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SpecialtyFibrosarcoma_header_cell_0_1_0 Oncology Q1362453?uselang=en#P1995Fibrosarcoma_cell_0_1_1

Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. Fibrosarcoma_sentence_0

It is usually found in males aged 30 to 40. Fibrosarcoma_sentence_1

It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. Fibrosarcoma_sentence_2

It also involves the periosteum and overlying muscle. Fibrosarcoma_sentence_3

Presentation Fibrosarcoma_section_0

Adult-type Fibrosarcoma_section_1

Individuals presenting with fibrosarcoma are usually adults thirty to fifty-five years old, often presenting with pain. Fibrosarcoma_sentence_4

Among adults, males have a higher incidence for fibrosarcoma than females. Fibrosarcoma_sentence_5

Infantile-type Fibrosarcoma_section_2

In infants, fibrosarcoma (often termed congenital infantile fibrosarcoma) is usually congenital. Fibrosarcoma_sentence_6

Infants presenting with this fibrosarcoma usually do so in the first two years of their life. Fibrosarcoma_sentence_7

Cytogenetically, congenital infantile fibrosarcoma is characterized by the majority of cases having a translocation between chromosomes 12 and 15 (notated as t(12;15)(p13;q25)) that results in formation of the fusion gene, ETV6-NTRK3, plus individual cases exhibiting trisomy for chromosomes 8, 11, 17, or 20. Fibrosarcoma_sentence_8

The histology, association with the ETV6-NRTK3 fusion gene as well as certain chromosome trisomies, and the distribution of markers for cell type (i.e. cyclin D1 and Beta-catenin) within this tumor are similar to those found in the cellular form of mesoblastic nephroma. Fibrosarcoma_sentence_9

Indeed, mesoblastic nephroma and congenital infantile sarcoma appear to be the same disease with the exception that mesoblastic lymphoma originates in the kidney whereas congenital infantile sarcoma originates in non-renal tissues. Fibrosarcoma_sentence_10

Pathology Fibrosarcoma_section_3

The tumor may present different degrees of differentiation: low grade (differentiated), intermediate malignancy and high malignancy (anaplastic). Fibrosarcoma_sentence_11

Depending on this differentiation, tumour cells may resemble mature fibroblasts (spindle-shaped), secreting collagen, with rare mitoses. Fibrosarcoma_sentence_12

These cells are arranged in short fascicles which split and merge, giving the appearance of "fish bone" known as a herringbone pattern. Fibrosarcoma_sentence_13

Poorly differentiated tumors consist in more atypical cells, pleomorphic, giant cells, multinucleated, numerous atypical mitoses and reduced collagen production. Fibrosarcoma_sentence_14

Presence of immature blood vessels (sarcomatous vessels lacking endothelial cells) favors the bloodstream metastasizing. Fibrosarcoma_sentence_15

There are many tumors in the differential diagnosis, including spindle cell melanoma, spindle cell squamous cell carcinoma, synovial sarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor and biphenotypic sinonasal sarcoma. Fibrosarcoma_sentence_16

Diagnosis Fibrosarcoma_section_4

Ancillary testing for fibrosarcoma includes IHC, where vimentin is positive, cytokeratin and S100 are negative, and actin is variable. Fibrosarcoma_sentence_17

In animals Fibrosarcoma_section_5

Dogs Fibrosarcoma_section_6

Fibrosarcoma occurs most frequently in the mouth in dogs. Fibrosarcoma_sentence_18

The tumor is locally invasive, and often recurs following surgery. Fibrosarcoma_sentence_19

Radiation therapy and chemotherapy are also used in treatment. Fibrosarcoma_sentence_20

Fibrosarcoma is also a rare bone tumor in dogs. Fibrosarcoma_sentence_21

Cats Fibrosarcoma_section_7

In cats, fibrosarcoma occurs on the skin. Fibrosarcoma_sentence_22

It is also the most common vaccine-associated sarcoma. Fibrosarcoma_sentence_23

In 2014, Merial launched Oncept IL-2 in Europe for the management of such feline fibrosarcomas. Fibrosarcoma_sentence_24

Bostock DE, et al. Fibrosarcoma_sentence_25

performed a study of cats that had fibrosarcomas excised and were followed for a minimum of 3 years, or until death. Fibrosarcoma_sentence_26

Two factors, tumor site and mitotic index, were found to be of prognostic significance, but tumor size, duration of growth, and histologic appearance were not. Fibrosarcoma_sentence_27

Following removal of fibrosarcomas from the flank in 6 cats, none died as a result of the tumor but 24 of 35 (70%) cats with fibrosarcoma in the skin of the head, back, or limbs were euthanatized because of local recurrence, usually within 9 months of surgery. Fibrosarcoma_sentence_28

See also Fibrosarcoma_section_8


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