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Journal of Korean Medical Science Feb 2001The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very...
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.
Topics: Adult; Heart Neoplasms; Humans; Male; Myxosarcoma
PubMed: 11289388
DOI: 10.3346/jkms.2001.16.1.111 -
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 -
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 -
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 -
Annals of Surgery May 1964
Topics: Amputation, Surgical; Bone Neoplasms; Carcinoma, Squamous Cell; Chondrosarcoma; Congenital Abnormalities; Fibrosarcoma; Hemipelvectomy; History; Humans; Infant, Newborn; Leg; Liposarcoma; Melanoma; Myxosarcoma; Osteomyelitis; Osteosarcoma; Pathology; Pelvic Neoplasms; Photomicrography; Radiography; Rhabdomyosarcoma; Sarcoma
PubMed: 14153804
DOI: 10.1097/00000658-196405000-00004 -
The Cornell Veterinarian Apr 1992A large fibrotic mass originating from the cecal base was discovered upon surgical exploration of the abdomen in a Thoroughbred mare with a history of chronic colic and...
A large fibrotic mass originating from the cecal base was discovered upon surgical exploration of the abdomen in a Thoroughbred mare with a history of chronic colic and weight loss. The mass protruded intraluminally resulting in partial obstruction. Surgical excision was not feasible due to the location of the mass and the inability to exteriorize it adequately from the abdominal cavity. The mass was fibrous with a shiny, gelatinous material present throughout the neoplasm. Histologically, large confluent spaces filled with mucopolysaccharides were identified by staining with Alcian blue. The diagnosis of myxosarcoma was based upon finding of atypical fibroblastic cells, mucinous stroma, local invasiveness, and metastasis to the regional lymph nodes. Myxomatous tumors have not previously been documented to occur in the equine intestinal tract.
Topics: Animals; Cecal Neoplasms; Colic; Female; Horse Diseases; Horses; Myxosarcoma
PubMed: 1623729
DOI: No ID Found -
BMJ Case Reports Jul 2018Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue sarcoma which was initially observed in acral sites and characterised by spindle cells, pleomorphic...
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.
Topics: Adult; Ankle Joint; Bone Neoplasms; Combined Modality Therapy; Fibrosarcoma; Humans; Lung Neoplasms; Lymphatic Metastasis; Male; Myxosarcoma; Neoplasm Recurrence, Local; Soft Tissue Neoplasms; Tomography, X-Ray Computed; Whole Body Imaging
PubMed: 30021730
DOI: 10.1136/bcr-2018-224259 -
Animals : An Open Access Journal From... May 2024Neoplasia has been reported in lizards, but more research is needed to accurately document the prevalence and prognosis of the various known neoplasms that affect...
Neoplasia has been reported in lizards, but more research is needed to accurately document the prevalence and prognosis of the various known neoplasms that affect lizards. This study reviewed medical records from an online database, the Exotic Species Cancer Research Alliance (ESCRA), and reviewed published literature to determine the prevalence of neoplasia, malignancy, metastasis, treatment strategies, and outcomes by species and sex. Records from 55 individual lizards, 20 different species, and 37 different tumors were identified. In the literature, 219 lizards, 59 species, and 86 unique tumors were identified from 72 published case reports. Potential signalment factors such as age, sex, and species were evaluated to see if they affected case outcome. Additional factors including neoplasia type, presence of metastasis, and types of pursued treatments were also evaluated. Statistical analysis was performed to determine whether a factor was significantly associated with animal death due to the identified neoplasia or with animal survival or death due to other causes (non-neoplastic outcomes). Komodo dragons and savannah monitors were more likely to die from neoplasia compared to other lizard species. Cases where the status of metastasis was unknown were significantly associated with death due to neoplasia. Having an unknown status of male versus female was significantly associated with non-neoplastic outcomes of death. Leukemia and islet cell carcinoma were significantly associated with death due to neoplastic causes. Chondrosarcoma, myxosarcoma, osteosarcoma, and squamous cell carcinoma were significantly associated with non-neoplastic outcomes of death. Surgery alone and radiation therapy alone each were significantly associated with non-neoplastic outcomes of death, while lizards not receiving treatment were significantly associated with death due to neoplasia. Benign neoplasia was significantly associated with non-neoplastic outcomes of death. These results will aid in the improved diagnosis and management of neoplasia in lizard species, as well as expanding our understanding of prognostic indicators of neoplasia in lizards.
PubMed: 38791614
DOI: 10.3390/ani14101395 -
Oncology Research and Treatment 2020The aim of this retrospective analysis is to understand the natural history of myxofibrosarcoma (MFS), in particular whether the prognosis can be influenced by...
BACKGROUND
The aim of this retrospective analysis is to understand the natural history of myxofibrosarcoma (MFS), in particular whether the prognosis can be influenced by histologic grade.
METHODS
We reviewed 229 adult patients with primary MFS of the limbs. We analyzed disease-specific survival (overall survival [OS]) and local recurrence (LR).
RESULTS
Median age was 70 years (range, 19-92). Sixteen (7.0%) were grade 1, 38 (16.6%) grade 2, and 175 (76.4%) grade 3. A worse OS was found in grade 3 MFS (73.1%) than in grade 2 and 1 MFS (91.9 and 100%, respectively) at 5 years (p = 0.031). Locally recurred MFS had a worse OS (p = 0.018). A better LR-free rate (100% at 5 years) was observed in grade 1 MFS; however, a similar rate was observed between grade 2 and 3 tumors (77.1 and 80.0% at 5 years, respectively, p = 0.412).
CONCLUSIONS
Grade 3 MFS has the worst prognosis. Grade 1 MFS have the lowest risk of LR. These data could help identify a high-risk patient group, thus selecting a more careful follow-up for higher-risk patients. Since MFS mostly affects the elderly population, it might be useful to reserve adjuvant treatments (radiotherapy and chemotherapy) to higher-risk patients.
Topics: Adult; Aged; Extremities; Female; Fibrosarcoma; Follow-Up Studies; Humans; Male; Middle Aged; Myxosarcoma; Neoplasm Grading; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Soft Tissue Neoplasms; Survival Rate; Young Adult
PubMed: 32268331
DOI: 10.1159/000506844 -
Thorax Jul 1972Pulmonary blastomas are extremely rare subpleural tumours consisting of relatively well-differentiated branched tubular glands which resemble fetal lung tissue embedded...
Pulmonary blastomas are extremely rare subpleural tumours consisting of relatively well-differentiated branched tubular glands which resemble fetal lung tissue embedded in a malignant mesodermal stroma. The previous 13 established cases reported up to June 1969 are now supplemented by a fourteenth. The patient was a 32-year-old man who developed acute pain in the right chest followed by a haemorrhagic pleural effusion. At thoracotomy a yellowish-white necrotic and vascular tumour was located lying loosely in the fissure between the upper and the middle lobes. The histological appearance of the tumour was unusual in that the mesodermal element was very variable and in different areas simulated fibrosarcoma, leiomyosarcoma, lipomyxosarcoma, and malignant haemangiopericytoma. This diversity of mesodermal proliferation is best explained on the basis that the tumour has originated in an embryonic or pleuripotential type of mesenchyme, the site of which is probably in the periphery of the lung.
Topics: Adult; Fibrosarcoma; Hemangiopericytoma; Humans; Leiomyosarcoma; Lung; Lung Neoplasms; Male; Myxosarcoma; Pleural Effusion
PubMed: 5075621
DOI: 10.1136/thx.27.4.487