Osteoblasts are nothing but bone forming cells (‘osteo’= bone & ‘blasts’= forming cells). When they start to undergo too much proliferation and become neoplastic, they form a malignant tumour called Osteosarcoma. Osteosarcoma is characterized by immature bone or osteoid tissue formed by neoplastic cells. However, fibrous or cartilaginous tissue may also co-exist or even predominate.
What is telangiectatic osteosarcoma (TO) ?
Telangiectatic osteosarcoma is basically an unusual and special variant of osteosarcoma, with its own distinct radiologic, gross and microscopic features.
There is a conventional type of osteosarcoma. In addition to that, various rare subtypes of osteosarcoma also have been described. These include the small-cell, parosteal, periosteal, intracortical, intramedullary (based on its location) and the giant cell-rich and telangiectatic sarcomas (based on its histology).
Almost 3% to 12% of osteosarcomas are of the telangiectatic subtype.
Paget was the first to describe telangiectatic osteosarcoma. However, Ewing was the first to consider and describe it as a variant of osteosarcoma.
What are the clinical features of telangiectatic osteosarcoma ?
Age & Site: There is a male predominance of the disease, with a male to female ratio of 2:1. The average age at presentation is 17 years old, the age range varying from 15 to 20 years old.
The most common location at presentation is the metaphyses of long bones, especially of the bones around the knee joint. Most lesions are localized to the metaphyseal region.
Clinical Presentation of Telangiectatic Osteosarcoma: The clinical presentation of Telangiectatic Osteosarcoma is more or less like the Conventional Osteosarcoma. Generally, the most common signs and symptoms are recent local pain, soft tissue mass, or both. Some cases present with pathologic fracture. Patients with Telangiectatic Osteosarcoma are at higher risk of pathologic fractures than Conventional Osteosarcoma. Osteosarcoma patients with a pathologic fracture have an increased risk of recurrence at site, poorer survival, and a greater need for amputation than those who do not have this fracture.
Telangiectatic osteosarcomas are hastily progressing lesions affecting the medullary and cortical bones, with ill-demarcated margins.
How is the telangiectatic osteosarcoma diagnosed ?
The main modalities used in the identification and diagnosis of Telangiectatic osteosarcoma are-
- Radiography for detecting the bone lesion : Amongst all the imaging modalities, the traditional plain x-rays guide us most towards the diagnosis. The radiographic appearance of Telangiectatic Osteosarcoma is predominantly that of a lytic, destructive bone tumour without sclerosis. Cortical bone destruction (outer layer of bone) is invariably present, with the pattern of bone destruction described as geographic and/or moth-eaten.
A Codman’s triangle is seen as in the conventional type. It signifies new bone formation on the outer surface of bone/ periosteum. - MRI and bone scintigraphy : MRI is used to investigate the tumour extent. A bone scintigraphy study demonstrates the fluid component (cystic, dialted blood vessels) and uptake in the septa.
- CT scans : These are used to detect metastasis. CT of the chest and abdomen is performed to identify any organ metastases Osteoid matrix can be identified in CT scans and is found in the septae.
- Histopathological Investigations : Histopathology and immunohistochemistry are requisite for confirmation of Telangiectatic osteosarcoma in the biopsied tissues. Microscopic examination is the only definitive diagnostic aid in such cases.
The bone tissue that is found in the tumour is neoplastic. It is not formed by normal osteoblasts, but by tumour osteoblasts. The osteoblasts seen are pleomorphic and anaplastic.
Telangiectatic osteosarcoma shows dilated, large blood-filled cystic spaces lined or traversed by atypical stromal cells which produce lace-like bony tissue/ osteoid. Lesions are largely hemorrhagic or necrotic.
Histologically, telangiectatic osteosarcoma can be separated into low-grade (better behaved lesions with mild to moderate nuclear atypia and few mitoses) and at the other end, into high-grade lesions (aggressive tumours with marked anaplasia and high index of mitotic activity).
The cut-section of an affected bone: Note the predominantly cystic, blood-filled cavity.
Microscopic image of the affected bone: numerous, large blood-filled spaces with thick septa.
What can telangiectatic osteosarcoma be confused with ?
It is easy to confuse the Telangiectatic osteosarcoma with an Aneurysmal Bone Cyst. However, the presence of atypical and/or overtly malignant cells is enough to rule out the diagnosis of Aneurysmal Bone Cyst.
How is telangiectatic osteosarcoma treated ?
Most people with an osteosarcoma necessitate tailored treatment regimens. The three basic modalities for cancer treatment include surgery, chemotherapy and/or radiotherapy, and now, even immunotherapy.
The modern management protocols utilize neoadjuvant chemotherapy (chemotherapy administered prior to surgery), which is then followed by surgical resection.
On the whole, the prognosis for patients with Telangiectatic Osteosarcoma, has improved significantly and is comparable to conventional osteosarcoma owing to neoadjuvant chemotherapy.
What is the follow-up required after the treatment of telangiectatic osteosarcoma ?
After your treatment is completed, you will require regular check-ups by your doctor and x-ray examination. The radiographic & advanced imaging techniques are non-invasive techniques with diagnostic capability and are to be used for follow-up after surgery and to locate potential sites of metastasis. These check-ups will have to continue for several years. If you have notice any problems or recent symptoms, inform your doctor, as soon as possible.
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
