The Canadian Cancer Society defines rare cancer as “a cancer that is diagnosed in less than 6 of every 100,000 people in Canada each year.” Examples include penile cancer, male breast cancer, ureter cancer etc. In Canada, rare cancers account for 20% of new cancer cases. There is, however, little information about rare cancers, challenges with diagnosis, and few treatment options.
Often, information about rare cancers is hard to come by, and survivors might feel isolated in their journey; therefore, it is always good to talk about one’s illness with professionals and family members, as well as finding others with the same disease, perhaps through a support group or an online forum.
Rare Blood and Bone Marrow Cancers
Acute Lymphoblastic Leukemia (ALL) is a cancer that originates in the stem cells of the blood. Stem cells are basic cells that go on to become distinct types of cells with different jobs. Blood stem cells go on to become blast cells. These are immature blood cells. Leukemia occurs when there is an excessive production of blast cells. Acute lymphoblastic leukemia originates in atypical lymphoid stem cells and develops rapidly. ALL is the most frequently diagnosed form of leukemia in young children but for adults, it is the least frequently occurring of the 4 major forms of leukemia.
Information from: Canadian Cancer Society
For more information, visit the Canadian Cancer Society.
Chronic Lymphocytic Leukemia (CLL) originates in atypical lymphoid stem cells and generally develops slowly over the course of months or years. In the vast majority of CLL cases (95%), the atypical lymphoid stem cells turn cancerous or malignant. In adults, CLL is one of the most prevalent forms of leukemia; however, in children, CLL is very rare.
Information from: Canadian Cancer Society
For more information, visit the Canadian Cancer Society
Chronic Myeloid Leukemia (CML) occurs when the bone marrow produces an excessive amount of granulocytes as a result of a genetic mutation called the BCR-ABL gene. The mutation alters the cells to prevent them from developing into normal granulocytes. These unusual granulocytes that have the BCR-ABL gene may be referred to as leukemia cells or CML cells. Over time, as the disease develops, the CML cells accumulate in the bone marrow and the blood, pushing out the healthy blood cells, and restricting their ability to function. CML is a chronic disease because it develops quite slowly.
Information from: Canadian Cancer Society
For more information, visit the Canadian Cancer Society
Myelodysplastic Syndromes: Myelodysplastic syndromes (MDS) represent a collection of diseases where the bone marrow produces an insufficient number of healthy blood cells. Rather, the bone marrow produces immature blood cells referred to as blast cells that don’t function normally. These cells accumulate in the bone marrow and in the blood, and push out healthy blood cells, resulting in less healthy red blood cells, white blood cells and platelets.
From: Canadian Cancer Society
For more information, visit Canadian Cancer Society
Myeloproliferative Neoplasms (MPNs): Myeloproliferative neoplasms (MPNs) are a collection of blood diseases where the bone marrow overproduces one or more types of blood cells. A myeloproliferative neoplasm begins when one blood stem cell undergoes a mutation and reproduces. Some MPNs may progress into acute myeloid leukemia. MPN’s are categorized as blood cancers because the blood cells are produced uncontrollably; however, MPNs typically progress at a slow pace, so they only require mild treatments. The three primary types of MPNs are:
- Myelofibrosis: A type of myeloproliferative neoplasm where scar tissue accumulates in the bone marrow, preventing the production of sufficient healthy blood cells. From: Canadian Cancer Society.
- Polycythemia Vera: Polycythemia Vera occurs when the body produces an excessive number of red blood cells and in some cases, white blood cells or platelets.
- Essential thrombocytopenia: Essential thrombocythemia occurs when the body produces an excessive number of platelets.
From: Canadian Cancer Society
For more information, visit Canadian Cancer Society.
Rare Non-Hodgkin's Lymphoma
A rare type of non-Hodgkin lymphoma, this is a severe type of cancer that impacts the T lymphocytes. Anaplastic Large Cell Lymphomas can vary, with some being intense and able to rapidly spread, while others develop more slowly.
Information from: Cleveland Clinic
For more information, visit Cleveland Clinic.
CNS lymphoma is a type of rare cancer that targets your central nervous system (CNS). The cancer can develop in the brain, spinal cord, spinal fluid or behind the eye. CNS lymphoma is able to spread rapidly throughout your central nervous system, and can lead to alterations in your vision and muscle weakness.
Information from: Cleveland Clinic
For more information, visit Cleveland Clinic.
Marginal zone lymphomas (MZLs) are a type of slow-developing B-cell non hodgkin lymphomas. Various types of Marginal zone lymphomas exist but all types originate in the lymphoid tissue, which can be found in your stomach, lymph nodes and spleen.
Information from: Cleveland Clinic
For more information, visit: Cleveland Clinic
Lymphoplasmacytic lymphoma occurs when certain white blood cells experience changes that cause them to become cancerous. These cancerous cells can accumulate in the bone marrow, pushing out healthy blood cells. The cancerous cells can also accumulate in other regions of the body, like the lymph nodes and the spleen.
Information from: Mayo Clinic
For more information, visit: Mayo Clinic and Lymphoma Canada
Burkitt lymphoma is a severe and rapidly growing type of non-Hodgkin lymphoma that originates in B cells. Burkitt lymphoma can arise in the lymph nodes, but also in other organs or tissues. This cancer can spread to the central nervous system, reaching the brain or spinal cord.
Information from: Canadian Cancer Society
For more information, visit: Canadian Cancer Society
Mediastinal large b-cell lymphoma is a severe and rapidly growing form of non-Hodgkin Lymphoma that occurs in the thymus or lymph nodes in the middle of the chest. When the lymph nodes surrounding the lungs and behind the breastbone swell, this can lead to symptoms, including chest discomfort or a cough.
Information from: Canadian Cancer Society
For more information, visit: Canadian Cancer Society
This is an intense and rapidly growing form of non-hodgkin lymphoma. Lymphoblastic lymphoma is quite similar to acute lymphoblastic leukemia (ALL) and both are treated similarly. Lymphoblastic lymphoma and ALL vary based on the location of the cancerous cells. Cancer cells are primarily located in the lymph nodes in lymphoblastic lymphoma but in ALL, cancer cells are generally located in the bone marrow and in some cases, the lymph nodes.
From: Canadian Cancer Society
For more information, visit Canadian Cancer Society
Rare Hodgkin Lymphomas
Nodular lymphocyte-predominant Hodgkin lymphoma is an uncommon form of blood cancer that develops when lymphocytes behave unusually. NLPHL is rare, and develops slowly as the product of genetic mutations targeting genes in the B lymphocytes. NLPHL represents a small percentage of all Hodgkin lymphoma cases.
From: City of Hope Cancer Centers
For more information, visit City of Hope Cancer Centers and Canadian Cancer Society
Rare Brain and Spine Cancers
Astrocytoma: astrocytoma is a type of tumour that can occur in the brain and spinal cord. It begins in the astrocytes of the central nervous system (CNS). Astrocytomas are located in the cerebellum, cerebrum, central areas of the brain, brain stem and spinal cord.
From: Canadian Cancer Society
For more information: Canadian Cancer Society
A type of tumour that forms from the membranes that line the brain and spinal cord, known as the meninges. A meningioma is not categorized as a brain tumor; however, it may exert pressure on the nearby brain, nerve and vessels. It is the most frequent type of tumor that develops in the head. The majority of meningiomas develop at a very slow pace, and they can progress over a period of time without presenting symptoms. However, in some cases, their impact on nearby brain tissue, nerves, or vessels may lead to serious disability.
From: Mayo Clinic
For more information: Mayo Clinic
Rare Breast Cancers
Phyllodes Tumours: Phyllodes tumour is a rare tumour that originates in the breast connective tissue. Generally, phyllodes tumours are non-cancerous (benign) but in some cases, they can be cancerous or possess characteristics of both non-cancerous and cancerous tumours, known as borderline tumours.
From: Canadian Cancer Society
For more information, visit: Cleveland Clinic
Paget disease of the breast emerges as a rash or as other skin variations on the breast and nipple, and typically occurs only on one breast. The majority of people with Paget disease of the breast also experience invasive ductal carcinoma or ductal carcinoma in situ in the breast.
From: Canadian Cancer Society
For more information, visit Canadian Cancer Society
Male Breast Cancer: Male breast cancer is a rare cancer that starts as a growth of cells in male breast tissue. Breast cancer is generally perceived as a condition that affects women but all individuals are born with some amount of breast tissue, and as a result anyone can develop breast cancer. Male breast cancer is not common and mostly occurs in older men but it can develop at any age.
From: Mayo Clinic
For more information, visit: Mayo Clinic
Inflammatory Breast Cancer: A rare form of breast cancer that occurs when cancer cells obstruct the lymph vessels in the breast skin, leading to the reddening or darkening of the breast skin and inflammation. Inflammatory breast cancer progresses and spreads rapidly, and typically, by the time it is diagnosed, inflammatory breast cancer has moved to the lymph nodes or different organs. Inflammatory breast cancer occurs more frequently in younger women and women with an African background.
- From: Canadian Cancer Society
- For more information, visit: Canadian Cancer Society
Rare Gastrointestinal Tract Cancers
Cholangiocarcinoma (bile duct cancer) is a form of cancer that develops in the bile ducts that transport bile (digestive juice). Bile ducts attach your liver to your gallbladder and to your small intestine. Cholangiocarcinoma mainly affects individuals above 50, but it can develop at any age.
From: Mayo Clinic
For more information, visit Mayo Clinic
Duodenal cancer is a rare type of cancer that occurs in the duodenum, which makes up the first portion of the small intestine. The duodenum works to break down partially digested food into nutrients but cancer in this region affects this process. In the beginning stages, duodenal cancer generally produces no symptoms but as the cancer progresses, it may interfere with proper digestion, leading to symptoms such as nausea, abdominal pain or constipation.
From: Cleveland Clinic
For more information, visit Cleveland Clinic
Anorectal melanoma is a rare type of cancer that begins in the region surrounding the anus/rectum. It is also referred to as anorectal mucosal melanoma (ARMM). The rectum makes up the last section of the large bowel. The anus is the opening at the end of the rectum where stool leaves the body. Melanoma refers to cancer that begins from cells known as melanocytes. Typically, melanoma begins in the skin, but some melanocytes are also located in the mucosa. The mucosa is the tissue covering many regions of the body. Melanoma that occurs in the mucosa is known as mucosal melanoma, and it can develop in various regions of the body such as the region in and around the anus or rectum.
From: Macmillan Cancer Support
For more information, visit Macmillan Cancer Support
Rare Gynecologic Cancers
Germ Cell Tumours: Germ cell tumors are a collection of cells that are produced from reproductive cells known as germ cells. Germ cells become eggs in the female reproductive system but in the male reproductive system, they become sperm. The majority of germ cell tumors occur in the ovaries or the testicles, although they can occur elsewhere in the body, for example, in the belly, brain and chest. Germ cells that form outside the ovaries and testicles are classified as extragonadal germ cell tumours. This is a rare occurrence that it is not completely understood.
From: Mayo Clinic
For more information, visit Mayo Clinic.
Fallopian Tube Cancer: Fallopian Tube Cancer develops in the fallopian tubes, the ducts that transport eggs from the ovaries to the uterus. Generally, fallopian tube cancer originates in epithelial tissue and behaves like ovarian cancer and primary peritoneal cancer. Fallopian tube cancer is rare and may result in ovarian cancer. Individuals at a greater risk are those with a BRCA gene mutation.
From: Cleveland Clinic
For more information, visit Cleveland Clinic
Vulvar Cancer: Vulvar cancer begins with the growth of cells on the vulva. The vulva is the region of the skin that encircles the urethra and vagina. The vulva also includes the clitoris and labia. Vulvar cancer typically develops as a mass or a sore on the vulva, frequently resulting in itching. Vulvar cancer can develop at any age but it is typically diagnosed in older adults.
From: Mayo Clinic
For more information, visit Mayo Clinic and the Canadian Cancer Society
Rare Peritoneal Cancers
Primary Peritoneal Cancer: A rare type of cancer that develops in the thin layer of tissue surrounding your abdomen known as the peritoneum. The peritoneum also surrounds the bladder, intestines, rectum and uterus, and it is composed of epithelial cells designed to protect the surfaces of your body. The peritoneum also facilitates the movement of organs inside the abdomen through producing fluid. Primary peritoneal cancer develops when the cells within the peritoneum grow excessively. Symptoms aren’t usually detected until the cancer has advanced and for this reason, the majority of individuals end up being diagnosed at a later stage.
From: Cleveland Clinic
For more information, visit Cleveland Clinic
A highly rare form of cancer that targets the peritoneal cavity which includes the abdomen and pelvis, and fills them with cancerous cells that form mucin. Mucin is a substance that makes up one of the components of mucus. It resembles jelly. Pseudomyxoma peritonei does not produce a tumour that can metastasize. Rather, the cancer begins as a cancerous polyp in the appendix. When the polyp bursts through the appendix, it sets off a surge of mucin-producing cancer cells that, over a period of time, impact the digestive system. The surge of jelly-like mucin explains why PMP is sometimes referred to as “jelly belly.”
From: Cleveland Clinic
For more information, visit: Cleveland Clinic
Rare Thymus Cancers
Thymus cancer originates in the cells of the thymus, and is a rare type of cancer. The thymus is a small gland located at the top region of the chest and in between the lungs and beneath the breastbone. The thymus produces T cells that move throughout the body to assist in fighting infection and disease. In addition, the thymus produces hormones and various other substances to help T-cells establish and maintain proper immune system functioning. Thymus cells may become altered and stop growing normally. These events can result in non-cancerous (benign) tumours for example, thymic cysts and thymolipomas. Thymus cell changes can also lead to cancer in certain instances. Typically, thymus cancer originates in the epithelial cells of the thymus, and is known as thymoma and thymic carcinoma.
From: Canadian Cancer Society
For more information, visit Canadian Cancer Society, BC Cancer, and Cleveland Clinic
Rare Urinary Tract Cancers
Ureteral Cancer: Ureteral cancer is characterized by a growth of cells that begins in the ureters. The ureters are components of the urinary tract, transporting urine made by the kidneys to the bladder. These tubes link the kidneys to the bladder. Ureteral cancer is rare, and typically occurs in older adults and in individuals who have experienced bladder cancer.
From: Mayo Clinic
For more information, visit Mayo Clinic
Penile Cancer: Penile cancer originates in or on the penis. It frequently begins in the skin cells of the penis. Cells in the penis can undergo changes that lead them to stop functioning normally. These shifts can result in non-cancerous or precancerous conditions, but they may also lead to cancer. Typically, penile cancer begins in the squamous cells, which are located in the skin and head of the penis. Cancer that begins in the squamous cells in the penis are referred to as squamous cell carcinoma (SCC) of the penis. SCC can occur virtually anywhere on the penis, but generally it forms on the foreskin in uncircumcised men or the glans. Other types of penile cancer, including rare forms, like adenocarcinoma, melanoma and basal cell carcinoma (BCC) can also occur.
From: Canadian Cancer Society
For more information: visit Canadian Cancer Society or BC Cancer.
Rare Cancer Organizations and Blogs
Canadian Organization for Rare Disorders (CORD): “CORD is Canada’s national network for organizations representing all those with rare disorders. CORD provides a strong common voice to advocate for health policy and a healthcare system that works for those with rare disorders. CORD works with governments, researchers, clinicians and industry to promote research, diagnosis, treatment and services for all rare disorders in Canada.” Visit them here.
Canadian Rare Disease Network: “The CRDN is a pan-Canadian network that unites the country’s leading clinical, scientific, and patient expertise to improve the lives of the millions of children and adults affected by a rare disease across Canada. By bringing together all partners and mobilising the rare disease community, the CRDN aims to accelerate innovative scientific and medical advances, and improve standards of care across Canada for patients and their families.” Visit them here.
- Rare Cancer Alliance (USA)
- Rare Cancer Research Foundation (USA)
- Rare Cancer Coalition | National Organization for Rare Disorders (USA)
- MyPART Network Patient Advocacy Partners – NCI (USA)
- EURACAN (Europe)
- ERDERA – European Rare Diseases Research Alliance
- EURORDIS – Rare Diseases Europe
- Rare Cancers Australia
- Australian Rare Cancer Portal