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Chondrosarcoma

Chondrosarcoma is a malignant neoplasm that predominantly is made of cartilage. It occurs most frequently in long bones, the pelvic girdle and the scapula. Chondrosarcoma is the second most frequent primary malignant tumor of bone.


Presentation

The preliminary signs of chondrosarcoma involve dull and deep pain in the affected area which is usually experienced in the night hours. The mean time from the diagnosis of chondrosarcoma from the onset of pain is usually 19.4 month in low grade tumors and much faster in high grade once [6].

In addition, there may be swelling accompanied by tenderness in the affected area. One of the major factors that distinguish bone cancer from other type of bone disorders is that there is persistent pain in the affected area which is generally felt during night. Another factor that needs mention is that individuals with chondrosarcoma often present with fractures after a minor fall or accident. This one feature is important for distinguishing bone cancer from other disorders of the bone.

Leg Edema
  • We report a case of a 45-year-old female who presented with a 5-month history of left leg edema in 2015. Contrast-enhanced computed tomography showed a large mass in the left iliac vein with scattered calcifications.[ncbi.nlm.nih.gov]
Thyroid Nodule
  • When CS arises from thyroid cartilage, it may clinically present as a thyroid nodule. Here we report a rare case of CS of thyroid cartilage misinterpreted as medullary thyroid carcinoma.[ncbi.nlm.nih.gov]
Stridor
  • Suspicion should be raised if a patient presents with classical signs and symptoms; i.e. dysphonia, inspiratory stridor, dysphagia, odynophagia, neck mass, or persistent cough.[ncbi.nlm.nih.gov]
  • A 60-year-old gentleman presented with progressively increasing cough, severe stridor, and production of phlegm for approximately 2 years.[ncbi.nlm.nih.gov]
Sore Throat
  • We report a case of a 71-year-old male smoker who presented with sore throat, dysphagia, and odynophagia for 3 months.[ncbi.nlm.nih.gov]
Inspiratory Stridor
  • Suspicion should be raised if a patient presents with classical signs and symptoms; i.e. dysphonia, inspiratory stridor, dysphagia, odynophagia, neck mass, or persistent cough.[ncbi.nlm.nih.gov]
Persistent Cough
  • Suspicion should be raised if a patient presents with classical signs and symptoms; i.e. dysphonia, inspiratory stridor, dysphagia, odynophagia, neck mass, or persistent cough.[ncbi.nlm.nih.gov]
Progressive Visual Loss
  • A 45-year-old woman presented with a 7-month history of amenorrhea and a 3-month history of progressive visual loss in the left eye.[ncbi.nlm.nih.gov]
Hip Pain
  • We report a case of a 50-year-old female who presented with 6 months of left hip pain. Initial radiographs demonstrated an osteolytic lesion with adjacent area of sclerosis in the proximal left femur.[ncbi.nlm.nih.gov]
  • We report herein a case involving the left femoral head in a 25-year-old female with a 3-month history of worsening left hip pain.[ncbi.nlm.nih.gov]
  • Review Topic QID: 3091 2 Clear cell chondrosarcoma ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 2 (OBQ10.136) A 60-year-old female complains of progressive right hip pain.[orthobullets.com]
Joint Deformity
  • Wide excision or amputation is recommended when severe joint deformity or soft tissue involvement interferes with hand function.[ncbi.nlm.nih.gov]
Neck Mass
  • Suspicion should be raised if a patient presents with classical signs and symptoms; i.e. dysphonia, inspiratory stridor, dysphagia, odynophagia, neck mass, or persistent cough.[ncbi.nlm.nih.gov]
  • Examination revealed a level 3 neck mass and positron emission tomography imaging revealed a metabolically active right laryngeal mass. Biopsies revealed a cartilaginous neoplasm.[ncbi.nlm.nih.gov]
Amenorrhea
  • A 45-year-old woman presented with a 7-month history of amenorrhea and a 3-month history of progressive visual loss in the left eye.[ncbi.nlm.nih.gov]

Workup

The following are the diagnostic procedures employed for detecting chondrosarcoma:

  • X-ray of the bone is done to analyze the presence of lesions. In the results, if tumors are present, the bone appears to be thinned and expanded. The extent of damage to the bone depends on the grade of the cancer [7].
  • Biopsy is carried out to analyze the type of the tumor cells [8]. 
  • Imaging tests such as CT scan and MRI scan are used to examine spread of the cancer to other parts of the body. The most common area to get affected is the lungs and a CT scan may reveal this.
  • Bone scan is a more sensitive test as compared to bone X-ray. 

Treatment

The type of treatment employed depends on the nature, size and location of the tumor. The grade at which the cancer is detected also greatly affects the type of treatment method to be employed. The following methods are applied for treating chondrosarcoma:

  • Surgery: Surgical removal of the tumor forms the basis of treatment of chondrosarcoma. If the cancer has been detected in the early stages, then limb amputation can be spared. However, it is not always possible to avoid limb amputation which is necessary to avoid spread of cancer to other parts of the body.
  • Proton therapy has been widely used for its positive results in treating chondrosarcoma. In this, powerful radiation is administered which attacks on the tumor cells and spares the other healthy tissues of the body.
  • The use of conventional chemotherapy may have limited application in the treatment of mesenchymal and dedifferentiated types of chondrosarcoma [9]. The use of doxorubicin and cisplatin chemotherapeutic agents in treatment of sensitive cancer cells has been implored in some inoperable cases of chondrosarcoma [10].

Prognosis

Prognosis of chondrosarcoma greatly depends on how early the cancer was detected and treatment initiated. When individuals are diagnosed during the grade I of the disease, prognosis is favorable and the survival rate 5 years after proper treatment is 90%. However, in those when the tumors have become aggressive, the prognosis is poor with only 10% patients surviving the treatment period.

There are high chances that the tumors develop back and therefore it is advised that regular checkup be carried out to detect early signs of new tumor development.

Complications

Complications can develop when the cancer has not been diagnosed in the initial stages. As a result, it can metastasize to other parts of the body. In addition, nerve dysfunction can also develop in patients with chondrosarcoma.

Etiology

The exact cause that triggers the development of chondrosarcoma is unknown. It is however believed that chondrosarcoma may be present as a secondary condition in individuals with other medical illness such as:

Chondrosarcoma may also affect some children who have received either chemotherapy or radiation therapy for treatment of other types of cancer. Some genetic studies also suggests that chondrosarcoma is associated with genetic anomalies like the loss of heterozygosity in many gene loci [3].

Epidemiology

Chondrosarcoma is a rare form of cancer affecting about 600 patients in United States each year. This form of cancer accounts for about 20 to 27% of malignant bone tumors [4]. It affects more males than females, with a male to female ratio of 1.5:1.

Sex distribution
Age distribution

Pathophysiology

Chondrosarcomas are classified into 2 types depending on their source of origin: Primary and secondary. Primary chondrosarcoma occurs spontaneously unlike secondary type that develops as a result of other associated disease conditions. Chondrosarcoma has also been divided into various grades depending on the extent of spread of the disease.

Grade I chondrosarcoma develops at slow rate and is less aggressive in nature. The malignant cells appear similar to those of normal cells of cartilage tissues. Grade II and III are characterized by rapid growing tumor cells which are more aggressive in nature [5]. The cells can appear abnormal and can spread to the neighboring sites such as lymph nodes and other organs. Grade IV chondrosarcoma is defined as anaplastic tumors having abnormal growth pattern.

Prevention

So far, no guidelines have been formulated to prevent chondrosarcoma. However, if individuals can recognize unusual signs and symptoms and seek medical help then the cancer can be treated at the initial stages. This can help prevent the spread of the cancer to other parts of the body and improve the life expectancy rate.

Summary

Chondrosarcoma is a type of cancer that develops in the bones and joints. It is a rare form of cancer and usually affects adults aged 40 years and above. Chondrosarcoma can affect any part of the body that contains cartilages; but primarily begins with development of tumor cells in the arms, legs and or pelvis [1]. Chondrosarcoma may be classified as primary and secondary depending on its source [2].

Chemotherapy and radiation therapy are not effective in treating such type of cancer. Surgical removal of the malignant tumors along with other methods is employed for treating chondrosarcoma.

Patient Information

Definition

Chondrosarcoma is a rare form of bone cancer that develops in the bone and joints. Males more commonly fall prey to such type of cancer. Adults aged 40 years and above are more prone to develop chondrosarcoma. The common areas of tumor development include areas of arm, legs and pelvis.

Cause

There are two type of chondrosarcoma: primary and secondary. The exact cause that triggers the development of the primary type is not yet known. However, secondary chondrosarcoma is thought to develop as a result of certain types of disease conditions.

Symptoms

The tumors cells are large in size and measures more than 5 cm. Individuals usually experience a dull and deep pain in the affected area during night. In addition, the patients also complain of inflammation and tenderness in the areas that do not go away with medication.

Diagnosis

The condition is diagnosed using bone X-ray that helps in identifying the tumor locations. Imaging studies such as CT scan and MRI help in detection of the spread of the cancer to other parts of the body. Biopsy of the tumor cells is done to analyze the nature of the cells. Bone scan may also be required as it provides additional information about the tumor cells.

Treatment

Surgery is often the first line of treatment followed by proton therapy to destroy the cancer cells without affecting the healthy tissues of the body.

References

Article

  1. Healey JH, Lane JM. Chondrosarcoma. Clin Orthop Relat Res. Mar 1986; 119-29.
  2. Mankin HJ, Cantley KP, Schiller AL, Lippiello L. The biology of human chondrosarcoma. II. Variation in chemical composition among types and subtypes of benign and malignant cartilage tumors. J Bone Joint Surg Am. Mar 1980; 62(2):176-88.
  3. Bovee JV, Cleton-Jansen AM, Kuipers-Dijkshoorn NJ, et al. Loss of heterozygosity and DNA ploidy point to a diverging genetic mechanism in the origin of peripheral and central chondrosarcoma. Genes Chromosomes Cancer. Nov 1999; 26(3):237-46.
  4. Dorfman HD, Czerniak B. Bone Tumors. St Louis, Mo: Mosby; 1998:353-440.
  5. Ryzewicz M, Manaster BJ, Naar E, Lindeque B. Low-grade cartilage tumors: diagnosis and treatment.Orthopedics. Jan 2007; 30(1):35-46; quiz 47-8.
  6. Gitelis S, Bertoni F, Picci P, Campanacci M. Chondrosarcoma of bone. The experience at the Istituto Ortopedico Rizzoli. J Bone Joint Surg Am. Oct 1981; 63(8):1248-57.
  7. Douis H, Saifuddin A. The imaging of cartilaginous bone tumours. II. Chondrosarcoma. Skeletal Radiol. 2013; 42(5):611-26 
  8. Simon MA, Biermann JS. Biopsy of bone and soft-tissue lesions. J Bone Joint Surg Am. Apr 1993; 75(4):616-21.
  9. Italiano A, Mir O, Cioffi A, Palmerini E, Piperno-Neumann S. Advanced chondrosarcomas: role of chemotherapy and survival. Ann Oncol. 2013; 24(11):2916-22 
  10. Van Oosterwijk JG, Herpers B, Meijer D. Restoration of chemosensitivity for doxorubicin and cisplatin in chondrosarcoma in vitro: BCL-2 family members cause chemoresistance. Ann Oncol. 2012; 23(6):1617-26 

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Last updated: 2019-07-11 22:04