patoloji-ders-notlari
Title
Serdar Balcı
Mesenchymal Tumors
Serdar BALCI, MD
Tumors are classified according to germ layers
- Epithelial
- Endodermal
- Ectodermal
- Neural
- Mesenchymal
Mesenchymal
- Lymphoid
- Soft Tissue
- Bone
- Peripheral Nerves
- Supporting Tissues in all organs
- Each organ system has its own mesenchymal tumors
- Vascular tumor can be seen in skin, liver, GI tract, brain, …
- Undifferentiated mesenchymal tumors
Mesenchymal Tumors
- Benign
- Common in general population
- Malignant
- Uncommon
- Lethal
- Names are given according to tissue of origin
- Malignant Mesenchymal Tumor
- Used as a generic term for a tumor when tissue origin is not identified
- Malignant Mesenchymal Tumor
Sarcomas mostly metastasize via blood vessels
**Osteoid tissue does not have lymphatics **
Pathology Secrets Third Edition
Paget diseases of bone → increased risk of osteosarcomas
AIDS, HHV8 → Kaposi sarcomas
Neurofibromatosis type 1 → multiple neurofibromas → malignant peripheral nerve sheath tumors
Retinoblastoma patients → after treatment risk of osteosarcomas
Li Fraumeni → Osteosarcomas
Age
Rhabdomyosarcomas <5 yrs
Osteosarcoma 6-15 yrs
Environmental Effects
Osteogenic sarcoma → radioactive phosphorus painters early 20th century
Liver angiosarcoma → radioactive Thorotrast use as a radiologic imaging contrast material
Robbins Basic Pathology
http://www.iarc.fr/en/publications/list/bb/
Robbins Basic Pathology
Benign adipose tissue tumor
Lipoma
Rubin’s Pathology 7th Ed
Malignant Adipose Tissue Tumor
Liposarcoma
Rubin’s Pathology 7th Ed
Malignant Adipose Tissue Tumor
Dedifferentiated Liposarcoma
Malignant mesenchymal tumors when lose their differentiation are called dedifferentiated
Rubin’s Pathology 7th Ed
Malignant Adipse Tissue Tumor
Myxoid Liposarcoma
Rubin’s Pathology 7th Ed
Fibromatosis
Related to Gardner Syndrome
Rubin’s Pathology 7th Ed
Malignant Fibroblast Tumor
Fibrosarcoma
Rubin’s Pathology 7th Ed
Undifferentiated Pleomorphic Sarcoma
Malignant Fibrous Histiocytoma
Rubin’s Pathology 7th Ed
Malignant Fibroblast Tumor
Fibrosarcoma
Robbins Basic Pathology
Robbins Basic Pathology
Benign skeletal muscle tumor
Rhabdomyoma
**Enzinger and Weiss’s Soft Tissue Tumors **
Malignant skeletal muscle tumor
Rhabdomyosarcoma
Rubin’s Pathology 7th Ed
Malignant skeletal muscle tumor
Rhabdomyosarcoma
Rubin’s Pathology 7th Ed
Malignant skeletal muscle tumor
Rhabdomyosarcoma
Rubin’s Pathology 7th Ed
Benign smooth muscle tumor
Leiomyoma
Rubin’s Pathology 7th Ed
Malignant smooth muscle tumor
Leiomyosarcoma
Rubin’s Pathology 7th Ed
Malignant smooth muscle tumor
Leiomyosarcoma
Rubin’s Pathology 7th Ed
Malignant smooth muscle tumor
Leiomyosarcoma
Rubin’s Pathology 7th Ed
GIST Gastrointestinal Stromal Tumor
- Stomach, GI Tract
- Benign to Malignant
- 75-80% oncogenic, gain-of-function mutations tyrosine kinase c-KIT
- receptor for stem cell factor
- 8% have mutations that activate a related tyrosine kinase, platelet-derived growth factor receptor A (PDGFRA)
- Either mutation is sufficient for tumorigenesis
- Interstitial cells of Cajal
- **GISTs appear to arise from, or share a common stem cell **
- express c-KIT
- Located in the muscularis propria
- Serve as pacemaker cells for gut peristalsis
Rosai and Ackerman’s Surgical Pathology
Rosai and Ackerman’s Surgical Pathology
Benign vascular tumor
Hemangioma
Rubin’s Pathology 7th Ed
Rubin’s Pathology 7th Ed
Malignant Vascular Tumor
Angiosarcoma
Rubin’s Pathology 7th Ed
Angiosarcoma
Most important malignant vascular tumor, occurs most often in soft tissues
May involve the bones, internal organs, and any part of the body
composed of malignant endothelial cells forming irregular anastomozing channels or solid nests
Prognosis depends on the size of the lesions and the extent of spread
HHV8 related malignant vascular tumor
Kaposi Sarcoma
Rubin’s Pathology 7th Ed
Kaposi sarcoma
Vascular tumor
Dark red and bluish skin plaques and nodules
Can also affect internal organs
Herpes virus type 8
Related to HIV
Clinicopathologic forms of Kaposi sarcoma
- Classic sporadic form
- Locally invasive, recurrent hemorrhagic lesions on the legs of elderly men
- May be associated with some other internal malignancy
- Acquired immunodeficiency syndrome (AIDS)–associated epidemic form
- Male homosexuals suffering from AIDS
- Skin, lymph nodes, and internal organs, gastrointestinal tract
- Posttransplantation Kaposi sarcoma
- A few months to many years after solid organ transplantation
- Skin or internal organs
- Endemic African Kaposi sarcoma
- Children in areas of sub-Saharan Africa
- Rapidly developing lymphadenopathy
Robbins Basic Pathology
Benign nerve sheath tumor
Schwannoma
Rosai and Ackerman’s Surgical Pathology
**Malignant peripheral nerve sheath tumor **
Rosai and Ackerman’s Surgical Pathology
Robbins Basic Pathology
Benign bone tumor forming cartilage
Osteochondroma
Rubin’s Pathology 7th Ed
Benign bone tumor forming cartilage
Enchondroma
Rubin’s Pathology 7th Ed
Malignant bone tumor forming cartilage
Chondrosarcoma
Rubin’s Pathology 7th Ed
Chondrosarcoma
Second most common primary malignant bone tumor.
Peak incidence in the 40- to 60-year-old
Axial skeleton—pelvis, vertebra, shoulder, and proximal parts of the femur and radius.
Chondrosarcomas are slower-growing tumors than osteosarcomas
Untreated tumors generally have a better prognosis than osteosarcomas
Chondrosarcomas do not respond well to chemotherapy
Prognosis depends on the resectability of the tumor, its histologic grade, and the presence or absence of hematogenous metastases
Robbins Basic Pathology
Malignant bone tumor forming osteoid
Osteosarcoma
Rubin’s Pathology 7th Ed
Robbins Basic Pathology
Osteosarcomas
Most common primary bone tumor
Peak incidence in second decade of life
Metaphysis of long bones of the extremities
60% of cases found around the knee joint
Invades locally and metastasizes hematogenously to the lungs
Very malignant (Without therapy there is high mortality
Combined chemotherapy with surgery is curative in 80% of childhood and adolescent tumors
Old-age osteosarcomas have a less favorable response to therapy
Any tumor spreading with a bite?
Tasmanian Devil Facial Tumour Disease