Definition of Malignant Mesenchymal Neoplasms: A usually aggressive malignant mesenchymal cell tumour most commonly arising from muscle, fat, fibrous tissue, bone, cartilage, and blood vessels. Sarcomas occur in both children and adults. The prognosis depends largely on the degree of differentiation (grade) of the tumour. Representative subtypes are liposarcoma, leiomyosarcoma, osteosarcoma, and chondrosarcoma.
2B50 Chondrosarcoma primary site
2B50.0 Chondrosarcoma of bone or articular cartilage of limbs
2B50.1 Chondrosarcoma of bone or articular cartilage of pelvis
2B50.2 Chondrosarcoma of bone or articular cartilage of ribs sternum or clavicle
2B50.Y Chondrosarcoma of bone or articular cartilage of other specified sites
2B50.Z Chondrosarcoma of bone or articular cartilage of unspecified sites
Definition of Osteosarcoma primary site: A usually aggressive malignant bone-forming mesenchymal tumour, predominantly affecting adolescents and young adults. It usually involves bones and less frequently extraosseous sites. It often involves the long bones (particularly distal femur, proximal tibia, and proximal humerus). Pain with or without a palpable mass is the most frequent clinical symptom. It may spread to other anatomic sites, particularly the lungs.
2B51 Osteosarcoma primary site
2B51.0 Osteosarcoma of bone or articular cartilage of jaw
2B51.1 Osteosarcoma of bone or articular cartilage of limbs
2B51.2 Osteosarcoma of bone or articular cartilage of pelvis
2B51.Y Osteosarcoma of bone and articular cartilage of other specified sites
2B51.Z Osteosarcoma of bone and articular cartilage of unspecified sites
Definition of Ewing sarcoma primary site: A small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ewing’s sarcoma/peripheral neuroectodermal tumour. It affects mostly males under age 20, and it can occur in soft tissue or bone. Pain and the presence of a mass are the most common clinical symptoms.
2B52 Ewing sarcoma primary site
2B52.0 Ewing sarcoma of bone or articular cartilage of limbs
2B52.1 Ewing sarcoma of bone or articular cartilage of pelvis
2B52.2 Ewing sarcoma of bone or articular cartilage of ribs
2B52.3 Ewing sarcoma of soft tissue
Definition of Ewing sarcoma of soft tissue: A rare malignant neoplasm of the soft tissues. It is typically a disease of children and young adults. It is characterised by t(11:22) (q24: q12) resulting in the expression of EWS/FLI-1 chimeric transcript. Most commonly occurs in the paravertebral region, chest wall, pelvis and lower extremities.
2B52.Y Ewing sarcoma of bone and articular cartilage of other specified sites
2B52.Z Ewing sarcoma of bone and articular cartilage of unspecified sites
2B53 Fibroblastic or myofibroblastic tumour primary site
2B53.0 Myxofibrosarcoma primary site
2B53.1 Fibroblastic or myofibroblastic tumour of skin
2B53.Y Other specified fibroblastic or myofibroblastic tumour primary site
2B53.Z Fibroblastic or myofibroblastic tumour primary site unspecified
Definition of Fibroblastic or myofibroblastic tumour primary site unspecified: A pleomorphic sarcoma characterised by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern.
2B54 Unclassified pleomorphic sarcoma primary site
2B54.0 Unclassified pleomorphic sarcoma of skin
Definition of Unclassified pleomorphic sarcoma of skin: A rare malignant neoplasm arising from the skin. It is characterised by the presence of spindle cells in a storiform pattern and histiocytes with abundant cytoplasm.
2B54.1 Unclassified pleomorphic sarcoma of retroperitoneum or peritoneum
2B54.Y Unclassified pleomorphic sarcoma primary site other specified site
2B54.Z Unclassified pleomorphic sarcoma primary site unspecified site
Definition of Rhabdomyosarcoma primary site: Rhabdomyosarcoma is a malignant soft tissue tumour which develops from cells of striated muscle. It is the most common form of tumour found in children and adolescents.
2B55 Rhabdomyosarcoma primary site
2B55.0 Rhabdomyosarcoma of the oral cavity or pharynx
2B55.1 Rhabdomyosarcoma of respiratory or intrathoracic organs
2B55.2 Rhabdomyosarcoma of male genital organs
2B55.Y Rhabdomyosarcoma other specified primary site
2B55.Z Rhabdomyosarcoma unspecified primary site
2B56 Angiosarcoma primary site
2B56.0 Angiosarcoma of heart
2B56.1 Angiosarcoma of skin
Definition of Angiosarcoma of skin: A malignant tumour arising from the endothelial cells of the blood vessels. Microscopically, it is characterised by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphoedema are at increased risk of developing angiosarcoma.
2B56.2 Angiosarcoma of breast
Definition of Angiosarcoma of breast: A malignant vascular neoplasm arising from the breast.
2B56.3 Angiosarcoma of liver
Definition of Angiosarcoma of liver: A malignant vascular neoplasm arising from the liver.
2B56.Y Angiosarcoma other specified primary site
2B56.Z Angiosarcoma unspecified primary site
Definition of Kaposi sarcoma primary site: A malignant neoplasm characterised by a vascular proliferation which usually contains blunt endothelial cells. Erythrocyte extravasation and hemosiderin deposition are frequently present. The most frequent site of involvement is the skin; however it may also occur internally. It generally develops in people with compromised immune systems including those with acquired immune deficiency syndrome (AIDS).
2B57 Kaposi sarcoma primary site
2B57.0 Kaposi sarcoma of lung
2B57.1 Kaposi sarcoma of skin
Definition of Kaposi sarcoma of skin: A Kaposi sarcoma arising from the skin. It presents with patches, plaques, or nodules.
2B57.2 Kaposi sarcoma of gastrointestinal sites
2B57.Y Kaposi sarcoma of other specified primary sites
2B57.Z Kaposi sarcoma of unspecified primary site
2B58 Leiomyosarcoma primary site
2B58.0 Leiomyosarcoma of retroperitoneum or peritoneum
2B58.1 Leiomyosarcoma of uterus
2B58.2 Leiomyosarcoma of stomach
Definition of Leiomyosarcoma of stomach: This is a malignant nonepithelial tumour that arises from cells lining the stomach that develop into smooth-muscle.
2B58.Y Leiomyosarcoma other specified primary site
2B58.Z Leiomyosarcoma unspecified primary site
Definition of Liposarcoma primary site: Liposarcoma, a type of soft tissue sarcoma, describes a group of lipomatous tumours of varying severity ranging from slow-growing to aggressive and metastatic. Liposarcomas are most often located in the lower extremities or retroperitoneum, but they can also occur in the upper extremities, neck, peritoneal cavity, spermatic cord, breast, vulva and axilla.
2B59 Liposarcoma primary site
2B59.0 Liposarcoma of soft tissue of limb
2B59.1 Liposarcoma of retroperitoneum or peritoneum
2B59.2 Liposarcoma of male genital organs
2B59.Y Liposarcoma other specified primary site
2B59.Z Liposarcoma unspecified primary site
Definition of Synovial sarcoma primary site: A malignant neoplasm characterised by the chromosomal translocation t(X;18)(p11;q11). It can occur at any age, but mainly affects young adults, more commonly males. Although any site can be affected, the vast majority of the cases arise in the deep soft tissues of extremities, especially around the knee. Microscopically, synovial sarcoma is classified as monophasic (with a spindle or epithelial cell component) or biphasic (with both spindle and epithelial cell components). Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph nodes.
2B5A Synovial sarcoma primary site
2B5A.0 Synovial sarcoma of soft tissues of limb
2B5A.1 Synovial sarcoma of respiratory or intra-thoracic organs
2B5A.Y Synovial sarcoma other specified primary site
2B5A.Z Synovial sarcoma unspecified primary site
Definition of Gastrointestinal stromal tumour primary site: This is the most common mesenchymal tumour that arises in the gastrointestinal tract. It is generally immunohistochemically positive for CD117 (KIT), phenotypically paralleling Cajal-cell differentiation, and most examples contain KIT- or PDGFRA- activating mutations. It is most frequent in the stomach and to a lesser degree in the small intestine. The prognosis depends on the tumour size and the mitotic activity.
2B5B Gastrointestinal stromal tumour primary site
2B5B.0 Gastrointestinal stromal tumour of stomach
Definition of Gastrointestinal stromal tumour of stomach: A gastrointestinal stromal tumour that arises from the stomach. It covers a spectrum of benign to malignant mesenchymal neoplasms and includes most gastric smooth muscle tumours, leiomyoblastomas, and tumours formerly called gastrointestinal autonomic nerve tumours.
2B5B.1 Gastrointestinal stromal tumour of small intestine
Definition of Gastrointestinal stromal tumour of small intestine: A gastrointestinal stromal tumour that arises from the small intestine. It usually affects adults over fifty years of age. The majority of cases have spindle cell morphology. The prognosis depends on the tumour size and the mitotic activity.
2B5B.Y Gastrointestinal stromal tumour of other gastrointestinal sites
2B5B.Z Gastrointestinal stromal tumour of unspecified gastrointestinal sites
Definition of Endometrial stromal sarcoma primary site: A malignant, infiltrating mesenchymal tumour arising from the uterine corpus, cervix, vagina, and the ovary. Based on its morphologic characteristics, it is classified as either a low grade or an undifferentiated (high grade) stromal sarcoma. The low grade endometrioid stromal sarcoma is characterised by the presence of oval to spindle-shape cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. Numerous small vessels are also present. The undifferentiated stromal sarcoma is characterised by an aggressive clinical course, the presence of significant cellular atypia, pleomorphism, and high mitotic activity.
2B5C Endometrial stromal sarcoma primary site
2B5D Malignant mixed epithelial mesenchymal tumour primary site
2B5D.0 Malignant mixed epithelial mesenchymal tumour of ovary
Definition of Malignant mixed epithelial mesenchymal tumour of ovary: Malignant mixed epithelial mesenchymal tumour of the ovary is a rare and very aggressive neoplasm presenting most commonly in postmenopausal women and is composed of adenocarcinomatous and sarcomatous elements and, depending on the types of these elements, can be classified as homologous or heterologous. It often has a poor prognosis.
2B5D.1 Malignant mixed epithelial and mesenchymal tumour of corpus uteri
Definition of Malignant mixed epithelial and mesenchymal tumour of corpus uteri: A primary malignant neoplasm of the uterine corpus characterised by the presence of an epithelial and a mesenchymal component. This category includes carcinosarcoma, carcinofibroma, and adenosarcoma.
2B5D.Y Malignant mixed epithelial mesenchymal tumour other specified primary site
2B5D.Z Malignant mixed epithelial mesenchymal tumour unspecified primary site
2B5E Malignant nerve sheath tumour of peripheral nerves or autonomic nervous system primary site
2B5F Sarcoma not elsewhere classified primary site
2B5F.0 Sarcoma not elsewhere classified of uterus
2B5F.1 Sarcoma not elsewhere classified of retroperitoneum or peritoneum
2B5F.10 Myosarcomas of omentum
2B5F.1Y Other specified sarcoma not elsewhere classified of retroperitoneum or peritoneum
2B5F.1Z Sarcoma not elsewhere classified of retroperitoneum or peritoneum unspecified
2B5F.2 Sarcoma not elsewhere classified of other specified sites
2B5F.3 Sarcoma not elsewhere classified primary site unknown
2B5G Myosarcoma of uterus part not specified
2B5H Well differentiated lipomatous tumour primary site
2B5J Malignant miscellaneous tumours of bone or articular cartilage of other or unspecified sites
2B5K Unspecified malignant soft tissue tumours or sarcomas of bone or articular cartilage of other or unspecified sites
2B5Y Other specified malignant mesenchymal neoplasms
2B5Z Malignant mesenchymal neoplasm of unspecified type
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