Adrenal carcinoma is an aggressive cancer originating in the adrenal glands, with very limited incidence. These include the adrenal cortex, which is responsible for the secretion of important hormones like cortisol, aldosterone, and adrenaline, which are crucial for the functioning of the human body.
Epidemiology
Adrenal carcinomas are rare, with the incidence estimated to be around 0.6 to 1.67%. Although in southern Brazil the incidence is 10 –15 time that of the normal population, this is attributable to a mutation on the P53 gene.
Anatomy
Pathophysiology
Origin: Adrenal carcinoma develops in the cortex of the adrenal glands, which is the outer layer of the organ. The adrenal cortex is involved in secretion of hormones such as cortisol, aldosterone and sex hormones.
Genetic Mutations: There are several genes’ mutations and abnormalities which can cause adrenal carcinoma. For example, TP53 gene, CTNNB1 genes, and chromosomes 11 alteration commonly occurred.
Hormonal Overproduction: It may lead to the overproduction of hormones in the adrenal glands. This may result in hormone-related diseases including Cushing’s disease resulting from excessive cortisol, Conn’s disease resulting from excessive aldosterone or even virilization resulting from increased levels of androgens.
Local Invasion and Metastasis: Adrenal carcinoma is rapidly growing and has a high propensity to invade adjacent structures and organs. It also has the capacity to metastasize to other organs including the liver, lungs and lymph nodes, this makes the disease physiology a complex one.
Pathological Features: Adrenal carcinoma may be accompanied by hemorrhage and necrosis, and microscopically there is usually a high mitotic activity, atypical cells and necrosis. Pleomorphic architecture with high cellularity and Ki-67 proliferation rate can be observed in architecture of this type.
Diagnosis and Staging: The diagnosis of this disease very often prescribes imaging tests such as CT scans or MRI, biopsy, and blood tests to determine hormone levels. Staging aids in indicating the stage through which the disease has progressed and hence the treatment to be given.
Etiology
Genetic Predispositions: Although the exact causes of adrenal carcinoma have not been well identified, several factors can predispose an individual to the disease, and they include the following genetic disorders:
Li-Fraumeni Syndrome: An inherited condition which predisposes an individual to develop several forms of cancer.
Beckwith-Wiedemann Syndrome: A disease related to overgrowth and potentiality of certain types of cancers such as adrenal carcinoma.
Multiple Endocrine Neoplasia Type 1 (MEN1): A hereditary disease concerned with the development of tumor in many endocrine glands like adrenal gland.
Hormonal Factors: Hormonal alterations or hormonal imbalances may sometimes be related to adrenal tumors, although the relationship is not very clear with carcinoma.
Previous Adrenal Tumors: Some of those who have had concern of benign adrenal tumour including adrenal adenomas are likely to have carcinoma.
Environmental and Lifestyle Factors: No environmental or lifestyle factors have been associated with adrenal carcinoma but the common cancer risk factors such as smoking, obesity and exposure to some chemicals may be considered.
Other Medical Conditions: Inherited diseases that can predispose people to adrenal carcinoma include adrenal hyperplasia or some genetic disorders.
Genetics
Prognostic Factors
This disease has unfavourable outcome, especially if the tumor is diagnosed in the advanced stage, which is characteristic of adrenal carcinoma. It has been postulated that survival depends on the tumor size, stage at which it was diagnosed and response to treatment.
Clinical History
Age Group:
Being one of the rarest forms of cancer, adrenal carcinoma is considered a malignant tumor that does not spare any age. However, it is most diagnosed in adults that are aged between 40 and 60 years of age.
Acute Presentation: Adrenal carcinoma may also present acutely with acute abdominal pain, a palpable mass, or symptoms of adrenal crisis secondary to rapid tumor growth or hemorrhage. Subacute Presentation: It is usually chronic; symptoms include hormonal disorders, for example, Cushing’s syndrome, abdominal pain, and unexplained weight loss for some weeks or months. Chronic Presentation: It is a silent disease; thus, in its early stage, it may only present with nonspecific complaints, such as persistent abdominal pains or hormonal alterations, whereas the majority of patients are diagnosed during a metastatic stage of the cancer.
Differential Diagnoses
Adrenal Adenoma
Adrenal Metastasis
Pheochromocytoma
Primary Adrenal Lymphoma
Cushing’s Syndrome
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Surgical Treatment
Primary Treatment: For localized adrenal carcinoma, surgery is always considered a good option and is often performed. Surgery usually involves removal of entire tumor and in some cases the adrenal tissue that is adjacent to the tumor and at times the adrenal gland as well.
Adjuvant Therapy: Adjuvant therapy can be defined as any treatment given after the surgery to prevent the formation of a tumor again especially if the tumor was very large in size or there are other predisposing factors.
Radiation Therapy
Radiation therapy is usually given when surgery cannot be done and when there is local recurrence. It may also be used for the treatment of terminal conditions as a form of palliative measure.
Systemic Therapy
Chemotherapy: For advanced or metastatic adrenal carcinoma, chemotherapy is often used. Common regimens include the EDP (etoposide, doxorubicin, and cisplatin) regimen.
Targeted Therapy: For some patients, the individual molecular characteristics of the tumor may be used to justify the use of targeted therapies.
Hormonal Therapy: If the cancer produces excess hormones, medications to control hormone levels may be used.
Supportive Care
Symptom Management: This suggests palliative care which encompasses control of symptoms to improve the patient’s quality of life as an essential component of the treatment.
Psychological Support: Include patients in appropriate counselling or support groups that will enable them deal with the psychological side of the disease.
Pain Management: Medications or other forms of palliative care that aim to improve the quality of life usually address pain and discomfort.
Regular Monitoring: Regular follow-up with imaging and blood tests is necessary in the monitoring of disease processes and modification of treatment according to response.
Effectiveness of chemotherapeutic agents in treating Adrenal Carcinoma
Oncology
Doxorubicin: An anthracycline antibiotic that is a potent adjuvant with other agents in treating various cancers, including adrenal carcinoma. Etoposide: An antineoplastic agent of the topoisomerase inhibitor class that is used alone or in association with other neoplasm medications.
Cisplatin: A platinum agent which may be used as a part of combination therapies in treatment of adrenal carcinoma.
Mitotane: Particularly used for adrenocortical carcinoma; it’s an adrenolytic agent that suppresses the functioning of the adrenal cortex and can be employed in adjunctive and symptomatic treatment only.
Surgical Resection Procedure: Adrenalectomy is the only possible treatment for localized adrenal carcinoma, though recent studies indicate that chemotherapy and radiation could work also well. This is a surgical procedure where the adrenal gland is removed along with surrounding tissues or lymph nodes if they are infected with cancer. considerations: The extent of the surgery depends on the size of the tumor and how much the stage has advanced. Less invasive procedure is called the laparoscopic adrenalectomy, but this cannot be performed to cure large growths. Radiation Therapy Procedure: Radiation having more energy is used to destroy the cancer cells. It is used in types of cancer that cannot be removed through surgery or if there is recurrence of the disease after surgery. Considerations: It can be used for symptomatic relieving in cases when the tumor has metastasized to other organs or in combination with other techniques.
role-of-management-in-treating-adrenal-carcinoma
Diagnosis and Staging:
Imaging: CT, MRI scan, or PET scan to know the size and extension of the tumor.
Biopsy: This may be done if needed to rule out the disease.
Hormonal Evaluation: To confirm if the tumor is releasing hormones that may be causing some problems in the body.
Surgical Management:
Adrenalectomy: The primary treatment for localized adrenal carcinoma. Surgery involves removing the affected adrenal gland and possibly surrounding tissues if the cancer has spread locally.
Adjuvant Therapy:
Radiation Therapy: Given if there remains local disease or if surgery cannot be done.
Chemotherapy: Traditionally it is used in third line or as palliative treatment of metastatic adrenal carcinoma. It consists of mitotane, etoposide, doxorubicin, and cisplatin plan regimens.
Hormonal Therapy:
Mitotane: A drug employed for the purpose of controlling adrenal carcinoma by affecting adrenal cortex cells.
Follow-Up Care:
Surveillance Imaging: CT or MRI scans regularly to check for relapse or metastasis.
Hormone Levels Monitoring: They need counselling to approach and deal with any hormonal fluctuations that occur because of the cancer or its treatment.
Supportive Care:
Palliative Care: Covers all aspects of comfort to enhance a patient’s life by controlling the symptoms and effects of treatment.
Adrenal carcinoma is an aggressive cancer originating in the adrenal glands, with very limited incidence. These include the adrenal cortex, which is responsible for the secretion of important hormones like cortisol, aldosterone, and adrenaline, which are crucial for the functioning of the human body.
Adrenal carcinomas are rare, with the incidence estimated to be around 0.6 to 1.67%. Although in southern Brazil the incidence is 10 –15 time that of the normal population, this is attributable to a mutation on the P53 gene.
Origin: Adrenal carcinoma develops in the cortex of the adrenal glands, which is the outer layer of the organ. The adrenal cortex is involved in secretion of hormones such as cortisol, aldosterone and sex hormones.
Genetic Mutations: There are several genes’ mutations and abnormalities which can cause adrenal carcinoma. For example, TP53 gene, CTNNB1 genes, and chromosomes 11 alteration commonly occurred.
Hormonal Overproduction: It may lead to the overproduction of hormones in the adrenal glands. This may result in hormone-related diseases including Cushing’s disease resulting from excessive cortisol, Conn’s disease resulting from excessive aldosterone or even virilization resulting from increased levels of androgens.
Local Invasion and Metastasis: Adrenal carcinoma is rapidly growing and has a high propensity to invade adjacent structures and organs. It also has the capacity to metastasize to other organs including the liver, lungs and lymph nodes, this makes the disease physiology a complex one.
Pathological Features: Adrenal carcinoma may be accompanied by hemorrhage and necrosis, and microscopically there is usually a high mitotic activity, atypical cells and necrosis. Pleomorphic architecture with high cellularity and Ki-67 proliferation rate can be observed in architecture of this type.
Diagnosis and Staging: The diagnosis of this disease very often prescribes imaging tests such as CT scans or MRI, biopsy, and blood tests to determine hormone levels. Staging aids in indicating the stage through which the disease has progressed and hence the treatment to be given.
Genetic Predispositions: Although the exact causes of adrenal carcinoma have not been well identified, several factors can predispose an individual to the disease, and they include the following genetic disorders:
Li-Fraumeni Syndrome: An inherited condition which predisposes an individual to develop several forms of cancer.
Beckwith-Wiedemann Syndrome: A disease related to overgrowth and potentiality of certain types of cancers such as adrenal carcinoma.
Multiple Endocrine Neoplasia Type 1 (MEN1): A hereditary disease concerned with the development of tumor in many endocrine glands like adrenal gland.
Hormonal Factors: Hormonal alterations or hormonal imbalances may sometimes be related to adrenal tumors, although the relationship is not very clear with carcinoma.
Previous Adrenal Tumors: Some of those who have had concern of benign adrenal tumour including adrenal adenomas are likely to have carcinoma.
Environmental and Lifestyle Factors: No environmental or lifestyle factors have been associated with adrenal carcinoma but the common cancer risk factors such as smoking, obesity and exposure to some chemicals may be considered.
Other Medical Conditions: Inherited diseases that can predispose people to adrenal carcinoma include adrenal hyperplasia or some genetic disorders.
This disease has unfavourable outcome, especially if the tumor is diagnosed in the advanced stage, which is characteristic of adrenal carcinoma. It has been postulated that survival depends on the tumor size, stage at which it was diagnosed and response to treatment.
Age Group:
Being one of the rarest forms of cancer, adrenal carcinoma is considered a malignant tumor that does not spare any age. However, it is most diagnosed in adults that are aged between 40 and 60 years of age.
Acute Presentation: Adrenal carcinoma may also present acutely with acute abdominal pain, a palpable mass, or symptoms of adrenal crisis secondary to rapid tumor growth or hemorrhage. Subacute Presentation: It is usually chronic; symptoms include hormonal disorders, for example, Cushing’s syndrome, abdominal pain, and unexplained weight loss for some weeks or months. Chronic Presentation: It is a silent disease; thus, in its early stage, it may only present with nonspecific complaints, such as persistent abdominal pains or hormonal alterations, whereas the majority of patients are diagnosed during a metastatic stage of the cancer.
Adrenal Adenoma
Adrenal Metastasis
Pheochromocytoma
Primary Adrenal Lymphoma
Cushing’s Syndrome
Surgical Treatment
Primary Treatment: For localized adrenal carcinoma, surgery is always considered a good option and is often performed. Surgery usually involves removal of entire tumor and in some cases the adrenal tissue that is adjacent to the tumor and at times the adrenal gland as well.
Adjuvant Therapy: Adjuvant therapy can be defined as any treatment given after the surgery to prevent the formation of a tumor again especially if the tumor was very large in size or there are other predisposing factors.
Radiation Therapy
Radiation therapy is usually given when surgery cannot be done and when there is local recurrence. It may also be used for the treatment of terminal conditions as a form of palliative measure.
Systemic Therapy
Chemotherapy: For advanced or metastatic adrenal carcinoma, chemotherapy is often used. Common regimens include the EDP (etoposide, doxorubicin, and cisplatin) regimen.
Targeted Therapy: For some patients, the individual molecular characteristics of the tumor may be used to justify the use of targeted therapies.
Hormonal Therapy: If the cancer produces excess hormones, medications to control hormone levels may be used.
Supportive Care
Symptom Management: This suggests palliative care which encompasses control of symptoms to improve the patient’s quality of life as an essential component of the treatment.
Oncology, Other
Supportive Environment:
Psychological Support: Include patients in appropriate counselling or support groups that will enable them deal with the psychological side of the disease.
Pain Management: Medications or other forms of palliative care that aim to improve the quality of life usually address pain and discomfort.
Regular Monitoring: Regular follow-up with imaging and blood tests is necessary in the monitoring of disease processes and modification of treatment according to response.
Oncology, Other
Oncology
Doxorubicin: An anthracycline antibiotic that is a potent adjuvant with other agents in treating various cancers, including adrenal carcinoma. Etoposide: An antineoplastic agent of the topoisomerase inhibitor class that is used alone or in association with other neoplasm medications.
Cisplatin: A platinum agent which may be used as a part of combination therapies in treatment of adrenal carcinoma.
Mitotane: Particularly used for adrenocortical carcinoma; it’s an adrenolytic agent that suppresses the functioning of the adrenal cortex and can be employed in adjunctive and symptomatic treatment only.
Oncology, Other
Surgical Resection Procedure: Adrenalectomy is the only possible treatment for localized adrenal carcinoma, though recent studies indicate that chemotherapy and radiation could work also well. This is a surgical procedure where the adrenal gland is removed along with surrounding tissues or lymph nodes if they are infected with cancer. considerations: The extent of the surgery depends on the size of the tumor and how much the stage has advanced. Less invasive procedure is called the laparoscopic adrenalectomy, but this cannot be performed to cure large growths. Radiation Therapy Procedure: Radiation having more energy is used to destroy the cancer cells. It is used in types of cancer that cannot be removed through surgery or if there is recurrence of the disease after surgery. Considerations: It can be used for symptomatic relieving in cases when the tumor has metastasized to other organs or in combination with other techniques.
Oncology, Other
Diagnosis and Staging:
Imaging: CT, MRI scan, or PET scan to know the size and extension of the tumor.
Biopsy: This may be done if needed to rule out the disease.
Hormonal Evaluation: To confirm if the tumor is releasing hormones that may be causing some problems in the body.
Surgical Management:
Adrenalectomy: The primary treatment for localized adrenal carcinoma. Surgery involves removing the affected adrenal gland and possibly surrounding tissues if the cancer has spread locally.
Adjuvant Therapy:
Radiation Therapy: Given if there remains local disease or if surgery cannot be done.
Chemotherapy: Traditionally it is used in third line or as palliative treatment of metastatic adrenal carcinoma. It consists of mitotane, etoposide, doxorubicin, and cisplatin plan regimens.
Hormonal Therapy:
Mitotane: A drug employed for the purpose of controlling adrenal carcinoma by affecting adrenal cortex cells.
Follow-Up Care:
Surveillance Imaging: CT or MRI scans regularly to check for relapse or metastasis.
Hormone Levels Monitoring: They need counselling to approach and deal with any hormonal fluctuations that occur because of the cancer or its treatment.
Supportive Care:
Palliative Care: Covers all aspects of comfort to enhance a patient’s life by controlling the symptoms and effects of treatment.
Adrenal carcinoma is an aggressive cancer originating in the adrenal glands, with very limited incidence. These include the adrenal cortex, which is responsible for the secretion of important hormones like cortisol, aldosterone, and adrenaline, which are crucial for the functioning of the human body.
Adrenal carcinomas are rare, with the incidence estimated to be around 0.6 to 1.67%. Although in southern Brazil the incidence is 10 –15 time that of the normal population, this is attributable to a mutation on the P53 gene.
Origin: Adrenal carcinoma develops in the cortex of the adrenal glands, which is the outer layer of the organ. The adrenal cortex is involved in secretion of hormones such as cortisol, aldosterone and sex hormones.
Genetic Mutations: There are several genes’ mutations and abnormalities which can cause adrenal carcinoma. For example, TP53 gene, CTNNB1 genes, and chromosomes 11 alteration commonly occurred.
Hormonal Overproduction: It may lead to the overproduction of hormones in the adrenal glands. This may result in hormone-related diseases including Cushing’s disease resulting from excessive cortisol, Conn’s disease resulting from excessive aldosterone or even virilization resulting from increased levels of androgens.
Local Invasion and Metastasis: Adrenal carcinoma is rapidly growing and has a high propensity to invade adjacent structures and organs. It also has the capacity to metastasize to other organs including the liver, lungs and lymph nodes, this makes the disease physiology a complex one.
Pathological Features: Adrenal carcinoma may be accompanied by hemorrhage and necrosis, and microscopically there is usually a high mitotic activity, atypical cells and necrosis. Pleomorphic architecture with high cellularity and Ki-67 proliferation rate can be observed in architecture of this type.
Diagnosis and Staging: The diagnosis of this disease very often prescribes imaging tests such as CT scans or MRI, biopsy, and blood tests to determine hormone levels. Staging aids in indicating the stage through which the disease has progressed and hence the treatment to be given.
Genetic Predispositions: Although the exact causes of adrenal carcinoma have not been well identified, several factors can predispose an individual to the disease, and they include the following genetic disorders:
Li-Fraumeni Syndrome: An inherited condition which predisposes an individual to develop several forms of cancer.
Beckwith-Wiedemann Syndrome: A disease related to overgrowth and potentiality of certain types of cancers such as adrenal carcinoma.
Multiple Endocrine Neoplasia Type 1 (MEN1): A hereditary disease concerned with the development of tumor in many endocrine glands like adrenal gland.
Hormonal Factors: Hormonal alterations or hormonal imbalances may sometimes be related to adrenal tumors, although the relationship is not very clear with carcinoma.
Previous Adrenal Tumors: Some of those who have had concern of benign adrenal tumour including adrenal adenomas are likely to have carcinoma.
Environmental and Lifestyle Factors: No environmental or lifestyle factors have been associated with adrenal carcinoma but the common cancer risk factors such as smoking, obesity and exposure to some chemicals may be considered.
Other Medical Conditions: Inherited diseases that can predispose people to adrenal carcinoma include adrenal hyperplasia or some genetic disorders.
This disease has unfavourable outcome, especially if the tumor is diagnosed in the advanced stage, which is characteristic of adrenal carcinoma. It has been postulated that survival depends on the tumor size, stage at which it was diagnosed and response to treatment.
Age Group:
Being one of the rarest forms of cancer, adrenal carcinoma is considered a malignant tumor that does not spare any age. However, it is most diagnosed in adults that are aged between 40 and 60 years of age.
Acute Presentation: Adrenal carcinoma may also present acutely with acute abdominal pain, a palpable mass, or symptoms of adrenal crisis secondary to rapid tumor growth or hemorrhage. Subacute Presentation: It is usually chronic; symptoms include hormonal disorders, for example, Cushing’s syndrome, abdominal pain, and unexplained weight loss for some weeks or months. Chronic Presentation: It is a silent disease; thus, in its early stage, it may only present with nonspecific complaints, such as persistent abdominal pains or hormonal alterations, whereas the majority of patients are diagnosed during a metastatic stage of the cancer.
Adrenal Adenoma
Adrenal Metastasis
Pheochromocytoma
Primary Adrenal Lymphoma
Cushing’s Syndrome
Surgical Treatment
Primary Treatment: For localized adrenal carcinoma, surgery is always considered a good option and is often performed. Surgery usually involves removal of entire tumor and in some cases the adrenal tissue that is adjacent to the tumor and at times the adrenal gland as well.
Adjuvant Therapy: Adjuvant therapy can be defined as any treatment given after the surgery to prevent the formation of a tumor again especially if the tumor was very large in size or there are other predisposing factors.
Radiation Therapy
Radiation therapy is usually given when surgery cannot be done and when there is local recurrence. It may also be used for the treatment of terminal conditions as a form of palliative measure.
Systemic Therapy
Chemotherapy: For advanced or metastatic adrenal carcinoma, chemotherapy is often used. Common regimens include the EDP (etoposide, doxorubicin, and cisplatin) regimen.
Targeted Therapy: For some patients, the individual molecular characteristics of the tumor may be used to justify the use of targeted therapies.
Hormonal Therapy: If the cancer produces excess hormones, medications to control hormone levels may be used.
Supportive Care
Symptom Management: This suggests palliative care which encompasses control of symptoms to improve the patient’s quality of life as an essential component of the treatment.
Oncology, Other
Supportive Environment:
Psychological Support: Include patients in appropriate counselling or support groups that will enable them deal with the psychological side of the disease.
Pain Management: Medications or other forms of palliative care that aim to improve the quality of life usually address pain and discomfort.
Regular Monitoring: Regular follow-up with imaging and blood tests is necessary in the monitoring of disease processes and modification of treatment according to response.
Oncology, Other
Oncology
Doxorubicin: An anthracycline antibiotic that is a potent adjuvant with other agents in treating various cancers, including adrenal carcinoma. Etoposide: An antineoplastic agent of the topoisomerase inhibitor class that is used alone or in association with other neoplasm medications.
Cisplatin: A platinum agent which may be used as a part of combination therapies in treatment of adrenal carcinoma.
Mitotane: Particularly used for adrenocortical carcinoma; it’s an adrenolytic agent that suppresses the functioning of the adrenal cortex and can be employed in adjunctive and symptomatic treatment only.
Oncology, Other
Surgical Resection Procedure: Adrenalectomy is the only possible treatment for localized adrenal carcinoma, though recent studies indicate that chemotherapy and radiation could work also well. This is a surgical procedure where the adrenal gland is removed along with surrounding tissues or lymph nodes if they are infected with cancer. considerations: The extent of the surgery depends on the size of the tumor and how much the stage has advanced. Less invasive procedure is called the laparoscopic adrenalectomy, but this cannot be performed to cure large growths. Radiation Therapy Procedure: Radiation having more energy is used to destroy the cancer cells. It is used in types of cancer that cannot be removed through surgery or if there is recurrence of the disease after surgery. Considerations: It can be used for symptomatic relieving in cases when the tumor has metastasized to other organs or in combination with other techniques.
Oncology, Other
Diagnosis and Staging:
Imaging: CT, MRI scan, or PET scan to know the size and extension of the tumor.
Biopsy: This may be done if needed to rule out the disease.
Hormonal Evaluation: To confirm if the tumor is releasing hormones that may be causing some problems in the body.
Surgical Management:
Adrenalectomy: The primary treatment for localized adrenal carcinoma. Surgery involves removing the affected adrenal gland and possibly surrounding tissues if the cancer has spread locally.
Adjuvant Therapy:
Radiation Therapy: Given if there remains local disease or if surgery cannot be done.
Chemotherapy: Traditionally it is used in third line or as palliative treatment of metastatic adrenal carcinoma. It consists of mitotane, etoposide, doxorubicin, and cisplatin plan regimens.
Hormonal Therapy:
Mitotane: A drug employed for the purpose of controlling adrenal carcinoma by affecting adrenal cortex cells.
Follow-Up Care:
Surveillance Imaging: CT or MRI scans regularly to check for relapse or metastasis.
Hormone Levels Monitoring: They need counselling to approach and deal with any hormonal fluctuations that occur because of the cancer or its treatment.
Supportive Care:
Palliative Care: Covers all aspects of comfort to enhance a patient’s life by controlling the symptoms and effects of treatment.
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