American singer, songwriter, and musician Little Richard (born Richard Wayne Penniman) died on May 9 at the age of 87. His agent, Dick Alen, :
"Little Richard passed away this morning from bone cancer in Nashville. He was living with his brother in Nashville. He was battling for a good while, many years. I last spoke to him about two or three weeks ago. I knew he wasn't well but he never really got into it, he just would say 'I'm not well.' He's been suffering for many years with various aches and pains. He just wouldn't talk about it much." It was not disclosed what type of bone cancer, primary or metastatic (see below), that Little Richard had.
Richard was an influential figure in popular music for seven decades and had several nicknames, including "The Innovator," "The Originator," and "The Architect of Rock and Roll." His high energy performance style with frenetic piano playing and shouted vocals, along with his flamboyant attire propelled Little Richard to rock and roll royalty. His number one singles, "Tutti Frutti," "Long Tall Sally," and "Good Golly Miss Molly" are credited with adding black rhythm and blues into rock and roll. Artists such as the Beatles, the Rolling Stones, Elton John, and Elvis Presley all said that they were influenced by Little Richard's music and persona.
Little Richard received a Lifetime Achievement Grammy in 1993, and was inducted into the first class of the Rock and Roll Hall of Fame in 1986 and the Blues and Rhythm and Blues Halls of Fame in 2015.
Bone Cancer
Several different kinds of tumors can grow in bones: primary bone tumors, which form from bone tissue and can be malignant (cancerous) or benign (not cancerous), and metastatic tumors (tumors that develop from cancer cells that formed elsewhere in the body and then spread to the bone). Malignant primary bone tumors (primary bone cancers) are less common than benign primary bone tumors. Both types of primary bone tumors may grow and compress healthy bone tissue, but benign tumors usually do not spread or destroy bone tissue and are rarely a threat to life.
Primary bone cancers are included in the broader category of cancers called sarcomas. Bone cancers are rare and account for much less than 1% of all new cancers diagnosed. According to SEER, in 2020, an estimated 3,600 new cases of primary bone cancer (0.2% of all new cancer cases) will be diagnosed in the United States. Some 1,720 bone cancer deaths are predicted to occur this year.
Types of Primary Bone Cancer
Types of primary bone cancer are defined by which cells in the bone give rise to them.
Osteosarcoma
Osteosarcoma arises from bone-forming cells called osteoblasts in osteoid tissue (immature bone tissue). This tumor typically occurs in the arm near the shoulder and in the leg near the knee in children, adolescents, and young adults but can occur in any bone, especially in older adults. It often grows quickly and spreads to other parts of the body, including the lungs. Risk of osteosarcoma is highest among children and adolescents ages 10 to 19. Males are more likely than females to develop osteosarcoma. Among children, osteosarcoma is more common in blacks and other racial/ethnic groups than in whites, but among adults it is more common in whites than in other racial/ethnic groups. People who have Paget disease (a benign bone condition characterized by abnormal development of new bone cells) or a history of radiation to their bones also have an increased risk of developing osteosarcoma.
Chondrosarcoma
Chondrosarcoma begins in cartilaginous tissue. Chondrosarcoma most often forms in the pelvis, upper leg, and shoulder and usually grows slowly, although sometimes it can grow quickly and spread to other parts of the body. Chondrosarcoma occurs mainly in older adults (over age 40). The risk increases with advancing age. A rare type of chondrosarcoma called extraskeletal chondrosarcoma does not form in bone cartilage. Instead, it forms in the soft tissues of the upper part of the arms and legs.
Ewing sarcoma
Ewing sarcoma usually arises in bone but may also rarely arise in soft tissue (muscle, fat, fibrous tissue, blood vessels, or other supporting tissue). Ewing sarcomas typically form in the pelvis, legs, or ribs, but can form in any bone. This tumor often grows quickly and spreads to other parts of the body, including the lungs. The risk of Ewing sarcoma is highest in children and adolescents younger than 19 years of age. Boys are more likely to develop Ewing sarcoma than girls. Ewing sarcoma is much more common in whites than in blacks or Asians.
Chordoma
Chordoma is a very rare tumor that forms in bones of the spine. These tumors usually occur in older adults and typically form at the base of the spine (sacrum) and at the base of the skull. About twice as many men as women are diagnosed with chordoma.
What are the possible causes of bone cancer?
Although primary bone cancer does not have a clearly defined cause, researchers have identified several factors that increase the likelihood of developing these tumors.
Previous cancer treatment with radiation, chemotherapy, or stem cell transplantation. Osteosarcoma occurs more frequently in people who have had high-dose external radiation therapy (particularly at the location in the body where the radiation was given) or treatment with certain anticancer drugs, particularly alkylating agents; those treated during childhood are at particular risk.
Certain inherited conditions. A small number of bone cancers are due to hereditary conditions. For example, children who have had hereditary retinoblastoma are at a higher risk of developing osteosarcoma, particularly if they are treated with radiation. Members of families with Li-Fraumeni syndrome are at increased risk of osteosarcoma and chondrosarcoma, as well as other types of cancer. Additionally, people who have hereditary defects of bones have an increased lifetime risk of developing chondrosarcoma.
Certain benign bone conditions. People over age 40 who have Paget disease of bone are at increased risk of developing osteosarcoma.
What are the symptoms of bone cancer?
Pain is the most common symptom of bone cancer, but not all bone cancers cause pain. Persistent or unusual pain or swelling in or near a bone can be caused by cancer or by other conditions. Other symptoms of bone cancer include a lump (that may feel soft and warm) in the arms, legs, chest, or pelvis; unexplained fever; and a bone that breaks for no known reason (this is called a pathological fracture, which is a broken bone that breaks because of a disease rather than an injury).
Metastatic Bone Cancer
Cancer that metastasizes to the bones from other parts of the body is called metastatic (or secondary) bone cancer and is referred to by the organ or tissue in which it began. In adults, cancerous tumors that have metastasized to the bone are much more common than primary bone cancer. A reported that solid tumors frequently metastasize to bone, and that "these bone metastases are associated with shortened survival and increased risk of serious bone complications during the patients' remaining lifespan." The most common origin sites for metastatic bone cancer were breast (36%), lung (16%), and colorectal (12%). The mean time to bone metastasis was 400 days (1.1 years). Patients with stage IV cancer at diagnosis had the highest incidence of bone metastasis, often diagnosed within 30 days.
Treatment for Bone Cancer
The goal of management for patients with bone cancer (both primary and metastatic) include:
- Pain management
- Preservation/reservation of function
- Minimizing the risk of skeletal-related events, such as a pathologic fracture, spinal cord compression, necessity for radiation to bone (for pain or impending fracture), or surgery to bone
- Bone stabilization
- Enhancing local tumor control
Primary bone cancer
Surgery is the primary treatment for primary bone cancer, although there have been favorable advances in the use of chemotherapy for neoadjuvant (pre-surgical) therapy in osteosarcoma.
The surgeon removes the entire tumor with negative margins (that is, no cancer cells are found at the edge of the tissue removed during surgery). The surgeon may also use special surgical techniques to minimize the amount of healthy tissue removed along with the tumor.
Dramatic improvements in surgical techniques and preoperative tumor treatment have made it possible for most patients with bone cancer in an arm or leg to avoid radical surgical procedures (that is, removal of the entire limb). However, most patients who undergo need reconstructive surgery to regain limb function.
Although osteosarcoma is believed to be relatively radiation insensitive, recent improvements in radiation therapy have renewed interest in this modality for patients responsive to chemotherapy for whom surgery might be debilitating.
Metastatic bone cancer
Treatment for metastatic bone disease is partially dependent on the primary tumor origin. Multimodality treatment may be necessary with origin-specific systemic chemotherapy along with radiation therapy (external beam or stereotactic body radiotherapy) and/or surgical intervention.
External beam radiation is often useful for pain reduction in skeletal metastases, which can be complete in up to 1/3 of patients. Osteoclast inhibitors (including ) slow or reverse the progression of skeletal metastases and reduce the likelihood of skeletal-related events. They also have some modest analgesic effect.
Clinical trials for patients with primary bone cancers can be found . For those with metastatic bone cancer, click .
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country, including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.