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Archives of Pathology & Laboratory... Oct 2014Myxoinflammatory fibroblastic sarcoma (MIFS) is a malignant mesenchymal neoplasm most frequently arising in the distal extremities of adults, which usually behaves in a... (Review)
Review
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.
Topics: Antigens, Neoplasm; Chromosomes, Human, Pair 1; Chromosomes, Human, Pair 10; Diagnosis, Differential; Fibrosarcoma; Gene Amplification; Gene Rearrangement; Histone Acetyltransferases; Humans; Hyaluronoglucosaminidase; Liposarcoma, Myxoid; Myxosarcoma; Prognosis; Proteoglycans; Receptors, Transforming Growth Factor beta; Soft Tissue Neoplasms; Transcription Factors; Translocation, Genetic
PubMed: 25268202
DOI: 10.5858/arpa.2013-0549-RS -
The Canadian Veterinary Journal = La... Jul 2014A 5-year-old male Australian bearded dragon (Pogona vitticeps) was presented with a 2-month history of a periocular mass. The clinical evaluation included a physical...
A 5-year-old male Australian bearded dragon (Pogona vitticeps) was presented with a 2-month history of a periocular mass. The clinical evaluation included a physical examination, hematology, biochemistry, and radiographs. The mass was treated surgically and diagnosed as myxosarcoma. Strontium-90 plesiotherapy was attempted, but the mass recurred 5 mo later.
Topics: Animals; Anti-Inflammatory Agents, Non-Steroidal; Diagnosis, Differential; Eye Neoplasms; Lizards; Male; Meloxicam; Myxosarcoma; Neoplasm Recurrence, Local; Radiotherapy, Adjuvant; Strontium; Thiazines; Thiazoles
PubMed: 24982518
DOI: No ID Found -
BMJ Case Reports Feb 2014Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft...
Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised specimen were consistent with reticular/microcystic variant of schwannoma. This case report emphasises that the diagnosis of reticular/microcystic schwannoma should always be considered in cases where myxoid sarcomas are suspected as it can mimic malignant myxoid sarcomas, clinically and radiologically, thereby avoiding aggressive intervention and overtreatment.
Topics: Diagnosis, Differential; Female; Forearm; Humans; Magnetic Resonance Imaging; Middle Aged; Myxosarcoma; Neurilemmoma; Soft Tissue Neoplasms
PubMed: 24557478
DOI: 10.1136/bcr-2013-202963 -
Acta Orthopaedica Et Traumatologica... 2013Myxoinflammatory fibroblastic sarcoma (MIFS) is a recently described, rare low-grade sarcoma. Generally located in the upper and lower extremities, MIFS clinically...
Myxoinflammatory fibroblastic sarcoma (MIFS) is a recently described, rare low-grade sarcoma. Generally located in the upper and lower extremities, MIFS clinically mimics a benign cystic mass and is composed of spindle-like or atypical cells and mixed inflammatory infiltrates located in the fibroblastic myxoid stroma. Radiologic images and macroscopic appearance generally resemble a lobulated mass with irregular margins. We present a case of a tumoral mass with neoplastic cells at the center and a smooth surface with a previously undefined appearance. Myxoinflammatory fibroblastic sarcoma is significantly difficult to distinguish clinically from benign lesions and the surgeon should consider the possibility of malignancy in lesions located at the extremities.
Topics: Ankle; Diagnosis, Differential; Female; Fibrosarcoma; Humans; Middle Aged; Myxosarcoma; Radiography; Soft Tissue Neoplasms; Treatment Outcome
PubMed: 24509225
DOI: 10.3944/aott.2013.2476 -
Journal of the American Veterinary... Apr 2013
Topics: Animals; Dog Diseases; Dogs; Female; Heart Neoplasms; Heart Valve Diseases; Myxosarcoma
PubMed: 23547667
DOI: 10.2460/javma.242.8.1067 -
Chirurgia (Bucharest, Romania : 1990) 2013Retroperitoneal tumours represent a particular oncological pathology. No other human pathology is so deceiving and scarce in specific clinical symptoms as these tumours....
INTRODUCTION
Retroperitoneal tumours represent a particular oncological pathology. No other human pathology is so deceiving and scarce in specific clinical symptoms as these tumours. Usually borrowing the symptoms of nearby organs they are discovered in advanced or incurable stages.
MATERIAL AND METHOD
We have tried to present to you the challenge of diagnosing some retroperitoneal tumours. One of the rarest signs was by far the paraneoplastic syndrome or the secretion of active biological substances. The study group is composed of histopatologically diagnosed patients. The vast majority were selected from urology and general surgery wards, but there were cases from gynaecology, neurosurgery and even from endocrinology and gastroenterology.
RESULTS
Paraneroplastic syndrome represents the secretion of various substances by the tumour, substances that make changes at bioumoral level. In our study we found 3 cases of 32 patients (9,37%) with paraneoplastic syndrome. What is to be noticed is that because of this syndrome the diagnosis was more difficult and was indirectly referred to a retroperitoneal tumour. We would like to present these cases and their particularities.
CONCLUSIONS
Every case was a surgical and diagnostic challenge. Biologic active substance secretion or the paraneoplastic syndrome makes the clinical picture even more complicated for primitive retroperitoneal tumours. The clinical symptoms of these tumours require a more careful approach of these patients. Close co-operation with other medical specialties in cases such as ours is mandatory.
Topics: Diagnosis, Differential; Female; Fibrosarcoma; Follow-Up Studies; Humans; Male; Myxosarcoma; Paraneoplastic Syndromes; Radiography; Retroperitoneal Neoplasms; Treatment Outcome; Vena Cava, Inferior
PubMed: 23464765
DOI: No ID Found -
World Journal of Surgical Oncology Nov 2012Myxofibrosarcoma is a common sarcoma in the extremities of older people, but is rare in the head and neck region. Here, we report the case of a 42-year-old male patient... (Review)
Review
Myxofibrosarcoma is a common sarcoma in the extremities of older people, but is rare in the head and neck region. Here, we report the case of a 42-year-old male patient in whom myxofibrosarcoma generated from the sinus piriformis. Histopathologically, the tumor was characterized by spindle cellular proliferation with moderate cellular density in fibromyxoid stroma. Immunohistochemically, the tumor cells showed positive reactivity for vimentin, Ki-67, smooth muscle actin, and CD34, but negative staining for S-100. Based on these results, the tumor was diagnosed as a low-grade myxofibrosarcoma. Resection of the tumor was performed via a transcervical approach. The patient's postoperative clinical course was uneventful and no local recurrence or distant metastasis has been found so far. The pathology, clinical characteristics, and treatment of myxofibrosarcoma are also reviewed.
Topics: Adult; Fibrosarcoma; Humans; Male; Myxosarcoma; Pharyngeal Neoplasms; Prognosis; Review Literature as Topic
PubMed: 23152982
DOI: 10.1186/1477-7819-10-245 -
International Journal of Clinical and... 2012Primary and metastatic cardiac sarcomas represent a heterogeneous group of rare neoplasms with varying clinical course and diverse histogenetic line of differentiation....
BACKGROUND
Primary and metastatic cardiac sarcomas represent a heterogeneous group of rare neoplasms with varying clinical course and diverse histogenetic line of differentiation. To date, there exist no uniform guidelines for their surgical and/or oncological treatment.
METHODS
We evaluated retrospectively all patients undergoing cardiac surgery for primary or secondary cardiac sarcoma in the period 1999-2011 at the Erlangen Heart Centre to analyze their clinicopathological spectrum, treatment and outcome.
RESULTS
Five patients (3 women & 2 men; mean age: 46 years; range: 28-81 years) had primary cardiac sarcomas (6.7% of primary cardiac tumors) and 4 had cardiac metastasis from soft tissue sarcoma (1 case each of osteosarcoma, myxoid liposarcoma, alveolar soft part sarcoma and pleomorphic spindle cell sarcoma). Primary sarcomas were located in the left atrium (n=3), left ventricle (n=1) and right atrium (n=1). Histological types were myxosarcoma (3), pleomorphic undifferentiated sarcoma (1) and angiosarcoma (1). Four patients died at 2-64 months (mean, 24.5 months). Sarcoma metastasis to the heart developed at a mean of 109.5 months from initial diagnosis (range, 5-240 months). Three of them died of disease at a mean of 14 months after cardiac surgery and one is disease free 34 months after heart transplantation for metastasis.
CONCLUSIONS
Primary and metastatic cardiac sarcomas are very heterogeneous in their histological appearance, clinical presentation and course of the disease. Radical surgery combined with chemoradiation is promising in patients with resectable disease and may significantly prolong survival. Cardiac transplantation represents an emerging strategy for patients with isolated unresectable cardiac involvement.
Topics: Adult; Aged; Aged, 80 and over; Cardiac-Gated Imaging Techniques; Chemoradiotherapy, Adjuvant; Female; Germany; Heart Neoplasms; Heart Transplantation; Humans; Male; Metastasectomy; Middle Aged; Retrospective Studies; Sarcoma; Survival Analysis; Time Factors; Tomography, Spiral Computed; Treatment Outcome
PubMed: 23119110
DOI: No ID Found -
Annals of Surgical Oncology Jan 2013Myxofibrosarcomas (MFS) are a historically heterogeneous group of tumors that exhibit a propensity for local recurrence. The objectives of this study were to analyze the...
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.
Topics: Adult; Age Factors; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols; Brain Neoplasms; Chemotherapy, Adjuvant; Disease-Free Survival; Female; Fibrosarcoma; Humans; Kaplan-Meier Estimate; Lower Extremity; Lung Neoplasms; Male; Middle Aged; Myxosarcoma; Neoplasm Grading; Neoplasm Recurrence, Local; Neoplasm, Residual; Proportional Hazards Models; Radiotherapy, Adjuvant; Retrospective Studies; Soft Tissue Neoplasms; Torso; Upper Extremity; Young Adult
PubMed: 22890594
DOI: 10.1245/s10434-012-2572-3 -
Chang Gung Medical Journal 2011Primary neoplasms of the heart are rare. Malignant cardiac myxoma, or so-called myxosarcoma, accounts for about 6% of primary malignant cardiac tumors. Cerebral... (Review)
Review
Primary neoplasms of the heart are rare. Malignant cardiac myxoma, or so-called myxosarcoma, accounts for about 6% of primary malignant cardiac tumors. Cerebral metastasis of malignant cardiac myxoma is extremely rare; only three cases have been reported and two of them included an autopsy study. The case described herein is the first reported brain metastasis combined with multiple cerebral aneurysms originating from primary cardiac myxosarcoma. The true incidence of cerebral myxomatous aneurysm is unknown and the pathogenesis of myxomatous aneurysm formation has not been fully defined. The current hypothesis favors that tumor materials from cardiac myxomas embolize into the vasa vasorum of the peripheral arteries and subsequently prolifere in the vessel wall. This then leads to a weakening of subintimal tissue, such as the internal elastic lamina, with subsequent aneurysm formation. The prognosis of cardiac myxosarcoma is very poor. Although malignancies most likely develop from the mesenchymal cells, they are difficult to treat with any modality (operation, chemotherapy, radiotherapy or transplantation) because these tumors have usually undergone extensive spread by the time the diagnosis is made.
Topics: Adult; Brain Neoplasms; Female; Heart Neoplasms; Humans; Intracranial Aneurysm; Myxosarcoma
PubMed: 21733362
DOI: No ID Found