Telangiectatic Osteosarcoma

Updated: April 16, 2024

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Background

Telangiectatic osteosarcoma is a malignant bone tumor of a primary type. It is the rare and aggressive form. Osteosarcoma develops more frequently than any other types of primary bone cancer. 

Dilated blood vessels within the osteosarcoma tissue are considered as a distinguishing feature of the telangiectatic type of this tumor. The patients usually show up with pain and sometimes a palpable mass in the site of the affected bone. 

It may appear as a destructive and lytic lesion with areas of radiolucency and soft tissue mass. Due to the presence of extensive vascular spaces and there may be a risk of spontaneous hemorrhage within the tumor which leads to further complications. 

Epidemiology

The majority of patients affected by telangiectatic osteosarcoma are young adults . The mean age at presentation is 17.5 years.The single anatomic site is the distal femoral metaphysis in which represents 42 percentage of cases which is followed by the proximal tibia or proximal humerus and the proximal femur . Flat bones are relatively rare and occurring in about 8 percent of cases of non-appendicular non-spinal sites. Infrequent sites of telangiectatic osteosarcoma are reported and incude the scapula and mandible . If the spine are even rarer and accounting for 2 percent of all cases of primary vertebral osteosarcomas. 

Anatomy

Pathophysiology

It arises from the neoplastic transformation of mesenchymal cells and which give rise to bone. Microscopically it displays a worrisome appearance with irregular cells and shapes. The cancerous cells surround and permeate these vascular areas by forming partitions among them by resulting in a honeycomb or network-like pattern under examination. 

A defining histological feature of telangiectatic osteosarcoma is the existence of dilated and blood-filled spaces within the tumor tissue. Commonly called aneurysmal cavities and these areas lend the growth its signature look. 

Etiology

It frequently arises due to genetic vulnerabilities and inherited disorders that elevate susceptibility have been linked. Alterations in TP53, a tumor suppressor gene, commonly underlie osteosarcoma cases. Other conditions involving mutations responsible for bone formation and mending may enhance risk as well. 

Exposure to ionizing rays is a known determinant for osteosarcoma initiation. This can arise from therapeutic rays or environmental radiation contact. 

Genetics

Prognostic Factors

The presence of metastases at diagnosis particularly in the lungs is a negative prognostic factor which leads to a poor prognosis.The response of the tumor to preoperative chemotherapy is an important prognostic factor. The anatomical location of the tumor within the bone can impact prognosis. Tumors in the distal femur or proximal tibia may have varying prognoses. Limb-sparing surgery is often attempted when feasible and successful limb-sparing surgery is generally associated with improved quality of life. 

Clinical History

Age group: A rare bone cancer called telangiectatic osteosarcoma usually affects young adults and adolescents between the ages of 10 and 30, peaking in the teens and early twenties. 

Physical Examination

Swelling:Examines visible or palpable swelling around the affected bone. 

Tenderness:Palpation identifies areas of tenderness by indicating tumor presence. 

Skin Examination:Checks for signs of ulceration or texture changes. 

Lymph Node Examination:Assesses enlargement of regional lymph nodes by potentially indicating metastasis. Neurovascular Examination: Assesses nerve and blood vessel function in affected limb. 

Peripheral Pulses:Checks presence and strength of peripheral pulses. 

Age group

Associated comorbidity

Tumors with dilated blood arteries and spontaneous bleeding can lead to problems such as restricted mobility and a worse quality of life particularly if the cancer affects weighing bones or joints. Psychological stress can be brought on by the difficulties associated with cancer therapy and illness uncertainty. 

Associated activity

Acuity of presentation

A typical sign of telangiectatic osteosarcoma is pain, which is usually progressive and confined to the damaged bone. Tumor site swelling may be seen due to the tumor’s ability to expand the bone. 

Differential Diagnoses

Aneurysmal Bone Cyst 

Giant Cell Tumor of Bone 

Ewing Sarcoma 

Fibrous Dysplasia 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Neoadjuvant Chemotherapy: It can decrease the tumor before surgery by making it easier to remove and possibly improving the condition. 

Surgical Intervention: It involves the surgical removal of tumor 

Adjuvant Chemotherapy:Following the surgery patients often undergo additional chemotherapy to specifically address any lingering cancer cells and lower the chances of the cancer reoccurence. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-non-pharmacological-approach-for-telangiectatic-osteosarcoma

Radiation therapy and surgical resection are two non-pharmacological treatments. For adolescents and teenagers the limb-sparing surgery is frequently performed although amputation may be required. Radiation therapy is not the main course of treatment however it may be in some circumstances. The goal of innovative medicines like as immunotherapy and treatments based on nanotechnology is to provide less harmful and more effective treatments. 

Use of Alkylating agent

Ifosamide: Often taken in conjunction with other chemotherapy drugs, ifosfamide is a chemotherapy drug that prevents replication of DNA and cell growth. 

use-of-intervention-with-a-procedure-in-treating-telangiectatic-osteosarcoma

Surgery:During a limb-sparing surgical operation the tumor is removed completely by removing a portion of healthy tissue. Rebuilding soft tissues and bones may be required in some circumstances in order to restore look and functioning. Amputation: If surgery to save limbs is not feasible because of the size or position of the tumor and then amputation can be an option. 

Biopsy: A biopsy is usually conducted before to definitive surgical surgery. A little sample of tumor tissue is taken during the biopsy in order to be examined histologically. 

use-of-phases-in-managing-telangiectatic-osteosarcoma

Diagnosis Phase:The doctor will evaluate your condition, perform imaging tests like MRI and CT scans and may collect a tissue sample for further diagnosis confirmation. 

Surgical Intervention:surgical removal achieve local control of the tumor. Removal of the tumor while preserving the affected limb and often followed by reconstructive procedures. 

Supportive Care:Supportive care measures to manage pain and address nutritional needs. 

Monitoring and Follow-up:Detect and manage any recurrence or late effects of treatment. 

 

Medication

Media Gallary

Telangiectatic Osteosarcoma

Updated : April 16, 2024

Mail Whatsapp PDF Image



Telangiectatic osteosarcoma is a malignant bone tumor of a primary type. It is the rare and aggressive form. Osteosarcoma develops more frequently than any other types of primary bone cancer. 

Dilated blood vessels within the osteosarcoma tissue are considered as a distinguishing feature of the telangiectatic type of this tumor. The patients usually show up with pain and sometimes a palpable mass in the site of the affected bone. 

It may appear as a destructive and lytic lesion with areas of radiolucency and soft tissue mass. Due to the presence of extensive vascular spaces and there may be a risk of spontaneous hemorrhage within the tumor which leads to further complications. 

The majority of patients affected by telangiectatic osteosarcoma are young adults . The mean age at presentation is 17.5 years.The single anatomic site is the distal femoral metaphysis in which represents 42 percentage of cases which is followed by the proximal tibia or proximal humerus and the proximal femur . Flat bones are relatively rare and occurring in about 8 percent of cases of non-appendicular non-spinal sites. Infrequent sites of telangiectatic osteosarcoma are reported and incude the scapula and mandible . If the spine are even rarer and accounting for 2 percent of all cases of primary vertebral osteosarcomas. 

It arises from the neoplastic transformation of mesenchymal cells and which give rise to bone. Microscopically it displays a worrisome appearance with irregular cells and shapes. The cancerous cells surround and permeate these vascular areas by forming partitions among them by resulting in a honeycomb or network-like pattern under examination. 

A defining histological feature of telangiectatic osteosarcoma is the existence of dilated and blood-filled spaces within the tumor tissue. Commonly called aneurysmal cavities and these areas lend the growth its signature look. 

It frequently arises due to genetic vulnerabilities and inherited disorders that elevate susceptibility have been linked. Alterations in TP53, a tumor suppressor gene, commonly underlie osteosarcoma cases. Other conditions involving mutations responsible for bone formation and mending may enhance risk as well. 

Exposure to ionizing rays is a known determinant for osteosarcoma initiation. This can arise from therapeutic rays or environmental radiation contact. 

The presence of metastases at diagnosis particularly in the lungs is a negative prognostic factor which leads to a poor prognosis.The response of the tumor to preoperative chemotherapy is an important prognostic factor. The anatomical location of the tumor within the bone can impact prognosis. Tumors in the distal femur or proximal tibia may have varying prognoses. Limb-sparing surgery is often attempted when feasible and successful limb-sparing surgery is generally associated with improved quality of life. 

Age group: A rare bone cancer called telangiectatic osteosarcoma usually affects young adults and adolescents between the ages of 10 and 30, peaking in the teens and early twenties. 

Swelling:Examines visible or palpable swelling around the affected bone. 

Tenderness:Palpation identifies areas of tenderness by indicating tumor presence. 

Skin Examination:Checks for signs of ulceration or texture changes. 

Lymph Node Examination:Assesses enlargement of regional lymph nodes by potentially indicating metastasis. Neurovascular Examination: Assesses nerve and blood vessel function in affected limb. 

Peripheral Pulses:Checks presence and strength of peripheral pulses. 

Tumors with dilated blood arteries and spontaneous bleeding can lead to problems such as restricted mobility and a worse quality of life particularly if the cancer affects weighing bones or joints. Psychological stress can be brought on by the difficulties associated with cancer therapy and illness uncertainty. 

A typical sign of telangiectatic osteosarcoma is pain, which is usually progressive and confined to the damaged bone. Tumor site swelling may be seen due to the tumor’s ability to expand the bone. 

Aneurysmal Bone Cyst 

Giant Cell Tumor of Bone 

Ewing Sarcoma 

Fibrous Dysplasia 

Neoadjuvant Chemotherapy: It can decrease the tumor before surgery by making it easier to remove and possibly improving the condition. 

Surgical Intervention: It involves the surgical removal of tumor 

Adjuvant Chemotherapy:Following the surgery patients often undergo additional chemotherapy to specifically address any lingering cancer cells and lower the chances of the cancer reoccurence. 

Orthopaedic Surgery

Radiation therapy and surgical resection are two non-pharmacological treatments. For adolescents and teenagers the limb-sparing surgery is frequently performed although amputation may be required. Radiation therapy is not the main course of treatment however it may be in some circumstances. The goal of innovative medicines like as immunotherapy and treatments based on nanotechnology is to provide less harmful and more effective treatments. 

Orthopaedic Surgery

Doxorubucin: It is an anthracycline which acts by tampering with cancer cells DNA to prevent them from proliferating and dividing. 

Cisplatin: It produces DNA adducts, which damage DNA and result in cell death. 

Oncology, Radiation

Ifosamide: Often taken in conjunction with other chemotherapy drugs, ifosfamide is a chemotherapy drug that prevents replication of DNA and cell growth. 

Orthopaedic Surgery

Surgery:During a limb-sparing surgical operation the tumor is removed completely by removing a portion of healthy tissue. Rebuilding soft tissues and bones may be required in some circumstances in order to restore look and functioning. Amputation: If surgery to save limbs is not feasible because of the size or position of the tumor and then amputation can be an option. 

Biopsy: A biopsy is usually conducted before to definitive surgical surgery. A little sample of tumor tissue is taken during the biopsy in order to be examined histologically. 

Orthopaedic Surgery

Diagnosis Phase:The doctor will evaluate your condition, perform imaging tests like MRI and CT scans and may collect a tissue sample for further diagnosis confirmation. 

Surgical Intervention:surgical removal achieve local control of the tumor. Removal of the tumor while preserving the affected limb and often followed by reconstructive procedures. 

Supportive Care:Supportive care measures to manage pain and address nutritional needs. 

Monitoring and Follow-up:Detect and manage any recurrence or late effects of treatment. 

 

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