Condition Lookup
Sub-Category:
Rare Cancers
Number of Conditions: 6
Carcinoid Tumors
Specialty: Oncology
Category: Sarcomas
Sub-category: Rare Cancers
Symptoms:
flushing; diarrhea; abdominal pain; wheezing; heart valve problems; weight loss; fatigue
Root Cause:
Carcinoid tumors arise from neuroendocrine cells, which produce hormones such as serotonin and other chemicals. These tumors often grow slowly and can secrete hormones that affect various body systems.
How it's Diagnosed: videos
Diagnosis is typically through imaging studies such as CT scans, MRIs, or PET scans, and blood tests measuring elevated levels of serotonin or 5-HIAA (a serotonin metabolite). Biopsy and histopathological examination confirm the diagnosis.
Treatment:
Treatment may include surgery to remove the tumor, somatostatin analogs (like octreotide) to control symptoms, chemotherapy, and in some cases, liver-directed therapies (such as ablation or embolization) if the tumors have spread to the liver.
Medications:
Somatostatin analogs (e.g., octreotide and lanreotide ) are often prescribed to control symptoms and inhibit hormone secretion. These medications are classified as peptide receptor drugs and can help reduce flushing and diarrhea. Chemotherapy agents may also be used, depending on the tumor's progression, such as streptozocin and temozolomide , which are alkylating agents.
Prevalence:
How common the health condition is within a specific population.
Carcinoid tumors are rare, accounting for about 0.5% of all cancers, but the incidence of neuroendocrine tumors is rising, with an estimated prevalence of 2-5 cases per 100,000 people per year.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
Risk factors include age (more common in older adults), a family history of neuroendocrine tumors, certain inherited genetic syndromes (such as MEN1 and von Hippel-Lindau syndrome), and conditions that affect the gastrointestinal tract like Crohn's disease.
Prognosis:
The expected outcome or course of the condition over time.
The prognosis depends on the location and spread of the tumor. Localized tumors have a relatively good prognosis with surgery, but metastatic or advanced cases can be more challenging to treat. The 5-year survival rate for localized carcinoid tumors is around 80%, while it drops significantly with distant metastases.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Potential complications include carcinoid syndrome (which leads to flushing, diarrhea, and heart valve problems), liver metastasis, and malnutrition due to nutrient malabsorption from the gastrointestinal symptoms.
Neuroendocrine Tumors (NETs)
Specialty: Oncology
Category: Sarcomas
Sub-category: Rare Cancers
Symptoms:
abdominal pain; diarrhea; flushing; wheezing; fatigue; unexplained weight loss; heart palpitations; skin rashes
Root Cause:
Neuroendocrine tumors are cancers that develop from neuroendocrine cells, which have characteristics of both nerve cells and hormone-producing cells. These tumors can occur in various parts of the body, most commonly the gastrointestinal tract, pancreas, and lungs. They can secrete hormones, leading to symptoms like flushing and diarrhea.
How it's Diagnosed: videos
Diagnosis is through imaging techniques such as CT scans, MRIs, or PET scans to locate the tumor, blood tests for elevated hormone levels (e.g., chromogranin A, serotonin), and biopsy for histological confirmation.
Treatment:
Treatment often involves surgical resection of the tumor, if localized, and medications such as somatostatin analogs (e.g., octreotide or lanreotide) to manage symptoms. Chemotherapy and targeted therapies (e.g., everolimus, sunitinib) may be used for advanced or metastatic disease. In some cases, liver-directed treatments (like embolization) or peptide receptor radionuclide therapy (PRRT) are used for metastases.
Medications:
Somatostatin analogs like octreotide and lanreotide help control the symptoms and reduce hormone secretion. These are classified as peptide receptor drugs. For advanced NETs, chemotherapy agents like temozolomide or streptozocin (alkylating agents) and targeted therapies like everolimus (an mTOR inhibitor) and sunitinib (a tyrosine kinase inhibitor) may be used.
Prevalence:
How common the health condition is within a specific population.
The incidence of NETs has been increasing, with an estimated prevalence of around 2-3 cases per 100,000 individuals per year. NETs are rare but more common in older adults, with gastrointestinal and pancreatic NETs being the most frequent.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
Risk factors include age (more common in people over 50), a family history of NETs, genetic conditions like multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau syndrome, and neurofibromatosis type 1.
Prognosis:
The expected outcome or course of the condition over time.
Prognosis varies based on tumor type, location, and stage at diagnosis. For localized NETs, the 5-year survival rate is generally favorable (70-80%), while advanced or metastatic NETs have a lower survival rate (around 30-40%).
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Complications include metastasis to the liver or other organs, carcinoid syndrome (which includes symptoms like flushing, diarrhea, and heart valve issues), hormonal imbalances, and malabsorption due to gastrointestinal involvement.
Small Intestine Cancer
Specialty: Oncology
Category: Sarcomas
Sub-category: Rare Cancers
Symptoms:
abdominal pain; unexplained weight loss; nausea; vomiting; jaundice; blood in stool; changes in bowel habits; fatigue
Root Cause:
Small intestine cancer develops in the cells of the small intestine, often affecting the epithelial or neuroendocrine cells. The exact cause is unknown, but genetic mutations and conditions like Crohn's disease, celiac disease, and Lynch syndrome can increase the risk.
How it's Diagnosed: videos
Diagnosis is typically through imaging studies like CT scans, MRIs, or endoscopy (e.g., capsule endoscopy or push enteroscopy). Biopsy is used to confirm the diagnosis through histological examination of the tumor tissue.
Treatment:
Treatment involves surgery to remove the tumor if detected early. Chemotherapy (e.g., 5-fluorouracil, capecitabine, or oxaliplatin) is often used for advanced disease. Radiation therapy may be used in some cases. Targeted therapy or immunotherapy may also be considered depending on tumor type.
Medications:
Chemotherapy drugs like 5-fluorouracil (an antimetabolite) and oxaliplatin (a platinum-based chemotherapy drug) are commonly used. Targeted therapies like bevacizumab (an anti-VEGF monoclonal antibody) and immunotherapies such as pembrolizumab (an immune checkpoint inhibitor) may be prescribed for advanced or metastatic cases.
Prevalence:
How common the health condition is within a specific population.
Small intestine cancer is very rare, representing less than 2% of all gastrointestinal cancers. Its incidence is estimated to be about 2-3 cases per 100,000 people per year.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
Risk factors include genetic conditions such as Lynch syndrome, familial adenomatous polyposis (FAP), and Crohn's disease. Celiac disease and a history of radiation therapy to the abdomen also increase risk.
Prognosis:
The expected outcome or course of the condition over time.
The prognosis depends on the stage of the cancer at diagnosis. For localized small intestine cancer, the 5-year survival rate can be as high as 80%, while for metastatic disease, the prognosis is much poorer, with a 5-year survival rate closer to 30%.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Potential complications include bowel obstruction, bleeding, malnutrition, and metastasis to the liver or lymph nodes. In some cases, the cancer can lead to the development of secondary tumors or complications from chemotherapy.
Peritoneal Mesothelioma
Specialty: Oncology
Category: Sarcomas
Sub-category: Rare Cancers
Symptoms:
abdominal pain; swelling in the abdomen; unexplained weight loss; nausea; vomiting; loss of appetite; fatigue; bloating; constipation
Root Cause:
Peritoneal mesothelioma is a rare cancer that affects the peritoneum, the lining of the abdomen. It is most commonly caused by asbestos exposure, which leads to the development of malignant cells that grow and invade the peritoneal lining.
How it's Diagnosed: videos
Diagnosis typically involves imaging studies such as CT scans, MRIs, or ultrasounds to detect fluid buildup (ascites) and tumors in the abdomen. A biopsy is needed for definitive diagnosis, often performed through laparoscopy or peritoneal biopsy.
Treatment:
Treatment options include surgery (e.g., cytoreductive surgery) to remove as much of the tumor as possible, often followed by heated chemotherapy (HIPEC) to target remaining cancer cells. Chemotherapy (e.g., pemetrexed and cisplatin) is used for advanced cases, and radiation therapy may also be applied.
Medications:
Common chemotherapy drugs used to treat peritoneal mesothelioma include pemetrexed (an antifolate drug) and cisplatin (a platinum-based chemotherapy agent). These are used in combination to help shrink tumors and manage the disease. In some cases, targeted therapies or immunotherapy may be considered.
Prevalence:
How common the health condition is within a specific population.
Peritoneal mesothelioma is extremely rare, accounting for approximately 10-20% of all mesothelioma cases. The overall incidence is low, with an estimated 300-500 new cases diagnosed annually in the United States.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
The primary risk factor is exposure to asbestos, particularly in occupations like construction, shipbuilding, and manufacturing. Other risk factors include genetic mutations and a history of prior radiation exposure.
Prognosis:
The expected outcome or course of the condition over time.
The prognosis for peritoneal mesothelioma is poor, with a median survival rate of 12-24 months, depending on the stage at diagnosis. Patients who undergo cytoreductive surgery with HIPEC may have improved outcomes, but survival is still limited.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Complications include bowel obstruction, ascites (fluid buildup in the abdomen), malnutrition, and metastasis to other parts of the body, particularly the lungs. The treatment itself can also cause side effects like fatigue, nausea, and immune suppression.
Extragonadal Germ Cell Tumors
Specialty: Oncology
Category: Sarcomas
Sub-category: Rare Cancers
Symptoms:
abdominal pain; swelling or mass; chest pain; coughing; shortness of breath; unexplained weight loss; fever; night sweats
Root Cause:
Extragonadal germ cell tumors (EGCTs) arise from germ cells that are typically found in the gonads (ovaries or testes) but develop in areas outside of these organs, such as the mediastinum, retroperitoneum, or pineal gland. These tumors are believed to be the result of abnormal migration of germ cells during fetal development.
How it's Diagnosed: videos
Diagnosis typically involves imaging techniques such as CT scans, MRIs, or chest X-rays to locate the tumor. Blood tests to measure tumor markers like alpha-fetoprotein (AFP) and human chorionic gonadotropin (hCG) are also used. A biopsy is necessary for histological confirmation.
Treatment:
Treatment for extragonadal germ cell tumors often involves a combination of surgery, chemotherapy, and sometimes radiation therapy. The standard chemotherapy regimen includes bleomycin, etoposide, and cisplatin (BEP). Surgery is used to remove the primary tumor when feasible.
Medications:
Chemotherapy agents such as bleomycin (an antibiotic used as a chemotherapy agent), etoposide (a plant alkaloid), and cisplatin (a platinum-based chemotherapy drug) are used to treat extragonadal germ cell tumors. These drugs are classified as alkylating agents, antimetabolites, and plant alkaloids , respectively.
Prevalence:
How common the health condition is within a specific population.
Extragonadal germ cell tumors are rare, representing about 1-5% of all germ cell tumors. The incidence is lower than gonadal germ cell tumors, with approximately 2-3 cases per million people per year.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
Risk factors include age (most common in adolescents and young adults), history of gonadal germ cell tumors, and certain genetic syndromes like Klinefelter syndrome. In rare cases, a family history of germ cell tumors or other related conditions may increase risk.
Prognosis:
The expected outcome or course of the condition over time.
The prognosis for extragonadal germ cell tumors depends on the tumor's location, stage at diagnosis, and response to treatment. If caught early and treated aggressively, the 5-year survival rate can be around 70-90%, but survival rates drop significantly for metastatic or advanced disease.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Potential complications include metastasis to the lungs, liver, or other organs, recurrence after treatment, and side effects of chemotherapy such as organ toxicity (e.g., to the lungs or kidneys), fertility issues, and secondary cancers.
Plasmacytomas
Specialty: Oncology
Category: Sarcomas
Sub-category: Rare Cancers
Symptoms:
bone pain; fatigue; unexplained weight loss; anemia; frequent infections; weakness; numbness or tingling in limbs
Root Cause:
Plasmacytomas are abnormal growths of plasma cells, a type of white blood cell that produces antibodies. These tumors can occur in bone (solitary plasmacytoma of bone) or soft tissue (extramedullary plasmacytoma). They may develop in isolation or as part of a more widespread condition like multiple myeloma.
How it's Diagnosed: videos
Diagnosis involves imaging techniques like X-rays, CT scans, or MRIs to detect bone lesions or soft tissue masses. Bone marrow biopsy and blood tests (such as serum protein electrophoresis for monoclonal proteins) are used to confirm the presence of abnormal plasma cells. A biopsy of the tumor is performed for histological diagnosis.
Treatment:
Treatment typically involves localized radiation therapy or surgical resection, depending on the tumor's location. In some cases, chemotherapy or stem cell transplantation may be used if the plasmacytoma is part of a more systemic disease like multiple myeloma. For extramedullary plasmacytomas, radiation is often the treatment of choice.
Medications:
Chemotherapy agents like melphalan (an alkylating agent) and cyclophosphamide (another alkylating agent) may be used for advanced disease or when plasmacytomas are associated with multiple myeloma. Additionally, corticosteroids like dexamethasone can be prescribed to reduce inflammation and manage symptoms.
Prevalence:
How common the health condition is within a specific population.
Plasmacytomas are rare, with solitary plasmacytomas representing only about 3% of all plasma cell neoplasms. The incidence is estimated at 0.2-1 case per 100,000 people per year.
Risk Factors:
Factors or behaviors that increase the likelihood of developing the condition.
Risk factors include age (more common in older adults), a family history of plasma cell disorders, and conditions like multiple myeloma. Previous radiation therapy and certain infections (like HIV) may also increase risk.
Prognosis:
The expected outcome or course of the condition over time.
The prognosis for solitary plasmacytoma is generally favorable, especially if it is diagnosed early and treated with surgery or radiation. The 5-year survival rate for localized plasmacytomas is around 80-90%. However, if plasmacytomas progress to multiple myeloma or involve multiple sites, the prognosis worsens significantly.
Complications:
Additional problems or conditions that may arise as a result of the original condition.
Complications can include bone fractures due to bone involvement, pain management issues, spinal cord compression if the tumor involves the spine, and recurrence of the plasmacytoma. If it transforms into multiple myeloma, the complications and prognosis worsen.