Secondary lymphedema is a condition characterized by swelling of the soft tissues in which an excessive amount of lymph has accumulated, and is caused by certain malignant diseases such as Hodgkin's disease and Kaposi sarcoma.[1]:849
Secondary
Lymphedema
By Joachim Zuther, on August 20th,
2012
Secondary
lymphedema results from an identifiable damage leading to disruption or
obstruction of normally-functioning lymph vessels and/or lymph nodes and may present itself in the
extremities, trunk,
abdomen, head and neck
and external genitalia.
Worldwide, the
most widespread cause of secondary lymphedema is an infection with a
thread-like worm named wucheria bancrofti, which leads to a condition
known as filariasis. This is a tropical disease, endemic in more than 80
countries in Africa, India, Southeast Asia, and South America, as well as in
the Pacific Islands and the Caribbean.
According to the
World Health Organization, 1.3 billion individuals are threatened by the
disease and over 120 million people are currently affected by it, with about 40
million individuals being disfigured by lymphedema and suffering from recurrent
infections and other secondary conditions. To read more about this condition,
please refer to a previous entry on this site by clicking here.
The highest
incidence of secondary lymphedema in the United States is observed following
surgery and radiation for malignancies, particularly among those individuals
affected by breast
cancer.
Other than skin
cancer, breast cancer is the most common type of malignancy among women in the
United States. All women are at risk for developing breast cancer; males are
also affected at a ratio of one male to 100 females (1).
With increasing
age, the greater a woman’s chance of developing breast cancer increases, with
the majority of breast cancer cases occurring in women over 50 years of age.
While breast cancer is less common at a young age (e.g., women in their
thirties), younger women tend to have more aggressive breast cancers than older
women, which may explain why survival rates are lower among younger women.
Incidence also varies within ethnic groups and geographical location within the
U.S.
Generally it
can be said that one out of eight women in the U.S. will develop breast cancer
during the course of their lives. Almost 227,000 new
cases of breast cancer in females and 2200 in males are
estimated in the United States in 2012 (2).
Why does
lymphedema develop?
Any type of
surgery, specifically procedures that require the removal of lymph nodes, can
cause the onset of lymphedema. Surgical procedures in cancer therapy, such as
breast conserving (lumpectomy) or more extensive breast surgery (mastectomy)
commonly include the removal (dissection) of lymph nodes, with subsequent
damage to lymph vessels.
Many
individuals receive radiation therapy following the surgical procedure, which
may aggravate the situation.
The goal of
these procedures is to eliminate the cancer cells and to save the patient’s
life. A side effect in lymph node removal is the disruption in the transport
of lymphatic fluid.
The lymphatic
system consists of lymph vessels and lymph nodes throughout the body. Lymph vessels
collect lymphatic fluid, which is composed of protein, water, fats, and waste
products from cells. These vessels transport the fluid to the lymph nodes,
where waste products and foreign materials are filtered out from the fluid.
After passing several groups of lymph nodes, the lymph vessels return the fluid
back to the blood.
When the vessels
are damaged, the flow of lymphatic fluid is compromised. If the remaining
lymph vessels that are unaffected by the surgery are not able to compensate for
the damaged vessels, lymphatic fluid accumulates in the tissues. This
accumulation of lymphatic fluid results in abnormal swelling, most commonly
affecting the upper and lower extremities; however, other parts of the body may
be affected as well.
Other surgeries,
to include treatment of melanoma, cancer affecting the genitourinary and
gynecologic systems, cancers in the head and neck region, or soft tissue
malignancies, generally include the removal of lymph nodes with subsequent
disruption of lymphatic pathways, which may cause the onset of
lymphedema.
Radiation therapy,
specifically if combined with the surgical removal of lymph nodes, can cause
scarring in soft tissue and inflammation of lymph nodes and lymph vessels,
which may also contribute to the development of secondary lymphedema.
Less common causes
for secondary lymphedema include surgeries other than for the treatment of
malignancies, or trauma disrupting the flow of lymph. Tumors growing in the
soft tissues can become large enough to cause a physical block on lymphatic
structures subsequently obstructing the normal flow of lymph.
Secondary cases of
lymphedema may occur immediately following the surgical procedure and/or
radiation, within a few months, a couple of years, or twenty years or more
after treatment. The average time of onset is between 14 and 24 months
post-surgically, with an increased number of cases over time. Some individuals
may never experience any symptoms; however, the risk of development of
secondary lymphedema lasts a life time.
There is no
consistency in the data on the incidence of lymphedema, and most statistics
that are available are those on breast cancer related lymphedema (BCRL)
affecting the upper extremities.
It was
reported that the five-year cumulative incidence of lymphedema following breast
cancer surgery in women was 42%; of the affected women, 80% developed
lymphedema within two years and 89% within three years (3). A study, which was
published in 2001 (conducted by Petrek et al), followed 263 patients after
mastectomy and complete axillary dissection. At 20 years after treatment, 49%
reported lymphedema; of those, 77% noted onset within three years after
surgery, and the remaining women developed lymphedema in the arm at a rate of
almost 1% per year (4).
Prevention
Patient education
about the possibility of developing secondary lymphedema, discussion of the
risk factors and risk reduction
practices, combined with appropriate surveillance and prompt
reporting of symptoms following cancer treatment, can limit the incidence and
progression of secondary lymphedema.
A recent study (5)
including patients who received treatment for breast cancer, determined that
patients who received information about the possible onset of secondary
lymphedema demonstrated significantly reduced symptoms when compared with
patients who did not receive this information. Women who received information
about lymphedema were significantly less likely to report heaviness in the
extremity, arm swelling, impaired shoulder mobility, and breast swelling.
The NLN’s
position statement on risk reduction practices (6) serves as a valuable
resource.
Early treatment of
secondary lymphedema by a qualified therapist is of paramount importance to
limit progression of the swelling and to avoid complications often associated with untreated or
incorrectly treated lymphedema.
The treatment of
choice for this condition is complete decongestive therapy (CDT), a combination
of modalities including manual lymphatic drainage, the application of padded
short-stretch bandages, use of compression garments, therapeutic exercise, and
skin care. Details of these treatment elements are available by clicking here.
References:
(1) http://www.cancer.org/acs/groups/content/@epidemiologysurveilance/documents/document/acspc-029771.pdf
(2) http://cancer.gov/cancertopics/types/breast
(3) http://cebp.aacrjournals.org/content/19/11/2734.long
(4) http://onlinelibrary.wiley.com/doi/10.1002/1097-0142(20010915)92:6%3C1368::AID-CNCR1459%3E3.0.CO;2-9/abstract
(5) Fu, MR, Chen,
CM, et al (2010). The effect of providing information about lymphedema on the
cognitive and symptom outcomes of breast cancer survivors, Annals of
Surgical Oncology, 17:1847-1853.
(6) http://www.lymphnet.org/pdfDocs/nlnriskreduction.pdf
Secondary
Lymphedema
By Joachim Zuther, on August 20th,
2012
Secondary
lymphedema results from an identifiable damage leading to disruption or
obstruction of normally-functioning lymph vessels and/or lymph nodes and may present itself in the
extremities, trunk,
abdomen, head and neck
and external genitalia.
Worldwide, the
most widespread cause of secondary lymphedema is an infection with a
thread-like worm named wucheria bancrofti, which leads to a condition
known as filariasis. This is a tropical disease, endemic in more than 80
countries in Africa, India, Southeast Asia, and South America, as well as in
the Pacific Islands and the Caribbean.
According to the
World Health Organization, 1.3 billion individuals are threatened by the
disease and over 120 million people are currently affected by it, with about 40
million individuals being disfigured by lymphedema and suffering from recurrent
infections and other secondary conditions. To read more about this condition,
please refer to a previous entry on this site by clicking here.
The highest
incidence of secondary lymphedema in the United States is observed following
surgery and radiation for malignancies, particularly among those individuals
affected by breast
cancer.
Other than skin
cancer, breast cancer is the most common type of malignancy among women in the
United States. All women are at risk for developing breast cancer; males are
also affected at a ratio of one male to 100 females (1).
With increasing
age, the greater a woman’s chance of developing breast cancer increases, with
the majority of breast cancer cases occurring in women over 50 years of age.
While breast cancer is less common at a young age (e.g., women in their
thirties), younger women tend to have more aggressive breast cancers than older
women, which may explain why survival rates are lower among younger women.
Incidence also varies within ethnic groups and geographical location within the
U.S.
Generally it
can be said that one out of eight women in the U.S. will develop breast cancer
during the course of their lives. Almost 227,000 new
cases of breast cancer in females and 2200 in males are
estimated in the United States in 2012 (2).
Why does
lymphedema develop?
Any type of
surgery, specifically procedures that require the removal of lymph nodes, can
cause the onset of lymphedema. Surgical procedures in cancer therapy, such as
breast conserving (lumpectomy) or more extensive breast surgery (mastectomy)
commonly include the removal (dissection) of lymph nodes, with subsequent
damage to lymph vessels.
Many
individuals receive radiation therapy following the surgical procedure, which
may aggravate the situation.
The goal of
these procedures is to eliminate the cancer cells and to save the patient’s
life. A side effect in lymph node removal is the disruption in the transport
of lymphatic fluid.
The lymphatic
system consists of lymph vessels and lymph nodes throughout the body. Lymph vessels
collect lymphatic fluid, which is composed of protein, water, fats, and waste
products from cells. These vessels transport the fluid to the lymph nodes,
where waste products and foreign materials are filtered out from the fluid.
After passing several groups of lymph nodes, the lymph vessels return the fluid
back to the blood.
When the vessels
are damaged, the flow of lymphatic fluid is compromised. If the remaining
lymph vessels that are unaffected by the surgery are not able to compensate for
the damaged vessels, lymphatic fluid accumulates in the tissues. This
accumulation of lymphatic fluid results in abnormal swelling, most commonly
affecting the upper and lower extremities; however, other parts of the body may
be affected as well.
Other surgeries,
to include treatment of melanoma, cancer affecting the genitourinary and
gynecologic systems, cancers in the head and neck region, or soft tissue
malignancies, generally include the removal of lymph nodes with subsequent
disruption of lymphatic pathways, which may cause the onset of
lymphedema.
Radiation therapy,
specifically if combined with the surgical removal of lymph nodes, can cause
scarring in soft tissue and inflammation of lymph nodes and lymph vessels,
which may also contribute to the development of secondary lymphedema.
Less common causes
for secondary lymphedema include surgeries other than for the treatment of
malignancies, or trauma disrupting the flow of lymph. Tumors growing in the
soft tissues can become large enough to cause a physical block on lymphatic
structures subsequently obstructing the normal flow of lymph.
Secondary cases of
lymphedema may occur immediately following the surgical procedure and/or
radiation, within a few months, a couple of years, or twenty years or more
after treatment. The average time of onset is between 14 and 24 months
post-surgically, with an increased number of cases over time. Some individuals
may never experience any symptoms; however, the risk of development of
secondary lymphedema lasts a life time.
There is no
consistency in the data on the incidence of lymphedema, and most statistics
that are available are those on breast cancer related lymphedema (BCRL)
affecting the upper extremities.
It was
reported that the five-year cumulative incidence of lymphedema following breast
cancer surgery in women was 42%; of the affected women, 80% developed
lymphedema within two years and 89% within three years (3). A study, which was
published in 2001 (conducted by Petrek et al), followed 263 patients after
mastectomy and complete axillary dissection. At 20 years after treatment, 49%
reported lymphedema; of those, 77% noted onset within three years after
surgery, and the remaining women developed lymphedema in the arm at a rate of
almost 1% per year (4).
Prevention
Patient education
about the possibility of developing secondary lymphedema, discussion of the
risk factors and risk reduction
practices, combined with appropriate surveillance and prompt
reporting of symptoms following cancer treatment, can limit the incidence and
progression of secondary lymphedema.
A recent study (5)
including patients who received treatment for breast cancer, determined that
patients who received information about the possible onset of secondary
lymphedema demonstrated significantly reduced symptoms when compared with
patients who did not receive this information. Women who received information
about lymphedema were significantly less likely to report heaviness in the
extremity, arm swelling, impaired shoulder mobility, and breast swelling.
The NLN’s
position statement on risk reduction practices (6) serves as a valuable
resource.
Early treatment of
secondary lymphedema by a qualified therapist is of paramount importance to
limit progression of the swelling and to avoid complications often associated with untreated or
incorrectly treated lymphedema.
The treatment of
choice for this condition is complete decongestive therapy (CDT), a combination
of modalities including manual lymphatic drainage, the application of padded
short-stretch bandages, use of compression garments, therapeutic exercise, and
skin care. Details of these treatment elements are available by clicking here.
References:
(1) http://www.cancer.org/acs/groups/content/@epidemiologysurveilance/documents/document/acspc-029771.pdf
(2) http://cancer.gov/cancertopics/types/breast
(3) http://cebp.aacrjournals.org/content/19/11/2734.long
(4) http://onlinelibrary.wiley.com/doi/10.1002/1097-0142(20010915)92:6%3C1368::AID-CNCR1459%3E3.0.CO;2-9/abstract
(5) Fu, MR, Chen,
CM, et al (2010). The effect of providing information about lymphedema on the
cognitive and symptom outcomes of breast cancer survivors, Annals of
Surgical Oncology, 17:1847-1853.
(6) http://www.lymphnet.org/pdfDocs/nlnriskreduction.pdf