Malignant Mesenchymal Neoplasms – Definitions & ICD 11 Codes

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.

Exclusions:

• Mesenchymal tumours of meninges (2A01.1)
• Tumours of cranial or paraspinal nerves (2A02.1)

Chondrosarcoma primary site

ICD 11 Code For Chondrosarcoma primary site

  2B50  Chondrosarcoma primary site

Exclusions:

• Osteosarcoma, primary site (2B51)

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

Osteosarcoma primary site

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.

ICD 11 Code For Osteosarcoma primary site

  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

Ewing sarcoma primary site

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.

ICD 11 Code For Ewing sarcoma primary site

  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

Fibroblastic or myofibroblastic tumour primary site

ICD 11 Code For Fibroblastic or myofibroblastic tumour primary site

  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.

Unclassified pleomorphic sarcoma primary site

ICD 11 Code For Unclassified pleomorphic sarcoma primary site

  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.

Inclusions:

• malignant fibrous histiocytoma of skin

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

Rhabdomyosarcoma primary 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.

ICD 11 Code For Rhabdomyosarcoma primary site

  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

Angiosarcoma primary site

ICD 11 Code For Angiosarcoma 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.

Inclusions:

• Kupffer cell sarcoma of liver

2B56.Y Angiosarcoma other specified primary site

2B56.Z Angiosarcoma unspecified primary site

Kaposi sarcoma 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).

ICD 11 Code For Kaposi sarcoma primary site

  2B57  Kaposi sarcoma primary site

Coded Elsewhere:

• Human immunodeficiency virus disease associated with Kaposi sarcoma (1C62.3)

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

Leiomyosarcoma primary site

ICD 11 Code For Leiomyosarcoma 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

Liposarcoma 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.

ICD 11 Code For Liposarcoma primary site

  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

Synovial sarcoma 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.

ICD 11 Code For Synovial sarcoma primary site

  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

Gastrointestinal stromal tumour 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.

ICD 11 Code For Gastrointestinal stromal tumour primary site

  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

Endometrial stromal sarcoma primary site

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.

ICD 11 Code For Endometrial stromal sarcoma primary site

  2B5C  Endometrial stromal sarcoma primary site

Malignant mixed epithelial mesenchymal tumour primary site

ICD 11 Code For Malignant mixed epithelial mesenchymal tumour 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

Malignant nerve sheath tumour of peripheral nerves or autonomic nervous system primary site

ICD 11 Code For Malignant nerve sheath tumour of peripheral nerves or autonomic nervous system primary site

  2B5E  Malignant nerve sheath tumour of peripheral nerves or autonomic nervous system primary site

Exclusions:

• Malignant peripheral nerve sheath tumour of cranial or paraspinal nerves (2A02.10)

Sarcoma not elsewhere classified primary site

ICD 11 Code For Sarcoma not elsewhere classified primary site

  2B5F  Sarcoma not elsewhere classified primary site

2B5F.0 Sarcoma not elsewhere classified of uterus

Coded Elsewhere:

• Endometrial stromal sarcoma, primary site (2B5C)
• Leiomyosarcoma of uterus (2B58.1)
• Rhabdomyosarcoma of corpus uteri (2B55.Y)

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

Myosarcoma of uterus part not specified

ICD 11 Code For Myosarcoma of uterus part not specified

  2B5G  Myosarcoma of uterus part not specified

Coded Elsewhere:

• Leiomyosarcoma of uterus (2B58.1)
• Rhabdomyosarcoma of corpus uteri (2B55.Y)

Well differentiated lipomatous tumour primary site

ICD 11 Code For Well differentiated lipomatous tumour primary site

  2B5H  Well differentiated lipomatous tumour primary site

Malignant miscellaneous tumours of bone or articular cartilage of other or unspecified sites

ICD 11 Code For Malignant miscellaneous tumours of bone or articular cartilage of other or unspecified sites

  2B5J  Malignant miscellaneous tumours of bone or articular cartilage of other or unspecified sites

Exclusions:

• Neoplasms of haematopoietic or lymphoid tissues

Unspecified malignant soft tissue tumours or sarcomas of bone or articular cartilage of other or unspecified sites

ICD 11 Code For Unspecified malignant soft tissue tumours or sarcomas 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

Other specified malignant mesenchymal neoplasms

ICD 11 Code For Other specified malignant mesenchymal neoplasms

  2B5Y  Other specified malignant mesenchymal neoplasms

Malignant mesenchymal neoplasm of unspecified type

ICD 11 Code For Malignant mesenchymal neoplasm of unspecified type

2B5Z  Malignant mesenchymal neoplasm of unspecified type