treatment were undertaken in the remaining patients. Twenty-four cases relapsed before 12 months, and 12 relapsed before 3 years. Twenty-four died before 12 months, nine before 2 years, and three after 2 years. At present there is no evidence of disease in eight patients: three out of 25 fibrosarcomas (one more than 1 year, one more than 13 years, and one more than 14 years), three out of 15 chondrosarcomas (one more than 1 year, one more than 13 years, and one more than 14 years), and two out of 15 osteosarcomas (one more than 18 years). The prognosis is gloomy. However, an early diagnosis and radical surgery contribute to an improvement in the course of the disease.